Cystic Fibrosis and You [Second edition] 9780773574021

Citation preview

CF and You A Guide for Adolescents 2nd Bdítíon

Y.G. gorne1Ø, Ν.Β. ΜACDOnR1d, 1. Cappellí, J. ΜcGrath S C.B. Heíck

IIlυstr~ted by gyla MacDonald

CARLETON UNIVERSITY PRESS

O Yolanda Korneluk and Carleton University Press, 1996 Published by Carleton University Press for the Children's Hospital of Eastern Ontario ISBN 0-88629-309-X Printed and bound in Canada by Tri-Co Printing, Ottawa

Canadian Cataloguing in Publication Data Main entry under title: CF and you: a Guide for adolescents 2nd ed. ISBN 0-88626-309-X 1 Cystic fibrosis. I. Korneluk, Y. G. (Yolanda Gloria). 616.3'7 C96-900873-2 RC858.C95C24 1996 Cover design: Your Aunt Nellie

CARLETON UNIVERSITY PRESS Q~ldη's FOwκωαοπ la randaöon de πi8pítal Øe-fa-ts

This project was supported by the Children's Hospital of Eastern Ontario

CONTENTS PREFACE

víí

ACKNOWLEDGEMENTS

VIII

Part One: PHYSICAL ASPECTS OF CYSTIC FIBROSIS INTRODUCTION

What is CF? How is CF diagnosed? How did I get CF? What causes CF? Is there any way to find out who is a carrier of the CF gene?

3 3 3 4 4 6

THERAPIES FOR THE RESPIRATORY SYSTEM

7

Introduction

7

Physiotherapy

What does physiotherapy involve? What is school physiotherapy? What happens if I stop doing physiotherapy? Exercise

Can I exercise instead of doing physiotherapy? Is there anything I should know or do before I start an exercise program?

8 8 9 10 10 10 10

Supplemental (extra) oxygen

11

Antibiotics

11 11 12

Do I need to take antibiotics? How are antibiotics taken? Anti-inflammatory agents

12

Drugs to "thin" lung mucus

13

Say NO to cough medicines

13

Organ transplants

13

THERAPIES FOR THE DIGESTIVE SYSTEM

14

Introduction

14

Enzyme replacement When should I take the enzymes? Why is the dosage of enzymes different from person to person? What happens if I don't take the enzymes and I need them? What happens if I take too many enzymes? What if I feel uncomfortable taking enzymes in public?

15 15

Movement of food through the large intestine (GI motility)

17

CF and weight control What sort of diet will help me gain weight? I think I'm overweight. Should I go on a weight-loss diet? What are jejunostomy and gastronomy tubes? Will having a jejunostomy or gastronomy tube interfere with any of my activities? What are the pros and cons of having a jejunostomy or gastronomy tube?

17 17 18 18

NEW THERAPIES

20

Other complications associated with CF Intestinal obstruction Diabetes Liver disease Heat prostration (Heat collapse) Finger clubbing Barrel chest Pneumothorax Hemoptysis Cor pulmonale Heartburn

21 21 21 21 22 22 22 23 23 23 23

Hospitalization and CF

24

When does the doctor hospitalize someone with CF?

24

15 16 16 16

18 19

Infection control and cepacia

24

Common tests

25

What has research discovered about CF?

27

Part Two: PERSONAL ASPECTS OF CF INTRODUCTION

31

Relationships Should I talk about CF? Who should I tell? When and how should I tell? Boyfriends/Girlfriends Relatives Impact of CF on parents

31 31 31 32 32 33 33

School Should my teachers be told about CF? Who should tell them? What should I say? l'm afraid of getting behind in my school work if l'm hospitalized l'm nervous about going to high school. Is this normal? Can I participate in sports?

34 34 34 34 34 35 35

Social activities Are cigarettes and marijuana harmful? Will alcohol affect someone with CF differently? Will my doctor tell my parents if I ask him/her about things like drugs and alcohol? Can someone with CF travel? Can someone with CF get a driver's licence? How do I explain my cough?

36 36 37 37 37 38 38

Self image

38

Sexuality and reproduction Males Females

39 39 40

Birth control and sexually transmitted diseases Sexual desire and deciding when to have sex Should someone with CF have a baby?

