Ethics Rounds: a Casebook in Pediatric Bioethics [1 ed.] 9781610023672, 9781610023665

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Pediatric Collections Ethics Rounds: A Casebook in Pediatric Bioethics

Edited by John D. Lantos, MD

Pediatric Collections Ethics Rounds: A Casebook in Pediatric Bioethics

Edited by John D. Lantos, MD Associate Editor, Pediatrics Professor of Pediatrics, University of Missouri–Kansas City Director, Children's Mercy Hospital Bioethics Center

Published by the American Academy of Pediatrics 345 Park Blvd. Itasca, IL 60143 The American Academy of Pediatrics is not responsible for the content of the resources mentioned in this publication. Web site addresses are as current as possible but may change at any time. Products are mentioned for information purposes only. Inclusion in this publication does not imply endorsement by the American Academy of Pediatrics. © 2019 American Academy of Pediatrics. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means—electronic, mechanical, photocopying, recording, or otherwise—without prior written permission from the publisher. Printed in the United States of America APC015 Print ISBN: 978-1-61002-366-5 eBook ISBN: 978-1-61002-367-2

Pediatric Collections Ethics Rounds: A Casebook in Pediatric Bioethics

About AAP Pediatric Collections Pediatric Collections is a series of selected pediatric articles that highlight different facets of information across various AAP publications, including AAP Journals, AAP News, Blog Articles, and eBooks. Each series of collections focuses on specific topics in the field of pediatrics so that you can keep up with best practices, and make an informed response to public health matters, trending news, and current events. Each collection includes previously published content focusing on specific topics and articles selected by AAP editors. Visit http://collections.aap.org to view a list of upcoming collections.

TABLE OF CONTENTS 1

Introduction Lantos JD

End-of-Life Decisions

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Introduction | End-of-Life Decisions Lantos JD

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A Premature Infant With Necrotizing Enterocolitis Whose Parents Are Jehovah's Witnesses Meadow W, Feudtner C, Antommaria AH, Sommer D, Lantos JD

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A Saudi Family Making End-of-Life Decisions in the PICU Sayeed S, Padela A, Naim MY, Lantos JD

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Symbolic Resuscitation, Medical Futility, and Parental Rights Frader J, Kodish E, Lantos JD

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Palliative Sedation With Propofol for an Adolescent With a DNR Order Johnson LM, Frader J, Wolfe J, Baker JN, Anghelescu DL, Lantos JD

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Parental Refusal of a Liver Transplant for a Child With Biliary Atresia Cronin DC, Squires J, Squires R, Mazariegos G, Lantos JD

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When Life-Sustaining Treatment Is Withdrawn and the Patient Doesn’t Die Kutzsche S, Partridge JC, Leuthner SR, Lantos JD

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Ethics Rounds: A Casebook in Pediatric Bioethics

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TABLE OF CONTENTS 42

Is Treatment Futile for an Extremely Premature Infant With Giant Omphalocele? Feltman D, Stokes T, Kett J, Lantos JD

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Cross-Cultural Differences in Communication About a Dying Child Cochran D, Saleem S, Khowaja-Punjwani S, Lantos JD

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Are We Allowed to Discontinue Medical Treatment in This Child? Leeuwenburgh-Pronk WG, Miller-Smith L, Forman V, Lantos JD, Tibboel D, Buysse C

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Two Infants, Same Prognosis, Different Parental Preferences Antommaria AHM, Collura CA, Antiel RM, Lantos JD

Different Philosophies

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Introduction | When Doctors and Parents Have Different Philosophies Lantos JD

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A 6-Month-Old With Vaccine-Hesitant Parents Opel DJ, Feemster KA, Omer SB, Orenstein WA, Richter M, Lantos JD

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Should Pediatric Practices Have Policies to Not Care for Children With Vaccine-Hesitant Parents? Alexander K, Lacy TA, Myers AL, Lantos JD

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When Parents Refuse a Septic Workup for a Newborn Simpson E, Moon M, Lantos JD

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Should All Deaf Children Learn Sign Language? Napoli DJ, Mellon NK, Niparko JK, Rathmann C, Mathur G, Humphries T, Handley T, Scambler S, Lantos JD

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Should Neonatologists Give Opinions Withdrawing Life-sustaining Treatment? Blumenthal-Barby JS, Loftis L, Cummings CL, Meadow W, Lemmon M, Ubel PA, McCullough L, Rao E, Lantos JD

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TABLE OF CONTENTS Genomics

101 Introduction | Ethical Issues in Genomics Lantos JD

103 Genomic Contraindications for Heart Transplantation Char DS, Lázaro-Muñoz G, Barnes A, Magnus D, Deem MJ, Lantos JD

110 Please Test My Child for a Cancer Gene, but Don’t Tell Her Bester J, Sabatello M, van Karnebeek CDM, Lantos JD

117 Testing Children for Adult-Onset Genetic Diseases Caga-anan ECF, Smith L, Sharp RR, Lantos JD

Severe Disability

125 Introduction | Ethical Issues Surrounding Permanent Severe Disability in Childhood Lantos JD

127 Mechanical Ventilation for a Child With Quadriplegia Novotny WE, Perkin RM, Mukherjee D, Lantos JD

132 An Infant With Trisomy 18 and a Ventricular Septal Defect Janvier A, Okah F, Farlow B, Lantos JD

138 A Baby With Meningococcemia and Septic Shock Leeuwenburgh-Pronk WG, Smith PJ, van Vught AJ, Lantos JD, Tibboel D, de Hoog M, Buysse C

Research Ethics

145 Introduction | Research Ethics Lantos JD

147 Should an IRB Approve a Placebo-Controlled Randomized Trial of Analgesia for Procedural Pain in Neonates? Bellieni CV, Taddio A, Linebarger JS, Lantos JD

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TABLE OF CONTENTS 151 When Is Waiver of Consent Appropriate in a Neonatal Clinical Trial? Schreiner MS, Feltman D, Wiswell T, Wootton S, Arnold C, Tyson J, Lantos JD

158 Risks in a Trial of an Innovative Treatment of Duchenne Muscular Dystrophy

Bos W, Estra AE, Pinxten W, Mayer MP, Lantos JD

163 Randomized n-of-1 Trials: Quality Improvement, Research, or Both? Samuel JP, Burgart A, Wootton SH, Magnus D, Lantos JD, Tyson JE

Law and Health Policy

171 Introduction | Issues in Law and Health Policy Lantos JD

173 Can a Patient Designate His Doctor as His Proxy Decision Maker? Black PG, Derse AR, Derrington S, Lantos JD

178 Who Should Get the Last PICU Bed? Wightman A, Largent E, Del Beccaro M, Lantos JD

184 Was Sarah Murnaghan Treated Justly? deSante J, Caplan A, Hippen B, Testa G, Lantos JD

192 Should We Tell Parents When We’ve Made an Error? Bell SK, Mann KJ, Truog R, Lantos JD

197 When a Surgical Colleague Makes an Error Antiel RM, Blinman TA, Rentea RM, Gonzalez KW, Knott EM, Juang D, Oyetunji T, Holcomb GW III, Angelos P, Lantos JD

202 The Dilemma of Predicting Violent Radicalization Rousseau C, Ellis BH, Lantos JD

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Introduction he ethical issues that arise in pediatrics are very different from those that arise in other clinical settings. The

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differences arise partly because young children cannot make decisions for themselves. Thus, the principle of

autonomy, a principle that is so important in resolving the dilemmas that arise in the care of adults, is irrelevant in ethical dilemmas involving young children. It is partially relevant in older children and adolescents. Furthermore, autonomy is, essentially, a procedural principle. It doesn’t claim to determine what choice is right. It only specifies who should be empowered to make the decision about what is right. Without the ability to fall back on autonomy, doctors, parents, and ethicists who are caring for children must make substantive decisions about what is best for those children. To do so, they rely on a number of ethical considerations. They always try to do what is best for the child.1 Sometimes, however, they must balance what is best for the child with what is best, overall, for the family.2 At the very least, doctors and parents try to avoid any choice that will cause irreversible harm to the child.3 Decisions for children are always made with an eye toward the child’s future. That requires considerations about long-term health outcomes4 as well as about the child’s right to make decisions about her own health care in the future.5 All of these considerations can be useful. None of them is obviously superior to the others or necessarily determinative in any particular case. In the end, we are left with thresholds of benefit and harm, between which lies a zone of parental discretion.6 This collection presents a series of cases that highlight some ethical dilemmas that arise in pediatrics. The use of cases to teach bioethics is controversial. The dominant theoretical approach to bioethics in America today relies on ethical principles such as autonomy, beneficence, and justice. Many people are familiar with the principal-based approach articulated by Beauchamp and Childress in their influential book, Principles of Biomedical Ethics.7 Beauchamp and Childress suggest that 4 principles—autonomy, beneficence, non-maleficence, and justice—provide an analytical framework that can help clinicians think through difficult ethical dilemmas and choose the best solution. This approach is theoretically appealing. A large part of its appeal is its promise of providing a principled solution to moral problems. Arras quotes a graduate of a bioethics training course that used this approach, “This [method] is what our student-doctors need. It’s really objective, based on principles, just like a science.”8 Arras contrasts the principled approach with a case-based, or casuistic, method of analyzing ethical dilemmas. He notes, “The casuistical method has much in common with the method of the common law. On both fronts, we reason from the ‘bottom up’ (from cases to fleshed-out principles) rather than from the ‘top down’ (as most versions of applied ethics imply). The principles themselves are always subject to further revision and specification. Final judgments usually turn on a fine-grained analysis of the particularities of the case.”8 Over the years, though, many people have advocated a case-based approach to ethical analysis and teaching. Miller and colleagues suggest that the roots of such an approach can be found in the work of American pragmatist philosopher John Dewey.9 Dewey suggests that, in the real world, people respond to ethical dilemmas in 4 steps. First, we experience a “felt difficulty.”10 Then, we describe the difficulty. Third, we suggest possible solutions. Finally, we observe (or experiment) in order to see whether the proposed solutions should be accepted or rejected. This 4-step process is one that should be familiar from both clinical medicine and clinical ethics. Both are case-based disciplines built on a foundation of theoretical principles. In medicine, those principles come from physiology and psychology. In ethics, they come from our experiences as those experiences are analyzed for moral content. There is something familiar about such an approach to ethical dilemmas. It is the method of newspaper advice columns. Somebody faces a difficult choice. They are not sure about what ought to be done. They ask for advice. The advice columnist gives a recommendation and reasons for that recommendation. The advice is practical. Such an approach should be familiar to physicians because case presentations are commonly used to teach clinical skills. Toulmin pointed out the similarities between case studies in ethics and in clinical medicine: “Ethics and clinical medicine are both prime examples of the concrete fields of thought and reasoning in which the theoretical rigor of geometrical argument is unattainable: fields in which we should above all strive to be

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reasonable rather than insisting on a kind of exactness that ‘the nature of the case’ does not allow.”11 In that article, Toulmin went on to specify more similarities between clinical medicine and applied ethics: We may view the problems of clinical medicine and the problems of applied ethics as two varieties of a common species. Defined in purely general terms, such ethical categories as “cruelty” and “kindness,” “laziness” and “conscientiousness,” have a certain abstract, truistical quality: before they can acquire any specific relevance, we have to identify some actual person, or piece of conduct, as “kind” or “cruel,” “conscientious” or “lazy,” and there is often disagreement even about that preliminary step. Similarly, in medicine: if described in general terms alone, diseases too are “abstract entities,” and they acquire a practical relevance only for those who have learned the diagnostic art of identifying real-life cases as being cases of one disease rather than another.11 Toulmin notes that “Aristotle pioneered the general doctrine that principles never settle ethical issues by themselves: that is, that we can grasp the moral force of principles only by studying the ways in which they are applied to, and within, particular situations.” Similarly, we can never become excellent diagnosticians by studying pathophysiology alone. We also need to learn how to talk to patients, to elicit a medical history and a description of the patient’s current ailments, and then to put the specific knowledge together with the scientific framework. Such non-theoretical, case-based moral reasoning has been criticized for being unanchored and relativistic. Arras, for example, notes that “[C]asuistry, for all its usefulness as a method, is nothing more (and nothing less) than an ‘engine of thought’ that must receive direction from values, concepts and theories outside of itself.”12 The case-based method also has its defenders. Smith notes that it is the basis of much moral theology and identifies some fundamental features of modern casuistry.13 “The absence of a clear moral consensus is assumed. Moral certainty is found in the acceptance of a moral verdict on particular cases and the basic intellectual strategy is extrapolation from the morally more certain to the less certain.” Casuistry builds on a foundation of broadly agreed on moral intuitions that are then applied to the analysis of a particular case.14 Casuistry relies on reference to paradigm cases. These are cases that illustrate a particular sort of dilemma and a specific solution to that dilemma. A casuist who is analyzing a new case will try to decide which paradigm cases or precedents apply to the new case. Kaebnik described the casuistic process: “The guiding idea in casuistry is the notion that one reaches a judgment in a new case by considering how it is analogous to ‘paradigms’—cases about which there is a considered consensus concerning the right judgment.”15 Over the years, the study of pediatric bioethics has generated a set of paradigm cases. These are our common heritage and our touchpoint with the past. We tell and retell the legal case of Prince v Massachusetts16 and the all-purpose maxim that parents are free to make martyrs of themselves but not of their children. We tell and retell the tale of a Baby Doe, a baby born with Down syndrome and esophageal atresia who died because his parents, guided by their obstetrician, would not authorize life-saving surgery.17 We remember Rudy Linares who held a PICU staff at bay with a .44 Magnum while he removed his son from life-support.18 We know of Baby L from Boston,19 Baby K from Virginia,20 and Charlie Gard from the United Kingdom.21 These well-analyzed cases help by becoming touchstones of moral reasoning. The method of casuistry—or case-based moral reasoning—is to analyze new and unique cases by comparing them to older cases that have already been analyzed and about which a societal consensus has been reached. The cases in this collection were first published in Pediatrics as part of the series entitled “Ethics Rounds.” The structure of each is similar. A vignette, based on real cases, describes a situation in which clinicians or administrators need to make a choice. In some, the parents want to continue treatment and the clinicians want to stop. Some of the cases deal with dilemmas about what to disclose to parents. Others deal with intra-professional conflicts. In each case, then, we sought experts to respond by saying what they would do and why. Some of the respondents consider themselves to be bioethicists. Others are experienced clinicians. Some are parents. Many had been involved in the actual cases and thus had a direct personal stake in the outcome. The goal in putting these together in this way was to seek different sorts of expertise, different perspectives, and different insights into the real-world dilemmas that arise in the care of children.

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The cases focus on situations in which a doctor or other child health professional must make a choice. The choices have 5 key features.22 First and foremost, all of the choices are bad. If there were a good choice among a bunch of bad ones, there would be no ethical dilemma. Put another way, there are good ethical reasons not to make each of the available choices. A child health professional who must make an ethically troubling choice will certainly feel some moral distress. The goal of ethical analysis is not to eliminate moral distress. The goal, instead, is to both minimize and understand that distress. A second key feature of the choices in ethically complex cases is that all of the options are legal. This is important because, while the focus of the cases is not on the legal issues, doctors must be aware of legal constraints. There are situations in which law and ethics conflict and in which the ethically appropriate thing to do violates the law. But if something is clearly illegal, then one must consider the implications of civil disobedience. In this collection, we do not deal with such issues. Third, reasonable people will disagree about which choice is ethically preferable. This follows from the first 2 features of these choices. Because reasonable people will disagree about the ethically best choice, the point of ethical analysis is to give compelling reasons for one’s preferred choices. Such analysis sometimes leads to agreement about which bad choice is preferable. The fourth feature of these choices is that they are ones in which fundamental ethical principles of bioethics are in conflict. Thus, citing principles in support of one’s argument, while important, usually does not resolve the conflict. It is not enough, for example, to say that as reasonable people, we should always do what is in the baby’s best interest, invoking the principle of beneficence. Another reasonable person might counter by invoking the principle of autonomy to claim that parents have the right to make the decision or the principle of justice to say that concerns about resource allocation should determine the outcome of the case. The tough cases are tough precisely because they are the ones in which the principles conflict. The final key feature is that a decision must be made. There is no neutral or default position. One must either disclose the truth or not, withdraw life support or continue it, approve a research protocol or not. These are real-world dilemmas that reflect the complex world of clinical practice, clinical research, and health policy. The cases all come from the actual experiences of doctors, nurses, social workers, and other clinicians. Some include parents’ perspectives. In their responses, writers draw on both personal experiences and, in some cases, on a rich body of scholarship in pediatric bioethics that has developed over the last few decades. They invoke many familiar ethical principles, including beneficence for the child, autonomy for the parents, and each child’s right to an open future. They have concerns about justice, fairness, cost-effectiveness, conscience, and professionalism. They invoke ideas from philosophy, religion, law, economics, literature, psychology, and social sciences. This collection does not offer a single philosophical framework to analyze these ethical conundrums. Instead, it presents the cases and asks experienced clinicians and bioethicists to explain how they analyze the issues, which choices they would make, and why. This eclectic approach suggests that there is no overarching ethical framework for deciding such cases. That is not quite true. It would be more accurate to say that there are many ethical frameworks that cannot be reduced to one. Experienced clinical ethicists recognize the need for an ecumenical approach to theory. This ecumenical approach takes for granted that rules and principles are always tentative and provisional and may need to be revised or rejected. This approach also has its roots in Dewey’s pragmatism. In Dewey’s view, “[R]ules and principles are not absolutely decisive or final; they have the logical value of working hypotheses for the satisfactory resolution of morally problematic situations. In the process of ethical inquiry and efforts to resolve moral problems, we clarify, test, and revise our moral rules and principles.”9 That summary of Dewey’s conception of moral principles suggests the theme of this collection and the reason why a casebook in pediatric bioethics might be more relevant to clinicians than an abstract philosophical treatise. There is a tradition of such casebooks. In one of the earliest published casebooks in clinical ethics, Brody23 defended an approach that he called “the model of conflicting appeals.” This model recognizes that “both parties in the patient-physician relationship are subject to a wide variety of moral appeals.” They must balance their consideration of appeals to rights, to respect for persons, to virtues, to cost-effectiveness, and to justice. These different appeals sometimes lead to different conclusions. For some people, appeals to justice outweigh appeals to rights or virtues. Others weigh matters differently. Brody notes that, inevitably, this leads to

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conclusions that don’t please everyone. He writes, “We need to treat the development of complex moral theories, like the development of complex scientific theories, as a tentative process….”23 Brody elaborates how such reasoning works: “There are no rules dictating which level of theory is most appropriate at a given stage of inquiry. Theories are formed at many levels of generality, ranging from simple generalizations covering a small portion of the data to complex theories covering the whole area of inquiry.”23 With that open-ended philosophy in mind, these cases should be read as the beginning of a discussion about pediatric bioethics, rather than the end of one. The best way to use these cases is as the starting point for reflection on similar cases. Each response was generated independently. Commenters do not always agree. Readers are not expected to, either.

John D. Lantos, MD Associate Editor, Pediatrics Professor of Pediatrics, University of Missouri–Kansas City Director, Children’s Mercy Hospital Bioethics Center References 1. Kopelman LM. The best interest standard as threshold, ideal, and standard of reasonableness. J Med Phil. 1997;22:271–289 2. Groll D. Four models of family interests. Pediatrics. 2014;134(suppl 2):S81–S86 3. Diekema DS. Parental refusals of medical treatment: the harm principle as threshold for state intervention. Theor Med Bioeth. 2004;25(4):243–264 4. Lantos JD. Ethical problems in decision making in the neonatal ICU. N Engl J Med. 2018;379(19):1851–1860 5. Millum J. The foundation of the child’s right to an open future. J Soc Philos. 2014;45:522–538 6. Gillam L, Wilkinson D, Xafis V, et al. Decision-making at the borderline of viability: who should decide and on what basis? J Paediatr Child Health. 2017;53:105–111 7. Beauchamp TL, Childress JF. Principles of Biomedical Ethics. 7th ed. Oxford, UK: Oxford University Press; 2012 8. Arras J. Principles and particularity: the roles of cases in bioethics. Indiana Law J. 1994;69:983–1014 9. Miller FG, Fins JJ, Bachetta MD. Clinical pragmatism: John Dewey and clinical ethics. J Cont Health Law & Pol. 1996;13:27–51 10. Dewey J. How We Think. New York, NY: DC Health & Co; 1910:72. https://archive.org/details/how wethink000838mbp. Accessed June 21, 2018. 11. Toulmin S. How medicine saved the life of ethics. Persp Biol Med. 1982;25:736–750 12. Arras J. Getting down to cases: the revival of casuistry in bioethics. J Med Phil. 1991;16:29–51 13. Smith DH. The moral theology or the casuistic tradition. Anglican Theo Rev. 2012;94:595–615 14. Hunter KM. A science of individuals. J Med Phil. 1989:14:193–212 15. Kaebnik GE. On the intersection of casuistry and particularism. Kenn Inst Ethics J. 2000;10:307–322 16. Prince v Massachusetts, 321 US 158 (1944). https://supreme.justia.com/cases/federal/us/321/158/. Accessed August 9, 2018 17. Lantos JD. Baby Doe five years later: implications for child health. N Engl J Med. 1987;317:444–447 18. Lantos JD, Miles SH, Cassel CK. The Linares affair. Law Med Health Care. 1989;17:308–315 19. Paris JJ, Crone RK, Reardon F. Physician refusal of requested treatment. The case of Baby L. N Engl J Med. 1990;322:1012–1015 20. Annas GJ. Asking the courts to set the standard of emergency care—the case of Baby K. N Engl J Med. 1994;330:1542–1545 21. Lantos JD. The tragic case of Charlie Gard. JAMA Peds. 2017;171:935–936 22. Lantos JD. What we talk about when we talk about ethics. Hastings Cent Rep. 2014;44(1 suppl):S40–S44 23. Brody B. Life and Death Decision Making. New York, NY: Oxford University Press; 1988

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End-of-Life Decisions

Introduction | End-of-Life Decisions John D. Lantos, MD, Associate Editor, Pediatrics he way we die has changed. Death used to happen despite all available treatments. Today, death usually follows

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a decision to withhold or withdraw some potentially life-prolonging therapy. Doctors and parents must decide

whether further intervention will be helpful or harmful. Psychologist Barbara Sourkes1 describes what she calls “the living-dying interval.” She writes, “In the past, the illness trajectory moved directly from diagnosis to death, with little intervening time or space.” Today, she says, there is a prolonged middle phase, that can include “a cycle of remissions and relapses, a gradual downhill course, or prolonged remission implying a cure.”1 In this living-dying interval, we have many choices. Each case is different. Each family is different. Case studies illustrate some of those differences in ways that “acquaint us with specific and intensified repertories of emotions, experiences, and possibilities.”2 For parents, the intensity of emotions is overwhelming. Many parents have written about the experience. Novelist Aleksandar Hemon3 wrote about the moment when he first heard that his 2-year- old daughter Isabel had a brain tumor. He could not grasp the enormity of the news that he was receiving. “I didn’t comprehend… it was beyond my experience, belonging to the domain of the unimaginable and incomprehensible.”3 When novelist Lorrie Moore4 was told that her baby had a Wilms tumor, she foolishly imagined that, perhaps, they’d done an ultrasound on the wrong patient. Hemon and Moore both explain, too, that it was almost unbearably painful to watch their children undergo invasive procedures in the intensive care unit (ICU). Hemon writes, “In the ICU, we found her entangled in a web of IVs, tubes, and wires, paralyzed by Rocuroniun. We spent the night watching her, kissing the fingers on her limp hand, reading or singing to her.”3 Moore describes the powerlessness that she felt when she went to visit her baby in the ICU, “It is a horror and a miracle to see him. He is lying in his crib in his room, tubed up, splayed like a boy on a cross. Seeing him like that, she wants to pick him up and run from the ICU, to rescue him from the medical torture that he is undergoing.”4 Moore’s baby survives. Hemon’s baby dies. Their narratives capture the radical uncertainty and the tumultuous emotions that constitute the parents’ experience of a life-threatening illness in their child. As they were going through the experiences and making decisions, they had no idea whether the decisions were the right ones. Ultimately, the rightness or wrongness of those decisions would be determined by the outcome, an outcome that could not be known at the time that the decisions needed to be made. These narratives capture key components of the experience of death and dying in childhood today—uncertainty, helplessness, denial, fear of the future, anger at the doctors and the brutal nature of medical care today, and, ultimately, a profound sense of isolation. Because we now possess the technology to keep children alive for months or years when they have no hope of recovery or cure, we must decide whether their last days or weeks of life should be spent in the ICU, tethered to machines, isolated, and in pain. Surely, it seems, for such children, it is ethical, humane, and compassionate to change the goals of treatment. Instead of providing therapy to prolong their lives, we should only provide therapy that relieves their pain or facilitates loving relationships with family. But there is an ever-present flipside. It is not always clear when the chances for cure are gone, when to give up hope. Always lurking in the background of such decisions are concerns that we may be giving up too soon on a child who might ultimately survive. We all know stories and have experienced cases in which a child beat the odds and not only survived but thrived. How, then, should we think through the delicate issues in these cases? In this section, we present a number of different cases and responses. Some raise issues of different religious or cultural beliefs about medical treatment. In some cases, the question is whether to tell parents the whole truth. Some address the gray zone between aggressive palliation and euthanasia. These cases only hint at the endless variation in clinical situations, family experiences, and possible ethical responses.

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References 1.

Sourkes B. The Deepening Shade: Psychological Aspects of Life-Threatening Illness. Pittsburgh PA: University of Pittsburgh Press; 1982:55

2.

Shattuck R. Proust’s Binoculars: A Study of Memory, Time, and Recognition in A La Recherche du Temps Perdu. New York, NY: Random House; 1963:134

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3.

Hemon A. The aquarium: a child’s isolating illness. The New Yorker. June 13, 2011

4.

Moore L. People like that are the only people here. Birds of America. New York, NY: Picador; 1988:215

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A Premature Infant With Necrotizing Enterocolitis Whose Parents Are Jehovah’s Witnesses AUTHORS: William Meadow, MD, PhD,a Chris Feudtner, MD, PhD,b Armand H. Matheny Antommaria, MD, PhD,c Dane Sommer, DMln,d and John Lantos, MDe Department of Pediatrics, University of Chicago, Chicago, Illinois; bDepartment of Pediatrics, University of Pennsylvania, Philadelphia, Pennsylvania; cDepartment of Pediatrics, University of Utah, Salt Lake City, Utah; and dChaplaincy Services and eDepartment of Pediatrics, Children’s Mercy Hospital, Kansas City, Missouri a

ABBREVIATION NEC—necrotizing enterocolitis www.pediatrics.org/cgi/doi/10.1542/peds.2010-0079 doi:10.1542/peds.2010-0079 Accepted for publication Apr 13, 2010 Address correspondence to John Lantos, MD, Children’s Mercy Bioethics Center, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2010 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.

With this article, we introduce a new feature in Pediatrics called “Ethics Rounds.” The goal of this new feature is to present clinical cases that raise ethical issues. We present each case to pediatricians and bioethicists, who comment on the case on the basis of the information that they have at the time. We then give these same commentators the conclusion of the case and ask for further reflections. The case is printed in italic font, and comments are in normal, Roman font. To protect patient privacy, we change certain facts of each case. Thus, although the reports are based on real cases, the names, ages, location, and gender of the patient may be changed if they are not relevant to the central issues of the case. We invite readers to submit cases to the editor of Ethics Rounds: John Lantos, MD ([email protected]). Our first case involves an extremely premature infant who developed necrotizing enterocolitis (NEC). We presented the case, as it evolved, to 2 experienced pediatrician-bioethicists, Dr William Meadow and Dr Chris Feudtner. We then asked another pediatricianbioethicist, Armand H. Matheny Antommaria, who has training in religious ethics to comment on the case and the discussion.

CLINICAL ETHICS CASE REPORT Infant Smith was born at 25 weeks’ gestation by emergency cesarean delivery after placental abruption. His birth weight was 475 g. He was stable for the first 4 days. A head ultrasound on day 2 showed a grade II intraventricular hemorrhage on the right. He had oxygen saturation levels of 94% on low ventilator settings. On day 5, Infant Smith experienced a decrease in oxygen saturation and abdominal distension. An abdominal radiograph showed free air in the abdomen. A surgical consult was obtained, and an exploratory laparotomy was scheduled. As part of the consent for surgery, the parents were asked to consent for a blood transfusion. The parents became very agitated. For the first time since the infant was admitted they stated that they were Jehovah’s Witnesses and that they would not consent to the use of blood or blood products. At a care conference, the parents asked more questions about the nature of the surgery: Was it necessary? Could they wait? Could it be done “bloodlessly?” Would the surgery and the blood transfusion guarantee that the infant’s life would be saved? After discussing these issues, the parents stated that they would not consent to the surgery unless the surgeons would promise not to use blood products. The mother stated, “I would rather just hold my baby and see what happens than give my consent for something that is wrong in the eyes of God.” An ethics consultation was held.