41 41 42

Independence

42

Health

43 43 44 45 45 45 45

Sometimes I find it hard to deal with the people at the CF clinic Transfer to adult care Why should I go to the adult CF clinic? When will I transfer to the adult CF clinic? Health deterioration Death and dying Part-time jobs and careers

Is there anything special I should consider when planning a career or taking a part-time job? Do I have to mention CF at a job interview? What should I tell an employer? Is there anyone who can help me with course choices or career plans?

46 46 47 47 47

CONCLUSION

48

GLOSSARY

49

PREFACE This booklet was designed to answer questions commonly asked by adolescents with Cystic Fibrosis (CF). The first section deals with the physical aspects of CF. Making yourself knowledgeable about CF will increase your self-reliance and help you look after yourself. The second section deals with aspects of CF that might affect you personally. Some general strategies for coping with frequently encountered situations are provided. Ιn addition, a glossary which contains the meanings of words and phrases in bold face type can be found at the back of the handbook. This booklet is only a general guide. Since CF affects everyone differently, this booklet will not answer all of your questions. Speak to your doctor or someone else on staff at the CF clinic for individual questions or concerns.

ACKNOWLEDGEMENTS Though five names appear on the title page, the authors readily acknowledge that this book is the result of the concern, insight, time and effort of many. First of all, we would like to thank Dr. John Flood at Carleton University Press for agreeing to take this project on and especially for assigning us the tireless Pauline Mckillop as project editor. Ms Mckillop's patience, precision, editorial skills and sense of humour made the production of the second edition of this book a pleasant and rewarding experience. We thank the Press's designer Barbara Cumming for the successful layout of the pages—the result of her talent and many skipped lunches. We would also like to thank the following health care providers at the Children's Hospital of Eastern Ontario for their input and commitment to this project: Anne Smith, Murielle Dagenais, Deanna Stoski, Marie Shinmoto, Monica Brown, Deb Clement, John Goodman and Anne Schlieper. In addition, we would like to thank Organon Canada and the Children's Hospital Foundation for their financial support. Most importantly, we would like to thank the following adolescents for their willingness to lend their expertise on being a teen with CF as well as for their courage which continues to be an inspiration to us all: Lisa, Christine, Kyle, Michael, Jessica, Justin, Adrienne, Max and James.

Part One PHYSICAL ASPECTS OF CYSTIC FIBROSIS

Physical Aspects of Cystic Fibrosis

INTRODUCTION What is Cystic Fibrosis? Cystic Fibrosis (CF) is an inherited condition affecting approximately 1 in every 2000 children born in Canada. The effects of CF primarily involve the function of the exocrine glands. The exocrine glands are glands that secrete their products outside the body and on the surface of internal organs via ducts. Tears, mucus, sweat, and digestive juices are all examples of substances secreted by exocrine glands. CF causes these glands to produce abnormally thick and sticky secretions. This causes problems in the respiratory and digestive systems. The extent to which each system is involved varies from person to person. Thus, the symptoms experienced by each individual will also vary. You may have heard of endocrine glands. Endocrine glands secrete hormones that are responsible for growth, sexual maturation, etc. CF does not affect these glands directly, but malnutrition may affect growth and `rate of sexual maturation.

How is CF diagnosed? The sweat test is the standard test used to confirm the diagnosis of CF. A sweat test measures the amount of salt in a person's sweat. People with CF have much more salt in their sweat than people without CF. When an infant or child shows symptoms of CF, such as frequent lung infections, failure to grow, or intestinal and digestive problems, the doctor will order a sweat test to find out if the child has CF. The sweat test cannot be used to predict how severely a person will be affected by CF. More recently, DNA (deoxyribonucleic acid) testing has been used to determine whether a child has CF. A positive test means that the patient has two altered copies of the CF gene.

3

4

Fan. 'ou How did 1 get CF? Everyone inherits traits or characteristics from both of their parents. Hair and eye colour are examples of inherited traits. Traits are transmitted by genes. Genes are tiny structures within the cells of the body that are made up of DNA. Genes provide the blueprints that determine the structure and function of cells in the body. Genes are always present in pairs, one inherited from the mother and the other from the father. CF occurs when a child inherits two copies of the CF gene that are altered or mutated. People who inherit one or two altered CF genes are known as carriers of the CF gene. It is estimated that 1 in every 20-25 people is a carrier of the CF gene. A person who has one altered version of the CF gene paired with a normal version/copy does not develop the disease or show any symptoms. In order to inherit CF, a person must inherit two altered CF genes. This can happen only if both parents are carriers. Each time two carriers of one altered gene (1(400 marriages) conceive, there is a 25% chance that two CF genes will be passed on to their child, a 50% chance that their child will not have the disease but will be a carrier, and a 25% chance that the child will neither be a carrier nor have the disease. People with CF are also carriers of two altered versions of the CF gene, and they have a 100% chance of passing an altered version of the CF gene onto their children.