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William Meadow First, this infant must be small for gestational age. If not, he would be closer to 600 g. So, his prognosis is a little better than his birth weight alone would suggest. I would want to know a little more about his respiratory status. Is he stable on pressures of 22/4 centimeters of water with a fraction of inspired oxygen (FIO2) of 40%? Or 28/4 centimeters of water and an FIO2 of 80%? That is important to know, because the more lung disease the infant has, the worse his long-term prognosis is. I am surprised that the doctors did not already know that the parents were Jehovah’s Witnesses. Didn’t it come up when the mother needed surgery? I would not agree to try surgery without the option of giving blood. This infant is too small and the surgery too big. We have learned that relatively stable infants can do well with quite low hematocrit levels. I have managed many Jehovah’s Witness infants and allowed their hematocrit levels to drift into the teens, and we have had infants born at 1100 g get through the NICU without a blood transfusion. However, that would not be possible in this case. He needs surgery to survive, and to have surgery, he will need blood. Generally, my first impulse in this case would be to say that parents are not allowed to withhold blood transfusions from their children. It is a little complicated, because this infant’s prognosis is guarded as a result of the combination of small-for-gestationalage status, 450-g birth weight, lung disease, and NEC. If the parents had said, “We went on the Internet and saw that the outcome of babies with this constellation of illness is dismal. We want to withdraw life support,” I would probably support them. But, assuming the parents do not want to withdraw all life-sustaining therapy,

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I would get a court order to give blood during surgery. Chris Feudtner When I encounter an ethical problem, whether in the role of clinician or ethics consultant, I organize my approach by asking a set of routine questions. Although I will present them in a neat sequential list, in practice they all fill my mind simultaneously as I work toward some provisional answers. First, what is going on and who is and needs to be involved? This sounds so naive, yet I am sure that if we asked different people caught up in this scenario that question, we would get different answers. The clinicians likely see a premature infant with new-onset NEC that, if left untreated by surgery, almost certainly will be fatal; the parents likely see a child whom they love being caught in a situation that tests the basic tenets of their faith, specifically regarding the transfusion of blood products. I anticipate that emotions run high on both sides. I do not work alone: I would (and did, in fact, when gathering my thoughts about this case) talk with the surgeons, neonatologists, and other members of the ethics-consultation services and would involve nurses, the parents, and perhaps members of their faith community in the discussions. Second, what are some key facts? For this premature infant, born at 25 weeks’ gestation, the initial probability of survival at the time of birth was 65%; having now survived to day-oflife 5, that probability of survival has risen to 75% to 80%. Now the infant probably has NEC, which in the first days to weeks after birth has an estimated mortality rate between 30% and 60%.1 The initial surgery to treat NEC in this age group would likely consist of placement of surgical drains. Although prognosis has a lot of uncertainty, approximately one-third of these infants would subsequently die, one-third

would require a second operation (and possibly more), and one-third would heal with no more surgical intervention. The grade II intraventricular hemorrhage does not change the prognosis for the worse at all. It is important (and interesting) to note that the likelihood of an infant born at 25 weeks’ estimated gestational age requiring a blood transfusion at some point is nearly 100%. In other words, the infant would almost certainly have needed a blood transfusion even if NEC had not occurred. In general, the potential harms of a blood transfusion are very rare. Whether the child or parents would suffer social shunning if the transfusion were to occur is another potential type of harm that should be assessed. Third, how much time do we have? Is the situation an immediate emergency, do we have a few hours hastened by clinical urgency, or are events unfolding at a pace at which we have days or even weeks to fully understand what is going on and make a decision? In this case, the infant’s condition is likely to deteriorate rapidly, measured in minutes to hours; if preserving life is our top priority, the quicker a decision is made, the better. Fourth, what are the key ethical concepts or frameworks that can guide our decision-making? Most readers are aware of similar cases, in which court orders were obtained to move ahead with blood transfusion without parental consent. The rationale for such decisions to countermand the preferences and beliefs of the family has to do with the state’s role in protecting children and advancing their best interests. More explicitly, the decision is based on prioritizing the child’s physical health over the family’s spiritual concerns, especially given the disproportional amount of benefit that the infant is likely to receive from the transfusion compared

with any physical harm from this course of action. Finally, what are our next steps, and what could go wrong with this plan? Given the urgency bordering on emergency of new-onset NEC, and the rough estimate that this infant’s chances of survival are 50% with routine surgery and blood transfusions, we should begin to seek a court order to authorize treatment. In parallel, we should be engaging in ongoing respectful conversations with the parents to describe our interpretation of the situation and our treatment plan, including securing a court order, while inviting their comments and critiques, participation, and partnership. We should also clarify whether they understand that, at some point, their infant is almost certainly going to require a blood transfusion; if they do not understand that, we need to explore why. As we move forward, we need to promote a forthright and supportive dialogue and keep a close eye on how the infant is doing clinically, because he is in a perilous state. If things go well for him, that is wonderful; if not, and further treatments have a disproportionate preponderance of probable harm compared with benefit, the rationale for our decisions up to this point may lead us to have to change course. The ethics-committee meeting included the neonatologist, surgery fellow, nurse practitioner, 2 primary nurses, intensive care nursery (ICN) social worker, chaplain, and nurse manager, as well as members of the hospital ethics committee and the parents. When asked about their preferences for treatment, the parents explained that they loved their new infant. They explained that their understanding of time might be different from that of “nonbelievers.” They said, “We would mourn the death of our child but

openly accept the brevity of this life for the glory that comes with being in the presence of God.” They asked about the possibility of bloodless surgery. The surgery fellow explained that “bloodless” techniques could not be used for an infant this small. The infant would certainly need multiple transfusions to survive. He explained that surgery should be performed immediately and that delaying surgery would worsen the prognosis. He also explained that, even with surgery, the chances for survival were low. The parents noted that the infant’s belly looked tighter and dark and that he did not seem to be responding to their touches as much. The mother reiterated, “Then I would just like to hold him and see what happens over the next 24 hours.” The parents were excused to return to their infant’s bedside. The team discussed the ethical balance between the parents’ autonomy to make decisions and the principle of beneficence for the patient. After a long discussion, the committee decided that the prognosis for this infant was sufficiently poor for the parents’ preferences to prevail. The parents once again were presented with the option of surgery or comfort care; they chose comfort care. They stayed at the infant’s bedside until he died 2 days later.

if you imagine that good ethics requires the consistent application of principles such as beneficence or autonomy. I prefer an approach that might be called “principled casuistry.” By that approach the principles are important, but they need to be adjusted to the peculiarities of the case. The interesting cases are all ones in which principles conflict. I often try to “reason backward,” that is, try to figure out what the best ending is likely to be, and then get there from where you are now. In this case, the likely ending was that the infant would die. We needed to care for the infant, but we also needed to care for the parents, which means trying to accommodate their beliefs in ways that do not lead us to do something that is manifestly against the interests of the infant. If the prognosis had been much better (if the infant had, eg, been born at 34 weeks’ gestation instead of 25 weeks), I would have been much more likely to go to court. One last point: when I do take Jehovah’s Witness parents to court, I try to warn them that it may not be pretty. I try to get them to understand that the judge may not be sympathetic, that I am on their side, that I am not angry with them, and that I will be there for them and their infant after the court hearing is over. Chris Feudtner

William Meadow Knowing what I knew at the time of the consult, I probably would have sought a court order for blood transfusions and gone ahead with the surgery. At that point, it seemed, the parents wanted everything done except a blood transfusion. However, I do not have a problem with the approach that the ethics committee took. It seems weird; I would not permit bloodless surgery, but I would allow no surgery at all. But, it only seems weird

We all would probably be well served to recall, from time to time, the Hippocratic aphorism regarding the practice of medicine: “Life is short, the art long, opportunity fleeting, experience perilous, judgment difficult.”1 In tough cases such as this one, with the perilous state of the patient and the press of fleeting time combined with clinical uncertainty, decisions have to be made relatively swiftly; the decision-making participants do not have the luxury of reading a neatly written case narrative

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and mulling things over. Still, in the mode of reflective practice, we can examine how this or other situations were handled and try to better understand how and why we (health care providers in general) make decisions the way that we do and how to improve this art. In retrospect, laudable aspects of how the clinicians handled this situation deserve praise, including the request for ethics consultative service, the representation of diverse points of view at the (presumably) expedited emergency ethics consultative meeting, and the respectful and forthright engagement with the parents. At the same time, this case raises 2 sets of intriguing questions. First, how and why did the fact that the parents were Jehovah’s Witnesses go unnoticed, especially given the 1 of 3 risk of dying that the infant had at the time of admission? What anticipatory counseling did the parents receive in the first days of hospitalization about the likelihood of not only blood transfusions but also other common clinical events? Answers to these questions might lead to quality improvement of routine parental and family assessment and counseling. Second, the committee’s decision seems to have hinged on judgment that “the prognosis for this infant was sufficiently poor,” such that the potential benefit for the child of receiving a blood transfusion dropped so low that the balance of considerations tipped in favor of honoring the parental power to make decisions on behalf of their child, but what constituted “sufficiently poor?” Perhaps the infant appeared sicker to the clinicians, yet on the basis of the facts that we have in front of us, the published data as mentioned above suggest that this patient had a 50/50 chance of survival or better. Perhaps not just the prospect of mortality but also morbidity was fac-

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tored into the prognosis; the data here suggest that an infant such as this one who survives NEC that required surgery has an 50% chance of still being on total parenteral nutrition 3 months later2 and subsequently elevated risks of poor growth and neurologic impairment (increased from 40% in infants with NEC who do not require surgery to 57% in infants with NEC who do require surgery).3 Does this prospect of mortality and morbidity, spelled out in quantitative terms, represent “sufficiently poor,” or is there a prognostic pessimism bias at work, similar to but in the opposite direction from the optimistic bias observed among oncologists regarding the prognosis for their patients? If so, what factors about the clinical scenario or the participants account for this phenomenon? Armand H. Matheny Antommaria This case raises significant issues about both the role of religious beliefs and the process of ethics consultation. Jehovah’s Witnesses reject the use of blood on the basis of their interpretation of the Bible and precedents in the history of Christianity. Relevant verses include Acts 15:28 –29: “For the Holy Spirit and we ourselves have favored adding no further burden to you, except these necessary things, to keep abstaining from things sacrificed to idols and from blood and from things strangled and from fornication.” This prohibition is understood to encompass both intravenous administration and individual cellular components such as platelets. Individual discretion is permitted regarding “minor fractions” such as immunoglobulin. They do not oppose other forms of medical treatment and emphasize alternatives to transfusion such as non-blood-volume expanders and blood-conservation surgical techniques.4 Members who voluntarily receive blood and do not repent are understood by the Jeho-

vah’s Witnesses to have revoked their memberships. Adult Jehovah’s Witnesses may refuse blood products even if they will die without them or even if third parties, such as their children, would be indirectly harmed. For minors, the presumption in favor of parental decisionmaking can be overridden if the decision is contrary to the child’s best interests. Some commentators reject parents’ religiously based evaluations of risks and benefits and assert that transfusion is justified to prevent death. Others seek to remain neutral regarding religious beliefs and instead protect the child’s future autonomy; the transfusion permits the child to mature and later decide whether to become a member of the religious tradition himself or herself. Interventions contrary to parents’ preferences must generally be necessary, discrete, highly effective, and without significant adverse effects. The primary focus should be on the child’s interests rather than the parents’ reasons. Although the ethics committee concluded that a transfusion was not justified because of the patient’s poor prognosis, it is not clear whether the prognosis was sufficiently poor at the onset or only became so poor over time. This raises the issue of whether there were avoidable delays in the process. ● Why weren’t the parents’ religious

beliefs identified earlier, such as while obtaining informed consent for transfusion as part of admission to the NICU? ● Was there sufficient information to

justify an ethics consult before the care conference, and could the 2 meetings been consolidated? ● How long did it take to schedule the

ethics consultation, and did the use of the full committee, rather than an individual consultant or small team, delay the process?

The limited information in the case description leaves open the possibility that a delay substantially affected the outcome.

EDITOR’S COMMENTS This case brings together the difficulties in prognostication for infants born at the threshold of viability, the added prognostic uncertainty associated with NEC, and the issue of whether parental religious beliefs should be

considered in decisions about lifesustaining treatment for an infant. If the infant’s prognosis for survival was sufficiently poor, then a decision not to proceed with surgery seems justifiable simply from the perspective of what is best for the infant. The challenge is to figure out how bad the prognosis must be to be considered sufficiently poor. In this case, the clinicians themselves disagreed. Some thought surgery would be beneficial, and others did not.

Disagreement among expert clinicians who are close to the case is itself an indication that the likely outcome is, at the very least, ambiguous or uncertain. Such disagreement might itself be a useful and reliable procedural criterion for deciding when parental choices ought to prevail. When doctors themselves disagree, parents should have the right to cast the deciding vote. —John Lantos, Section Editor

2006;355(8):856]. N Engl J Med. 2006;354(21): 2225–2234 3. Hintz SR, Kendrick DE, Stoll BJ, et al; NICHD Neonatal Research Network. Neurodevelopmental and growth outcomes of extremely low birth weight infants after necrotizing enterocolitis. Pediatrics. 2005;115(3):696 –703

4. Watch Tower Bible and Tract Society of Pennsylvania. How Can Blood Save Your Life? New York, NY: Watch Tower Bible and Tract Society of Pennsylvania; 2006. Available at: www.watchtower.org/e/hb/ article00.htm. Accessed February 11, 2010

REFERENCES 1. Lloyd GER, Chadwick J, Mann WN. Hippocratic Writings. New York, NY: Penguin; 1978 2. Moss RL, Dimmitt RA, Barnhart DC, et al. Laparotomy versus peritoneal drainage for necrotizing enterocolitis and perforation [published correction appears in N Engl J Med.

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A Saudi Family Making End-of-Life Decisions in the PICU In this “Ethics Rounds,” physicians must decide how far to go in respecting the wishes of a Saudi Arabian man whose infant is dying in the PICU. The man requests an approach to end-of-life decisions that is deeply rooted in both Saudi culture and the tenets of Islamic bioethics but at odds with prevailing US law and bioethics. We asked 3 physicians with expertise in cross-cultural issues to comment on the issues raised by this case. Dr Aasim Padela is an emergency medicine physician and a fellow in the Program on Medicine & Religion at The University of Chicago. Dr Sadath Sayeed is a neonatologist at Boston Children’s Hospital and on faculty in the Division of Medical Ethics at Harvard Medical School. Dr Maryam Naim is a pediatric intensivist at the Children’s Hospital of Philadelphia.

PART I Case Representation A 2-month-old boy was found unresponsive in his crib after his mother heard him choke shortly after feeding. His parents drove him to the emergency department, where he was apneic and pulseless. He had return of spontaneous circulation after 30 minutes of cardiopulmonary resuscitation and was transferred to the PICU. On physical examination, the infant has intermittent respiratory effort over the ventilator, no withdrawal from painful stimuli, and no pupillary response to light. Over the next few days, his neurologic examination remained limited to intermittent seizures and irregular respiratory effort. There are no other signs of brainstem function on serial examination. The parents are young and had recently emigrated from Saudi Arabia. The mother is wearing a full black burqa exposing only her face. She speaks no English. The father is wearing a tee shirt and jeans and speaks fluent English. A meeting is scheduled with both parents, the neurologists, social workers, PICU team, and an Arabic interpreter to discuss the prognosis and plans. Before the day of the meeting, the father meets alone with the attending physician. He tells her that he understands his son’s dire condition and bleak prognosis and asks that the physician discontinue the ventilator and let his son die but not tell his wife that they stopped the ventilator. She will, he says, accept the fact that the boy has died, but knowing the details would be devastating to her. Is it permissible to stop the ventilator in this case after discussing it only with the father? Aasim Padela Ethical dilemmas, and ethical choices, are socially produced. They are the products of context-specific interpretations of power dynamics, social practices, and cultural values.1 The field of bioethics tries to find areas of overlap among different moral traditions. By using this approach, we

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AUTHORS: Sadeth Sayeed, MD, JD,a Aasim Padela, MD, MSc,b Maryam Y. Naim, MD,c and John D. Lantos, MDd aHarvard Medical School, Boston Children’s Hospital, Boston, Massachusetts; bProgram on Medicine & Religion, The University of Chicago, Chicago, Illinois; cChildren’s Hospital of Philadelphia, Philadelphia, Pennsylvania; and dChildren’s Mercy Hospital, Kansas City, Missouri

KEY WORDS ethics, Islamic bioethics, bioethics, pediatric intensive care, withdrawal of life-support, infant www.pediatrics.org/cgi/doi/10.1542/peds.2011-1117 doi:10.1542/peds.2011-1117 Accepted for publication May 18, 2011 Address correspondence to John D. Lantos, MD, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64105. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2012 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding.

often find that peoples’ choices and preferences and values are often understandable even if we do not agree with them. So we must ask (1) why the father suggests his course of action, (2) whether it is appropriate within the family’s ethical framework, and (3) whether we can defend it ethically within our value system. This case has several morally relevant points: (1) our 2-month old patient is in a minimally conscious state with a relatively poor prognosis; (2) his family are recent emigrants from Saudi Arabia and can be reasonably assumed to be Muslim and thus informed, at least in some degree, by Islamic values; and (3) the father’s request to remove life support without informing his wife is controversial given the prevailing bioethical ideal within US culture of parental consensus when making such decisions. Importantly, in this case, the moral conflict over the father’s request lies not in his decision to remove life support but rather in his preference for providers’ to withhold information from, and potential decision-making authority allocation to, the mother. Several pieces of information are necessary to fully evaluate the father’s request. What is the exact boundary of his request to withhold information and his underlying rationale? Does he want providers not to engage in clinical prognostication with the mother or for providers not to relay to the mother the father’s request/decision to withdraw care? Does he want providers to lie? From a Muslim (separate from a normative Islamic tradition–based) bioethics perspective, there are several possible reasons that the father asks for the mother not to be told that life support is being withdrawn. He may feel motivated by a sense of responsibility to make the “tough” decisions within the family and shield others from the psychological distress of these decisions. This idea may be mediated by potential

differences between the parents in their understandings of the clinical situation or acculturative factors (hinted at by the way the scenario is presented [eg, the differences in clothing and language proficiency between the parents]). In many Muslim societies, family unity is prioritized over individual autonomy. The father may be motivated by a desire to maintain family unity and feel a dual parental consent process may lead to familial discord. In my own research, I have found that many Arab Muslim families find the choice to withdraw life support to be especially burdensome within their cultural context. Family members are often paralyzed when faced with such a decision due to their fear of culpability in front of other family members, and in front of God, for withdrawing care upon a loved one. Moving from the Muslim cultural context to an Islamic bioethical perspective, several other factors are worth mentioning. It is possible that the husband is motivated, at least partially, by Islamic tenets. Seeking medical care is not always an obligation, and thus it is not always obligatory to continue life support, according to the majority of Islamic scholars.2,3 Hence, life support can be withdrawn when the burdens, social, personal, or otherwise, are considered to outweigh possible benefits. Furthermore, medical experts are given wide latitude in determining the risk/benefits of medical care. This “authority” allows for the advice of a “righteous” physician to be considered beyond reproach ethically and legally. Hence, the father here, in following medical advice, may be making a choice to withdraw life support that is consonant within the family’s Islamic tradition. In addition, in the Islamic tradition, he is accountable for his family, both legally in this world and ethically in the hereafter. He may feel that the responsibility and burden to decide are his solely.

Given these religious and cultural considerations, some physicians who care for Muslim patients absolve family members of surrogate decision-making responsibility by couching the decision to withdraw life support as a purely medical decision. These providers do not ask families’ for formal consent to change code status. Rather, they use passive assent. Still, the question remains: is the father’s preferred approach tolerable to us as providers? I think that the best course of action should be to explore the reasons behind the father’s request and whether we feel that his actions are “ethically sound” according to the values of the family. My initial suggestion is for the health care providers, with or without ethics consultations, to inform the father of their medical ethics tradition and explain their moral, and potentially legal, concerns with his request. They could next offer to arrange a meeting with him, his wife, and, should the couple desire it, both an Islamic religious leader and a physician with insight into Muslim culture. Such a meeting would better elucidate his values and concerns. By entering into a dialogue, a middle ground may be found where neither side has to betray their moral positions all together. Sadath Sayeed This case raises interesting questions that start at the clinical bedside but move to the meta-ethical. For the purposes of the initial discussion, I make 2 assumptions. First, I assume that the information conveyed to the father is that the child has irreversible, devastating global neurologic damage. Second, I assume that there is consensus among the medical teams that it is now appropriate to withdraw life-sustaining interventions. (In reality, we should recognize that it often takes weeks to be sure of the prognosis and to reach such consensus.)

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Given these assumptions, the ethical issue then moves toward consideration of what has popularly been termed “respect for cultural differences.” The case offers a predictable, even stereotypical, picture of a Muslim couple from the Middle East, with a male authoritarian figure prepared to make all decisions. It is likely that, in Saudi Arabia, the father’s request would be respected with little or no hesitation (certainly without the need for an ethics consultation). It is also plausible that, in Saudi Arabia, the mother might accept her son’s tragic death and, while suffering the expected grief, she would not, in the months and years that follow, ever question her own role in the decisions that were made. She also would not struggle with guilt or carry regret that she did not “do everything possible” to save her son. She would accept her role, just as the father would accept his. These are not trivial considerations. It may be that it is precisely these outcomes that our more paternalistic Saudi counterparts feel a moral obligation to promote. Finally, let us imagine that the father explains all of this to the PICU attending in the private meeting. He reminds the attending that he knows his wife and their culture better than do the team of US specialists. He even offers to bring in a female physician friend of his who works in the Middle East as a consultant. Even with all these assumptions, I suspect that many clinicians and pediatric bioethicists in the United States would not abide by his wishes. We would insist that the case be dealt with by using the values of our (US) modern legal tradition and professional clinical ethics. We would assert that the wife has a right to participate in medical decisions that the husband cannot take away. So, when the rubber hits the road, we resist the temptation to yield to the persuasive considerations that the father has put forward. We do so, we tell

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ourselves, because even if the father is right and the mother might be better off, we worry about the broader moral implications of endorsing an idea of parental moral standing that does notfinally rest on equality for each individual. This scenario returns us to the challenge of what we might really hope to accomplish when we express a need for maintaining “cultural sensitivity.” Beyond listening to our patients’ parents in a way that sincerely acknowledges different values (not unimportant), do we really offer respect for those values? What might respect look like to the father in this case? Ultimately, it can mean only one thing: to do as he asks, for the reason that he asks, and accept that his love and concern for his wife lead him to different conclusions about his, and our, duties and obligations. If we are unprepared to go this far, then we need to accept that, when our value systems truly clash with those of our patients and families, we probably do not show much cultural respect. Instead, we assert universalism and insist that our own values are the ones that should be universalized. Maryam Y. Naim It is important to understand the context of this father’s request. To do so, it is necessary to understand gender roles in Saudi culture, which differ significantly from those in Western culture. Traditionally, women in Saudi Arabia live in a protected environment and are not part of decision-making in their family structure. Saudi women always have a male “guardian.” When unmarried, this is their father. After marriage, the responsibility of guardianship falls on a woman’s husband. The guardian is responsible for all decision-making for the woman, including decisions about whether she will be allowed to go to school, marry, or travel. The guardian also gives consent on her behalf for medical procedures.

Guardianship is closely tied with the concept of “namus” relating to a woman’s modesty and honor. Namus is a cultural concept and is an important asset for a woman in Saudi society. A woman must maintain her honor and that of her family; to maintain this honor, women cover themselves from head to toe and do not associate with men who are not related to them. This practice is called “khalwa” or “purdah.” If a woman performs a “dishonorable” act such as associating with a man who is not in her family, serious repercussions can occur. Not only can the woman be shunned from society but, in rare circumstances, an “honor killing” can occur in which a man kills a woman who performed a dishonorable act to maintain the family honor. Legally, in Saudi Arabia, children belong to their fathers, who have sole guardianship. Although these practices are viewed by many in the Western world as violations of women’s rights, they are widely accepted by the majority of women in Saudi Arabia. In conclusion, it is important for the attending physician to understand that the father of the infant is trying to protect his wife by excluding her from the decision-making process, which is a Saudi cultural norm.

PART II Case Representation The physicians decide that a family meeting is required and that the mother must know about the discussion to stop the ventilator. The medical team, including an Arabic interpreter, meets with both parents. They explain that the infant is only being kept alive by the ventilator, that his brain is injured beyond recovery, and that he has no chance of meaningful survival. The physician recommends that the ventilator be stopped. She asks the parents if they have any questions; neither parent does. The physicians and interpreter

leave the parents alone in the room to talk together. A few minutes later, the father comes out of the room alone. He tells the physician that his wife understands that the infant is dying and will die after they leave the hospital. He says that he and his wife will leave the hospital now and do not want to be there when the infant dies. He asks the physician to disconnect the ventilator after they are gone. The physician wants to have a conversation with the mother to be sure that she agrees and understands. Stating that his wife is devastated by the loss of their son, the father pleads with the physicians to let them both leave without further discussion and to withdraw the ventilator after they are gone. Is it permissible to withdraw the ventilator based on the father’s assurance that the mother agrees? If her assent is required, what are the reasons? If her assent is not required, why not? Sadath Sayeed After the family meeting with the interpreter, we can reasonably assume that the mother heard the facts and recommendations about her son’s condition and care. However, we cannot presume to know how much she understood or what her ultimate feelings are about the situation. Does our allegiance to a certain ideal of parental moral standing now obligate us to override the father’s latest request to simply let them be? Is it permissible to risk deep cultural offense for the sake of preserving our sense of ethical duty? This is where the case pushes us beyond the practical to the epistemological. How can we actually know that our moral ideas about parental roles and duties are superior to those of the Saudis? Are moral ideas that translate into practical clinical ethics truly universal or are they the product of local social and cultural norms, and historical attachments to certain strands of philosophical and/or

religious tradition? Before we further intrude upon the private grieving of this family, we ought to seriously consider these questions. My worries about whether this mother is acting autonomously reflect more general worries about the status of women in a society such as Saudi Arabia. I am particularly uncomfortable having a hand in perpetuating a system of gender inequality. I fear such a consequence to be one adverse effect of acquiescing to the father’s wishes in this case. Those worries are grounded in my sense that certain moral values pertaining to the inherent equality and dignity of each individual are universalizable. However, I also recognize that I really do not know if the mother wants a more active role in the decision-making in this case. It is all too easy to interpret her silence in a way that only reinforces my stereotypically informed ideas about how her freedom must be terribly constrained. I may be undermining her autonomy to live her life as she wishes, rather than promoting her autonomy by insisting that she live as I would have her live. There are dangers running in either direction. This uncertainty calls for humility on my part, as a physician, as an ethics consultant, and as a person genuinely concerned about how each of us relates to one another. I would abide by the father’s request in this case, because I am not convinced that my ideas about parental moral standing demand that I risk the immediate and future psychological harms that might accrue to this young couple. I am also not convinced that my ideas of moral value are so obviously and incontrovertibly superior to those of this family that I am willing to impose them. In the end, the tragedy of losing a child is this mother’s and this father’s and not ours. We go on to the next case, and they live with memories that we play

an instrumental role in shaping. Insisting that the mother verbalize her approval of the removal of life support seems to me more culturally imperialistic than respectful. Maryam Y. Naim According to our Western code of medical ethics, it is not permissible to stop the ventilator without permission from the mother. Withholding information and decision-making from her is a violation of her parental autonomy. It prevents her from participating in decisions that reflect her views of what is in the best interest of her child. However, this woman has been brought up in a different culture where decisions are made by male guardians (in this case, her husband). She participated in the family meeting and has heard about the prognosis of her child. The physician team should try to compromise with the fatherand talk to the motherand ask her whether she wants her husband to make these important decisions. This could be done by a female member of the team; that is, a resident, fellow, or attending asking the father for permission to talk to the mother on her own. Having a female talk to the mother on her own respects the Saudi cultural tradition of “khalwa”. The female physician could then confirm the mother’s understanding of the situation and whether she wants to delegate decision-making to her husband. If she does not want to participate in decision-making, this request should be honored by the physician team. Aasim Padela It is laudable that the patient care team facilitated a family meeting with an Arabic interpreter, presented the clinical scenario to both parents, and allowed both parents the time to ask questions and thendeliberate. Ultimately thefather agrees with the team’s recommendation to withdraw life support. At this point, I would argue that the medical team’s

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ethical responsibility has been discharged, and they should proceed in disconnecting life support. That the father voices a desire on behalf of both parents to not be present when the support is withdrawn, to me, raises no ethical controversy. Watching a loved one die is hard, and if people desire to not witness these moments of emotional and psychological hardship, I find no quarrel with that. Drawing from the pool of physician beneficence, a physician could easily justify such a course.

when life support is discontinued upon their loved one for psychological and moral reasons. I would not advocate one approach over the other but simply present the options to the family and honor their wishes.

Some may suggest that the parents should be at the bedside when the actual moment of death arrives, that being physically present would offer closure and facilitate the grieving process. A colleague of mine, a nurse who studies transcultural issues, recommends that we should offer the family a chance to make their loved one’s death a family moment where the physical presence of family spiritually supports the transition from this world to the hereafter. Such a course has its merits and has backing within a Muslim cultural framework where family members often recite prayers for forgiveness or read from the Qur’an to soothe the departing soul.

The more difficult issue is whether it is necessary to “have a conversation with the mother to be sure that she agrees and understands.” I assume that the attending physician is concerned about both her understanding and her agreement with the decision. One might ask whether this is an appropriate concern for her autonomy or whether it instead reflects the provider’s stereotypical view of Muslim women as uneducated and oppressed. I would argue that the mother does not have to “undergo” further conversations. She already chose to passively assent to the decision undertook by her husband. This decision is in accordance with medical recommendations. Further interrogating the mother does not offer any real benefits. Worse, it could harm the relationship between that family and the health care providers by showing a profound disrespect for their culture, their traditions, and their moral preferences.

However, another plausible Islamic framing of the proper role for families is also possible. By this view, one should not witness a morally questionable action to avoid a moral obligation to intervene. There are some Muslim familieswho find withdrawing life support for those who are minimally conscious orbrain dead to be unacceptable Islamically. Because most states do not allow a religious exemption for brain death, some families choose to be absent from the bedside

It is appropriate to be concerned for the mother’s autonomy. But even within an autonomy-oriented bioethics, an individual can authorize another to make decisions on his or her behalf. In this case, the principle of respect for persons should be considered preferable to that of respect for autonomy. Under this framework, the providers would view this family through their own cultural frame and ethical value system, rather than through a Western cultural

frame, with its 4 principles and its intense focus on individuality. This flexibility in moral outlook might avoid a rigid and inflexible practice-style that is antithetical to humanistic practice and does not meet the needs of a pluralistic and diverse society. We need to come together with patients to find shared values and goals that enable us to meet ethical standards of medical practice. When we do so, we may find that our goals (those of the patient/family and our own) may be the same even if the process in getting there may differ. John D. Lantos Bioethical paradigms are rooted in culture. We tend to think of our way of doing things as the only way because it reflects our own cultural beliefs and values. When confronted with a different culture, however, we have to go back to first principles and ask why, exactly, we do things the way we do. Is it because we are firmly convinced that our way is the right way for all people in all cultures at all times? Or is it because our way works well for us? The way we answer these questions determines how we respond to challenges for another cultural system. Personally, I worry about claims of universal moral values in situations such as this one. Decisions about removing life support are too new, in world history, to have been thoroughly analyzed from a moral, psychological, religious, or cultural perspective. Thus, I think deference to other views is appropriate. In this complex moral and ethical territory, we need to learn from one another.

REFERENCES 1. Orfali K. Parental role in medical decisionmaking: fact or fiction? A comparative study of ethical dilemmas in French and American neonatal intensive care

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units. Soc Sci Med. 2004;58(10):2009– 2022 2. Yacoub A. The Fiqh of Medicine. London, United Kingdom: Ta-Ha Publishers Ltd; 2001

3. Ebrahim AFM. An Introduction to Islamic Medical Jurisprudence. Durban, South Africa: The Islamic Medical Association of South Africa; 2008

Symbolic Resuscitation, Medical Futility, and Parental Rights AUTHORS: Joel Frader, MD,a,b Eric Kodish, MD,c and John D. Lantos, MDd,e Department of Pediatrics Medical Humanities/Bioethics, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; bPalliative Care, Children’s Memorial Hospital, Chicago, Illinois; cDepartment of Bioethics, Cleveland Clinic, Cleveland, Ohio; dChildren’s Mercy Bioethics Center, Children’s Mercy Hospital, Kansas City, Missouri; and eDepartment of Pediatrics, University of Missouri, Kansas City, Missouri

a

www.pediatrics.org/cgi/doi/10.1542/peds.2010-1379 doi:10.1542/peds.2010-1379 Accepted for publication May 21, 2010 Address correspondence to John D. Lantos, MD, Children’s Mercy Bioethics Center, Children’s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

What should doctors do when they are caring for a dying child with a complex, chronic condition and whose parents will not agree to palliative care? What if those parents are cognitively delayed and have lost legal custody of the child because of their inability to care for her? This month’s Ethics Rounds concerns a child with septo-optic dysplasia and respiratory failure. She is cared for by her grandmother and is legally a ward of the state. The doctors think that a do-not-resuscitate order is appropriate. Her grandmother agrees and is even ready to withdraw ventilator support. Her parents disagree. We asked 2 experts in bioethics consultation and palliative care to comment on this case. Joel Frader is Chief of General Academic Pediatrics and Medical Co-Director of Pediatric Palliative Care at Children’s Memorial Hospital in Chicago, Illinois. Eric Kodish is Chair of the Department of Bioethics at the Cleveland Clinic in Cleveland, Ohio.

Copyright © 2010 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.

CLINICAL ETHICS CASE REPORT D. K. is a 2-year-old girl with septo-optic dysplasia, microcephaly, seizure disorder, and severe developmental delay who is intubated for respiratory syncytial virus with respiratory failure. Her past medical history is significant for microcephaly and severe developmental delay since birth. Her baseline functioning is minimal—she has no purposeful movement and no speech or comprehension of language. She is unable to swallow and is given nutrition through a gastrostomy tube. Both of her parents are developmentally delayed and were unable to care for her at home. The state placed her with the maternal grandmother. Although the mother and father no longer have custody, they have been in the PICU and at the bedside daily since the patient was admitted to the hospital. The patient was admitted in respiratory failure and intubated. On the second hospital day, she suffered an acute respiratory decompensation on the ventilator and required a high-frequency ventilator at a fraction of inspired oxygen of 100%. Her primary care pediatrician and the PICU team recommended a do-not-resuscitate (DNR) order. The grandmother agreed that a DNR order and withdrawal of ventilator support is appropriate. The state child protection agency agreed to a DNR order if the hospital ethics committee approves it. The patient’s mother and father are adamantly opposed to a DNR order or withdrawal of ventilatory support. They are “hoping for a miracle.” The PICU’s attending physician called for an ethics consult. Our ethics question is: Is a DNR, with the consent of the grandmother and the state but over the objections of the parents, appropriate for this patient?

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Joel Frader From a “medical perspective,” a DNR order for D. K. seems to make sense. The child has a critical acute illness and a severe, presumably static encephalopathy, and under the circumstances, cardiac arrest would likely stem from prolonged ischemic damage because of inadequate gas exchange. In that case, cardiac compressions and inotropes probably would not work. If they did, she would likely suffer further brain damage. Pediatric intensivists and the child’s primary care physician could well consider a DNR order as part of a “big-picture” view of D. K.’s condition and invoke the child’s “best interest” as justification for limiting (further) resuscitation. If the state, as the child’s legally authorized decision-maker, and her primary caretaker both agree to a DNR order, then it would be legally permissible to write such an order. However, the matter needs closer scrutiny. First, no such thing as a universal medical perspective exists. If one queried many different pediatricians about this or similar cases, one would find a wide variety of responses. Some would rely on estimates of outcome for which no solid statistical data exist. Others would look at the child’s likely limited life span. Still others would draw on their own moral views, whether about resource allocation or the value of each human life. These preferences would likely rely on a complex and typically unconscious mix of scientific information, religion, social philosophy, and so on. Why should the views of D. K.’s doctors, or her grandmother, whatever their basis, automatically trump the views of the biological parents? Does the fact that her parents no longer have legal authority over D. K. mean that they no longer deserve moral consideration, either? The state presumably obtained responsibility for D. K.’s care because the

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parents could not consistently provide what D. K. needed to continue to live. We are not told that their failure reflected indifference or lack of care for their daughter, only that they could not manage her care. The grandmother apparently accepted day-to-day responsibility for D. K. with the aim of continuing the girl’s life. Now the parents strongly endorse that same objective—maintenance of D. K.’s life—albeit at a time the child’s life is threatened. Is it clear that the state, or more specifically an overwhelmed case worker who holds the state’s portfolio, and the grandmother have greater moral authority to consent to a DNR order than the parents? Before ignoring the parents’ feelings and preferences, one should want a substantive clinical assessment of the parents’ capabilities and the potential impact that not attempting resuscitation might have on them. Perhaps better communication techniques, using pictures or stories that would allow them to “connect” with their child’s condition or the likely failure of resuscitation, would help. Simply disregarding D. K.’s developmentally disabled parents may fail to provide adequate respect for the parents’ actual understanding and/or their emotional state. D. K.’s medical and psychosocial care team have more work to do. Eric Kodish This case, and all ethics consultations, can be appreciated as narrative. Cases play out over time, and the judgments we make at the beginning may differ from those at which we arrive in the end. Still, ethics consultants must respond to the facts as we know them, hoping that our advice will stand the test of time regardless of the next turn of events. The experienced ethics consultant accumulates a personal casebook, maps each case onto the template created by the ones that came before to

predict what might happen next in this case, and draws on that clinical story bank to craft ethical guidance. This case raises a number of questions. First, one must recognize that DNR status and withdrawal of ventilator support are not necessarily linked. The primary care pediatrician and PICU team do not go as far as D. K.’s grandmother in terms of treatment limitation. Does the health care team have consensus around what degree of limitation is in the patient’s best interests? The state child protection agency seems to take a processoriented approach, looking to the ethics committee to ensure thorough review before a decision on DNR is made. But, what would the authorities say if the committee recommended stopping the ventilator? D. K.’s maternal grandmother is in a complicated position. She does not have legal custody, but she has responsibility for D. K. I would want to know more about this family. How long has D. K. lived with her grandmother? Does the grandmother have any help caring for this challenging child? Is she just exhausted? I would also want to know about the paternal grandparents and maternal grandfather, if they are in the picture. Finally, the patient’s parents may have no legal standing, but they have important moral significance in this story. It would be cruel and disrespectful to completely exclude them from the decision-making process and ignore their hope. An excellent ethics consultant might help the key stakeholders to use their moral imagination to make a substituted judgment decision from the perspective of D. K. herself, but that would be quite a stretch given her past medical history. The better alternative would be to make an objective assessment of burdens versus benefits of continued support, keeping focus on the objective rather than the subjective to the extent possible. Clarity

about the distinction between DNR and stopping the ventilator is paramount: a 1-step-at-a-time approach necessitates these decisions to be uncoupled, and consideration of code status should precede any discussion of withdrawal. Finally, pediatric ethics requires that we prioritize the question “What is the right thing to do?” over the question of “Who gets to make the decision?” Focusing on the latter question would be the easy thing to do but the wrong way to look at this case. The ethics committee discussed several options. Some argued that the grandmother’s wishes should prevail, because she has custody and wants to do what the doctors are recommending. Others thought that the parents should have the right to decide because they would bear the greatest emotional burden from their child’s death. In the end, a consensus was reached that the ethics committee would recommend that the infant not receive cardiopulmonary resuscitation (CPR). Discussion then focused on how this should be communicated to the family. Some suggested a family meeting with the parents and grandmother present. Others suggested just telling the grandmother and leaving it up to her to communicate with the parents. One doctor suggested that the best solution would be a “slow code” in which doctors would attempt a brief, symbolic resuscitation. This, he argued, would allow the child to die but would not force the parents to acquiesce in a decision to withhold therapy from their child or to confront the grandmother about her complicity in the decision. Is such a slow code ethically permissible? Joel Frader The staff now wonders about “show” or “slow” codes to help the parents cope. In other words, the doctors and

nurses would go through the motions of resuscitation without actually conforming to the standards of good medical practice (adequate compression, ensuring oxygenation, medications to support circulation, etc). Can one ethically justify such behavior, recognizing the motivation to help the parents? It’s tempting, but it is not right. Such deception fails on a number of levels. As Sissela Bok argued some time ago, lying often goes awry. Humans cannot easily sustain the lie; somebody finds out, and the fact of the deception gets back to those one hoped to deceive. The revelation adds anger and mistrust to the disappointment that the original understanding was mistaken. Regaining that trust, if at all possible, takes tremendous time and effort. The more widely known the deception, the more individuals wonder and worry about whether they should trust their health care professionals. “Will my doctor keep her promise to keep my secrets from my lover/husband/children?” Although this lie seems acceptable, what about the next possible deception? Does that one involve a more serious violation of a prima facie duty to tell the truth? In D. K.’s case the professionals may feel tempted to excuse a show code because of the parents’ mental handicaps, but that only compounds the ethical violation. Such reasoning fails to respect the importance of trust and fidelity we owe all persons and perpetuates generally poor treatment of those with disabilities. Finally, the show code seems to be an attempt to avoid having to do the hard work of “owning up” to an institutional decision to put the parents’ wishes aside. Although the professionals, institutional officials, the primary care taker (grandmother), and the state may have acceptable reasons for enforcing a DNR order, the decision not to resuscitate does not let them all off the hook of having to com-

municate the decision clearly and compassionately to the parents. That discussion will be hard. We can all understand the wish to avoid it, but such conversations go with the territory of being a physician. We have to have them, so we should learn to conduct them well. We owe D. K.’s parents honesty and good psychosocial care regardless of what happens to their daughter. Eric Kodish Two new questions are raised, and I will dispense with the first and easier one first. Despite the suggestion of a well-meaning doctor, the ethics committee cannot and must not give explicit approval for a slow code. The long-term implications of suggesting that slow-code status would be morally acceptable in this case are untenable. It would lead to an erosion of trust and a cascade of problematic consequences. Patients, families, and health care professionals all need to rely on the good-faith assumption that when CPR is attempted it will be done with vigor and genuine hope for success. If the ethics committee were to provide an imprimatur of ethical legitimacy to the concept of a slow code, that good faith would be shattered for future cases. Doubt would permeate the clinical atmosphere. If you are going to do something, do it right. Charades are not acceptable when it comes to life-and-death matters. The second question is more complex and challenging. Although I generally disagree with those who contend that clinical ethics consultation is really just communication mediation, there is an element of truth to the assertion. What is the best choreography of communication in this narrative? Good clinicians are sensitive to familial burden and seek to minimize the pain in what are always tragic situations. Insisting that the patient’s grandmother be the

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messenger would add insult to injury. I would advocate for a family meeting with all present and frame the decision as agonizing but ultimately in the best interest of D. K. I would also be sure to elicit and attend to the spiritual care needs of the family at this final chapter in the life of their daughter. The ethics consultant recommended that the attending physician inform the grandmother of the decision to write a DNR order and withhold resuscitation. When the attending physician went to speak to the grandmother, the parents were there. In discussion, they all agreed that the infant should get some CPR. The grandmother said, “Okay, but only 3 chest compressions.” The parents agreed. The next day, the infant arrested, and a code was called. The infant got 3 chest compressions and then died. Joel Frader It it important to note that at the time of this discussion both the grandmother and the parents had an opportunity to participate in a discussion of what to do in case of cardiopulmonary arrest. Unfortunately, it seems that the grandmother and, by inference, the attending physician exploited the parents’ limited understanding of CPR. An attempt to explain the meaning of an arrest and what might constitute a real resuscitation would have been appropriate. If the medical team and the ethics committee really believed that CPR would fail under any circumstance for this infant, the only harm in a 10-minute effort with adequate compressions and usual medications would have been the “waste” of time involved. The staff could have used that time to teach resuscitation to inexperienced nurses, residents, and others.

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Professionals need to learn to put their interests—and feelings—aside in many circumstances, and this case provides a good example of such an occasion. Assuming that the physicians take care to ensure the infant’s comfort, either by having determined her inability to respond to pain or by providing analgesia, a legitimate resuscitation attempt would not harm the child and would preserve veracity for all concerned. Eric Kodish The dénouement of this narrative is disturbing. The ethics consultant must not become the ethics police, but some follow-up debriefing and education might be helpful for the future. Three chest compressions do not a resuscitation make. Although a DNR order would have arguably been the most ethically sound outcome, a full-fledged effort at resuscitation would have been much preferable to the showcode approach that was taken. By acceding to the grandmother’s request, the PICU team was practicing “a la carte” medicine, and in this admittedly difficult clinical situation, the team took the easy way out. I would convene a “what can we learn from this difficult case?” teaching session within a couple weeks of D. K.’s death and work with the PICU team to try to understand their approach and to enhance their understanding of why this outcome was problematic from the perspective of clinical ethics. We all need to keep learning from cases.

EDITOR’S COMMENTS Doctors’ primary ethical obligations are to the child who is their patient. They must try to do what is in the child’s interest and not cause harm or

pain to the child. However, they have ethical obligations to the family as well. Often, those obligations conflict. For this dying child, a full code was too much, for medical and humanistic reasons. For this dying child’s family, no code was too little, for emotional reasons. A limited and symbolic code (3 chest compressions) was just right. However, such symbolic codes do not fit well into the either-or dictates of our current ethical or regulatory environment. The grandmother got it. She understood that her granddaughter was dying. She realized that her daughter did not want to give up. She tried to find the best compromise between tragic reality and illusory hope. She recognized that CPR would be symbolic but also that symbols are important. Many arguments can be made against symbolic codes. They boil down to arguments against deception. Such concerns are valid if we tell the family that we are going to “do everything” and then do not. Such deception did not occur in this case. This case is not about deception. It is about the power to decide how to describe what was happening and what would be done. It was about the authority to determine what should count as truth-telling or deception. In a tragic case such as this one, that power and authority properly belongs to the family. They have the right to tell their own story. The grandmother exercised that power and authority wisely. We should learn from her. —John Lantos, Section Editor

Palliative Sedation With Propofol for an Adolescent With a DNR Order Liza-Marie Johnson, MD,a Joel Frader, MD,b Joanne Wolfe, MD, MPH,c,d Justin N. Baker, MD,a Doralina L. Anghelescu, MD,a John D. Lantos, MDe

Death from cancer is often painful. Usually, the pain can be relieved in ways that allow patients to remain awake and alert until the end. Sometimes, however, the only way to relieve pain is to sedate patients until they are unconscious. This method has been called palliative sedation therapy. Palliative sedation therapy is controversial because it can be misunderstood as euthanasia. We present a case in which an adolescent who is dying of leukemia has intractable pain. Experts in oncology, ethics, pain management, and palliative care discuss the trade-offs associated with different treatment strategies. ThE CASE Diamond is a 17-year-old girl with relapsed acute lymphoblastic leukemia, 7 months’ post–allogeneic bone marrow transplant. Over the past 2 months, her percentage of leukemic blasts has been increasing, and she has no cure-directed chemotherapy available for her current disease. She has been followed by the palliative care team since her relapse and was recently admitted to the hospital for pain control. During this admission, she developed respiratory distress and fever and was diagnosed with bilateral pneumonia. After extensive discussion of the options, Diamond’s mother decided to forgo transfer to the ICU and escalation in respiratory support while continuing all other therapeutic interventions, including antibiotics and total parental nutrition. A do-not-attemptresuscitation order was placed in the chart specifying that Diamond should not be treated with mechanical ventilation or cardiopulmonary resuscitation. Throughout the course of this admission, the palliative care and pain management teams intensively

treated escalating symptoms of pain and agitation with limited success. Despite significant opioid dose escalation, opioid rotation, and the use of adjuvant medications, Diamond continued to be highly agitated with grunting, moaning, and reports of high levels of pain. Additional strategies included a fentanyl infusion of 1000 μg/hour and then rotation to an equianalgesic dose of hydromorphone. Despite these measures, Diamond remained uncomfortable. A ketamine infusion, scheduled lorazepam, and haloperidol were added, without significant relief of her distress. Diamond’s suffering was extremely distressing to her family and the bedside nurses. The palliative care team suggested that Diamond may be a candidate for palliative sedation therapy (PST) using propofol. The medical team had concerns regarding the timing and appropriateness of the decision for PST, because Diamond’s pneumonia appeared to be improving with therapy. Her mother was agreeable to PST and asked the team to give her a dose that would quickly “end this suffering.” An ethics consult was called to determine the

abstract

aDivision

of Oncology Hospitalist Medicine, St. Jude’s Hospital and Research Center, Memphis, Tennessee; bPediatric Palliative and End-of-Life Care, Ann and Robert H. Lurie Children’s Hospital, Chicago, Illinois; cPediatric Palliative Care, Dana-Farber Cancer Institute, Boston, Massachusetts; dPediatric Palliative Care, Boston Children’s Hospital, Boston, Massachusetts; and eDepartment of Pediatrics, Children’s Mercy Kansas City, Kansas City, Missouri

Drs Johnson, Frader, Wolfe, Baker, Anghelescu, and Lantos contributed to the design of this paper, the drafting of the manuscript, the review of the manuscript, and approved the final version. DOI: https://doi.org/10.1542/peds.2017-0487 Accepted for publication Feb 14, 2017 Address correspondence to John D. Lantos, MD, Department of Pediatrics, Children’s Mercy Kansas City, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2017 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose. To cite: Johnson L-M, Frader J, Wolfe J, et al. Palliative Sedation With Propofol for an Adolescent With a DNR Order. Pediatrics. 2017;140(2):e20170487

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appropriateness of using PST in this young adult patient.

Drs Johnson, Baker, and Anghelescu Comment The death of one’s child is a lifealtering experience. Parents may experience negative physical and psychological morbidities, including depression, anxiety, complicated grief, and posttraumatic stress disorder. End-of-life care for dying children is an interdisciplinary effort that includes anticipatory counseling, symptom management, and comprehensive psychosocial, spiritual, and bereavement care. Parents will always remember the last days of their child’s life. We do not want Diamond’s mother to remember a death marked by suffering and uncontrolled pain. The perception of uncontrolled suffering can cause significant distress to both family and staff. How then to treat patients with refractory symptoms at the end of life?

PST is the use of sedative medications to address intolerable and refractory suffering by a purposeful reduction in patient awareness, even to the point of unconsciousness. Medications commonly used in PST include benzodiazepines, anesthetics (propofol and ketamine), opioids, and antipsychotics often at doses that achieve continuous deep sedation. The goal when initiating PST is comfort, not preservation of function or consciousness. PST is a rarely used intervention of “last resort.” The goal is not to hasten death, but to relieve distress from refractory and distressing physical symptoms.1 In evaluating patients like Diamond, it is important to determine if the difficult-to-manage symptoms are (1) burdensome to the patient and family and (2) truly refractory. Many patients who initially appear to have refractory symptoms will respond to expert consultation from palliative care and pain teams and the maximization of traditional

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therapies. Such maximization may include rapid opioid escalation, rotation to an alternative opioid, nonopioid adjuvant medications, nonpharmacologic approaches, or interventional approaches.2–4 In the vignette, the patient is already followed by the pain and palliative care teams and continues to have high levels of pain and suffering that are distressing to her family. To the extent that Diamond has the capacity to participate in conversations, the medical team should solicit her perspective. Some children may desire family interaction and will tolerate greater levels of pain if doing so comes with greater lucidity.

We recommend that PST be used only when the medical team and family are in consensus that the child is imminently dying, with imminently dying defined as a patient with a terminal diagnosis, unlikely to recover from his/her current illness, and unlikely to survive more than several days or weeks. Although the goal of PST is not to hasten death (ie, euthanasia), there exists a risk of respiratory depression (and death) with the doses needed for adequate relief of suffering in some patients. This potential double effect is rare when PST is used by clinicians adequately trained in this intervention, but it is a consideration when evaluating the appropriateness of PST in pediatric patients. In Diamond’s case, it would be helpful to have an interdisciplinary team meeting to clarify her prognosis and goals of care. Although it seems that the pneumonia may be responding to therapy, Diamond’s progressive leukemia appears lifelimiting, and she seems to be at risk for progressive organ dysfunction, coagulopathy, and continued pain from her advancing disease. A team meeting may identify other interventions that may be helpful with symptom management (ie, palliative chemotherapy) and allow the primary team to verbalize their

concerns about the timing and appropriateness of PST. Members of the clinical team may be unfamiliar with the purpose of PST and may not understand that the goal is the relief of suffering and not to hasten death. A clinical ethics consultant or health care ethics committee should be involved.

Before initiating PST, we recommend a family care conference to clarify the family’s understanding of their child’s prognosis (ie, imminently dying). The team should assess the severity of symptoms and tolerability of the suffering and use shared decision-making approaches to determine if PST is consistent with the patient’s and family’s preferences and values. It is important to involve the child in an age-appropriate manner and to focus on an objective evaluation of the child’s suffering, not the parents’.

Diamond’s mother appears receptive to PST and has specifically requested “a dose of something to quickly end this suffering.” Among parents whose children have died of cancer, over 10% considered making a request to hasten their child’s death, and as high as one-third of parents consider making this request if a child has uncontrolled pain at the end of life.5 Clear and comprehensive communication about the purpose of PST (relief of suffering), the risks and benefits of therapy, and the plan for assessing comfort and titrating sedation should be clearly outlined to family and staff. In adult studies on PST, achievement of adequate analgesia may be associated with life prolongation rather than hastening of death.6 Objective criteria for monitoring children on PST may help families and clinicians evaluate the level of sedation and, in some circumstances, a trial of sedation may be indicated to determine if it is still indicated. We generally recommend, when using PST, the withholding and withdrawing of all interventions

the parties understand the moral issues and facilitate a plan of action acceptable to most participants in Diamond’s care?

FIGURE 1

Algorithm for clinicians considering PST. ANH, artificial nutrition and hydration; DNAR, do not attempt resuscitation; IDT, interdisciplinary team; POLST, physician order for life-sustaining treatment. (Reprinted with permission from Johnson LM, Snaman JM, Cupit MC, Baker JN. End-of-life care for hospitalized children. Pediatr Clin North Am. 2014;61[4]:842.)

not directly related to optimizing comfort. We discuss other end-oflife preferences and identify the desired location of death. Depending on the availability of local hospice services, PST may be available in the home setting. Alternatively, patients can remain hospitalized in a quiet, private inpatient room without the restrictions that are often associated with an ICU. We encourage pediatric institutions to develop policies and procedures for the use of sedative medications, such as propofol, outside of the ICU or procedural areas when used for PST.

In summary, PST is an uncommon but important intervention reserved for those children with refractory suffering at the end of life. Although the concept of PST is well described in the adult palliative care and oncology literature, it has received less attention in the pediatric literature, with publications primarily limited to case series and case reports.7 Numerous position statements and clinical guidelines for the consideration of PST have

been published by experts in adult palliative care1; however, there is a dearth of pediatric-specific guidance. Given the particularly complex medical and social situation that often surrounds a dying child, particularly a child with refractory physical symptomatology, there is a great need for new approaches to pain and symptom control. It may be helpful for institutions to develop standard operating procedures and follow a clinical algorithm (Fig 1) when considering a pediatric patient for PST.8

Dr Joel Frader Comments

Diamond’s case illustrates tensions between the palliative care team’s recommendation for PST and the medical team’s belief that PST will preclude Diamond’s surviving her acute infection. Frustration and anxiety likely affect the palliative care clinicians because of the patient’s resistance to remarkably high opioid doses. Complicating the situation is the mother’s request for euthanasia. How can ethics consultants help

One wonders where to find Diamond’s perspective. Has the mother voiced Diamond’s view, in a kind of substituted judgment (although that term generally does not apply when making decisions for minors)? Or does Diamond’s mother express a “best interest” view of what to do or, alternatively, project the mother’s desire for relief from Diamond’s apparent misery? If we knew more about Diamond’s experience with her cancer and its treatment, what she went through with her transplant, her beliefs about the meaning of her condition and the value she attached to symptom relief or the potential for additional time to interact with family or the world, we might have an easier time deciding on the path ahead. Diamond may have shared some of her views with her parents, friends, pastor, or members of her clinical team, shedding light on her attitudes and preferences. The ethics team should explore whether Diamond left clear indications about what she would want under the circumstances. Although her status as a minor complicates the legal weight one could place on her “advance directives,” surely at age 17, one would have strong moral grounds for respecting her wishes. Palliative care clinicians have tools to help adolescents explore and articulate their views about how they want to die.9,10 Barriers do exist to completing documents or having advance care planning conversations with teenagers,11,12 even when everyone agrees that a cure cannot happen and death will come sooner than later. Some families feel that such efforts place too great a burden on the adolescent; some parents insist that they will all “just know the right thing to do when the time comes” and that planning only increases the pain

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of ongoing life. Some clinicians repeatedly say it is “too soon” to initiate such communication, although, as happened with Diamond, waiting incurs the risk of the patient losing the capacity to participate. Teenagers may feel reluctant to raise the matter on their own, worrying that talking will upset their parents or even clinicians. Some adolescents, when queried, really do not want to talk about their futures and defer to their parents, although one cannot always identify those patients without asking them directly. Some empirical data suggest that children, like many adults, welcome opportunities to discuss foreseeable death, ask questions, and have a say regarding care they might receive or the way they wish family and friends remember and honor them after death.13,14 The ethical challenge involves allowing minors to meaningfully express their views without excessive disruption of the patient’s emotional and social world. In Diamond’s case, not having a clear view of her preferences makes it harder to resolve the tensions surrounding her care.15

Two ethical issues remain. First, what ought to be the goals of care? The “big picture” suggests prolonging life per se might not serve Diamond’s interests, especially without a reasonable prospect of adequately controlling ongoing pain, delirium, etc. Nothing here suggests that curing pneumonia will lessen her overall symptom burden; indeed, improving her respiratory status might only prolong her suffering. Using PST typically means forgoing other treatments, including routine fluids and nutrition, antibiotics, and other interventions that extend life. Cases like Diamond’s can be complicated because the infusions necessary to provide adequate analgesia and sedation may maintain circulatory integrity longer than family and clinicians appreciate or find comfortable. The ethics team and the

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palliative care clinicians must ensure that all involved parties understand how PST might actually delay death.

The second ethical issue is whether professionals may honor Diamond’s mother’s request for a swift end to Diamond’s ordeal. In the United States, the simple answer remains, “no.” Euthanasia equals homicide, a crime in our country (unlike, in specific situations, in the Netherlands and Belgium and a few other jurisdictions). Physician aid-in-dying, legal in some states in the United States, is an altogether different matter in which physicians provide prescriptions for medications that capacitated adult patients with terminal conditions may take to hasten their own deaths. Even where physician aid-in-dying is legal in the United States, minors cannot participate. Patients like Diamond, with diminished consciousness, lack the capacity to take the deathinducing medicines, even if they are adults. Whether we should allow euthanasia, for adults or (some) children, in the United States, is quite another issue. Most palliative care clinicians claim good palliative care makes euthanasia unnecessary. Cases like Diamond’s, however, push us to reconsider. Even if we provide effective PST, what purpose do we serve by continuing her existence in an anesthetized state? In Diamond’s case, ending her life swiftly may make sense. It seems ethically justified to hasten death in limited circumstances when continued life provides no benefit; when continued, palliative treatment may, somewhat paradoxically, prolong death; and, most controversially, when the extended life increases the suffering of those providing loving care for the patient. Clearly defining the conditions that permit euthanasia so as to prevent abuse, either of individuals or classes of patients (such as those with disabilities), strikes many as

daunting. Nevertheless, it seems time to try to find agreement on this. Unfortunately, religious and political conflicts in the United States make it unlikely we will see legalization of euthanasia any time soon.

Dr Joanne Wolfe Comments

Let’s consider the unknowns about this case. We do not know the extent to which Diamond, the patient, had the opportunity to participate in decision-making and advance care planning. We do not know whether there are any other loved ones, such as another parent, involved in Diamond’s life. Most importantly, we do not know the patient and family goals of care in the context of her refractory leukemia.

Let’s assume the approach to Diamond’s care has been optimal and unfolded as follows. Diamond’s care has been guided by an interdisciplinary oncology team from the time of diagnosis. She has a longstanding relationship with a social worker, and when she relapsed after transplant, the social worker introduced “Voicing My Choices”10 to her. They carefully worked through this advance care planning booklet developed by and for adolescents and young adults. In completing the booklet, among other hopes, she indicated that, “If a doctor and another health care provider both decide that I am close to death and likely to die within a short period of time, and life-support treatment would only delay the moment of my death: I would want to have a natural death.” Once completed, Diamond shared this document with her mother. Let’s now assume that at the time of the clinical team’s discussion with Diamond’s mother about her resuscitation status, Diamond was not able to participate in discussions given her profound respiratory distress and high levels of sedation. The interdisciplinary team meets with her mother and conducts a highquality goals of care discussion.16 To

begin, they reflect on Diamond as a person and talk about her strengths and passions. They then use the “ask tell ask” strategy to disclose her poor prognosis. First Diamond’s mother is asked, “Is it ok to talk about how Diamond is doing?” Once her mother agrees, she is told that her daughter has refractory leukemia complicated by severe pneumonia. The team additionally explains that they are worried that if she is intubated she is not likely to get better and they would be facing decisions about discontinuing the ventilator down the road. After this discussion, Diamond’s mother is asked what she thinks would be most important to Diamond in light of this prognosis. Her mother references Diamond’s documentation of her preferences and agrees to the recommendation that a do-not-attempt-resuscitation order be written on her behalf. At the same time, she expresses the hope that Diamond recovers from the pneumonia with continued antibiotics and nutrition support. Her goals are blended, as is not uncommon,17 and she hopes her daughter lives as long as possible and as well as possible, despite her refractory leukemia.

Fast forward to the current scenario; although the pneumonia is improving, Diamond’s discomfort is escalating. Why is this? The most likely explanation is disease progression potentially resulting in bone and visceral pain, with possible central nervous system involvement as well. Let’s also assume that Diamond is not a candidate for additional disease-directed therapy because of organ dysfunction. Let’s additionally presume that goals of care have been revisited with Diamond’s family and that it has been effectively communicated to them that she is dying from progressive leukemia. Through this discussion, the family now hopes that Diamond lives as comfortably as possible for whatever time she has left. The team

recommends palliative sedation to achieve this goal. The team also recommends, and the family agrees, to discontinue antibiotics and intravenous nutrition so as not to inadvertently prolong her dying.

What about the family’s request to give Diamond a dose that will quickly “end this suffering?” Is this a request for euthanasia? Experience suggests that such requests are not rare when a child’s suffering is extreme. Indeed, in a survey of 141 parents of children who died of cancer, 13% reported that they considered requesting hastened death for their child, and these considerations tended to increase with an increase in the child’s suffering from pain.5 Thus, palliative sedation is an especially important intervention when conventional approaches do not achieve patient comfort. If comfort is achieved and Diamond’s final days are peaceful, her family’s grief may also be eased.18

Dr John D. Lantos Comments

The distinction between palliative sedation and euthanasia is subtle but real. It turns on the intention of the doctor who is administering treatment. The goal of palliative sedation is to relieve suffering. The goal of euthanasia is to cause death. Palliative sedation can sometimes cause death. In the context in which palliative sedation is used, it may be difficult to know whether it caused death or whether death would have occurred anyway. But causation is not the key issue; intention is. That difference in intention may not seem like an adequate safeguard. But, in the context of a dying patient with intractable suffering, doctors have a moral obligation to try to relieve suffering. If that can be done without causing death, it should. If it cannot, then the obligation to relieve suffering is adequate justification for administering a treatment that may also shorten a patient’s life.