What causes CF? In 1989, Canadian and American scientists working together discovered the gene responsible for the development of symptoms of CF. The gene is on chromosome 7 and codes for a protein called CFTR (Cystic Fibrosis Transmembrane Regulator). The defective CFTR is responsible for the abnormally thick and sticky secretions produced by the exocrine glands. The discovery of the CF gene has improved our understanding of CF, which has in turn improved our treatment of the disease. Eventually, it is hoped that CF will be controlled and damage to the lungs and other organs prevented.

Physical Aspects of Cystic Fibrosis

THE INHERITANCE PATTERN OF CF carrier parents

ί25%

25%

50%

chance unaffected

chance unaffected carrier

50% chance affected

chance affected

Fig. la When both parents are carriers

non-carrier parent

CF- parent

carrier parent

CF-parent

ϊί

50% chance unaffected carrier

Fig. lb When one parent has CF and the other is a carrier

membrane spanning domain

chloride channel cell membrane

~ιι ~ιι Q,~ιc e~c 100% chance unaffected carrier Fig. lc When one parent has CF and the other is nota carrier

regulatory domain

nucleotide binding domains

Fig. 2 Cystic Fibrosis Transmembrane Regulator

5

Cf • ~ `•~ is there any way to find out who is a CF carrier?

People with CF are carriers, as are both of their parents. The discovery of the CF gene has made carrier testing more reliable. Genetic tests can identify the more common mutations of the CF gene, but there are more than 400 known abnormal variations of this gene. Because of this, a negative DNA test result for CF means only that the person does not have any of the common abnormalities of the CF gene. It is possible that the person may be a carrier of a rare version of the CF gene that is not identified by the test for the common variations of the gene. The CF gene test can be done on DNA extracted from blood cells, or it can be done on cells that are brushed from the inside of the cheek using a cotton swab. The sweat test cannot be used to find out whether someone is a carrier of the altered CF gene.

Physical Aspects of Cystic Fibrosis

THERAPIES FOR THE RESPIRATORY SYSTEM Introduction

In order to live, each cell of the body needs to take in oxygen and get rid of carbon dioxide. This process is called respiration. Respiration begins when air is taken into the nose and mouth and then passed through the trachea into the lungs. Once in the lungs, the air enters a network of increasingly smaller tubes, the bronchi and the bronchioles, which end in millions of small air sacs called alveoli. Oxygen moves across the alveolar wall in order to be taken up by the body. Air moves in and out of the lungs due to the contraction and relaxation of the diaphragm and chest wall, which are controlled by the nervous system.

Fig. 3 The respiratory system The lungs and nasal cavities are lined with cells that secrete mucus. The mucus humidifies the air and collects germs and dust particles, which are then easily removed by coughing, sneezing, and swallowing. This helps to keep the lungs and nasal passages clear. In CF, however, the mucus is very thick and glue-like and not easily expelled, so that it has a tendency to remain in the lungs and block the breathing passages. Germs collect and multiply in the lungs, especially in areas where the thickened mucus is stuck. The CFTR abnormality in CF also makes germs stick to the walls of the bronchioles. The body reacts to

7

CF and You bronchial obstruction

inflammajion

infection

t

bronchial wall damage ---bronchhectasis permanent lung danage lung failure Fig. 4 The process of lung damage in patients with CF this infection by having cells come in to try to kill the germs (inflammation). Unfortunately, the infection usually persists, and the inflammation reaction steadily increases. The inflammation and infection produce r scarring of the lung tissue. This damage further reduces the body's ability to clear the Iungs and leads to more severe lung damage and eventually to lung failure. The main goals in caring for the respiratory system areto clear the lungs of mucus and to control respiratory infections. Airway clearance techniques (ACT), antibiotics, and anti-inflammatory agents are the main methods used to control respiratory problems and decrease the rate of damage to the lungs. Physiotherapy