ABBREvIATION PST: palliative sedation therapy REFERENCES 1. Gurschick L, Mayer DK, Hanson LC. Palliative sedation: an analysis of international guidelines and position statements. Am J Hosp Palliat Care. 2015;32(6):660–671 2. Zernikow B, Michel E, Craig F, Anderson BJ. Pediatric palliative care: use of opioids for the management of pain. Paediatr Drugs. 2009;11(2):129–151 3. Anghelescu DL, Faughnan LG, Baker JN, Yang J, Kane JR. Use of epidural and peripheral nerve blocks at the end of life in children and young adults with cancer: the collaboration between a pain service and a palliative care service. Paediatr Anaesth. 2010;20(12):1070–1077 4. Maltoni M, Pittureri C, Scarpi E, et al. Palliative sedation therapy does not hasten death: results from a prospective multicenter study. Ann Oncol. 2009;20(7):1163–1169 5. Dussel V, Joffe S, Hilden JM, WattersonSchaeffer J, Weeks JC, Wolfe J. Considerations about hastening death among parents of children who die of cancer. Arch Pediatr Adolesc Med. 2010;164(3):231–237 6. Anghelescu DL, Hamilton H, Faughnan LG, Johnson LM, Baker JN. Pediatric palliative sedation therapy with propofol: recommendations based on experience in children with terminal cancer. J Palliat Med. 2012;15(10):1082–1090 7. Henderson CM, FitzGerald M, Hoehn KS, Weidner N. Pediatrician ambiguity in understanding palliative sedation at the end of life. Am J Hosp Palliat Care. 2017;34(1):5–19 8. Johnson LM, Snaman JM, Cupit MC, Baker JN. End-of-life care for hospitalized children. Pediatr Clin North Am. 2014;61(4):835–854 9. Wiener L, Battles H, Mamalian C, Zadeh S. ShopTalk: a pilot study of the feasibility and utility of a therapeutic board game for youth living with

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cancer. Support Care Cancer. 2011;19(7):1049–1054 10. Wiener L, Zadeh S, Battles H, et al. Allowing adolescents and young adults to plan their end-of-life care. Pediatrics. 2012;130(5):897–905 11. Freyer DR. Care of the dying adolescent: special considerations. Pediatrics. 2004;113(2):381–388 12. Rosenberg AR, Wolfe J, Wiener L, Lyon M, Feudtner C. Ethics, emotions, and the skills of talking about progressing disease with terminally ill adolescents: a review. JAMA Pediatr. 2016;170(12):1216–1223

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13. Weaver MS, Baker JN, Gattuso JS, Gibson DV, Sykes AD, Hinds PS. Adolescents’ preferences for treatment decisional involvement during their cancer. Cancer. 2015;121(24):4416–4424 14. Day E, Jones L, Langner R, BluebondLangner M. Current understanding of decision-making in adolescents with cancer: a narrative systematic review. Palliat Med. 2016;30(10):920–934 15. Hinds PS, Drew D, Oakes LL, et al. End-of-life care preferences of pediatric patients with cancer. J Clin Oncol. 2005;23(36):9146–9154

16. Waldman E, Wolfe J. Palliative care for children with cancer. Nat Rev Clin Oncol. 2013;10(2):100–107 17. Bluebond-Langner M, Belasco JB, Goldman A, Belasco C. Understanding parents’ approaches to care and treatment of children with cancer when standard therapy has failed. J Clin Oncol. 2007;25(17):2414–2419 18. Kreicbergs U, Valdimarsdóttir U, Onelöv E, Björk O, Steineck G, Henter JI. Care-related distress: a nationwide study of parents who lost their child to cancer. J Clin Oncol. 2005;23(36):9162–9171

ETHICS ROUNDS

Parental Refusal of a Liver Transplant for a Child With Biliary Atresia AUTHORS: David C. Cronin, MD, PhD,a Janet Squires, MD,b Robert Squires, MD,c George Mazariegos, MD,d and John D. Lantos, MDe aLiver Transplantation Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin; bChild Advocacy Program, dPediatric Transplantation, Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania; cUniversity of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania; and eUniversity of Missouri, Kansas City and Children’s Mercy Hospital, Kansas City, Missouri

KEY WORDS liver transplantation, maltreatment, medical ethics, neglect www.pediatrics.org/cgi/doi/10.1542/peds.2012-0567 doi:10.1542/peds.2012-0567 Accepted for publication Oct 5, 2012 Address correspondence to John D. Lantos, MD, University of Missouri, Kansas City, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2013 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.

Pediatricians are required by law to notify child protection agencies if parents are neglecting their child’s medical needs. Sometimes, however, it is difficult to determine when a particular parental choice ought to be reported. If the treatment is standard therapy and the consequences of nontreatment are dire, than parental refusal is usually considered neglectful. Organ transplants, however, represent a unique situation for 2 reasons. First, because there is a dire scarcity of organs, there are more people who need organs than there are organs to transplant. In this situation, it seems odd to force a transplant on a child whose parents do not want it, knowing that other parents are eager to have their child undergo a transplant. Second, transplantation is risky and requires lifelong follow-up and treatment. Thus, parental cooperation is essential. We present a case of a child who needs a liver transplant and whose parents refuse, and ask 2 surgeons, a gastroenterologist, and a child abuse specialist to comment on the case. David C. Cronin is director of the liver transplant program at the Medical College of Wisconsin. Robert Squires is a gastroenterologist, George Mazariegos is a transplant surgeon, and Janet Squires is chief of the child advocacy center, all at the University of Pittsburgh.

FUNDING: No external funding.

THE CASE A 10-month-old girl, Baby A, was admitted to the hospital because of worsening jaundice. She had been diagnosed with biliary atresia at 1 month of age, and a Kasai procedure was performed when she was 10 weeks old. Since then, she had been failing to thrive and had worsening jaundice. On the current admission, the patient’s weight was 8 kg (,5th percentile). Her abdominal girth was 50 cm with significant hepatomegaly. Her total protein was 6.0 g/dL with an albumin level of 2.5 g/dL. Alkaline phosphatase was 355 U/L, aspartate aminotransferase was 150 U/L, and alanine aminotransferase was 61 U/L. Her total bilirubin level was 20.5 mg/dL with a direct bilirubin of 18.0 mg/dL. Her prothrombin time was 17.0 seconds, partial thromboplastin time was 28.5 seconds, and her international normalized ratio was 1.8. Baby A’s physicians recommended a liver transplant and estimated that, without a transplant, she would not survive another year. Her parents did not consent to have her listed for a transplant. They believed that God was already healing their infant. They recounted the story of an uncle who had been told that he might need a kidney

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transplant and who was still alive and doing well 5 years later. Furthermore, they stated that they did not want their child “butchered.” They were also concerned about the burden of traveling to a transplant center 5 hours from their home for the procedure. Their physician was deeply disturbed by this decision. She felt that a liver transplant was the standard treatment of this condition and that it offered a 90% 5-year survival rate. She believed that it was medical neglect to refuse a liver transplant when the alternative was death within a few months. The physician requested an ethics consultation, asking whether it was permissible for the parents to refuse a liver transplant in this situation and, if not, whether the ethics committee would support a report to the child protection agency for medical neglect. David C. Cronin Writes The field of solid organ transplantation represents an area of medical practice that often must address issues of allocation of a scarce resource, justice, equity, and decisions of who should and who should not be allowed to benefit from the transplantation. In the United Statestoday, aliver transplantisstandardof-care for pediatric patients who have end-stage liver disease secondary to biliary atresia. Among pediatric patients, the most common cause of end-stage liver disease and the most common indication for a liver transplant is biliary atresia. The majority of these patients require liver transplantation before the age of 2 years. Unfortunately, although liver transplantation represents the single best and only definitive therapy associated with prolongation of life in the setting of end-stage liver disease, organs available for transplantation are most significantly limited for candidates weighing ,20 kg. This disparity between

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candidate need and organ supply is 6 times greater for pediatric candidates compared with adult candidates.1 Consequently, death while awaiting liver transplantation is a potential and real outcome. Due to the scarcity of organ availability, patients awaiting a liver transplant are triaged for organ allocation on a sickest-first basis. Determination of the sickest patient is represented by a prospectively validated severity-of-illness score referred to as the Pediatric End-Stage Liver Disease score; this scale is valued from 6 to 40, with increasing value representing an increasing risk of death awaiting liver transplantation.2 Baby A’s Pediatric End-Stage Liver Disease score was 30, representing a significant 30-day mortality risk without a liver transplant. Medically, this child represents the usual presentation of end-stage liver disease secondary to biliary atresia. In this particular case, the diagnosis of biliary atresia seems to be without question. The child presented early in life, underwent standard surgical therapy (Kasai procedure), and has progressed to end-stage liver disease as demonstrated by the laboratory values, physical examination, and failure to thrive. Although the patient has an indication for liver transplantation, the reason to pursue transplant at this point is her failure to thrive and worsening jaundice. Although the family has demonstrated commitment to good patient care (follow-up with physicians for diagnosis, surgical therapy, and postoperative care), they have a misconception about the difference between liver transplantation and kidney transplantation and the difference between the onset and reversibility of some forms of kidney failure and the progressive nature of liver failure in this situation. To discount the family’s concerns as irrelevant (as they are) would not be medically or

ethically appropriate. The health care team needs to present the information about the nature of the illness with and without liver transplantation as an option. The specific discussion that this child will die without a liver transplant must be conveyed. This must be done at a level that is understandable to the family and will most likely need to be conducted over a period of time. Furthermore, there is an opportunity to allay the concerns of “butchering” by reviewing the previous surgery (the Kasai procedure) and outlining the similarities in surgical scar and surgical procedure. The family’s repulsion may be a representation of fear and frustration. Taking a position for or against supernatural intervention is often not productive. In the end, we should not decry the possibility of miracles but rather incorporate divine intervention in addition to the appropriate medical/ surgical therapy. If after the educational and support measures and social service interventions are unsuccessful in moving the parents to accept transplantation as the appropriate treatment, the option of taking protective custody of the pediatric patient and acting in the child’s best interest is ethically and legally supported. In this situation, the parents have a duty to provide appropriate medical care but do not have the right to prevent appropriate medical intervention. This duty transcends religious objections in almost all circumstances. Ethically, the child does not have autonomous decision-making ability. As such, the pediatric patient should not be victim to inappropriate medical decisions that have significant lifethreatening or life-limiting consequences made by others (including the parents or surrogate caregivers). Parents and surrogate caregivers are responsible for providing appropriate medical care.

Legally, the state has an interest in the overall health and life of the most vulnerable members of society; in this case, pediatric patients. Consequently, there are clear mechanisms for health care providers to protect children at risk by taking protective custody and acting on a best interest basis. Here, the prohibition of liver transplantation on a religious basis would not be supported legally.3 In fact, because this is a child, many social support options exist to facilitate travel, housing support, and pharmaceuticals for the medical therapy. In the end, it is better to invest the time and effort to educate the family, answer questions, allay their fears, and work collaboratively to embrace the appropriate medical treatment. If unsuccessful, protective custody and prevention of medical neglect are ethically and legally justifiable but would not be an easy path. Robert Squires Writes Most children with biliary atresia have jaundice that begins in the first few weeks of life. Often, families assume that the problem is due to “normal newborn jaundice” that just goes away. It is natural to reject the notion that a child may have a serious liver problem when the child “looks okay.” From the earliest period of care, parents should be offered a thorough discussion, by using laymen’s terms and drawings, that will review the agespecific etiologies and help them understand what future problems and needs their child may have. It is not clear to me how this child was “diagnosed” with biliary atresia at age 1 month and had a Kasai procedure at age 10 weeks. The diagnosis of biliary atresia is usually made at the time of an operative cholangiogram, and the Kasai procedure is done immediately by using the same anesthesia. A cholangiogram should only be performed

in a center with the surgical capacity to do the appropriate intervention at the same time. I would worry, and try to address, the possibility that there were communication problems at that early stage of treatment. If biliary atresia is a potential diagnosis, the family must understand some general concepts going into the cholangiogram procedure: (1) biliary atresia is a fatal disease in the absence of a Kasai procedure (the natural history for children who do not have this procedure is that virtually all of them die of their liver disease before their third birthday); (2) after the Kasai procedure, careful and close medical follow-up will be necessary to avoid or manage complications related to biliary atresia (there are some children who live into adulthood with a successful Kasai); (3) not all Kasai procedures are successful; and (4) if the Kasai procedure is not successful, and if the jaundice has not cleared within 3 months after the procedure, then the child’s course will most likely be similar to those who did not receive a Kasai procedure, and an early death can only be averted with a liver transplant. The Kasai procedure should be performed between 4 and 8 weeks of age. The older the child is at the time of the procedure, the less likely that the child will establish satisfactory biliary drainage. The months after the Kasai procedure are crucial. The family must have a realistic knowledge of possible outcomes. If the procedure is successful, the elevated bilirubin levelswill fall to the nearnormal or normal range over the next 3 months. In other cases, the liver disease becomes progressive, and the child will need to be considered for liver transplantation. Early referral for a transplant evaluation, even as early as age 5 to 6 months for a child with biliary atresia and a failed outcome on a Kasai procedure, might give the family and

child the best opportunity for accessing resources and planning for the future. If I were consulted on this case, I would want to know the family’s understanding and experiences, as well as their concerns and anxieties. If parents do not seem to grasp the severity of the disease, do not accept responsibility, have inadequate resources, have competing problems that affect their ability to care for the child, or show signs of incapacitating fear or anxiety, supportive strategies are essential. I would work with other family members, such as grandparents, and other persons important to the parents, such as ministers or friends. A second medical opinion from another center may be important. Support from social services and referrals to available community resources, including attention to financial issues, is necessary. Facilitating meetings with other parents can be very helpful. Luckily, in the current case, there is time for patience, as the child’s deterioration is not imminent. I would do almost anything to gain the confidence of the parents, to support keeping the family together, and to work with the parents to provide life-saving medical care. However, if the parents remain completely opposed to the option of a liver transplant, I cannot accept the inevitability of the death for a child who could be helped by an available medical procedure with reasonable risks. This infant will die without a transplant. I believe such a case should be referred to the child protection authorities, and the judicial system must help decide what will be done. George Mazariegos Writes This scenario is a challenging situation. Multidisciplinary evaluation and working closely with the hepatologist seeing the child will help present a unified message to the family. As Dr Squires has emphasized, the key to helping the

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family make an informed decision is to be sure the family understands the child’s medical problem and the current data available regarding the benefits and the risks of organ transplant. I would evaluate for potential surgical and medical contraindications to transplant. These factors include a severe progressive systemic disease, a lifethreatening malignancy, or rare cardiopulmonary conditions that limit anesthesia and surgical options. There could be a temporary condition, such as sepsis, coagulopathy, or severe malnutrition, which might require treatment while waiting for a future transplant opportunity. From a technical point of view, there are few absolute contraindications. With improved surgical techniques and experience, previous relative contraindications to transplant in biliary atresia such as portal venous thrombosis or situs inversus have been eliminated. In this case, despite the fact that this is a young child who is small and who has had a previous failed outcome on a Kasai procedure, this infant would meet the criteria of a child who should do well in an experienced transplant center. Surgical possibilities include a deceased donor transplant, a living donor transplant, and a deceased donor split transplant. Thus, the prospect that this child will attain a suitable transplant in a reasonable time is high. Pediatric liver transplantation has excellent short- and long-term patient and graft survival outcomes.4 I would review with the family that the early (1year) survival is expected to be .90%; in fact, our recent experience shows survival at 98%.5 Studies of long-term survival, typically defined at 5- and 10year marks, show survival rates at ∼90%, with most children having highquality life parameters.6 I would remind parents that biliary atresia is a disease that does not recur after transplant, so that the child is cured of

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the original problem. The major focus of the care after transplantation is managing the complications of the medicines used for immunosuppression. There are major advances in this area, and many transplant recipients have a reasonable expectation to live with low-level immunosuppression. With a functioning liver, the child is expected to improve her nutrition and growth, which are essential for neurologic development. Finally, I would stress to the family the almost certain poor outcome without transplantation. There is no reasonable expectation that a very symptomatic infant can improve her nutrition and growth or to thrive with the current liver disease. Without a liver transplant, Baby A would not be expected to live beyond age 2 years. I do note the parents’ use of the word “butchered.” There is no question that transplant surgery is major and can be traumatizing. However, experienced centers understand the importance of treating the “whole child,” and great efforts are made to help the child and family cope. Our center has developed many resources to support families. Our major focus is return to normalcy for the child and family. A great resource for these parents would be to talk with other parents who have experienced a child having a transplant. Although there are clearly challenges and burdens, most families delight in the development and accomplishments of their child who has survived a lethal condition and who now has a real chance for a bright future. If all of our efforts fail and the parents of this child would continue their refusal of a liver transplant, I would call our hospital’s child protection team. In my assessment, with the known outcome of death for the child without a transplant, the case should be reviewed by child protection authorities, and I would be willing to participate in a legal process in which a judge might make the ulti-

mate decision. I would hope such action would not be necessary. Janet Squires Writes Parents are usually the appropriate surrogate medical decision-makers for their own children. We assume that they have the child’s interest at heart and are in the position to decide the best medical treatment. But there are limits. If parents choose a course of treatment that puts the child in imminent danger of harm, then physicians have a legal obligation to report such acts to the child protection system. Few pediatricians hesitate to report findings indicative of physical child abuse or sexual child abuse. Medical neglect is hard to define. Textbook discussions of medical neglect usually focus on time-limited interventions in which the benefits and risks are fairly easy to describe, such as a lifesaving blood transfusion. This case presents a myriad of more complex issues. Just a few of the complicating factors to be considered include:

 Organ transplant involves complex

technology. As new procedures and therapies become available, at which point do such interventions go from being “cutting edge” and research, to becoming standard of care?

 Organ transplants are not typically

curative. The recipient will have a chronic medical condition requiring ongoing medical care, often for a lifetime.

 The burden of treatment is significant. Surgery is invasive, and complications are common.

 Burden for a family of a child receiving a transplant is very high. Transplant centers may be geographically far from family homes. Parents must often give up work and take time from their other children.

 Courts are reluctant to mandate

medical treatments that do not have an end point. If a court were to mandate an organ transplant despite parental refusal, who will care for the child afterward?

 In this scenario, parents mention

a religious belief that God will heal their infant as 1 motive for their decision. Does it matter what parental reasons are given?

 Risk/benefit ratios may depend on the center providing the medical care. Outcome measures are not uniform for all centers.

The diagnosis of child medical neglect is mainly dependent on the level of harm the child will suffer from the lack of medical care that a reasonable person would choose; the definition of “reasonable” is obviously challenging. The diagnosis of medical neglect is generally independent of the motivations of the adult. Lack of resources should be recognized and addressed. Understanding other reasons that parents do not choose or provide “reasonable” medical care (eg, religious beliefs, mental health issues, competing family obligations) is important for attempts to work with the family. Fundamentally, however, pediatricians must assess when a child is being significantly harmed by medical choices of a parent or omissions of a parent’s care, and must call it medical neglect. In this particular case, I also would talk extensively with the parents and try any tactic to incorporate their wishes for their child. If they refused the transplant option, I also believe this is an appropriate case for a judge to decide. I would explain to the parents that a referral to the child protection system is not a criticism of them as parents, nor a challenge to the belief that they love their child. Rather, physicians have a legal obligation to recognize and report cases in which a child is at risk for

significant harm. Like the 2 transplant experts, I would do “almost anything” to avoid legal involvement but feel the certain death sentence for this child without intervention is not acceptable. John D. Lantos Comments In 1990, therewasa casein Saskatchewan, Canada, in which parents refused a liver transplant for their 10-month-old who had biliary atresia. The physicians sought a court order for transplant. The court sided with the parents.7 I can find no reported cases, since then, in the United States or Canada, in which physicians sought protective custody because parents were refusing a transplant. I suspect that not all parents consent to transplant. Instead, I think that physicians generally do not report such parents for neglect. Why? The child-centered arguments for overriding such parental refusals, as presented here, are compelling. Without treatment, the child will surely die. With treatment, the chances for a good outcome are excellent. Our reticence to seek protective custody comes, I think, from the dire scarcity of organs. Because there are not enough organs to go around, some people on waiting lists will die awaiting transplant. It seems wrong to force a transplant on an unwilling family if it means that a willing family might go without one. I suspect that such concerns, rather than the complete absence of parental refusals, is the reason why we have not seen court cases involving this situation. This is one of the very few situations in which such concerns about the dire scarcity of a life-saving resource might appropriately tip the balance against a decision that reflects what is clearly in the child’s best interest. As long as people are dying on the waiting list, this is wise health policy.

Dr Mazariegos Responds I do not believe organ scarcity is a major issue here. Wait list mortality has significantly improved over the past decade (Fig 1), even for the group with the highest usual death rate on the wait list; that is, infants aged ,1 year. For example, of 126 children listed for transplant aged ,1 year in 2009, a total of 7 on the waiting list nationally died, the fewest number that have ever died in 1 year over the past decade (Figs 1 and 2 present data from the 2010 report of DHHS’s Organ Procurement and Transplantation Network and Scientific Registry of Transplant Recipients).8 Split and live donor transplantation, along with improvements in allocation policy and pretransplant medical care, have made wait list mortality rare (but not yet zero) for children

FIGURE 1 Pretransplant mortality rates among pediatric patients wait listed for a liver transplant; grouped according to age.

FIGURE 2 Pediatric liver transplant rates according to age.

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in the United States. In fact, the most significant improvement in transplant rate has been in children aged ,1 year (Fig 2). Therefore, based on

the current data, I strongly disagree that organ scarcity might appropriately tip the balance against transplant for this child or be wise health

policy given the scenario described here: an otherwise healthy child with a reasonable chance for long-term success.

neglect: a guide for prevention, assessment and intervention. www.childwelfare.gov/pubs/ usermanuals/neglect/chaptertwo.cfm#fnb21. Accessed November 16, 2012 4. Berg CL, Steffick DE, Edwards EB, et al. Liver and intestine transplantation in the United States 1998-2007. Am J Transplant. 2009;9(4 pt 2):907–931 5. Shneider BL, Mazariegos GV. Biliary atresia: a transplant perspective. Liver Transpl. 2007; 13(11):1482–1495 6. Ng VL, Alonso EM, Bucuvalas JC, et al. Health status of children alive 10 years after pediatric liver transplantation performed in the

US and Canada: report of the studies of pediatric liver transplantation experience. J Pediatr. 2012;160(5):820–826.e3 7. Saskatchewan (Minister of Social Services) v. P.(F.) (K’aila), 1990, 69 Dominion Law Reports (4th) 134 (Saskatchewan Provincial Court) 8. Organ Procurement and Transplantation Network (OPTN) and Scientific Registry of Transplant Recipients. (SRTR). OPTN/SRTR 2010 Annual Data Report. Rockville, MD: Department of Health and Human Services, Health Resources and Services Administration, Healthcare Systems Bureau, Division of Transplantation; 2011

REFERENCES 1. Sweet SC, Wong HH, Webber SA, Horslen S, Guidinger MK, Fine RN, Magee JC. Pediatric transplantation in the United States, 1995–2004. Am J Transplant. 2006;6(5 pt 2):1132–1152 2. McDiarmid SV, Anand R, Lindblad AS; Principal Investigators and Institutions of the Studies of Pediatric Liver Transplantation (SPLIT) Research Group. Development of a pediatric end-stage liver disease score to predict poor outcome in children awaiting liver transplantation. Transplantation. 2002; 74(2):173–181 3. US Department of Health & Human Services, Administration for Children & Families. Child

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ETHICS ROUNDS

When Life-Sustaining Treatment Is Withdrawn and the Patient Doesn’t Die AUTHORS: Stefan Kutzsche, MD,a John Colin Partridge, MD,b Steven R. Leuthner, MD,c and John D. Lantos, MDd aDepartment

of Neonatology, Oslo University Hospital Ulleval, Oslo, Norway; bDepartment of Pediatrics, University of California, San Francisco, San Francisco, California; cDepartment of Pediatrics and Bioethics, Medical College of Wisconsin, Milwaukee, Wisconsin; and dChildren’s Mercy Hospital, Kansas City, Missouri KEY WORDS Ethics, palliative care, withdrawal of life-sustaining treatment, prognostication ABBREVIATION IUGR—intrauterine growth retardation Dr Kutzsche wrote the case presentation and his own commentary; Drs Partridge and Leuthner wrote their sections of the article; Dr Lantos conceptualized the article and wrote his section; and all authors approved the final manuscript. www.pediatrics.org/cgi/doi/10.1542/peds.2013-0413 doi:10.1542/peds.2013-0413 Accepted for publication Jul 9, 2013 Address correspondence to John D. Lantos, MD, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO, 64108. E-mail: [email protected]

abstract One of the most difficult decisions that doctors and parents must make is the decision to withdraw life-sustaining treatment. Doctors find it easier to withdraw treatments in situations where withdrawal will be rapidly fatal rather than in situations in which treatment withdrawal will lead to a prolonged dying process. Mechanical ventilation is usually such a treatment. Withdrawal of ventilation generally leads to the patient’s rapid demise. Doctors may tell parents that death will occur quickly after a ventilator is withdrawn. But what happens when the doctors are wrong and a patient survives without life support? What should doctors do next? We present a case in which that happened and asked 3 experts to comment on the case. Stefan Kutzsche is a senior consultant in neonatology at Oslo University Hospital Ulleval in Norway. John Colin Partridge is a neonatologist and professor of pediatrics at University of California, San Francisco. Steven R. Leuthner is a neonatologist and professor of pediatrics and bioethics at the Medical College of Wisconsin. They each recommend slightly different approaches to this dilemma. Pediatrics 2013;132:893–897

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2013 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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One of the most difficult decisions that doctors and parents must make is the decision to withdraw life-sustaining treatment. Doctors find it easier to withdraw treatments in situations where withdrawal will be rapidly fatal rather than in situations in which treatment withdrawal will lead to a prolonged dying process.1 Mechanical ventilation is usually such a treatment. Withdrawal of ventilation generally leads to the patient’s rapid demise. Doctors may tell parents that death will occur quickly after a ventilator is withdrawn. But what happens when the doctors are wrong and a patient survives without life support? What should doctors do next? We present a case in which that happened and asked 3 experts to comment on the case. Stefan Kutzsche is a senior consultant in neonatology at Oslo University Hospital Ulleval in Norway. John Colin Partridge is a neonatologist and professor of pediatrics at University of California, San Francisco. Steven R. Leuthner is a neonatologist and professor of pediatrics and bioethics at the Medical College of Wisconsin.

dysplasia, moderate retinopathy of prematurity, and a subependymal intraventricular bleed. At 3 weeks of age, the little boy was weaned to external continuous positive airway pressure. Respiratory failure developed at 3 months, secondary to persistent pulmonary hypertension and a right–left shunt. High-frequency oscillating ventilation and treatment with nitric oxide, sildenafil, and bosentan were started. However, frequent episodes of low oxygen saturation continued. About 2 months later, doctors and parents discussed the options of continuing or discontinuing ventilatory support. After the family meeting, the parents agreed to discontinue mechanical ventilation. Off the ventilator, the patient remained stable on 1 L/min of 40% oxygen by nasal cannula. Nasogastric feeds were continued. Morphine was given as required for discomfort. There was moderate respiratory distress but the child appeared not to be in pain. The parents were disconcerted because they had been led to believe that their son would soon die.

THE CASE An infant with severe intrauterine growth retardation (IUGR) was born at 26 weeks of gestation with a birth weight of 290 g. Extreme IUGR, oligohydramnios, and pathologic feto-placental circulation were recognized at weeks 13 and 18 respectively. The parents decided not to terminate pregnancy even when the mother developed severe preeclampsia. Elective cesarean delivery was performed after treatment with antenatal steroids; Apgar scores were 5 at 1 minute and 6 at 5 minutes. The child was intubated and transferred to the NICU. The parents were counseled about the probable poor outcome but wished to continue with intensive therapy. The neonatal course was complicated by the development of bronchopulmonary

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WHAT SHOULD THE DOCTORS DO NOW? Steve Leuthner writes: When we make the difficult decision to stop life-sustaining treatment of an infant, there is always some uncertainty about when, and whether, the infant will die. Every doctor knows cases in which the child survived longer than we had expected. Some survived for years. In this case, some clarifications might help guide further discussion with the family on the next steps. How much time has passed since the ventilator was stopped? If we are only an hour out, then I might discuss with the family how it could take hours to days for an infant to die. Ideally, this should have been discussed before the

withdrawal. I would assure them that this is not unexpected, that we will do what we can to control pain and discomfort, and that the most important thing now is to share this precious time with their infant. The parents should be encouraged to hold, bathe, and feed their infant. I would assure them that we will do what we can to control pain and discomfort. I would acknowledge how difficult yet normal it is for a parent to struggle with the wish for a quick, comfortable death. If, on the other hand, the infant were now stable for a few days or weeks, I might acknowledge that we made an error in our prediction and I would reevaluate the infant’s medical condition. I would reconvene the family, apologizing to them if they felt we had misled them, and again explain why we can’t always predict timing of death. I would then suggest that we need to talk about goals of care for the infant. What do we already know about the family values in this case? Clearly the motherput herownhealthand life atrisk when she developed preeclampsia, yet she chose not to terminate at a time the clinical situation appeared grave. We know that, despite counseling about poor outcome early in the NICU course, the family chose to continue intensive therapy. So I would ask, what is now different 5 months later? What are the reasons why the withdrawal of the ventilator was recommended and agreed to by the family? Perhaps the family wanted everything done as long as there was any hope of survival but, at the 5-month juncture, became convinced that aggressive support was futile. Alternatively, perhaps some level of “quality-of-life” threshold has been finally crossed for this family. Given the previous predictions/intuitions of death, the extreme premature gestation, the severe growth restriction and microcephaly, with an (admittedly small) intraventricular hemorrhage, and now

with severe bronchopulmonary dysplasia, pulmonary hypertension, and many episodes of hypoxia, some neurodevelopmental impairment is guaranteed. They may have agreed to withdraw the ventilator because they realized that the infant would either die or would survive with significant neurodevelopmental impairments.2 Understanding their values will help us understand how to help them. If the only reason they agreed to withdraw life support was because they believed that survival was impossible, then it may be appropriate to reinitiate intensive lifeprolonging treatment. If, on the other hand, they agreed to withdraw based on a benefit-burden proportionality and quality-of-life assessment, then things are not really different now. The infant still needs good palliative care. A palliative care service could have and now can help develop a new palliative care plan. So what are the current options to consider and offer this family? I see 3 possibilities. We could reintubate the infant and reinstitute an aggressive path of tracheostomy and ventilator support. This approach will likely only prolong the course of this terminal illness and increase suffering for the infant and the family during the dying process. A second option would be to continue the current care of oxygen, gavage feeds, and morphine. We would need to think about where to draw the line on life support as opposed to symptom control. Should we use continuous positive airway pressure? Antibiotics? All these possibilities need to be considered. And depending on these choices, we need to consider where this all can take place. One potential problem with this type of plan is that sometimes it seems we set the infant, the family, and ourselves up for a situation in which we are waiting and hopingforsomething badtohappen. That could lead to a bad psychological dynamic for family and staff alike.

A final option would be to continue down a comfort care–only path. This might entail weaning the oxygen completely and giving morphine as needed if more hypoxia and distress develop. We could consider withdrawing the artificial nutrition and hydration.

infant’s current treatment regimen is not particularly onerous, but the prognosis is either a poor neurodevelopmental outcome on the chance that he survives or a delayed death from pulmonary hypertension if he cannot survive extubated.

With any of these choices, the overwhelming likelihood is that the infant will die. The different approaches will not change that outcome, they only alter when he will die and under what circumstances. Those choices should be determined by the family’s values and preferences for how they’d like to take care of an infant in the last days or weeks of his life.

At this point, the medical team needs to readdress survival estimates, expected neurodevelopmental outcome, and the balance of burdens and benefits for longer-term survival of whatever duration. Reasonable treatment options appear to be: (1) to discontinue oxygen and medications and redirect the medical goals to comfort care, (2) to discontinue medications while continuing oxygen as a comfort measure, or (3) to continue all treatments in hopes that the persistent pulmonary hypertension will gradually improve. Restarting mechanical ventilation would likely not be efficacious and thus does not seem a reasonable option.

John Colin Partridge writes: Unexpected outcomes of medical care decisions inevitably complicate interactions with families of critically ill infants, leading to ethical dilemmas and unexpected changes in treatment plans. In the case of this markedly IUGR 290-g ex-26-week infant with pulmonary hypertension, providers and parents agreed to discontinue, and not reintroduce, mechanical ventilation. We must assume that the intent of extubating the infant was to prevent a later death despite therapy or to preclude survival with a very poor neurodevelopmental outcome. Both providers and parents presumably viewed death as a reasonable option and an expected, and imminent, outcome. The case does not relate important aspects of the earlier informedconsent process: (1) to what extent the counseling physicians prospectively discussed the possibility of a lingering death with the family, and (2) whether they attempted to ascertain the parents’ understanding of what would occur if the infant did not die soon after extubation. Now at 5 months of age and extubated, the infant is “stable,” with moderate respiratory distress on cannula oxygen. He is receiving morphine for discomfort, but shows no apparent signs of pain. The

If only short-term survival is foreseen, comfort care seems the most reasonable treatment option, and the one most aligned with the parents’ expectations. Oxygen might be considered a comfort measure, but the chance that it would prolong the infant’s death must be carefully weighed against the possible benefit of easing air-hunger. Analgesics should be prescribed to minimize pain, and sedatives used to treat agitation. A Do-Not-Resuscitate/Do-Not-Intubate order should be written and explained to the parents. Support for the parents and providers is critical to helping parents cope emotionally and to helping manage providers’ moral distress during what is likely to be a prolonged dying. In contrast in this case, it appears that the infant might survive for an uncertain period of time with treatments that are neither invasive nor particularly burdensome. Overall, the infant’s expected prognosis remains poor but not hopeless. Continuing, but not escalating, the treatment regimen seems a reasonable option, allowing time to determine the

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efficacy of ongoing therapy. This plan should be discussed with the parents, detailing how oral medications and cannula oxygen differ from mechanical ventilation. A full disclosure of the potential benefits and burdens of this waitand-see approach should be balanced by a description of what the infant would experience were the medical goals redirected to comfort care. The parents should be allowed time to consider the options and their accompanying consequences. Time may help parents come to accept the future of caring for a child with disabilities. The discussion should try to arrive at consensus about which plan would be in the infant’s best interests, and that selfperceived quality of life for some survivors of extreme prematurity, although not likely theirs, may be better than either providers or parents expect. In this case, it would seem appropriate for the parents to have the decisive voice in decisions about ongoing care. The medical team should offer necessary support services to the parents whether the infant continues to receive treatment, undergoes inpatient comfort care, or is discharged from the hospital or to hospice care. If the medical team cannot support a parental decision to allow comfort care against their recommendations, they must consider to what extent the infant’s clinical course after extubation changes their prior assessment that death would be an acceptable outcome of discontinuing mechanical ventilation. If they feel unable to follow the family’s wishes with regard to continuing medical treatments, it would be reasonable to explore transfer to another institution where the medical team could in good conscience follow the parents’ treatment preferences. Stefan Kutzsche writes: Medical care is not usually beneficial for children with extreme IUGR. This infant

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defied those odds and survived for several months, although with lifethreatening complications that continued to require frequent interventions. It became increasingly unlikely that the infant would ever be able to leave the hospital. As clinicians told the parents that they felt that their interventions were not of any benefit, the parents agreed to discontinue artificial ventilation. Doctors and parents both expected that the infant would die quickly. The plan, then, seemed to be as follows: 1. The infant would be extubated and doctors would see how the infant did. 2. The doctors and parents could both hope that the infant may make it. 3. If not, doctors would not reintubate. In my opinion, all members of the team should respect the infant’s unspoken right to live and the parents’ wishes to have their infant survive. The clinician’s role, at this point, is to strengthen the parents’ relationships with the child and prepare them in the practicalities of the withdrawal of life support. Parents must be told that even though the doctors did not expect their infant to live after withdrawal of ventilation, it would be possible that their son could live for days or even weeks. The parents hoped that their infant would survive. They are prepared for the worst. But when the infant confounded expectations and seemed to “want to live,” the parents also wanted their infant to live. The parents asked doctors for acting to benefit their child by sustaining their child’s life in a hope that his peaceful death could occur later. They expected clinicians to treat symptoms of discomfort, independently of whether their child received life-sustaining care or only palliative care. Their wishes were based on the principles to beneficence and avoiding harm.

This created a considerable dilemma. The parents may have lost confidence in the doctors’ability to predict the child’s outcome. The doctors may have felt that they let the parents down by not accurately predicting what would happen after the ventilator was discontinued. What should the doctors do now? The only achievable goal now was to build a stable therapeutic and empathic relationship between the parents and medical team. The latter needed to help the parents to understand what medical and nonmedical end points and goals might be achievable. In particular, they must support the parents coming to terms with the uncertainty of the infant’s life span, and in finding some meaning in their infant’s slow deterioration. Above all, the parents needed to know that the team had not abandoned them. The doctors and nurses should be careful to use neutral or positive language and not to suggest that the infant’s continued survival was a problem. John D. Lantos comments: In the United States, it is legal to withdraw life-sustaining medical treatment under a variety of circumstances. But it is not legal to give a lethal injection to a dying patient. The ethical difference between the 2 sorts of interventions may seem philosophically precious. After all, some say, the goal in both is to bring about the death of the patient in a situation where the prognosis is dismal.3 But one of the justifications for permitting the withdrawal of treatment but prohibiting active euthanasia is that we are sometimes incorrect in our prognosis. We may think that the withdrawal of life support will lead to a patient’s death. But it doesn’t. The most famous instance of this phenomenon is the case of Karen Ann Quinlan. Ms Quinlan survived for 9 years after her ventilator was withdrawn.4

We should always explain to parents that this outcome is possible and that it is not the withdrawal of the ventilator that causes death, it is the underlying disease. This may comfort parents by absolving them of responsibility for authorizing the death of a loved one. Cases like this one show that the distinction between treatment withdrawal and active euthanasia can be valid and morally important. Such cases should make us humble.

OUTCOME OF THE CASE The medical team and the parents assessedtheinfantdaily. Theparentswere helped to understand that the choice of palliative care did not mean they were failing their child. Opportunities were created for them to express emotions, and to review and honor their son’s life; they slowly prepared to bring their child home and were offered a step-down family room outside the neonatal unit. The infant was discharged at the age of 6 months on nasal oxygen insufflation,

oral morphine, and nasogastric feeding. The community health service supported both the patient and the family. Nurses were engaged for 7 night shifts a week and the hospital team visited daily. Although becoming increasingly difficult to sedate and needing more morphine, the infant remained responsive and interactive. After 3 weeks with palliative home care and a memorable time for the family, the boy died peacefully at home with his parents.

REFERENCES 1. Asch DA, Christakis NA. Why do physicians prefer to withdraw some forms of life support over others? Intrinsic attributes of life-sustaining treatments are associated with physicians’ preferences. Med Care. 1996;34(2):103–111

2. Meadow W, Lagatta J, Andrews B, Lantos J. The mathematics of morality for neonatal resuscitation. Clin Perinatol. 2012;39(4):941– 956

3. Perrett RW. Killing, letting die and the bare difference argument. Bioethics. 1996;10(2):131–139 4. Feinberg AW. The uncertain lessons of the Quinlan case. J Gen Intern Med. 1986;1(4): 267–268

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ETHICS ROUNDS

Is Treatment Futile for an Extremely Premature Infant With Giant Omphalocele? AUTHORS: Dalia Feltman, MD,a Theophil Stokes, MD,b Jennifer Kett, MD,c and John D. Lantos, MDd aNeonatology

Division, Evanston Hospital, Evanston, Illinois; Reed Medical Center, Bethesda, Maryland; cSeattle Children’s Hospital, Seattle, Washington; and dChildren’s Mercy Hospital, Kansas City, Missouri bWalter

KEY WORDS ethics, futility, omphalocele, prematurity, shared decision-making, palliative care, legal issues Dr Feltman conceptualized and designed the study and drafted the initial manuscript; Dr Stokes contributed substantially to the study conception and wrote and revised the manuscript; Dr Kett conceptualized the study and helped draft and revise the manuscript; Dr Lantos conceptualized the study and helped draft and revise the initial manuscript; and all authors approved the final manuscript as submitted. www.pediatrics.org/cgi/doi/10.1542/peds.2013-1001 doi:10.1542/peds.2013-1001 Accepted for publication Jul 9, 2013 Address correspondence to John D. Lantos, MD, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2014 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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abstract Doctors have no ethical obligation to provide futile treatment. This has been true since the time of Hippocrates who warned physicians not to treat patients who were “overmastered by their disease.” This principle remains valid today but, as the Society for Critical Care Medicine notes, it is difficult to identify treatment as absolutely futile in all but a few clinical situations. Far more common, they note, are “treatments that are extremely unlikely to be beneficial, are extremely costly, or are of uncertain benefit.” These, they say, “may be considered inappropriate and hence inadvisable, but should not be labeled futile.” So what should doctors do when they have a case that seems close to the futility threshold but does not, perhaps, quite cross it? In such cases, is it appropriate to make unilateral decisions to withhold life-sustaining treatment even if the family objects? Or should treatment be provided knowing that it might cause pain and suffering to an infant with no likelihood of benefit? To address these questions, we present a case of an extremely premature infant with a giant omphalocele and ask 3 neonatologists, Dr Dalia Feltman of Evanston Hospital, Dr Theophil Stokes of the Walter Reed Medical Center, and Dr Jennifer Kett, a neonatologist and fellow in bioethics at Seattle Children’s Hospital, to comment. Pediatrics 2014;133:123–128

Doctors have no ethical obligation to provide futile treatment. This ethical precept has been true since the time of Hippocrates, who warned physicians not to treat patient who were overmastered by their disease. This principle remains valid today but, as the Society for Critical Care Medicine notes, it is difficult to identify treatment as absolutely futile in all but a few clinical situations. Far more common, they note, are “treatments that are extremely unlikely to be beneficial, are extremely costly, or are of uncertain benefit.” These, they say, “may be considered inappropriate and hence inadvisable, but should not be labeled futile.”1 So what should doctors do when they have a case that seems close to the futility threshold but does not, perhaps, cross it? In such cases, is it appropriate to make unilateral decisions to withhold life-sustaining treatment even if the family objects? Or should treatment be provided, knowing that it might cause pain and suffering to an infant with no likelihood of benefit? To address these questions, we present a case of an extremely premature infant with a giant omphalocele and asked 3 neonatologists, Dr Dalia Feltman of Evanston Hospital, Dr Theophil Stokes of the National Naval Center, and Dr Jennifer Kett, a fellow in both neonatology and bioethics at Seattle Children’s Hospital, to comment.

THE CASE A neonatologist is asked at 2 AM to consult with a mother admitted for preterm labor at 25 weeks’ gestation. The pregnancy was complicated by a prenatal diagnosis of a large omphalocele on an ultrasound performed at 18 weeks. At that time, the parents spoke to neonatologists and surgeons about the omphalocele. Parents were planning to deliver at a tertiary care center to facilitate repair of the defect.

The mother now presents with spontaneous rupture of membranes, continuing contractions, and cervical dilation. The obstetricians say that she will deliver in the next few hours. Previous discussions with the parents did not broach the possibility of extremely premature birth. The neonatologist explains that because of the combination of extreme prematurity and severe congenital anomaly, delivery room resuscitation will be futile. She explains that the pediatric surgeon concurs with this assessment. She informs the family that the infant will be given excellent palliative care so that she will not suffer. She encourages the parents to hold their infant after she is born. The family is irate. The mother says, “This is wrong. You can’t just let her die. Please try to save her life. Do everything that you can.” The father says, “We are calling a lawyer. We demand that you do something to help our baby!” What should the neonatologist do? Dr Feltman Comments Whether a physician seeks limitation permission or simply explains that a futile treatment will be withheld, disagreement regarding the patient’s best interests may arise. The family may argue, plead, or threaten legal action for perceived abandonment. How should the medical team proceed? First, let’s examine the nature of a “futile” intervention as it pertains to this case. Youngner2 describes 4 criteria that are often used to support a claim that treatment is futile. These are as follows: (1) that the patient will have a poor quality of life; (2) that the treatment is physiologically ineffective; (3) that, although partially effective, the treatment cannot cure the underlying illness and will only postpone death; and (4) that the treatment has

an extremely low probability of being successful. Characterizing intervention as futile due to poor quality of life is least appropriate in this case. If this child could survive, but the parents and medical team had different views on whether certain aspects of her life would afford her a “reasonable” quality of life, this would warrant a different analysis, because parental wishes typically trump those of the medical team in such cases. Instead, in this case, the question is whether survival is possible because the infant is likely to have severe lung disease due both to prematurity and to the effects of the giant omphalocele. Aggressive delivery room interventions cannot be characterized as physiologically futile. They may be temporarily (hours to days to weeks) successful. This temporary success doesn’t alter the poor probability of survival. Treatment will be futile for anything but the temporary postponing of death. There are no reports of successful treatment of a giant omphalocele for an infant born at 25 weeks. Instead, experts strongly suggest that aggressive interventions may, at best, only postpone death for this infant for no more than a few weeks.3 Youngner2 argues that any futility arguments outside of physiologic futility (an example being providing cardiopulmonary resuscitation to a decapitated patient) are subject to the values of the patient, family, and physician. This case is not one of such physiologic futility. Instead, it is one in which treatment is deemed likely to cause pain and suffering and to only prolong the dying process. The question, then, is who should decide which action (resuscitation and intensive care or palliative care) is in the patient’s best interests. Lantos and Meadow4 summarize 3 commonly accepted pathways for when such disagreement occurs: (1) continued persuasion efforts, (2)

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intervention per family request, and (3) nonintervention by unilateral physician decision. They controversially suggest that, in some cases, a fourth option might be possible: to accede to the parents’ request for resuscitation but only perform cardiopulmonary resuscitation for a few minutes. These parents clearly love their infant and clearly feel betrayed by the doctors. They have prepared for aggressive surgeries knowing that death might occur. They don’t know how to deal with the new circumstances of an extremely preterm delivery. It is unlikely that the doctor will be able to persuade them to accept palliative care in the limited time that remains before delivery. Some argue that there is no need to solicit the parents’ permission to withhold attempts at life-sustaining treatments because such treatments are futile. By this argument, doctors have no obligation to offer or even discuss futile therapies. Thus, parents needn’t decide or even accede. In this case, intubation and ventilation might postpone death for days or weeks. Parents may feel that this is an appropriate goal of care. Perhaps they believe that such treatment is the right thing to do for their infant. If the neonatologist believes that the parents are acting in good faith and trying to do what is best for the infant, she could attempt resuscitation, explaining to the parents that this would be experimental, against physicians’ recommendations, and would inflict suffering without benefit. When one considers the 2 traditional aspects of a child’s “best interests” (present experiences and future interests), painful procedures are often justified because they may afford the patient a future that is better than the present. In our case, however, intubation, blood draws, mechanical ventilation, possible chest tubes, and chest compressions will be the means and the end. The burdensome treatments will not overcome the PEDIATRICS Volume 133, Number 1, January 2014

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condition; they will only allow for continued dependence on these interventions for the days or weeks before death. This situation highlights the dilemma that many clinicians feel. Overriding the primum non nocere principle in the absence of patient benefit, regardless of parental benefits, creates moral distress. Burt and Curtis5 characterize this visceral distress: “clinicians’ personal unwillingness to provide aggressive treatment that they themselves or their families would not want may arise from feelings of distaste, and even repugnance, that administering such treatments without reasonable prospect of success amounts to the infliction of physical abuse on dying or dead people.” Ultimately, intervening per this family’s request hurts both infant and physician, with questionable benefit to the family. What about a less than vigorous, symbolic effort at resuscitation? Lantos and Meadow4 argue that a modified resuscitation sometimes may be acceptable. They argue that some families in these situations “feel paralyzed and trapped.” For such families, they propose that doctors give ambiguous explanations of what will be done, that is, “we will continue to do everything we can that we think will help. And we will always do everything we can to make sure that he is not suffering. We will always be there, for him and for you.” For our case, limited ability to assess parents’ genuine inclinations makes this approach potentially dangerous. Particularly under time constraints, it is difficult to judge the parents’ reasons for wanting resuscitation. If judged incorrectly, the family may feel deceived if a less than fully aggressive resuscitation is performed. Additionally, the “slow” code typically refers to a patient who will most definitely die regardless of whether the code is slow or traditional; a slow code would not be appropriate in our case, for as previously discussed, aggressive

interventions could actually work for this infant to temporarily postpone death. Thus, this case comes down to a question of whether aggressive intervention that will only postpone death is so clearly against the infant’s best interests that it should not be provided even if the parents demand it. In my opinion, the burdens of treatment and the fact that survival in such a case is unprecedented justify nonintervention. Although unilateral decision-making is justified, this family deserves the “compassion, comfort, attendance, and shared grief” advocated by the rationale behind Lantos and Meadow’s “slow code.” This family has limited time to process inevitable death, perceiving abandonment. Whereas previous specialists delineated aggressive management, the neonatologist to their dismay “won’t do anything.” Assent should not be pursued; rather, condolences should be given because resuscitation would only prolong death. Focus should be redirected from what the physician will not do for the patient to what she will do. Attendance to comfort, elicitation of parents’ preferences regarding holding the infant, religious ceremonies, photography, etc, are the appropriate focus now that death is inevitable. Dr Stokes Comments Imagine that the neonatologist in this scenario possesses the ability to travel through time. She has traveled into the future and has seen that this infant died despite her best efforts at resuscitation. Having seen the future, she is absolutely certain that “delivery room resuscitation will be futile.” She is completely confident that her plan to provide “excellent palliative care so that she will not suffer” is ethically justified. Even so, the parents disagree. So what should the neonatologist do? The neonatologist seems to have the best interests of the infant in mind and has recommended a rational and ethical

course of action. Somehow, though, she has failed to adequately communicate with this family. I wonder whether a lack of empathy might be to blame. I would want to know more about her previous conversations with this family. Did she acknowledge the grief and the fear that this family was assuredly feeling? Did she find out if they had a name picked out for their infant? What was it like to learn of the omphalocele? What have their experiences been with doctors? What are they hoping for? What do they fear most? In taking the time to listen, learn, and feel with these parents, the doctor lays the foundation for a relationship based on trust and a promise to face hardship together. The doctor demonstrates that she is human, that she cares, and that she will be there when times get tough. Such a relationship is critical when agonizing decisions must be made. It does not appear to have been established in this case. This is a family who has been mentally preparing themselves for the birth of an infant with a major congenital anomaly. They have consulted with specialists and have learned about the obstacles facing their daughter after birth. They have been told that everything possible would be done to save their daughter. Now a doctor whom they have never met is telling them that it is hopeless to even try and save their infant. Is it futile to resuscitate an infant born at 25 weeks with a giant omphalocele? Giant omphaloceles are often associated with pulmonary hypoplasia, and at 25 weeks’ gestation this might indeed make resuscitation impossible. But pulmonary hypoplasia is difficult to diagnose prenatally. Estimates of gestational age often vary by 1 to 2 weeks. What if the infant was really 27 weeks, and what if the lungs weren’t hypoplastic? What if the omphalocele and intestines were intact at birth and amenable to a delayed surgical repair?

These questions cannot be answered until after birth. Clearly, the prognosis is not good. I believe that this infant will likely die. But this is my opinion, and I would have an honest discussion with the family about this. I would not tell them that delivery room resuscitation is futile. We would discuss both palliative care and delivery room resuscitation, and we would come up with a plan together. I would do my best to help them feel supported in their decision. If they wanted me to attempt resuscitation then I would. If they wanted me to provide palliative care then I would. If I started a resuscitation and it wasn’t working, then I would tell the parents that it wasn’t working, I would stop, and would then provide palliative care. Whichever course of action we decided on, I would assure them that I would be in the delivery room to care for their infant when she was born, and that we would continue to care for their daughter together. Dr Kett Comments Medical interventions that are not expected to benefit the patient are commonly referred to as futile. Although the attending physician does not provide her rationale in this case, it is likely that she believes that this infant, with 2 potentially life-threatening medicalconditions, is unlikely to survive. If there is no possibility that this infant can survive the immediate perinatal period, then aggressive delivery room resuscitation is indeed likely to be futile, and might even becruel. However, it isimportant thatour initial assessment of this infant’s prognosis be carefully analyzed. Extreme prematurity and giant omphalocele are, of course, extremely serious diagnoses. Infants born at 25 weeks face long NICU stays and may experience a number of life-threatening complications. However, the majority of infants born at this gestational age do survive, and many survive without serious

impairment. Because of this, the resuscitation of infants born at 25 weeks’ gestation and above is generally routinely recommended. Children born with giant omphalocele also face prolonged hospitalization and may likewise experience a number of serious complications. However, in the absence of other congenital anomalies, most full-term infants born with giant omphalocele survive to be discharged. Of course, the concern in this case is not each diagnosis as a separate entity, but that their combination would exponentially increase the likelihood of complications and therefore death. To begin with, other genetic or structural anomalies are common in infants with omphalocele. If present, many of these conditions might have a poor prognosis in the setting of extreme preterm birth. However, it may be possible to identify the features of some congenital anomalies on ultrasound before delivery to help make this assessment. Skin integrity is another area in which these conditions may impact one another. Extremely preterm infants have fragile, friable skin and can experience skin breakdown even from pulse oximeters and monitor leads. The care of an omphalocele membrane would be extremely challenging in this setting. In addition, infection risk is always a serious concern for extremely preterm infants, and the presence of an abdominal wall defect would be expected to increase this risk. Finally, these conditions are likely to severely amplify one another with regard to respiratory status. Extremely preterm infants are typically affected by respiratory distress syndrome and commonly require mechanical ventilation and surfactant administration after birth. Many infants born at this gestational age will go on to develop significant chronic lung disease. Respiratory insufficiency is also common in infants with giant omphalocele. If an infant with pulmonary

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insufficiency is also born extremely preterm, he or she simply may not have adequate lung function for survival. However, the degree of pulmonary insufficiency present can be difficult to predict prenatally. In addition, even severely preterm infants can have variable lung disease that is difficult to quantify before delivery. Clearly, the combination of extreme prematurity and giant omphalocele increases the risk of a number of complications. However, this situation does not mean that such complications are certain to occur. Respiratory status, skin integrity, andriskofinfection aredifficult or impossible to predict before delivery. Although pulmonary insufficiency is common in the setting of giant omphalocele, some infants with giant omphalocele do not have pulmonary insufficiency, and the respiratory complications of extreme prematurity alone are routinely managed successfully in the NICU. Likewise, extremely preterm infants do have surgical procedures on occasion, and caring for their wounds is a regular part of NICU care. Although thispatientisathighriskof complications and death, it is not certain that he or she will experience them. It is important to consider how much suffering this infant would be expected to endure in the pursuit of such a slim chance of survival. In the absence of complications, the most likely sources of discomfort would be mechanical ventilation and wound care. Mechanical ventilation, although certainly uncomfortable, is not generally thought to be painful. In fact, in many NICUs, infants who are mechanically ventilated do not receive analgesia or sedation. In addition, giant omphaloceles are not generally treated with primary closure. Staged bowel reduction or topical agents intended to epithelialize the existing membrane are typically used instead. Although these procedures may also be uncomfortable, they do not involve any type of surgical incision, PEDIATRICS Volume 133, Number 1, January 2014

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and therefore seem unlikely to be exceedingly painful. On the other hand, the treatment of potential complications might cause a great deal of suffering. For example, chest tube placement for pneumothoraces or the surgical repair of omphalocele rupture or an abdominal infection might cause significant pain. However, in the context of such complications, life-support measures could be reevaluated. It seems unlikely that an infant with giant omphalocele who is born at 25 weeks’ gestation could survive to discharge, but it is not impossible. In addition, in the absence of complications, it does not seem likely that this infant would experience a great deal of suffering. In this case, where the likelihood of success is small, but not zero, and where the infant would not necessarily experience great suffering, it seems reasonable to support the family’s strong desire to attempt resuscitation. In addition, the first several hours and days of life could provide information about lung function and skin integrity that could not be obtained prenatally. The decision to pursue continued lifesupport measures could be reevaluated as new clinical information became available. However, a careful review of this infant’s imaging studies should be performed before delivery, because the presence of other anomalies might drastically change the prognosis for this infant. In addition, the surgical team must be amenable to this plan, because they will be responsible for much of this infant’s treatment. Finally, this infant’s course must be closely monitored; should a clinical change occur that would cause significant suffering, the benefits of ongoing life support should be carefully reassessed. It is important that the care of this infant be at all times focused on her best interests, rather than the interests of the parents or the medical staff.

CASE REPORT: PART II The neonatologist suggests that she and the family meet together with the surgeon. The surgeon concurs with the futility assessment and says that an operation would not be feasible at this gestational age. The parents recognize that, without the possibility of surgery, resuscitation and neonatal intensive care would only cause pain. They say, “We don’t want baby to suffer if she can’t survive.” When the infant is born later that night, the neonatologist confirms the prematurity and the enormous abdominal defect. She notes a heart rate of 50. She wraps the motionless and apneic infant in a warm blanket and encourages parents to hold her and take pictures. A few minutes later, the infant dies. The family is left with the infant to grieve privately. Dr Stokes Comments This case illustrates the power of hope. Hope is a primal emotion, perhaps even a survival instinct. It is why discussions of futility are so fragile. The neonatologist’s statement that “delivery room resuscitation will be futile” seems to have triggered a fight or flight type of response in these parents. I wonder if the family’s response would have differed had the neonatologist said instead, “I think that delivery room resuscitation will be futile.” This simple rephrasing might have allowed the family to hear the conversation, while not completely overwhelming them with hopelessness. I recently counseled a family whom I was told was “unwilling” to accept their son’s diagnosis of trisomy 18. The diagnosis had been strongly suspected prenatally, and the infant had been admitted to the NICU after birth due to his small size, a small omphalocele, and an imperforate anus. An echocardiogram after birth had revealed a ductal dependent heart lesion. The NICU team was exasperated that the family would

not “accept reality” and were demanding that “everything be done.” I sat down with the mother and father and asked them to tell me how they were doing. For the next half hour the family told me the story of their pregnancy. The prenatal ultrasound and maternal blood tests were suggestive of trisomy 18. At each of their subsequent visits they heard that their son would be deformed and retarded and that there was no hope for survival. Ultimately this made them angry, and they stopped going to their prenatal visits at ∼30 weeks’ gestation. They told me that they couldn’t tolerate the negativity and hopelessness of the doctors any longer. The mother smiled when she told me about hearing her son cry for the first time in the delivery room. The father beamed when he recounted first seeing his son. Their son had proven all of the doctors wrong. He was alive. He had survived. Eventually, the mother asked me for my opinion, and I told them that I was pretty certain that their son had trisomy 18. Chromosome tests were pending, and thesewerelikelyto confirm thediagnosis. They nodded knowingly, and we talked further about trisomy 18 and how it had affected their son. I told them that their son was likely going to die of this condition, and we ultimately agreed that a palliative care plan would be best for him if the chromosomes confirmed our suspicions. At the end of our conversation, the father became tearful and thanked me profusely for my care of his son. In reality, I hadn’t really done anything for their son. I conveyed the same grim prognosis and recommended palliative

care just as the other doctors had. All I’d done differently was to listen and to feel. I was realistic while at the same time respectful of their hope. This was seemingly enough to allow them to hear what I had to say. It is not rational to maintain hope in the face of a hopeless situation, but that is the point that I am trying to make. Humans are rational and emotional creatures, and doctors must recognize this if we are to effectively communicate. Dr Feltman Comments As this patient illustrates, even in the “best” case scenario in which parents agree with the physician’s care plan, end-of-life decisions for a child whose extrauterine life has not yet begun are extremely difficult. Time constraints such as imminent delivery curtail opportunities for processing feelings and preferences, challenging relationshipbuilding between family and physician. Encounters in other high-velocity, highstakes settings such as the emergency department may present similar difficulties. When a treatment is deemed quantitatively or physiologically futile and therefore medically inappropriate, honest assertion of this and redirection to palliative interventions may strike the best balance between the patient’s best interests, physician integrity, and attendance to family grief.

successful, although it could be partially effective and, as a result, prolong the process of dying and increase the suffering of the dying patient. Should the doctor follow her own moral compass and refuse to provide a treatment that seems overwhelmingly likely to cause more harm than good? Or should she follow the parents’ requests and provide the treatment? Most of the time, such dilemmas arise because of the waywe have talked about diagnoses and options. We need to be careful about how we describe what can or cannot be done, and what we will or will not do. Sometimes, inadvertently, we err by either offering more hope than is justified or by limiting interventions too prematurely and absolutely. Decisions aboutresuscitation shouldnot be made before the infant is born. Even with an excellent prenatal diagnosis, there can be surprises in the delivery room. It is appropriate to assure the parents that a final decision will not be made until the doctor has a chance to examine and assess the newborn.

This case illustrates one of the most difficult moral dilemmas in all of medicine. Without intervention, a patient will die. Intervention is unlikely to be

The decision to withhold or withdraw treatment can happen before or after intubation, before or after chest compressions, and before or after resuscitation medications or cardioversion. In making the decision, it is crucial to consider the parents’ preferences and values. But it is not always right to allow those preferences to be determinative. Ultimately, the attending physician must make the decision about when and whether further intervention should be discontinued.

2. Youngner SJ. Who defines futility? JAMA. 1988;260(14):2094–2095 3. Tsakayannis DE, Zurakowski D, Lillehei CW. Respiratory insufficiency at birth: a predictor of mortality for infants with omphalocele. J Pediatr Surg. 1996;31(8):1088–1090, discussion 1090–1091

4. Lantos JD, Meadow WL. Should the “slow code” be resuscitated? Am J Bioeth. 2011;11(11):8–12 5. Curtis JR, Burt RA. Why are critical care clinicians so powerfully distressed by family demands for futile care? J Crit Care. 2003;18 (1):22–24

Dr Lantos Comments

REFERENCES 1. The Ethics Committee of the Society of Critical Care Medicine. Consensus statement of the SCCM’s Ethics Committee regarding futile and other possible inadvisable treatments. Crit Care Med. 1997;25:887– 891

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Cross-Cultural Differences in Communication About a Dying Child

Donald Cochran, MD,a Sarosh Saleem, MBBS, MBeth,b Sumaira Khowaja-Punjwani, CPB, MBeth, BScN,c John D. Lantos, MDb

There are more migrants, refugees, and immigrants adrift in the world today than at any time in the recent past. Doctors and hospitals must care for people from many different cultures, countries, and religious backgrounds. We sometimes find our own deeply held beliefs and values challenged. In this “Ethics Rounds,” we present a case in which a Pakistani immigrant family faces a tragic medical situation and wants to deal with it in ways that might be normative in their own culture but are aberrant in ours. We asked the American doctors and 2 Pakistani health professionals to think through the issues. We also invited the father to talk about his own experience and preferences. We conclude that strict adherence to Western ethical norms may not always be the best choice. Instead, an approach based on cultural humility may often allow people on both sides of a cultural divide to learn from one another. There are more migrants, refugees, and immigrants adrift in the world today than at any time in the recent past. Many people are insulated from the culture clashes that result from these dislocations. Doctors and hospitals are not. As we welcome the opportunity to care for people from many different cultures, countries, and religious backgrounds, we sometimes find our own deeply held beliefs and values challenged. In this “Ethics Rounds,” we present a case in which a Pakistani immigrant family faces a tragic medical situation and wants to deal with it in ways that might be normative in their own culture but are aberrant in ours. We asked the American doctors and 2 Pakistani health professionals to think through the issues. We also invited the father to talk about his own experience and preferences.

The Case A 6-year-old, previously healthy boy presented with ataxia, myoclonus, and cognitive decline. His symptoms had begun 3 months earlier with poor

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fine motor coordination and ataxia that had progressed. He ultimately received a diagnosis of subacute sclerosing panencephalitis (SSPE), a late complication of measles infection in infancy. SSPE is a progressive neurologic disease that has no treatment and no cure. Death is almost universal 1 to 3 years after onset, with mean survival of 18 months. The late stages include seizures, dementia, and a vegetative state.1

The parents immigrated to the United States from Pakistan 8 years earlier. Both parents spoke English fluently. They had 2 other children at home. The father was at the hospital more often. The mother was at home taking care of their other 2 children. After the diagnosis of SSPE was made, the father requested that his wife not be told of their son’s diagnosis, prognosis, or management plan, including possible upcoming procedures (gastrostomy tube, tracheotomy). The father said that the bad news was so painful to him,

abstract

aChildren’s Medical Center, Dallas, Texas; bChildren’s Mercy Bioethics Center, Kansas City, Missouri; and cAga Khan University, Karachi, Pakistan

All authors contributed to the design, drafting, and review of the manuscript, and approved the final manuscript as submitted. DOI: https://doi.org/10.1542/peds.2017-0690 Accepted for publication Mar 7, 2017 Address correspondence to John D. Lantos, MD, Department of Pediatrics, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2017 by the American Academy of Pediatrics FINaNCIaL DIsCLOsURe: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTeNTIaL CONFLICT OF INTeResT: The authors have indicated they have no potential conflicts of interest to disclose. To cite: Cochran D, Saleem S, Khowaja-Punjwani S, et al. Cross-Cultural Differences in Communication About a Dying Child. Pediatrics. 2017;140(5): e20170690

he wanted to shield his family and that he would break the news to his family when the time was right. “I know my wife better than anyone,” he said. In the meantime, he requested that all communication be with him only and that he would make all medical decisions.

This request caused moral distress and unrest among the medical team. One attending wrote in the chart that we had an “obligation” to ask the mother to come to the hospital so that we could tell her about her son. Others thought we should at least ask the mother what she wanted to know. There was a sense of urgency because his rapid deterioration raised concern about a more fulminant course that could mean death within months. How should the health team respond to the father’s request?

Donald Cochran, MD, Comments

The key question here is this: Should we tell the mother what we know against the father’s wishes? The providers should determine why he is making the request by asking, “What are your concerns about informing your wife?” One of the father’s stated reasons for the request was that he found the sad news painful and hard to bear. He wanted to spare his family the pain for the time being. His request was for more time and to let him tell his wife in his own way. He needed to think about how he would tell her, and he needed to build up the courage to do it. He reminded us that he knows his wife better than anyone. He may have had other experiences with her that led him to believe that the news should be handled delicately and would be handled best by him. We may not be privy to the mother’s medical history (particularly with mental illness) that would make the father even more protective. The mother could see that her son was deteriorating rapidly. The father recognized that

his progression led to a sense of urgency from the medical team, but he was consistent in his request for more time.

I think there may have been an assumption that the father was acting in a stereotypical Muslim male manner. I never got the impression that religious or patriarchal reasons were behind the request. It seemed to me that he was lovingly trying to protect his family. He showed respect to the female physicians. He never threatened the female attending’s authority or questioned her competency. In other words, he never acted as if his authority as the male head of household would be threatened if his wife were told. So putting an intention on the father’s request without exploring the reason for his request is wrong. What would be the harm of not telling the mother at that moment? We assume that she wants to know. That assumption is a deeply embedded cultural belief. Mothers in our culture would want to know. However, I think that asking the mother what she would want to know would be going against the spirit of the father’s request. The family unit is paramount in Pakistani society. By asking, we would be undermining the usual family interactions if the medical team inserted themselves into a family’s decision-making process.

Instead of making assumptions about a culture that I do not know much about, I think that the proper approach would be to respond as I would to any other father who made such a request. I would ask him about his concerns with informing his wife about their child’s illness, and I would tell him my concerns about not informing her. If the father had expressed that in his culture, the men make all the decisions, then we should respect that. Doing so would certainly be more challenging for some providers

to accept, because it goes against our own cultural norms. This is an American hospital, but do we have the obligation to insert our values, based on our own culture and traditions? I am not sure what would be gained. Striking the right balance between ethical norms and family needs requires skills in the art of medicine. The nuances of communicating and interacting are taught by experience. I would never lie to the mother or refuse to answer if she asked a question. But I would feel comfortable listening carefully to both father and mother and allowing truths to be revealed, as they inevitably will be, on their timetable rather than ours.

sarosh saleem, MBBs, MBeth, Comments

A pluralist, cosmopolitan society is a society which not only accepts difference, but actively seeks to understand it and to learn from it. In this perspective, diversity is not a burden to be endured, but an opportunity to be welcomed. Aga Khan IV, The Samuel L. and Elizabeth Jodidi Lecture at Harvard University Cambridge, Massachusetts, November 2015.

In a diverse, pluralistic culture, people hold very different beliefs. Such cultures thrive when we learn to respect and celebrate not only the values and customs we share but also our differences. But it is important to understand the basis of different beliefs. This case presents a contrast of cultures and values between the health care providers and a patient’s family. The concern raised by the providers is genuine. The custom, usual practice, and law in their culture all dictate that they should share medical information with both parents and involve them both in decision-making. The father’s request to withhold significant medical information about his child’s illness and progress toward death

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challenges this approach to doctor– family communication.

Before jumping to any conclusion, it is important to understand the sociocultural background of the family. Pakistan is a nonsecular, patriarchal society with its roots embedded in Islamic cultures and traditions. The Islamic religion gives equal rights to men and women. However, men have additional physical and economic responsibilities toward their families. Culturally, as well, men assume the roles and duties as head of the family and are considered responsible for making important decisions while caring for wives and children.

I assume that this Pakistani family is Muslim. In evaluating the father’s request, it is critical to distinguish between religious practices and cultural norms. The Quran, the central religious text of Islam, describes equal rights of men and women, portraying husband and wife in complementary rather than competitive roles. Sunnah (the life events and deeds of the Prophet Muhammad, peace be upon him) endorses shared decision-making by husband and wife in all matters of life. Men and women have equal social, legal, and moral status as human beings, according to Islam.2 The equitable differentiation between their roles and responsibilities in the family system endows a man to the duty of supporting and protecting his wife and children. Male dominance and authoritarianism are a strong cultural trait of South Asia, probably arising from social and cultural traditions of that region. Pakistan does have a patriarchal system, and it is customary for male family members (father, husband, brother, or son) to sign a consent form on behalf of a female patient or a child. These cultural traditions are commonly thought to be religious in nature. That is not correct, but it is a mistake

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made not only by outsiders but by misinformed Muslims too.

The death of a child can be devastating for any parent, anywhere in the world. Varied reactions can be expected. From a Western point of view, it may be strange and even shocking to seek to hold the news of the terminal illness of a child from the mother. From a Pakistani cultural perspective, it may be more understandable. They father may be trying to shield the mother from the shock and pain that this news may cause. In the absence of extended family, a common social support network in Pakistan, the father perhaps finds it appropriate to bear the burden of this sorrow and protect his wife for as long as he can. The father’s beliefs and preferences do not relieve the physicians of their responsibility to share important information with the patient’s mother. It is understandable that providers feel obligated to share the child’s medical information with his mother or at least ask her how much she would like to be involved. She might willingly say that all information should be shared and discussed with her husband only. Then, her wishes should be respected. But what if she wants to know?

Communication with understanding is the key to such situations where basic social values clash. Doctors should explain to the father that, with the progression of disease, it may not be possible to hold the truth much longer from his wife. Involving a religious or spiritual leader might help him realize and accept the inevitable truth. Muslims believe in divinely destined time of death. Prayer is meaningful near the time of death. An imam might help him see that his wife will probably want to be with the child and say special prayers at the end of his life.

sumaira Khowaja-Punjwani, CPB, MBeth, BscN, Comments In pediatric clinical setting the most common ethical question clinicians face is “Who has authority to make decisions for children?” This case extends that question by forcing professionals to ask, “Is it ever ethically permissible to not to tell a mother about the medical condition of child when requested by the father?” The question becomes more complex in the context of a profound cultural difference between the professionals and the father. In his culture, men are expected to make decisions for their family. They are obligated to do so.

I was born and raised in Pakistan. I live and work there now. I trained in bioethics in Karachi. We studied mostly Western ethics. I have always thought that this training was inadequate to deal with the issues that arise in Pakistani culture (and probably many others). Medical professionals are trained in an ethics that assumes that “one size fits all.” As a result, we health care professionals have become mechanical in our responses to challenging cases. It is offensive to us if a patient or patient caregiver makes a choice that diverges from routine or contrasts with what we believe is in their best interest. We learn that the patient’s autonomy must always be prioritized. We learn that, in pediatrics, both parents must participate in the process of shared decision-making. In many clinical scenarios, however, these ideals are unrealistic or inapplicable. We must learn to recognize that every individual is different, and not all share the same values.

Asian family values are very different from the Western family value system. In Eastern cultures the family unit includes the extended family. Traditional gender roles are common. The family patriarch is considered to be gatekeeper of private information and routinely makes health-related

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decisions for other family members.3 The concept of autonomy in East and West is distinctive. In the East, decisions about family matters are made either by the elder male member of the household or by consensus of elder members of house, which usually includes the father, the elder son, or an uncle. Women and children lack absolute individual autonomy in this culture. They are considered vulnerable. Therefore, the men of the house have an obligation to make decisions for the entire family and to consider the best interest of the entire family. The man’s job is tough and demanding. He must be emotionally strong almost all the time. To do so violates human nature.

This thoroughly patriarchal approach leads to odd situations. For instance, sometimes a 25-year-old elder son will make decisions for his 60-yearold, perfectly competent mother. This model, in which men make all the important decisions, is shared by many Eastern cultures. It is a strikingly different approach from that prevalent in many Western countries. For a medical team with a different cultural orientation, it is difficult to understand the background reasoning of the father’s request in this case. For me, a Pakistani nurse and bioethicist, it is easy.

Cultural humility is the best approach to effectively address such cases. This approach focuses on commitment and active engagement in a lifelong learning process. The clinicians should enter into an ongoing dialogue with patients, communities, colleagues, and themselves, based on the principles of cultural humility.4 “In a modern world where power imbalances exist, cultural humility is a process of openness, self-awareness, being egoless, and incorporating self-reflection and critique after willingly interacting with diverse individuals.”5 The ethical principle of autonomy is

focused on informed consent, shared decision-making, and each individual’s right to control what happens. The emphasis of cultural humility is to recognize power imbalances and not automatically give preference to the most powerful.

The best approach in this case is to acknowledge the father’s request and to gain his confidence by showing respect for his culture. This step should be followed by therapeutic communication to help him to learn that he needs to share information about the condition and prognosis of the child with his wife. Doctors and nurses should offer to help him through that difficult conversation. He needs to know that the medical team cannot lie to the mother, and thus they may need to dishonor his request to withhold information. For his wife to hear news from the doctors, rather than from him, would be the worst possible approach to communication in this case. Here is the most important lesson: Cultural factors that may be initially perceived as barriers may better understood as opportunities for enhanced, personalized treatment. In this case, the medical team perceived the problem as their difficult decision about whether, or to what degree, they could deviate from their routine protocol of shared decision-making. However, if they view these cross-cultural differences as opportunities to find the best in both cultures, these perceived barriers could be incorporated into a new plan about communication. That might ultimately benefit the care of this patient, this family, and future patients and families.

The Patient’s Father Comments

I would like to tell the story of our son Z’s illness from our perspective. I wrote this story myself, but I discussed it with my wife.

Around November 2015, Z’s teacher told us that Z was struggling with his

fine motor skills and that we needed to work with him to improve them. We took him to his primary doctor, and she told us that everything is normal and he would catch up with time. In February 2016, he even had a full physical examination. We were told that he was normal.

In March he started to lose his balance and fell down at times. We decided to consult the doctor again. The primary doctor referred us to neurology. We stayed for 3 days at the hospital, where they did some laboratory work and an MRI and EEG. Doctors suspected he had some form of ataxia and sent us home. We went back to hospital after 10 days because he was falling more and could barely sit upright. He again went through numerous tests, radiographs, and an MRI. At that point he received a diagnosis of SSPE. April was the most difficult time in our lives. We were losing our only son slowly and gradually. It was so painful for me to know what was coming next and to know that there was no cure. It was painful to know that my son would lose his sweet voice and would never be able to talk and call us Mom and Dad, that he would never be able to play soccer or watch his favorite movie, Cars, again. We would miss his hugs forever. To see your child like that and not be able to help was extremely painful.

I did not want my family to feel the pain that I was feeling. I wanted to reveal the diagnosis and prognosis at appropriate time and started mentally preparing them for the worst possible news of their lives. I know my family better than anyone else. I feared for my wife and family. I worried that my wife would not be able to bear the shocking news so soon after the initial correct diagnosis. I am very thankful to doctors and staff for their full cooperation and

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support. They respected my decision of not sharing his prognosis with my family for those few days. When I realized that my wife and other family members were mentally ready to accept the terrible news and seemed to be in a state when they could handle it, I asked the doctors to share the reality with them. I thank all the doctors and staff at Children’s, who took great care of Z and of us during his stay at the hospital.

John D. Lantos, MD, Comments

Often, ethical dilemmas initially seem to be about one thing, but analysis reveals that they are about something else entirely. In this case, the health professionals perceived the father through a distorting lens. They assumed that Western ethics about individualism and autonomy are universal and thus that the father was violating a universally recognized moral norm. That is clearly not the case. Our rules and our norms about individuality are as culturally embedded and as unique as those of any other culture. In many cultures, the family is the smallest unit of moral consideration. In those cultures,

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decisions are made by designated individuals within family systems. It is not clear whether 1 cultural approach is necessarily superior to another, or even what a study would look like to try to determine the safety and efficacy of either. We cannot randomly assign people to one culture or another. However, we can recognize the need for cultural humility. An approach based on cultural humility should always start with a willingness to question one’s own beliefs and cultural norms. Is our way necessarily the best way? What could we learn from other cultural approaches? Might there be a new synthesis that would arise if we thought about the strengths and weaknesses of each approach? Carefully considered compromises based on cultural humility will often be preferable to overconfidence, intolerance, and unwillingness to listen and learn.

aCKNOwLeDGMeNTs

We thank Dr Ladan Agharaokh for assistance in describing the case. We thank the patient’s father for offering his thoughts.

aBBRevIaTION SSPE: subacute sclerosing panencephalitis

ReFeReNCes

1. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J. 2002;78(916):63–70 2. Omar S. Marriage in Islam: life partnership or discriminatory family set up? An analysis of some protective legal and moral Shariah provisions for women with special reference to Surah An-Nisa. Available at: https://papers.ssrn. com/sol3/Delivery.cfm/SSRN_ID2492224_ code1438834.pdf?abstractid=2492224& mirid=1&type=2. Accessed September 5, 2014 3. Hilliard ME, Ernst MM, Gray WN, Saeed SA, Cortina S. Adapting pediatric psychology interventions: lessons learned in treating families from the Middle East. J Pediatr Psychol. 2012;37(8):882–892 4. Tervalon M, Murray-García J. Cultural humility versus cultural competence: a critical distinction in defining physician training outcomes in multicultural education. J Health Care Poor Underserved. 1998;9(2):117–125 5. Foronda C, Baptiste DL, Reinholdt MM, Ousman K. Cultural humility: a concept analysis. J Transcult Nurs. 2016;27(3):210–217

ETHICS ROUNDS

Are We Allowed to Discontinue Medical Treatment in This Child? Wendela G. Leeuwenburgh-Pronk, MDa, Laura Miller-Smith, MDb, Vicki Forman, RNc, John D. Lantos, MDb, Dick Tibboel, MD, PhDa, Corinne Buysse, MD, PhDa

One of the most difficult ethical dilemmas in pediatrics today arises when a child has complex chronic conditions that are not curable and cause discomfort with no prospect of any improvement on quality of life. In the context of medical futility, it is harmful to prolong medical treatment. The question is: How can medical treatment be discontinued when the child is not dependent on mechanical ventilation or ICU treatment? What is the appropriate palliative care and does it justify the use of sedatives or analgesics if this also might shorten life? One of the most difficult ethical dilemmas in pediatrics today arises when a child has complex chronic conditions that are not curable and cause discomfort with no prospect of any improvement on quality of life. In the context of medical futility, it is harmful to prolong medical treatment. The question is: How can medical treatment be discontinued when the patient is not dependent on mechanical ventilation or ICU treatment? What is considered proper palliative care and does it justify the use of sedatives or analgesics if this also might shorten life? We present such a case and seek commentary from experts with experience in such difficult cases. Laura Miller-Smith is a pediatric intensivist at Children’s Mercy Hospital in Kansas City, Missouri. Vicki Forman is a nurse at Verdugo Hills Hospital in Glendale, California, and the author of This Lovely Life: A Memoir of Premature Motherhood (2009). Wendela Leeuwenburgh-Pronk is a pediatrician, currently working at Emma Children’s Hospital in Amsterdam. Dick Tibboel and Corinne Buysse are pediatric intensivists at the Sophia Children’s Hospital in Rotterdam, Netherlands. They were the physicians involved in

abstract

the case. The parents consented to publication of the case.

THE CASE

a

Anna was born at term after an uneventful pregnancy. At birth she was diagnosed with Down syndrome, a small atrial septal defect type II, and respiratory distress due to stenosis of the choanae. Anna’s parents welcomed her into their life, never questioning the quality of life for and with a disabled child. During infancy, Anna developed multiple mysterious problems that seemed unrelated to the Down syndrome. She had feeding problems with the constant urge to vomit. The cause of these problems was unknown. At the age of 7 months, a gastrostomy and jejunostomy were placed, which alleviated her symptoms only mildly. She had intermittent severe respiratory distress, thought to be due to a combination of laryngotracheomalacia and severe obstructive sleep apnea due to stenosis of the choanae and hypotonia. At age 9 months, she remained dependent on oxygen supplementation and noninvasive positive-pressure respiratory support. A tracheotomy alleviated the symptoms of obstructive sleep apnea syndrome, but Anna

Sophia Children’s Hospital, Erasmus Medical Centre, Rotterdam, Netherlands; bChildren’s Mercy Hospital, Kansas City, Missouri; and cVerdugo Hills Hospital, Glendale, California

Drs Leeuwenburgh-Pronk, Miller-Smith, Lantos, Tibboel, Buysse, and Ms Forman helped conceptualize the project and contributed to the manuscript; and all authors reviewed and approved the final manuscript as submitted. Dr Leeuwenburgh-Pronk’s current affiliation is Emma Children’s Hospital, Academic Medical Centre, Amsterdam, Netherlands. www.pediatrics.org/cgi/doi/10.1542/peds.2014-0548 DOI: 10.1542/peds.2014-0548 Accepted for publication May 7, 2014 Address correspondence to Wendela G. Leeuwenburgh, MD, Department of Pediatrics, Emma Children’s Hospital, PO Box 22660,1100 DD Amsterdam, The Netherlands. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2015 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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suffered from sputum/saliva retention for which endotracheal suction several times every hour is necessary. An EEG revealed subclinical seizure activity. A skin culture grew methicillinresistant Staphylococcus aureus. Isolation further compromised her development because she could not go to the playroom or outside for a walk. Isolation, masks, robes, and hats limited normal human interaction. At the age of 11 months, she was able to focus only briefly and occasionally grasp a finger. She no longer showed any signs of recognition in response to her parents and did not smile. She was diagnosed with cerebral visual impairment and a mild conductive hearing loss. She exhibited constant movement, facial grimacing, choking, coughing, and vomiting. The medical team and parents together agreed to a do not resuscitate (DNR) order. The parents also requested withdrawal of positivepressure respiratory support. The PICU team was divided on the decision to withdraw medical treatment. The PICU team members consulted with specialists in developmental and genetic disorders, discuss the case with Anna’s family physician, and consult the university clinical ethics committee. In their opinion, the treatment so far had not established any improvement and considered no additional treatments available that would result in improvement of quality of life. Continuing medical treatment was unanimously regarded as futile. The medical team agreed to accede to the parents’ request to discontinue medical treatment. The parents then requested that, when medical treatment is discontinued, Anna will receive sufficient narcotics and benzodiazapines to sedate her. How would you respond?

LAURA MILLER-SMITH, MD, COMMENTS: The transition from pursuing cure to accepting death and providing palliative care can be difficult for

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health care providers. As highlighted in this case, that transition can be accompanied by moral distress and ethical uncertainty. Often, such distress and uncertainty are the result of less than optimal education for health care providers on the ethical and legal aspects of end-of-life care. The case illustrates a concern that often arises, spoken or unspoken, in situations of withdrawal of medical support. My first response in this scenario would be to ask the family to tell me more about the reasons why they are making this request. There is some ambiguity in what the parents might mean by “sedate.” I would want to hear them explain their concerns about their child. I would take the request as an opportunity to explain to them, in some detail, the process that we go through when we withdraw life support. I always try to clarify with the family what events will transpire when such therapies are withdrawn. I give them specifics about what support will be discontinued, and what will remain, what medication will be given and in what situations, the expected duration until the child will expire, and the clinical changes that will occur during that time period. I will frequently ask the parents what their concerns are surrounding these events. For most, the prevention of suffering, rather than hastening of death, is paramount. In the given scenario, the parents may be asking that their daughter not be allowed to suffer during removal of life-sustaining support. If that is the case, and their goals are now to minimize pain, agitation, and dyspnea, then narcotics and benzodiazepines are appropriate medications to accomplish these goals. I will always inform the family that the side effects of these medications can be respiratory suppression, but that we accept that risk of this complication because our priority is relief of pain and agitation.

Most parents are able to understand this concept of “double effect,” although their comfort with the secondary consequence is variable. I have had families request lower doses of medication (and, in some cases, even no sedation or anxiotolytics), because they do not want, in any way, to hasten their child’s death. Others have requested more of such medication to ensure that suffering is minimized, knowing that this may give them less time with their child. The process of treating end-of-life pain and distress, even knowing that such treatment might hasten death, has been labeled with many names. Some call it “terminal sedation” or “palliative sedation.”1–3 Regardless of the semantic nuances, the central ethical feature of such end-of-life care is that it requires that pain and suffering are refractory to standard medication doses. These attempts should be made before the escalation of sedation to the point of unconsciousness. In cases in which a patient is sedated to a level of unconsciousness, however, it is usually difficult to truly know whether the patient is suffering. Thus, the moral nuance comes down to the care providers’ intention. Is it to provide comfort and minimize pain, even if doing so requires higher doses of medication? Or is it to hasten death? If support is truly titrated to meet the patient’s need, and attempts have been made to use more routine dosing, then, ethically, terminal sedation or palliative sedation can be justified by the concept of “double effect.” Thus, I will increase the doses of medication I use to achieve my goal of pain relief. What, then, is the difference between such practices and active euthanasia? In the United States and many other countries, there is clearly a legal difference. Physicians may not legally give a patient medication with the explicit goal of hastening his or her death. However, the gray zone between prevention of pain and

facilitating a faster death comes down to the physician’s intention. With providing palliative medications, our intention must be to treat pain and discomfort. There have certainly been documented cases4–6 in which the physician’s intentions have been questioned by either other health care providers or even family members, and what may have otherwise been viewed as routine palliative care comes under scrutiny by either internal or external review. It is difficult to verify the physician’s intentions after an event. Because of the concern of legal ramifications, some physicians may feel uncomfortable giving the dose of medication that is needed to prevent suffering. This is true even though the legal risk is very low.4 Such liabilitydriven restraint also raises ethical concerns. My obligation is to treat my patient through the time of their death, and do my best to prevent the suffering that may accompany it. I should not hesitate to provide treatments that achieve these goals. I am transparent with both families and nursing staff about the goals of care, the medications and doses needed to achieve those goals, and my intentions. I carefully document such discussions in the medical record. This process should prevent subsequent second-guessing about intention. Institutional adoption of palliative care guidelines can also support the health care team in ensuring that adequate pain control is achieved, without fear of reprisal. Recently, after having the conversation with a parent about how I could use medications to treat pain and discomfort, but not to hasten death, the mother told me “No one would do this to my pet. Why are we doing it to my daughter?” For this mother, her daughter’s fate had been determined during her prolonged cardiac arrest. When the child was found to have severe neurologic insult that would not progress to

brain death, the family chose to withdraw support. Although they legally understood my limitations, they ethically had significant trouble with the fact that I could not use the tools at my disposal to “free” their daughter from her suffering. In their minds, her death was a fait accompli. We were only delaying the inevitable. Such cases are occasions to struggle with what it truly means to have a “good death.” Our dying patients’ families will never forget their final hours with their child. Those memories should not be tainted by fears that they, or we, did not do everything we could to ensure that their child died with dignity and minimal suffering.

VICKI FORMAN, RN, COMMENTS: For health care providers, Anna’s case is ethically complex. For Anna’s parents, it is heartbreaking. It may be difficult for health professionals to understand just how confusing, exhausting, and emotionally devastating it can be to go through a long and difficult course of medical treatment of a chronically ill child who is unresponsive to therapy. It is clear that Anna’s medical course was characterized by unpredictable complications that were not necessarily connected to her diagnosis of Down syndrome. We don’t know how her parents dealt with these surprises. Most parents fear having a disabled child. Yet, they also know that there are no guarantees that a child will be free of impairments that may have lifelong implications. Despite their fears of what it might be like to raise a child with developmental delays or impairments, they would never have chosen the alternative of a life without that child. Most parents want their children to have the best treatment no matter how disabled those children are or how poor the prognosis. Given all that, I would guess that these parents would not have come to their request for a DNR order lightly.

Anna, of course, has rights of her own. It is not surprising that the intensivists would question whether a DNR order was appropriate. Given the burdens of ongoing treatment and the uncertainty of the prognosis, however, such a parental request is logical and understandable. It is time to have a conversation regarding Anna’s long-term care and prognosis. For a child such as Anna, one complication can beget another. The absence of neurologic development, her significant complications, and the likelihood that further interventions will not improve her outcome should lead any provider to the ultimate questions: To what end are we continuing treatment? Would a DNR order and the withdrawal of medical treatment be the more humane choice? The PICU team members clearly have an ethical obligation to care for Anna. Parental requests to withhold or withdraw life-sustaining treatment of profoundly disabled children who are not otherwise terminally ill may seem to be requests to violate this obligation and thus are often greeted with horror. Some team members think they could never make similar requests or decisions. But Anna’s parents have the same obligation. No parent desires to make the request Anna’s parents made. In this case, however, I would assume that Anna’s parents were also considering Anna’s future. I would assume that their requests come from their consideration of what is in Anna’s best interest. After all, they had already consented to other interventions (tracheostomy, gastrostomy), indicating that they wanted life-preserving measures when those were likely to benefit Anna. I would see their request as coming from a desire to protect Anna from further suffering in the event of a catastrophe. Once the parents request to withdraw treatment, a more profound ethical dilemma arises. A DNR is one thing. Interventions that may hasten death

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at the time of withdrawal of therapy are something else entirely. Again, a caregiver could assume in this instance that the parents were looking for an “easy” end to Anna’s complicated life. Examined from the parents’ perspective, however, the request for pain relief and sedation may actually be a way to ensure that Anna’s transition from life to death would be pain-free. In cases in which a child is to be removed from life support, family members often focus, appropriately, on concerns about pain and suffering. If these concerns underlie the request for narcotics and sedation, the PICU team has a duty to explain as best as possible the dying process, what the parents can expect, what Anna may experience, and what interventions are best suited to ease her suffering, with the caveat that nothing can truly be predicted. It is reasonable to provide morphine to ease the work of breathing, or benzodiazepines to relieve visible agitation. It is not reasonable to medicate Anna in the absence of these symptoms, and the team is ethically bound to explain these conditions to Anna’s parents. Finally, what are the parents’ expectations about Anna’s death, now that they have made the decision to terminate treatment? Are they looking to the medication to ease Anna’s suffering or do they believe pain relief and sedation will make death somehow easier? The choice to implement a DNR and then withhold care is very abstract, whereas the death of a child is unbearably concrete. Despite their apparent belief that medications might ease Anna’s suffering, the team is dutybound to prepare the parents for the physiologic (and psychological) realities of Anna’s last hours. With honest communication about this process, perhaps Anna’s parents would feel more assured that pain relief and sedation may not be necessary, or that if they are, they will be administered appropriately.

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WENDELA G. LEEUWENBURGH-PRONK, MD, DICK TIBBOEL, MD, AND CORINNE BUYSSE, MD, COMMENT: The individual members of the PICU team had different opinions on the decision to withdraw medical treatment in this case because the diagnosis of Down syndrome is not generally associated with a poor quality of life. In this case, however, further treatment seemed futile to some because of the severe complications that Anna had developed in addition to the Down syndrome. Others, however, thought that her problems might still be successfully treated. In particular, they pointed out that Anna was able to breathe on her own. Thus, it seemed likely that she could be discharged from the PICU. After discharge, doctors could focus on the amelioration of the feeding problems and treatment of her seizures. The PICU team attempted to define Anna’s well-being in terms of both her current condition and her prospects for the future. They appropriately requested a second opinion from specialists in developmental pediatrics and in genetics. The PICU team also consulted the university clinical ethics committee. The process of evaluation was comprehensive. Over the course of several weeks these experts visited Anna multiple times. They interviewed her parents and the nurses caring for her. They also requested the opinion of Anna’s general practitioner. The general practitioner visited Anna in the hospital and discussed the situation with the parents and PICU doctor. The team then held a multidisciplinary meeting. The doctors, nurses, and social worker caring for Anna discussed her prognosis and quality of life. They concluded that the treatment so far had not led to any improvement and that it was unlikely that continued

treatment would lead to improvement. The medical team judged Anna’s current quality of life to be very bad with ongoing pain and suffering that was difficult to treat. Thus, continuing medical treatment was unanimously regarded as only prolonging suffering and disability and could therefore be considered harmful. Therefore, the medical team agreed that the parents’ request for discontinuation of medical treatment was appropriate. According to Dutch law, the active termination of a child’s life is illegal. It is legally justified, however, to withdraw treatment in case of medical futility and treat signs of discomfort with sedatives or analgesics. The question arising here is whether such treatment is proper palliative care or whether, instead, it is a prohibited hastening of death. The main purpose of this treatment must be to alleviate discomfort and not to shorten or end life. We explained that to the parents and provided treatment according to those principles.

JOHN D. LANTOS, MD, COMMENTS: In cases such as this, doctors and parents have been working together against tremendous odds in the hope that they can save the precious life of a child. The months of hard work have been an emotional roller coaster, with parents and professionals buffeted between hope and despair. In such cases, it is often difficult for everybody to simultaneously come to the realization that continued treatment is not in the child’s best interest. Hope springs eternal. But if, after consultation with experts, discussion among the professionals, input from the ethics committee, and heart-wrenching discussions with parents, it is everyone’s considered judgment that further life-prolonging treatment is not beneficial, then the withdrawal of such medical treatment is appropriate. At that point, the focus of medical therapy must be on the

treatment of the patient’s symptoms and efforts to make sure that the patient does not suffer during the dying process.

OUTCOME OF THE CASE Over the course of many days before Anna’s death, the responsible doctor talked with the parents openheartedly. The doctor’s goal was to maintain an atmosphere of transparency and mutual respect. This doctor placed Anna on her mother’s lap in preparation for withdrawing the supplemental oxygen. The doctor had propofol at hand to be used if Anna showed any signs of discomfort. After discontinuation of the oxygen supplementation, some signs of

discomfort were noted. Propofol relieved Anna’s discomfort. She died within a few minutes. In the course of weeks and months thereafter, the medical team met with Anna’s parents several times to reflect on the course of events. The parents repeatedly said that they appreciated the humane care given by the PICU team.

REFERENCES 1. Hasselaar JG. Palliative sedation until death: an approach from Kant’s ethics of virtue. Theor Med Bioeth. 2008;29(6): 387–396 2. Papavasiliou ES, Brearley SG, Seymour JE, Brown J, Payne SA; EURO IMPACT. From sedation to continuous sedation until death: how has the conceptual basis of

sedation in end-of-life care changed over time? J Pain Symptom Manage. 2013; 46(5):691–706 3. Putman MS, Yoon JD, Rasinski KA, Curlin FA. Intentional sedation to unconsciousness at the end of life: findings from a national physician survey. J Pain Symptom Manage. 2013;46(3):326–334 4. Goldstein NE, Cohen LM, Arnold RM, Goy E, Arons S, Ganzini L. Prevalence of formal accusations of murder and euthanasia against physicians. J Palliat Med. 2012; 15(3):334–339 5. Kuschner WG, Gruenewald DA, Clum N, Beal A, Ezeji-Okoye SC. Implementation of ICU palliative care guidelines and procedures: a quality improvement initiative following an investigation of alleged euthanasia. Chest. 2009;135(1):26–32 6. Lynøe N, Leijonhufvud M. Police in an intensive care unit: what can happen? J Med Ethics. 2013;39(12):772–775

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ETHICS ROUNDS

Two Infants, Same Prognosis, Different Parental Preferences Armand H. Matheny Antommaria, MD, PhDa, Christopher A. Collura, MDb,c,d,e, Ryan M. Antiel, MD, MAf, John D. Lantos, MDg,h

abstract

a

Ethics Center and Division of Hospital Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio; Departments of bPediatric and Adolescent Medicine, and f Surgery, Mayo Clinic; Rochester, Minnesota; cPalliative Care Fellow, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Boston, Massachusetts; d Department of Medicine, Boston Children’s Hospital, Boston, Massachusetts; eCenter for Bioethics, University of Minnesota, Minneapolis, Minnesota; gDepartment of Pediatrics, University of Missouri, Kansas City, Missouri; and hChildren’s Mercy Hospital Bioethics Center, Kansas City, Missouri

Drs Antommaria and Antiel conceptualized and designed the study and drafted the initial manuscript; Dr Collura contributed substantially to the study conception and reviewed and revised the manuscript; Dr Lantos conceptualized the study and helped draft and revise the initial manuscript; and all authors reviewed and approved the final manuscript. www.pediatrics.org/cgi/doi/10.1542/peds.2013-4044 DOI: 10.1542/peds.2013-4044 Accepted for publication June 12, 2014 Address correspondence to John D. Lantos, MD, Children’s Mercy Hospital Bioethics Center, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: jlantos@ cmh.edu PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2015 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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A central principle of justice is that similar cases should be decided in similar ways. In pediatrics, however, there are cases in which 2 infants have similar diagnoses and prognoses, but their parents request different treatments. In this Ethics Rounds, we present such a situation that occurred in a single NICU. Three physician-ethicists analyze the issues.

One of the toughest situations that arises in pediatrics is when 2 infants in the hospital at the same time have the same disease, the same prognosis, and require the same treatment, but, in 1 case, the parents want everything done, and, in the other case, the parents choose end-of-life comfort measures only. We know that different parental choices are permissible, when the indications for treatment are ambiguous and the prognosis uncertain. Yet it feels odd when the patients are in the hospital at the same time, perhaps side by side in the same unit, and caregivers work to keep 1 patient alive while providing palliative end-of-life care for the other. Such cases force us to look at the rationale for life-prolonging treatment or for palliative care. We present such a situation and ask for comments from Armand Antommaria, MD, PhD, a pediatric hospitalist and Director of the Ethics Center at Cincinnati Children’s Hospital Medical Center; Ryan Antiel MD, MA, a resident in surgery at the Mayo Clinic; and Chris Collura, MD, a neonatologist and bioethicist who is completing training in pediatric palliative medicine in the Harvard Palliative Care Fellowship.

THE CASES A 45-year-old G12P9 Amish mother delivered a premature infant at

33 weeks. After difficulty feeding, doctors diagnosed proximal esophageal atresia with tracheoesophageal fistula (TEF). The infant underwent TEF ligation with planned delayed anastomosis of the esophagus given the degree of gap length. Postoperatively, several attempts at extubation failed. Bronchoscopy revealed severe tracheomalacia at the level of the ligated TEF pouch. The medical team recommended tracheostomy with chronic mechanical ventilation for a period of months to years until the infant’s growth could optimize surgical repair. Head ultrasounds were normal. The family had many concerns. They had no electricity at home and realized that the infant would have to be placed in long-term care as long as mechanical ventilation was necessary. In addition, such an intervention would incur significant costs. Consistent with most Amish, the infant’s family refused statesponsored medical assistance including Medicaid. The family refused tracheostomy and requested compassionate extubation and comfort measures for their child. A second patient cared for concurrently in the same level III NICU had also been diagnosed with TEF. This neonate had been born by caesarian delivery as a premature twin at 31 weeks’

gestation to a 29-year-old G2P1 mother. Shortly after birth, a diagnosis of esophageal atresia with TEF was made. Unlike the first patient, primary anastomosis of the esophagus was performed. The postoperative course was complicated by cardiac tamponade secondary to pericardial effusion of parenteral nutrition from a central venous line. The infant recovered after emergent pericardiocentesis and prolonged cardiorespiratory resuscitation. The remaining hospital course was marked by multiple failed trials of extubation due to persistent airway obstruction. Severe distal tracheomalacia was diagnosed. Despite an unclear neurodevelopmental prognosis because of prolonged cardiopulmonary resuscitation, the medical team recommended tracheostomy with chronic mechanical ventilation for a period of months to years. The parents accepted these recommendations. The NICU staff was troubled by these decisions. Some felt that in case 1, treatment was more clearly in the infant’s best interests because the long-term prognosis was better. The staff struggled with the ethics of whether both decisions were permissible. Given the family’s beliefs that chronic technology-dependent intervention not consistent with their value-driven desires for their child, should the NICU medical team accept the parents’ request to withdraw ventilatory support in this Amish infant? Should they recommend treatment in the other case?

ARMAND ANTOMMARIA COMMENTS The initial component of any case analysis is clarifying the situation. In the first case, it would be important to clarify the implications of the parents’ religious beliefs for the patient’s care. The Amish are 1 of a group of denominations that

developed out of the Protestant Reformation collectively known as the Anabaptists (rebaptizers) because of their insistence on adult baptism. Contemporary Amish have different church districts that may interpret religious regulations differently.1 There may, therefore, be a wider range of options acceptable to the parents than the case description suggests. Although the Amish use alternative and traditional medicine, they also use contemporary, allopathic medicine.1,2 They are not opposed to technology per se. Instead, they adopt it selectively with the goal of building community and preserving social capital. As an example, the Amish forbid using 110-V current from public utility lines, but they do not prohibit all uses of electricity. Generators are permitted to power welders and carpentry tools and to recharge batteries. Inverters may be used to convert 12-V current from batteries into 110-V current. In some cases, exceptions have also been made for medical equipment. These distinctions are important to determine whether, in this case, it could be technically feasible and ethically acceptable to care for the patient at home. The Amish value self-reliance and community autonomy. They believe it is the church’s responsibility to care for its members. They therefore refuse to participate in Medicaid, Medicare, or commercial insurance programs. It would be important to clarify the degree to which the family’s objection to long-term care is based on familial or community obligations to care for the infant or on financial concerns. Additional financial resources may be available to the family within their own district or from adjoining districts. Some districts participate in an informal community-based hospital insurance called Church Aid. The amount of resources required for home mechanical ventilation might,

however, be considered poor stewardship.3 For the sake of argument, let us stipulate that either the treatment is ethically unacceptable to the parents or they are unable to pay for it. One could analyze the case using either a principle- or case-based approach. Preferring a case-based approach, I would frame the issue as whether the refusal to provide mechanical ventilation constitutes medical neglect. Parents have broad discretion in the care of their children, but the state is justified in intervening in cases of abuse or neglect. In addition to failing to provide adequate food or shelter, the failure to provide adequate medical care can constitute neglect. In evaluating suspected cases of neglect, the primary focus should be on the effects on the child rather than on the parent’s reasons. Even loving or well-intentioned parents can seriously harm their children. Religious justifications for refusing medical treatment therefore do not have special standing.4 The clearest cases of medical neglect involve the failure to provide discrete, lifesaving interventions that are highly efficacious and have limited side effects. The classic example is administering court-ordered blood transfusions to children whose parents are Jehovah’s Witnesses to treat acute blood loss. Although blood transfusions have risks, major risks are infrequent. The risk of contracting hepatitis B, for example, is ,1 in 800 000 U. Judicial decisions are less consistent when the condition is not immediately life threatening or the treatment is prolonged, has limited efficacy, or has significant side effects. Examples include chemotherapy and organ transplantation. For example, even though liver transplantation is a effective treatment of lifethreatening fulminant hepatic failure, it requires lifelong immunosuppression that entails significant risks including infection.

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At the other end of the spectrum, courts have usually declined to intervene in cases where the disease was disfiguring but not life threatening. Gathering accurate information regarding the conditions under which the courts have intervened is difficult because there is no systematic recording of trial court decisions. Prolonged mechanical ventilation is likely to fall within the gray zone. It can be lifesaving but is an ongoing intervention that entails significant risks. These risks include tracheal injury, respiratory infection, and impaired communication skills and swallowing.5 A traditional principle-based approach, focusing on the child’s best interests, also produces an indeterminate result. There is controversy about the effects of mechanical ventilation on the individual’s quality of life, the degree of burden on the caregivers, and how these factors should be weighed. Objectively, children who require prolonged mechanical ventilation tend to report decreased healthrelated quality of life compared with other children with chronic illness. Subjectively, they report their quality of life is acceptable.6 Caring for a technology-dependent child can be enriching, but it requires significant effort from the caregivers. These burdens may, however, be decreased by improved societal support.7 Even if this level of care giving is not morally obligatory, relinquishing custody would be an alternative to withdrawing life-sustaining treatment. A child could be cared for in an intermediate care facility or taken into state custody. Although children who lack a bonded caregiver have poorer developmental outcomes, it is not clear that their outcomes are so poor that they would be better off dead. Preferable alternatives might be a medical foster home, if the child may eventually be weaned off the ventilator, or adoption.

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The situation in the first case strikes me as exceptionally sad. Interpreting the case charitably, the patient’s parents love their child and are trying to do what they believe is best for him or her. Removing the child from their custody arguably increases the risk for a poor outcome. Although others may reasonably disagree, I am inclined to argue that the child’s interests should be primary and that mechanical ventilation would be an effective, time-limited intervention with a favorable benefit-to-risk ratio. I believe that state intervention would be justified if an appropriate placement were highly likely. With respect to the second case, it appears that the medical team also recommended a tracheostomy and continued mechanical ventilation. The parent’s preferences are not clear. If the parents were to refuse, one would need to consider whether the patient’s potential neurologic impairment alters the analysis of his or her interests. Most individuals with neurologic impairment are capable of having interests, including an interest in life-sustaining treatment. They should be protected against discrimination. Individuals with profound impairments may not, however, be capable of having interests, or their interests may be significantly reduced. More information would need to be provided about the patient’s potential neurologic outcome to fully analyze the second case. The key question, about which individuals may reasonably disagree, is whether, in these 2 similar situations, withdrawing mechanical ventilation constitutes medical neglect. If it does, both infants should receive mechanical ventilation, either as a result of parental consent or state intervention. If it does not, parents may choose to continue or withdraw mechanical ventilation based on their values and beliefs. In the latter case, different outcomes may be ethically appropriate.

Understanding why different outcomes may be ethically acceptable may help reduce the staff’s moral distress.

CHRISTOPHER COLLURA COMMENTS These families present what are most likely very different cultural, religious, and socioeconomic perspectives that inform disparate requests for their seriously ill infants. The Amish family relies on a value framework that heavily weighs the burden of the intervention as well as the financial resources of the family and community to contribute to their request to withdraw ventilator support. The other family acknowledges neuroprognostic uncertainty and elects to pursue tracheostomy and chronic mechanical ventilation. Analysis of these 2 divergent paths should prompt a systematic method in determining the ethical construct of each process. The American Academy of Pediatrics (AAP) has advocated for a patientcentered approach when considering intensive medical interventions for newborns. Any proposed treatment must be assessed as to whether it is consistent with the child’s best interests.8 In these cases, the medical teams must first consider the benefits and burdens of tracheostomy and chronic mechanical ventilation for these 2 children. The providers must weigh the likelihood of survival and cognitive and psychomotor prognosis against the invasive and long-term physical burden of the treatment. Tracheostomy for indications of airway obstruction has demonstrated favorable long-term survival and successful decannulation in the majority of patients.9,10 Tracheostomy may facilitate conscious engagement in emotional, physical, and intellectual activity for the 2 neonates described in the cases. Burden assessments often require value-laden judgments about an intervention’s impact on quality of

life. Limited data suggest that children who undergo tracheostomy face significant risk for serious postoperative complications including critical cannula obstruction, displacement, tracheal necrosis, and death. Less serious complications are much more common and include bleeding, granuloma, and skin wounds.2 The overwhelming majority of children with tracheostomy will experience lung infections.3 Many infants who receive tracheostomies have developmental delay and cognitive impairment,11,12,13 but it is difficult to disentangle the effect of the tracheostomy from the effect of their underlying diseases. Patients with tracheostomies and chronic ventilation require access to tertiary subspecialists, intensive home provider education, and home health care equipment, supplies, and staff. Tracheostomy and chronic mechanical ventilation may optimize both infants’ chances for long-term and developmentally engaged survival. Although invasive tracheostomy and chronic ventilation is burdensome and presents severe risks, the physical impact of the treatment does not clearly outweigh its benefits. The medical teams can confidently offer this intervention based on the best interests assessment. The question becomes whether this intervention is morally and legally obligatory. Just as the AAP advocates for a patient-centered approach, it also maintains the vital role of parents in decision-making. Parents’ cultural, religious, educational, social, and ethnic backgrounds often present complex values and decision-making perspectives that may diverge starkly from that of physicians. These views should be respected as parents and medical teams make rational decisions together concerning the care of the child.14 For the Amish patient, the parents request to withhold tracheostomy and withdraw the ventilator. The

decision considers the financial burden of long-term medical treatment on their family and community. The transition to home would likely have been precluded by the community’s restricted access to electricity and the rural geographic limitations to home care providers. Options for long-term care could have included a skilled nursing facility or medical foster home. The financial considerations of the Amish family described in the case are intrinsic to the social fabric of the Amish community. Although resource allocation is rooted within the cultural construct of the Amish, there is no societal precedent that allows it to be the sole driver determining withdrawal of life-sustaining medical treatment. Presuming the best intentions, the parents’ decision was additionally likely rooted in their own benefitburden assessment for their infant. Given their request to withdraw the ventilator, it may be reasonable to conclude that the parents gave less weight to their child’s survival than to the degree of physical impact invasive and long-term medical technology dependence would have on the infant’s quality of life. The medical decision-making regarding tracheostomy rarely presents itself as a clear benefit with minimal burden. Providers should override family wishes only when there is definitive evidence that the family’s surrogate decision-making is contradictory to the infant’s best interest.15 Although the best interest standard lends itself to offering the intervention, burdens of the proposed treatment are not minimal. Because of this, it is less clear whether this same assessment should morally compel state custody, invasive surgery, and long-term technology dependence against parental values. Medical decision-making for the Amish infant falls into a gray zone. The ethical framework for each case defaults to prioritize well-informed

parental authority on behalf of the child. All efforts should be made to medically educate the family, explore options for temporary medical foster care, long-term skilled nursing, or unconventional modes of treatment to facilitate the most informed choice. Ethics and legal consultation, team and family discussions, and thoughtful deliberations are warranted. Ultimately, the parents’ decision in both cases reflects their own values. These values seem to include, appropriately, concern about both the degree of physical burden on the infant’s life and financial concerns related to treatment. Given the uncertainty about outcome, it is ethically permissible to forgo tracheostomy in one case and to proceed with invasive interventions in the other. When the results of a benefit burden calculus are nondeterminative, then we should support the parents’ goals of care.

RYAN ANTIEL COMMENTS This case is framed against the backdrop of 2 families’ decisions for and against withdrawal of lifesustaining treatment compelled by their different cultural and religious backgrounds. It asks whether bedside determination of resource utilization is a valid component of parental decision-making. Is honoring the parent’s worldview, specifically in regard to available resources and expenses, something the physician can decide ad hoc? The AAP reminds pediatricians that when caring for critically ill children, they should consider the value systems of patients and their families beyond simply quantitative indicators of prognosis.14 However, the AAP has also declared that decisions regarding the withdrawal of life-sustaining treatment in children should not consider the financial interests of the parents or the limited resources of the family.8 In the Amish context, the

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AAP’s directive to consider the family’s social values in end-of-life decision-making directly conflicts with the obligation to discount concerns of financial means. The Amish believe in a strong separation between church and state and therefore refuse life and health insurance. Given limited or finite resources, the Amish must consider finances in the broader justice-driven context of the community. Should we allow these parents to decide differently based on their values? I predict that this case would not have been troubling to the team had the parents defended their decision for withdrawal of care with arguments using the traditional and accepted bioethics lexicon. The parents could have argued that the burdens of medical and surgical treatment outweighed the potential benefit and, therefore, seemed to impose unnecessary harm on their child. Comfort care, they could have argued, seemed to be in their child’s best interest. Had the argument been framed with only the individual in mind, it would have been more viscerally reassuring to the medical team; and they would have been able to honor the parents’ values regarding the benefits and burdens of a life sustained by mechanical ventilation. Instead, the parents considered their limited finances and the impact that continued care would have on their broader community. The medical team was inclined to think purely in terms of the individual’s medical burden, but the Amish parents challenged the assumption that burden is so narrowly defined. Although the best interest standard is frequently used in determining the necessity for state intervention when parents refuse medical treatment, the primary criterion by which this is determined remains unclear. It is not clear that the best interest of the child should be the sole factor in decision-making; the interests of

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children must be balanced against the welfare of others. Furthermore, any notion of best interest is intrinsically value laden. Instead it has been proposed that the threshold for state intervention should be the harm principle: does a parent’s refusal significantly increase the likelihood of serious morbidity when treatment options have a high likelihood of success?16,17 How is this case different from parental refusals of other lifesaving treatments? We are intolerant when Jehovah’s Witnesses parents refuse a blood transfusion for their infant because they believe it will result in the loss of the child’s salvation or when fundamentalist parents refuse chemotherapy for their child with leukemia because they believe God will heal their child without treatment. In these cases that seem black and white to the medical community, where the child is at significant risk of serious harm (ie, death), we call on the state to interfere. Although we grant parents significant freedom to make health care decisions for their child, we no longer believe that parental authority is absolute. Perhaps it is the invasiveness of the treatment and the projected burdens of prolonged mechanical ventilation at a long-term care facility and multiple surgeries that make this case fall within the gray zone. Here the benefit was not overwhelming enough to compel state intervention. Yet even this is arguable, for how could lifesaving tracheostomy with eventual decannulation not be overwhelming? We know that without treatment, the infant will die, as happened in this case. In contrast, infants who require tracheostomy placement and prolonged mechanical ventilation demonstrated average survival rates at 1, 3, and 5 years of life of 96%, 91%, and 90% respectively.12 MacIntyre has emphasized that different traditions constitute and mediate diverse visions of justice and

reason.18 This is in contrast to the liberal presumption that there exists a shared, ahistorical objective rationality, which can be used to resolve differences between competing socially embedded traditions. One’s background and metaphysical commitments largely determine one’s conception of justice and what constitutes reason. The enlightenment emphasis on individual rights shifted the moral unit of analysis from “persons-aspart-of-a-moral-community whose principle goal was a shared pursuit of the good life to persons-as-individualrational-moral-agents whose principle goal was the free pursuit of self-declared interests or preferences.”19 The Amish typify “persons-as-part-of-a-moral community” in ways somewhat baffling to our individualistic medical culture. Their very use of medicine is aimed at building community. Whereas modern medicine is inclined to think in terms of individuals, the Amish necessarily have to consider broader social implications. But how are we to consider the interest of an infant with an ambiguous future? An infant is not an asocial, autonomous, self-reliant agent but rather a dependent member of a particular moral community. As demonstrated by this case, physicians need to consider whether a community justice–based ethical vision should trump our traditional and supposedly objective standard.

JOHN D. LANTOS COMMENTS There is an irreducible tension in moral philosophy between principledriven approaches and case-based ones. Emerson famously wrote that “a foolish consistency is the hobgoblin of little minds.”20 Jonathan Sacks, former Chief Rabbi of the United Kingdom, thought otherwise when he wrote that “A world without values quickly becomes a world without value.”21 Rigorous application of principles runs the risk of ignoring

the particularities of unique situations. Case-based approaches can lead to an anchorless moral relativism. These cases, and the insightful commentaries, highlight the need (and the difficulty) of balancing these 2 approaches to moral reasoning. Neither can ever be completely adequate by itself. The challenge is to recognize the particular elements of seemingly similar cases that allow ethical defensible variability in our responses. This presents a challenge to doctors and nurses because it appears to be ethically inconsistent and thus unjust. It seems that infants with similar conditions should be treated similarly. An infant’s condition is never, however, just the medical condition. It also includes the cultural and religious values of the family and community within which the infant is born and would be raised. In complicated situations like these, when (1) the prognosis is questionable and (2) reasonable people disagree about the preferable course of treatment, a foolish consistency does not serve the best interests of children. Instead, deference to parents’ values and preferences may appropriately tip the scales of justice. REFERENCES 1. Kraybill DB. The Riddle of Amish Culture. Rev. ed. Baltimore, MD: Johns Hopkins University Press; 2001 2. Huntington GE. Health Care. In: Kraybill DB, ed. The Amish and the State. 2nd ed. Baltimore, MD: The Johns Hopkins University Press; 2001:163–189

3. Kotva JJ. The Anabaptist Tradition: Religious Beliefs and Health Care. Park Ridge, IL: The Park Ridge Center; 2002 4. Committee on Bioethics. American Academy of Pediatrics. Conflicts between religious or spiritual beliefs and pediatric care: Informed refusal, exemptions, and public funding. Pediatrics. 2013;132(5):962–965 5. Make BJ, Hill NS, Goldberg AI, et al. Mechanical ventilation beyond the intensive care unit. Report of a consensus conference of the American College of Chest Physicians. Chest. 1998; 113(suppl 5):289S–344S 6. Noyes J. Health and quality of life of ventilator-dependent children. J Adv Nurs. 2006;56(4):392–403 7. Carnevale FA, Alexander E, Davis M, Rennick J, Troini R. Daily living with distress and enrichment: the moral experience of families with ventilatorassisted children at home. Pediatrics. 2006;117(1). Available at: www.pediatrics. org/cgi/content/full/117/1/e48–e60

12. Singer LT, Kercsmar C, Legris G, Orlowski JP, Hill BP, Doershuk C. Developmental sequelae of long-term infant tracheostomy. Dev Med Child Neurol. 1989;31(2):224–230 13. Jiang D, Morrison GA. The influence of long-term tracheostomy on speech and language development in children. Int J Pediatr Otorhinolaryngol. 2003;67(suppl 1):S217–S220 14. American Academy of Pediatrics Committee on Bioethics. Ethics and the care of critically ill infants and children. Pediatrics. 1996;98(1):149–152 15. American Academy of Pediatrics Committee on Bioethics. American Academy of Pediatrics Committee on Bioethics: Guidelines on foregoing lifesustaining medical treatment. Pediatrics. 1994;93(3):532–536 16. Diekema DS. Parental refusals of medical treatment: the harm principle as threshold for state intervention. Theor Med Bioeth. 2004; 25(4):243–264

8. Bell EF; American Academy of Pediatrics Committee on Fetus and Newborn. Noninitiation or withdrawal of intensive care for high-risk newborns. Pediatrics. 2007;119(2):401–403

17. Ross LF, Aspinwall TJ. Religious exemptions to the immunization statutes: balancing public health and religious freedom. J Law Med Ethics. 1997;25(2–3):202–209, 83

9. de Trey L, Niedermann E, Ghelfi D, Gerber A, Gysin C. Pediatric tracheotomy: a 30-year experience. J Pediatr Surg. 2013;48(7):1470–1475

18. MacIntyre A. Whose Justice? Which Rationality? Notre Dame, IN: University of Notre Dame Press; 1988

10. Al-Samri M, Mitchell I, Drummond DS, Bjornson C. Tracheostomy in children: a population-based experience over 17 years. Pediatr Pulmonol. 2010;45(5): 487–493 11. Overman AE, Liu M, Kurachek SC, et al. Tracheostomy for infants requiring prolonged mechanical ventilation: 10 years’ experience. Pediatrics. 2013; 131(5). Available at: www.pediatrics.org/ cgi/content/full/131/5/e1491

19. Tilburt J. Shared decision making after MacIntyre. J Med Philos. 2011;36(2): 148–169 20. Emerson RW. Self-reliance. Available at: http://www.emersoncentral.com/ selfreliance.htm Accessed December 2, 2013 21. Sacks J. Has Europe lost its soul? Available at: http://www. catholiceducation.org/articles/social_ justice/sj0255.htm Accessed December 2, 2013

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Different Philosophies

Introduction | When Doctors and Parents Have Different Philosophies John D. Lantos, MD, Associate Editor, Pediatrics .

n many situations, parents are at a decided disadvantage compared to doctors because the doctors know more

I

about disease than do the parents. Thus, the parents are dependent on the doctor not just to care for their child

but also to give them the information that they need to make decisions. There are some situations, however, in which parents are well informed and have their own philosophy of care that differs from that of the doctor. Probably the most common such situation today is the decision about whether to immunize healthy children. In that situation, doctors and their professional societies generally recommend a well-studied and time-tested schedule of immunizations. Many parents, however, oppose routine immunization. This leads to a unique sort of clash between doctors and parents, one that is not resolvable using the usual tools of shared decision-making. In fact, some studies show that discussion between doctors and parents only leads to a hardening of positions on both sides, rather than a compromise on some middle ground.1 There are other instances of this sort of disagreement. It arises sometimes when a child has a rare disease. If the disease is so rare that doctors are unfamiliar with it, and if the parents are savvy enough to learn about their disease, the parents may develop more expertise than the doctors. Furthermore, in a world of social media, parents can easily connect with other parents whose children have similar conditions. A common example of this situation occurs today with trisomy 13 and 18.2 Most doctors rarely see cases of these chromosomal syndromes. But there are very active and sophisticated advocacy organizations and social media groups where parents can find information.3 Most such groups have a particular philosophy of care—they want all available treatments for their children. Most doctors recommend only comfort care for children with these syndromes. These differing philosophies of care can lead to intractable disagreements that are similar to those that arise about routine childhood immunizations. A third situation arises when a child is born with congenital deafness. Today, most people agree that such children should receive a cochlear implant. But should the child then be taught sign language as well as spoken language? Many parents and advocates within the deaf community think that becoming bilingual in that way will be best for the child. Many doctors think that learning to sign will interfere with a child’s development of oral language. What should doctors do when there is a medical treatment that is clearly medically indicated for a particular child and the parents not only do not want that treatment but, in refusing the doctors’ recommendations, the parents have both information and social support? What should parents do when they want a particular treatment and the doctors are unwilling to provide it? The answer today is to engage in a process of shared decision-making. That is, doctors should work with parents, try to understand their goals and values, and try to work out a plan of care that respects those goals and values. The doctor can often shape the discussion, but the ultimate decisions lie with the parents. Shared decision-making is seen as a middle ground between the extremes of excess physician paternalism and unbridled patient autonomy. Sometimes, however, doctors and parents are just too far apart in their views of what is best for the child. Sometimes, more conversation just leads to more antagonism, more deeply entrenched disagreements, and a breakdown in communication and trust. In some situations, doctors feel that they have no alternative but to either terminate their relationship with the family or to report parents to child protective services in order to ensure that children get the best treatment. Sometimes, parents harness the power of social media or seek legal counsel themselves in order to force doctors to provide treatments that the parents think are essential. Such cases highlight the existence of moral “gray zones” in which reasonable people disagree, there is no obviously right choice, societal consensus is lacking, and a decision must be made. There are risks to children from allowing gray zones to expand and become too broad. But there also are risks in attempts to eliminate any gray zone and, instead, have rigid policies that dictate choices in all situations. The discussions in these cases highlight the ways that different doctors, lawyers, philosophers, and parents think about such decisions.

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References 1.

Dube E, Gagnon D, MacDonald NE, et al. Strategies intended to address vaccine hesitancy: review of published reviews. Vaccine. 2015;33:4191–4203

2.

Pallotto I, Lantos JD. Treatment decisions for babies with trisomy 13 and 18. HEC Forum. 2017;29:213–222

3.

Guon J, Wilfond BS, Farlow B, et al. Our children are not a diagnosis: the experience of parents who continue their pregnancy after a prenatal diagnosis of trisomy 13 or 18. Am J Med Genet A. 2014;164A:308–318

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ETHICS ROUNDS

A 6-Month-Old With Vaccine-Hesitant Parents abstract Many primary care pediatricians find vaccine hesitancy to be one of the most frustrating situations that they face. Parents who refuse to vaccinate their children implicitly call into question an intervention that most pediatricians see as one of the safest and most effective health care interventions of all time. Many pediatricians respond by refusing to care for children whose parents refuse vaccines, and some may consider that the parent’s refusal warrants referral to child protective services. We present a case in which a pediatrician faces this dilemma, with responses from pediatricians in academia and private practice. Pediatrics 2014;133:526–530

AUTHORS: Douglas J. Opel, MD, MPH,a Kristen A. Feemster, MD, MPH, MSHPR,b,c Saad B. Omer, PhD,d,e MPH, MBBS, Walter A. Orenstein, MD,c Monica Richter, MD, PhD,f and John D. Lantos, MDg aSeattle Children’s Hospital, Seattle, Washington; bChildren’s Hospital of Philadelphia, Philadelphia, Pennsylvania; cUniversity of Pennsylvania Perelman School of Medicine in addition to Children’s Hospital; dEmory Vaccine Center, and eHubert Department of Global Health, Emory University Rollins School of Public Health, Atlanta, Georgia; fValley Children’s Clinic, Renton, Washington; and gChildren’s Mercy Hospital, Kansas City, Missouri

KEY WORDS vaccines, ethics, parental rights, law ABBREVIATION AAP—American Academy of Pediatrics Drs Opel and Orenstein conceptualized and designed the study and drafted the initial manuscript; Drs Feemster and Richter contributed to the study conception and drafted the initial manuscript; Dr Omer contributed substantially to the study conception and reviewed and revised the manuscript; Dr Lantos conceptualized the study, helped draft and revise the initial manuscript, and reviewed the final manuscript; and all authors approved the final manuscript as submitted. www.pediatrics.org/cgi/doi/10.1542/peds.2013-2723 doi:10.1542/peds.2013-2723 Accepted for publication Oct 10, 2013 Address correspondence to John D. Lantos, MD, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2014 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: Dr Feemster serves on the US Advisory Commission for Childhood Vaccines and receives reimbursement for time as a special government employee; the other authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: Dr Feemster received an honorarium from Pfizer, Inc, for participation on an advisory board regarding vaccine acceptance among clinicians, received an honorarium for participation in Influenza Forum Europe sponsored by Abbott Laboratories, and served as an expert witness for the defendant in a medical malpractice case about neonatal herpes simplex virus; the other authors have indicated they have no potential conflicts of interest to disclose.

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Many primary care pediatricians find vaccine hesitancy to be one of the most frustrating situations that they face. Parents who refuse to vaccinate their children implicitly call into question an intervention that most pediatricians see as one of the safest and most effective health care interventions of all time. Many pediatricians respond by refusing to care for children whose parents refuse vaccines, and some may consider that the parents’ refusal warrants referral to child protective services. In this month’s ethics rounds, we present a case that raises this issue. We then ask a group of experts to guide us through the ethically controversial choices. Respondents include Douglas J. Opel, a general pediatrician and bioethicist at Seattle Children’s Hospital; Monica Richter, a pediatrician in private practice in Renton, Washington; Saad B. Omer and Walter A. Orenstein of the Emory Vaccine Center; and Kristen Feemster, an infectious disease specialist at the Children’s Hospital of Philadelphia.

THE CASE You are seeing a 6-month-old boy for his health supervision visit in your primary care practice. This boy is completely unimmunized at his parents’ request. You explain to the parents, as you have on each of their well-child visits, that their son is at risk for a life-threatening disease. You once again strongly encourage them to immunize their son. They remain steadfast in their refusal because, as they tell you, they strongly believe that the risks of immunization outweigh the benefits. In particular, they are concerned about the safety of vaccines at his young age and believe that their child’s naturally strong immune system will protect him. Recently, you and your colleagues have been seeing a lot of pertussis cases in your practice, and according to the Department of Health, there have been 241 cases of pertussis in children ,1 year in your state in the last year. There PEDIATRICS Volume 133, Number 3, March 2014

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have been .2000 cases statewide across all age groups, which is a 1000% increase over the previous year and exceeds state-defined epidemic thresholds. As of yet, there have been no deaths from pertussis among children ,1 year. What would your next steps be in caring for this child and family? Response of Dr Douglas J. Opel This case illustrates the classic ethical tension in public health: weighing the competing values of individual choice and the common good. Pediatric providers are often at the center of this balancing effort. While trying to preserve a therapeutic alliance with parents, pediatric providers must decide at what point might it be ethically justifiable to intervene against parents’ wishes to protect their child’s or the public’s health. The presumption in US law is to respect parental autonomy and family privacy.1 Parents are presumed to best understand the needs and interests of their children. Parents are therefore allowed wide discretion in the decisions they make on behalf of their children. But parents’ rights are not unlimited. Those rights can be overridden when they make decisions that place their child’s health, well-being, or life in jeopardy. The benefits and burdens associated with a decision, and importantly, their likelihood of occurring, therefore are central to whether a parent’s decision reaches this harm threshold. To determine whether state intervention to override the parents’ wishes in this case would be justifiable, we must evaluate the benefits and burdens of all possible options to determine whether their refusal poses significant risk of serious harm to the child.2 We can begin this evaluation by focusing on the risk. The state is currently amid a pertussis epidemic: 241cases of pertussis among children ,1 year and .2000 cases total have been reported

this year in the child’s community. Although these numbers are hard to interpret principally because of underreporting and uncertainty about the total size of the population at risk, the cumulative incidence or an incidence rate offers a reasonable estimate of the level of risk to a child of contracting pertussis. We can develop an incidence estimate by extrapolating from recent pertussis epidemics. In the 2010 California and 2012 Washington State epidemics, the pertussis incidence in children ,6 monthswas 445.9 per 100 000 persons3 and in children ,1 year was 428 per 100 000, respectively.4 Therefore, the 1-year risk of pertussis during these epidemics was ∼0.4%. Applying this estimate to our case, I would argue that this level of risk, which, even during an epidemic, is ,1% risk to a child ,1 year old, is not high enough to justify seeking state intervention to interfere with the parents’ wishes. It is hard to argue that a ,1% of risk is “significant” when this level of risk approximates the risks associated with the DTaP (diphtheria-tetanus-acellular pertussis) vaccine itself: fever (,1%), hypotonic-hyporesponsive episode (,1%), and seizure (,1%).5 Risk, however, is only part of the equation: what is the harm associated with pertussis if a child ,1 year was to become infected? Children with pertussis suffer harm: $50% may be hospitalized, ∼25% will have major complications, and #1% will die3,6; and these risks are highest in the first 6 months of life. However, I would again argue that the level of harm a child is likely to experience is not serious enough to justify intervening against parent wishes. In Washington State, where our nonmedical exemption rate for required kindergartenentry immunizations is 4.2% (seventh worse in the nation), I am not aware of any case in which the state has compelled a parent to immunize their child against their wishes, in epidemic conditions or otherwise.

There are similar situations where we would likely respect parental refusal of a high-benefit, low-risk intervention because of the overall low risk of serious harm to the child, such as parenteral administration of vitamin K prophylaxis to a term infant. Although I would earnestly explore alternatives (for example, oral vitamin K), I would not compel a parent to give vitamin K to their child. Likewise, I think the most appropriate next step in caring for this unimmunized child would be to explore options to reduce his risk of contracting pertussis, such as ensuring that the parents and close contacts are up-todate with Tdap (tetanus toxoid, reduced diphtheria toxoid, and acellular pertussis, adsorbed): cocooning may decrease the risk of transmission to the child. I would also ensure that the parents are adequately informed about the symptoms of pertussis. All this said, it is hard to ignore the burdens an unvaccinated child poses to the community. These burdens can be significant: it cost .$175 000 to contain a 2008 measles outbreak in San Diego started by an intentionally unvaccinated boy.7 Although consideration of these burdens often extends beyond the doctorparent relationship and ultimately may not tip the balance in favor of limiting parental choice, other societal mechanisms may exist to hold parents accountable for their decisions. If others are harmed by a parent’s decision to refuse to vaccinate his or her child, for instance, perhaps that parent should be held liable.8 Response of Drs Saad B. Omer and Walter A. Orenstein There are 2 potential types of actions a physician can take in situations in which a family refuses to accept recommended vaccines: (1) decline further care to the family or (2) continue to provide care but use strategies to change their mind about vaccination

over time and mitigate effects of nonvaccination. Any decision in this context requires balancing 3 sets of interests: (1) interests of the child whose parents are refusing vaccines, (2) parental autonomy, and (3) interests of other children in the practice. Vaccines have been one of the most effective preventive tools for infant health. Therefore, vaccination has a clear benefit in protecting this infant from vaccinepreventable diseases, even after accounting for potential adverse events associated with each vaccine. Parental autonomy for children’s health-related decisions is well recognized (although this autonomy is not absolute).9 On the other hand, if this family is dismissed from the practice, they are likely to find a practice more sympathetic to their position on vaccines. Parents who refuse vaccines tend to seek care from providers who have less faith in vaccine safety compared with health care providers of vaccinated children.10 If the family seeks care from a physician more sympathetic to their position, there is a lower likelihood of an ongoing conversation regarding vaccine promotion to occur. Many parents who refuse vaccines for their young infants subsequently get their child vaccinated at older ages. In the case of this 6-monthold infant, parents may be more open to vaccination once the child is older. It is in the best interest of the child to continue receiving care from a “pro-vaccine” physician. Furthermore, the provision of some vaccines is better than total nonvaccination, and the pediatrician may be able to convince the family to accept some vaccines. Moreover, for some diseases such as influenza and pertussis, parents who refuse vaccines for their children should be strongly urged to get vaccinated themselves against these diseases to decrease the likelihood of transmission to their infants. Pediatric and family practice clinics have children who are unprotected

against vaccine-preventable diseases due to reasons other than vaccine refusal. For example, even for highly efficacious vaccines, some children do not mount a protective immune response (vaccine failures); some children have legitimate medical contraindications; and some children may be too young for vaccination, yet still susceptible (eg, an 11-month-old not yet vaccinated against measles). It could be argued that dismissing an unvaccinated child from a practice is in the interest of other children in the practice because unvaccinated children pose a risk to other children they interact with. However, in this situation, there are fewer restrictive options that preserve the interest of other children in the practice. For example, unvaccinated children could have a separate section of the waiting area. Moreover, they could be restricted to appointment times at the beginning or at the end of a clinic, minimizing their exposure to other children in the clinic. Any burden placed on vaccine-refusing families due to fewer appointment options would be reasonable because it does not substantially restrict parental autonomy while preserving interests of children whose parents do not refuse vaccines. We recognize that many practices may not have space for separate waiting rooms. However, if they do not, unvaccinated children could be asked to wait in their cars until an examination room is available. Another option to address vaccine refusal in clinical settings is to require that parents sign an informed declination form. These forms outline the rationale for vaccination, risks associated with nonvaccination, adverse events associated with various vaccines, and misconceptions regarding vaccines. Two good templates of such forms have been developed by the AmericanAcademyofPediatrics(AAP) and the Immunization Action Coalition.11,12

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There are no studies on the prevalence of such forms in pediatric practices in the United States. In summary, the overriding concern is what is in the best interests of the child and that interest is in continuing a relationship with the family with the hope over time to convince them to accept vaccination of their child. Discharging the family can compromise the health of the child by funneling the child to a provider who does not believe strongly in vaccines. Moreover, efforts of pediatricians to counsel families need to be reimbursed because discussions require physician time. Insurers should reimburse for these efforts. Response of Dr Kristen Feemster When parents refuse some or all of the recommended vaccines for their young child, the pediatrician must balance 3 things: (1) the relationship they have established with the child’s family, (2) a desire to respect the family’s preferences and beliefs, and (3) their desire to provide the best care possible to protect the health of the child. There are only 2 possible responses: to work with the family to encourage immunization or to request the family chose another pediatrician due to this conflict. The parents believe they are making the best decision for their child. You believe that they are refusing one of the most important elements of pediatric care. The AAP recently reaffirmed their recommendations for responding to parents who refuse immunization for their children. The policy statement concludes that “In general, pediatricians should endeavor not to discharge patients from their practices solely because a parent refuses to immunize a child.” Instead, pediatricians are encouraged to maintain the relationship in order to provide a platform for continued communication that could potentially result in a change in belief. I disagree. PEDIATRICS Volume 133, Number 3, March 2014

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Counseling parents is certainly appropriate. They should know that pertussis is actively circulating in many communities and that young infants are at highest risk of severe disease. Sharing this information with real-time data and personal stories could potentially be effective. This approach may help emphasize that the choice to forego vaccination is not between a vaccine and no vaccine, it is between a vaccine and being susceptible to a vaccine-preventable disease. Parents need to know that when children don’t get immunized, outbreaks of vaccine-preventable disease follow. This situation has occurred with measles, mumps, varicella, and pertussis in communities throughout the United States and Europe. Parents should also be informed that vaccines are one of the safest interventions recommended for children. Their approval requires more safety data than any other medication. Millions of vaccine doses are safely administered each year. If a vaccine-associated adverse event does occur, it is most commonly mild; serious adverse events are extremely rare, especially compared with the likelihood of a severe outcome from a vaccine-preventable disease like pertussis. No vaccine or medication is 100% risk free, nor is a decision to leave a child unvaccinated. If their decision does not change after this information is communicated, I would argue that the pediatrician is compelled to act as an advocate for the infant and the otherchildren in his or her practice. I would discharge this family from my practice. Routine immunization according to the Advisory Committee on Immunization Practices is a standard of pediatric care that offers significant direct benefit to both individual children and the community. As pediatricians, we are obligated to provide the standard of care and protect patients under our care based upon the central tenet of medical practice, “First do no harm.” By not

vaccinating this patient, we are playing a role in putting the infant at risk. As such, a pediatrician is justified in telling a family that he or she cannot with good conscience accept the parents’ decision to leave the child unvaccinated. A pediatrician also has a responsibility to the other children in his or her practice who would be at risk of exposure if this unvaccinated infant develops a vaccinepreventable disease. To maintain the profound public health impact we have seen from vaccines, immunizations cannot be optional. Accommodating refusals can send a message that vaccines are not really necessary. To confront the strong messages that question vaccine safety and efficacy and result in misperceptions, we must have equally strong messages in support of vaccination. It is important that the pediatrician listen to and acknowledge parents’ beliefs. One of the key contributors to rising vaccine hesitancy is an eroding trust in medicine and public health. If communication from pediatricians is to remain effective, we must do what we can to maintain trust. We cannot address concerns without knowing what they are. But when education and strong recommendations have not worked, action speaks louder than words. Response of Dr Monica Richter First, I would advise the parents that it is their decision whether to vaccinate their child. My role is to educate and make recommendations. Then I would ask them if they are open to discussing their decision. If not, then I recommend that they visit reputable Web sites such as those of the AAP or Centers for Disease Control and Prevention for more information. I will also tell them that I feel vaccines are one of the most important medical services that I provide and I will continue to revisit the subject at each visit. Vaccines are safe and effective, and there is no scientific

evidence that they cause autism. If they have any information to the contrary, I would like to review it with them. Then they are asked to read and sign the refusal-to-vaccinate form available from the AAP (revised September 2012). I should note that every nurse in my office reviews the vaccine records and discusses recommended vaccines with each family at each visit, well or sick, before I enter the room. This procedure emphasizes the importance of vaccines, and I know when families are opposed to vaccines as soon as I enter the room. If the parents are open to discussion, I would then ask them to tell me what they know about the immune system. From there it is easy to explain how the immune system gets stronger after each infection and how vaccines boost the immune system without the risk of disease. I would mention that vaccines are better than medications such as antibiotics or antiviral agents because the benefits of medication only last for as long as you take them, whereas the immunity from vaccines is long-lasting.

Furthermore, prevention of disease is always preferable to treating disease.

JOHN D. LANTOS COMMENTS

When parents continue to refuse immunizations, it is often because of cultural reasons (ie, some say, “people in my country do not vaccinate”) or because they know of someone who was reportedly harmed by a vaccine. I do not refuse treatment to these patients but continue to discuss the issue at each subsequent visit and with each subsequent child.

Parental vaccine hesitancy always exists in a semistable equilibrium with outbreaks of infectious disease.13 When children were dying of H1N1 influenza, and vaccine was in short supply, parents camped out on the steps of public health clinics to get vaccine for their children.14 When disease is common and deadly, parents want their children to be immunized. As a result, the prevalence of disease goes down. As the prevalence of disease goes down, more people refuse immunizations for their children. They are gambling. The odds are in their favor. At first, most of the kids are fine because herd immunity protects them. As herd immunity wanes, new outbreaks occur. Children die. As a result, more parents again choose to immunize their children. This dynamic has existed since the early days of vaccination.15 The dynamic will only change as a result of continued efforts at education by pediatricians, policy makers, and parents who have lost the gamble.

infections: results of a prospective multicenter surveillance study. Pediatrics. 1997; 100(6). Available at: www.pediatrics.org/ cgi/content/full/100/6/E10 Sugerman DE, Barskey AE, Delea MG, et al. Measles outbreak in a highly vaccinated population, San Diego, 2008: role of the intentionally undervaccinated. Pediatrics. 2010;125(4):747–755 Diekema DS. Choices should have consequences: failure to vaccinate, harm to others, and civil liability. 107 Mich Rev Law First Impressions 90 (2009) McDougall RJ, Notini L. Overriding parents’ medical decisions for their children: a systematic review of normative literature [published online ahead of print 2013]. J Med Ethics. doi:10.1136/medethics-2013-101446 Salmon DA, Pan WK, Omer SB, et al. Vaccine knowledge and practices of primary care providers of exempt vs. vaccinated children. Hum Vaccin. 2008;4(4):286–291

11. American Academy of Pediatrics. Documenting parental refusal to have their children vaccinated. Immunization. 2012. Available at: http:// www2.aap.org/immunization/pediatricians/pdf/ RefusaltoVaccinate.pdf. Accessed July 13, 2013 12. Immunization Action Coalition. Decision to not vaccinate my child. 2011. Available at: www.immunize.org/catg.d/p4059.pdf. Accessed July 13, 2013 13. Ofri D. The emotional epidemiology of H1N1 influenza vaccination. N Engl J Med. 2009; 361(27):2594–2595 14. Fletcher L, Kahn H. H1N1 Vaccine shortages create panic, worry. ABC News, October 26, 2009. Available at: http://abcnews.go.com/GMA/ OnCall/swine-flu-h1n1-flu-vaccine-shortage-panic/ story?id=8915650. Accessed August 14, 2013 15. Cherry JD, Harriman KH. Why do vaccinepreventable disease outbreaks occur in the United States. Available at: www.idse.net/ download/VPD_IDSE12_WM.pdf. Accessed August 14, 2013

I would closewithstoriesofchildrenwho have died unnecessarily because they were unvaccinated and emphasize the danger of pertussis in children under 1 year. The recent statistics are helpful. In addition, I would add that adults and adolescents often have milder forms of the disease and can expose their child unknowingly. My final statement is about personal experience with meningitis and how we saw several children with meningitis each year before the introduction of the Haemophilus influenzae type b vaccine. The parents usually agree to vaccinate or ask for Web sites with more information.

REFERENCES 1. Goldstein J. Medical care for the child at risk: on state supervention of parental autonomy. Yale Law J. 1977;86(4):645–670 2. Diekema DS. Parental refusals of medical treatment: the harm principle as threshold for state intervention. Theor Med Bioeth. 2004;25(4):243–264 3. Winter K, Harriman K, Zipprich J, et al. California pertussis epidemic, 2010. J Pediatr. 2012;161(6):1091–1096 4. Washington State Department of Health. Preliminary 2012 pertussis summary for Washington State. 2013. Available at: http:// doh.wa.gov/Portals/1/Documents/Pubs/348253-PertussisAnnualSummary.pdf. Accessed June 3, 2013 5. Ciofi degli Atti ML, Olin P. Severe adverse events in the Italian and Stockholm I pertussis vaccine clinical trials. Dev Biol Stand. 1997;89:77–81 6. Heininger U, Klich K, Stehr K, Cherry JD. Clinical findings in Bordetella pertussis

7.

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Should Pediatric Practices Have Policies to Not Care for Children With Vaccine-Hesitant Parents? Kenneth Alexander, MD, PhD,a,b Tomas A. Lacy, MD,c Angela L. Myers, MD, MPH,d John D. Lantos, MDe

One of the most divisive issues in pediatrics today concerns the proper response by pediatricians to parents who refuse routine childhood immunizations for their children. Many pediatricians refuse to care for such families. Others continue to provide care and continue to try to convince parents that the benefits of immunizations far outweigh the risks. Two of the most powerful arguments in favor of dismissing such parents are as follows: (1) their refusal suggests such lack of trust in the physicians’ recommendations that it undermines the basis for a meaningful physician– patient–parent relationship; and (2) unimmunized children present an unacceptable risk to other children in the physicians’ waiting rooms. This article examines those arguments. One of the most divisive issues in pediatrics today concerns the proper response by pediatricians to parents who refuse routine childhood immunizations for their children. Many pediatricians refuse to care for such families. Others continue to provide care and continue to try to convince parents that the benefits of immunizations far outweigh the risks. In this Ethics Rounds, we present a case of a pediatric practice trying to decide what their policies should be regarding immunizations. We asked experts in pediatric infectious disease to offer their advice.

THE CASE A group of pediatricians is trying to decide what their policy should be with regard to parents who refuse immunizations for their children. Some physicians in the group think that it is important to continue to care for the children and to keep trying to convince the parents to immunize their children. They cite

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the policy of the American Academy of Pediatrics (AAP),1 which states, “In general, pediatricians should avoid discharging patients from their practices solely because a parent refuses to immunize his or her child.” Other physicians in the group cite another line from that same policy, “When a substantial level of distrust develops, significant differences in the philosophy of care emerge, or poor quality of communication persists, the pediatrician may encourage the family to find another physician or practice.” The group calls the hospital ethicist to ask what course of action is best and why.

Angela Myers comments:

Physicians are obligated by oath to “do no harm.”2 Although this obligation sometimes conflicts with other obligations (eg, when we prescribe chemotherapy for a patient with cancer), it is the spirit of this oath that we carry with us every day. In addition, physicians are expected to

abstract

aDivision

of Infectious Diseases, Department of Pediatrics, Nemours Children’s Hospital, Orlando, Florida; bDepartment of Pediatrics, University of Central Florida College of Medicine, Orlando, Florida; cNemours Children’s Primary Care, Orlando, Florida; dDivision of Infectious Diseases, and eBioethics Center, Children’s Mercy Hospital and Clinics, Kansas City, Missouri

Drs Myers, Alexander, Lacy, and Lantos contributed to the design of the article, the drafting of the manuscript, and the review of the manuscript; and all authors approved the final version of the manuscript as submitted. DOI: 10.1542/peds.2016-1597 Accepted for publication May 12, 2016 Address correspondence to John D. Lantos, MD, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Copyright © 2016 by the American Academy of Pediatrics FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. FUNDING: No external funding. POTENTIAL CONFLICT OF INTEREST: Dr Alexander is a paid speaker and consultant for Merck Vaccines; the other authors have indicated they have no potential conflicts of interest to disclose.

To cite: Alexander K, Lacy TA, Myers AL, et al. Should Pediatric Practices Have Policies to Not Care for Children With Vaccine-Hesitant Parents?. Pediatrics. 2016;138(4):e20161597

provide beneficence to patients while minimizing maleficence.3

In the pediatric setting, immunization is second only to hand-washing in terms of “doing good” by promoting the health and well-being of children.4–7 However, this idea falls flat when a parent refuses to follow our recommendation to immunize their children. Of course, we recommend many things, and parents often do not follow our recommendations. Generally, we continue to care for their children and encourage them to lead healthier lives. I think immunizations are different. Let me explain why. The difference is in the implications that vaccine refusal has on other children. If we tell people to wear their seat belts and bicycle helmets, and they do not do so, they are only hurting themselves. If, as a result of not following our advice, something terrible happens, such as being thrown from a vehicle during a crash or experiencing an intracranial bleed from hitting their head on a curb, it happens only to that person. There is no risk to others conferred by their reckless decision.

The lack of risk to others is not the case with immunizations. When a nonimmunized child gets measles or an infection with Haemophilus influenzae type b, they have exposed other children to these life-threatening diseases. The exposure begins in the days before the illness even began, when they were completely asymptomatic. Secondary cases can be seen in closed communities of nonimmunized and underimmunized populations8 but can also be seen in larger populations.9,10 We recently cared for 2 children with H influenzae type b disease from a nonimmunized community. Substantial morbidity was seen in both cases, and 1 of the children died. This outcome led to a communitywide immunization effort through

the county health department.11 Similar patterns occurred, and on a much larger scale, in 2015. A large measles outbreak occurred in southern California, attributed to families with nonimmunized children visiting Disneyland. Sixteen percent of cases occurred in infants who were too young to be immunized. An additional 12% of cases occurred in children between the ages of 1 and 4 years, who would have had only 1 dose of measles-containing vaccine.7 Transmission of vaccine-preventable infections could occur in the community office setting in which a nonimmunized child with a communicable disease inadvertently transmits their infection to a young infant or a child who is immunosuppressed. This eventuality is even more likely when one considers the rate at which families are traveling internationally. In 2014 alone, >68 million people traveled from the United States to an international destination.12 A significant portion of these trips are to places in which immunization is much less common and therefore the risk of contracting a communicable disease is considerably higher. As in the Disneyland outbreak, the index case is often suspected or proven to be a nonimmunized individual who contracts the disease while traveling overseas and then becomes ill after arrival back home.7 Thus, the risk of exposure to a communicable disease in the waiting room of the physician’s office, while low, is not zero.

The presence of such risks creates a difficult dilemma for primary care pediatricians. All children deserve safe, high-quality medical care. Pediatricians, overall, are committed to the care of all children. However, that commitment to safe, high-quality care means that pediatricians have an obligation to make their offices and clinics as safe as they can possibly be. No child should be exposed to a potentially life-threatening

communicable disease in the physician’s office.

The 2012 policy statement from the AAP1 recognized the tension between pediatricians’ general obligations to care for children and their specific obligations to protect children from risks. The statement begins, “In general, pediatricians should endeavor not to discharge patients from their practices solely because a parent refuses to immunize a child.” I agree with this statement. In my view, pediatricians should make sincere and strenuous efforts to answer parents’ questions and address their concerns about immunizations.13 They should provide the evidence in a nonjudgmental way to convince families of the benefits of immunization. They should acknowledge the reality of a low, but not zero, risk of adverse reactions to immunization.14 In addition, pediatricians should consider a focused attempt to gain trust by providing the potentially most important of the recommended vaccines (eg, pneumococcal conjugate) per the recommended schedule, while allowing for delays of others (eg, polio) if it means that the child will eventually be fully immunized.

I am not advocating for an alternative immunization schedule. However, the AAP policy states that “As respect, communication, and information build over time in a professional relationship, parents may be willing to reconsider previous vaccine refusals.”15 Allowing some compromises up front may increase trust, and therefore willingness to immunize, later. Indeed, some of the strategies described here and detailed elsewhere have, in fact, been shown to increase immunization rates.16,17 However, pediatricians can and should only go so far. Not all families are open to discussion of this important issue. Some categorically

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refuse all preventive measures from birth. Families who refuse vitamin K, erythromycin ointment, and the initial hepatitis B vaccine are more likely to continue to refuse all vaccines in the future.18 The pediatrician has a perfect opportunity in the first few days of life to convince a parent that these preventive measures are important and, in rare instances, lifesaving. If the pediatrician is unable to gain trust during this initial period, what hope do they have of developing a respected partnership with a family? Partnership implies shared decision-making and a give-and-take relationship. This bond cannot exist when one party refuses to consider recommendations made by the other. In situations in which it is clear that further discussion will not be fruitful, it is appropriate for the pediatrician to refer the family to a different health care provider, one who shares their values and with whom they can establish a trusting and nonconflictual relationship.18

Pediatricians do not want to dismiss any patients or families from their practice. The AAP understands this objective and endorses it. However, the AAP policy also appropriately recognizes that there are uncommon circumstances in which the pediatrician and family are at an impasse, and the only logical conclusion is to sever the relationship. The primary reason why it is sometimes appropriate to sever the pediatrician-family relationship is that the child may get higher quality care from a pediatrician who shares the parents' values. A pediatrician whose concerns about transmission of infectious disease in the waiting room leads to anxiety about other patients and frustration with parents and may not be able to provide quality care.

Kenneth Alexander and Thomas Lacy Comment: We are all concerned that the presence of unvaccinated children

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in our offices poses a small but real risk of transmitting vaccinepreventable diseases to our other patients, especially to those who are immunocompromised, those who cannot be immunized, and those who, by no fault or choice of their own, face the prospect of significant morbidity or mortality should they acquire a vaccinepreventable disease. We often believe that nonvaccinating parents, by not immunizing their own children, knowingly (albeit unintentionally) place our vulnerable patients at risk. Given that nonimmunizing parents knowingly place other peoples’ children at risk, it is understandable that many pediatric practices view nonimmunization as a breach of both physician–family trust and patient safety that merits a severance of the relationship with the family. In practices who choose to exclude unvaccinated families, parents are generally given a period of time to get their child's immunizations up to date, after which time they are advised to seek alternative care. Such deadlines often lead families to get their children immunized. Unfortunately, some families (no one knows how many) simply disappear from the practice to an unknown quality of care. Thus, although exclusion may be a successful means of encouraging nonimmunizing parents to accept vaccination for their children, and may also increase the safety of our vulnerable patients by reducing their exposure to nonimmunized children, at least in the office waiting room, what is the cost of this action to excluded children? Countering the argument that nonvaccinated families should be dismissed from practices is the belief that children are still best cared for by pediatricians, even children in families who refuse to immunize. This argument is powerful. We believe that an nonimmunized child will fare better in the care

of a pediatrician (who, even if a family refuses to immunize, will still advocate on behalf of the child, recommending bicycle helmets, bathwater safety, healthy diet, and safe storage of firearms) than in the care of a less qualified practitioner (who may recommend disproven or unproven and potentially dangerous remedies, such as homeopathy or naturopathy). By continuing to care for the child, the pediatrician can continue promoting immunization and will continue providing other recommended health and safety guidance and intervention.

When addressing the question of exclusion of families for failing to immunize their children, our ethicist colleagues and the AAP provide us with mixed messages.1 On the one hand, they advocate nonabandonment. On the other hand, they permit physicians to choose whom to serve. When debating the matter of excluding nonimmunizing families from pediatric practices, the principles of beneficence and nonmaleficence are often cited. Unfortunately, applying these principles is challenging and gives us opposing answers. On the one hand, pediatricians can justify excluding nonimmunizing families from their practices because pediatricians believe that nonimmunized children place other patients at risk, especially in the medical office. By excluding nonimmunizing families, pediatricians believe that they are protecting their most vulnerable patients. Thus, excluding nonimmunized children from a practice can be viewed as form of beneficence toward the vulnerable, protecting them from harm. On the flip side, excluding a nonvaccinated child from a practice could be viewed as detrimental to that child's health, provided we believe that pediatricians do good beyond simply immunizing children. From this vantage, excluding nonvaccinating families from our

practices denies children the benefits of our nonimmunization-related health promotion efforts. From this view, exclusion of nonimmunizing families from our practices can be viewed as a maleficent action. Furthermore, we doubt that exclusion of nonvaccinating families would have the desired effect of protecting our vulnerable children. Insofar as it is unlikely that a family excluded from a practice for refusing to immunize their children is unlikely to seek immunization elsewhere, exclusion itself probably does nothing to decrease the proportion of nonimmunized children. Because excluded parents are not likely to seek vaccination elsewhere, thus leaving excluded children unvaccinated, exclusion may well maintain the possibility that our at-risk patients will be exposed to vaccine-preventable diseases out in the world, if not in our office waiting rooms; another maleficent act. Thus, ethical analysis based on beneficence and nonmaleficence points us in 2 different directions; excluding unimmunized children from practice is a beneficent act performed for the benefit of the vulnerable, but excluding children is a maleficent act toward the nonimmunized child because we are denying them the benefits of our nonvaccinationrelated care, potentially with the added cost of continued nonimmunization. Applying the principles of beneficence and nonmaleficence to the question of excluding nonimmunizing families from our practices leaves us in an ethical fog of opposing conclusions. Maybe we need a tool beyond beneficence and nonmaleficence.

Perhaps the ethical principle we should use to address the exclusion question is that of double effect.19 The principle of double effect asserts that when an intended positive action carries with it a smaller and unintended negative effect, the action

is still positive overall. The principle of double effect requires that:

• The intended effect is positive • The positive effect is intended

• The negative effect is not intended as a means to the positive effect, nor as an end in itself • The positive effect outweighs the negative effect • Continual effort is made to minimize the negative effect

Applying the principle of double effect to the question of excluding nonimmunizing families from our practices, I am confident that good pediatric primary care brings to children and their families frequent substantial benefits that extend beyond vaccination. Good primary care is a definite and substantial positive, even without immunization. To assess the degree of unintended negative effect (the risk that unimmunized children pose to vulnerable children in our waiting rooms), we must keep in mind that the rates of vaccine-preventable diseases (with the exceptions of human papilloma virus infection and influenza) are low compared with the rates of nonimmunization.) For example, in 2014 in California, there were 9 157 390 people aged