What does physiotherapy involve? Physiotherapy may involve a variety of methods used to clear mucus from the breathing passages (ACTS). The traditional method of physiotherapy used in treating CF is postural drainage, vibration, & percussion, which are together known as PDVP. This method relies on percussion to dislodge mucus from the breathing passages, postural drainage to take advantage of gravity to move mucus upwards, and vibration to help move mucus from smaller to

Physical Aspects of Cystic Fibrosis

larger airways so that it can be coughed up. The lungs are divided into five sections called lobes. Three of the lobes are located in the right lung, and two are in the left lung. Each lobe is further divided into several segments. Different postural drainage positions are used to drain different segments of each lobe. In addition to PDVP, you may be asked by your physiotherapist or doctor to try other ACTs. The active cycle of breathing techniques (or ACBTs) involve different types of huffs, usually done in postural drainage positions, to move mucus up in the lungs. Autogenic drainage (AD) is a method of controlled breathing done in a sitting position. The positive expiratory pressure (PEP) mask involves breathing Fig. 5 The lobes of the lung out through a small opening. This is also done in a sitting position. The advantage of ACBTs, AD, and the PEP mask is that, once they are learned, you don't need anybody to help you with them, nor do you need to carry a lot of bulky equipment around with you. This is a great advantage when you are away from home. These techniques also make you more aware of what is happening in your lungs and can provide you with important feedback on changes in your condition. What is school physiotherapy?

Some adolescents need physiotherapy at school. When this is necessary, physiotherapy can be done in a private place during the least disruptive time. School physiotherapy allows adolescents who need it to take part more fully in school activities. If you think you need school physiotherapy, speak to your physiotherapist, clinic nurse, or doctor.

9

What happens if I stop doing physiotherapy? If you stop doing physio, mucus builds up in the lungs, providing an ideal breeding ground for germs. This can eventually lead to serious infections, inflammation, scarring of the lung tissue, and a decrease in the effectiveness of your lungs. Initially, the build-up of mucus in the bronchioles does not produce any symptoms, and you might not know that lung damage is occurring. In some cases, more than half of the lung is destroyed before symptoms, such as not being able to run as far or as fast as before, are noticed. E xercise Can I exercise instead of doing physiotherapy? Exercise will help to loosen mucus from the lungs. Some activities, such as swimming, jogging, basketball and cycling, will also help build the muscles used in breathing. Exercise alone, however, is not enough to clear all the mucus from the lungs, especially from the lower lobes. The upside-down position used in postural drainage is more effective than exercise for clearing the mucus from the lower lobes. For this reason, it is recommended that you continue physiotherapy along with an exercise program. Is there anything / should know or do before i start an exercise program? It is recommended that everyone check with their doctor before beginning an exercise program. You may find the following tips useful: • Choose an activity you really like, otherwise you won't stick to your exercise schedule. • Start your exercise program slowly, and don't do too much the first time out. Slowly build up the amount of time that you spend at your activity. • Get a friend or family member to join you. You'll be able to motivate each other.

Physical Aspects of Cystic Fibrosis • Keep at it. It will probably take a month before you begin to notice any changes or see any results. • You will probably have to eat more to make up for the energy you are using while exercising. Speak to the nutritionist or someone else at the CF clinic about making changes in your diet before you begin your exercise program. Supplemental (extra) oxygen

With severe CF, the ability of the lungs to deliver oxygen to the blood may be so impaired that it restricts the persons' activities. In these cases, extra oxygen delivered by mask or nasal tubes allows the person to be more active. At home, oxygen is usually supplied by a machine which takes oxygen from the room air and concentrates it. Outside the home, a portable liquid oxygen tank is more practical. Oxygen therapy is used only when the level in the blood is low. The need for supplemental oxygen can by tested by oximetry (or 02Sat). Antibiotics

Do i need to take antibiotics? People with CF are prone to catch infections caused by two types of bacteria (germs): staphylococcus (or Staphylococcus aureus) and pseudomons (or Pseudomons aeruginosa). The abnormal CFTR makes these germs stick better to the cells lining the tubes of the lungs. Thus, in contrast to people without CF. CF patients often have bacteria in their lungs. By taking a throat swab or sputum sample, the doctor can determine which kind of bacteria are present and then determine the best type of antibiotic therapy. The function of antibiotics is to decrease the number of bacteria and to prevent them from growing and increasing lung damage. Although bacteria are present in the lungs of people with CF from a very young age, antibiotics are usually reserved for occasional flare-ups. Some people, however, get a lot of respiratory infections and may need to be on antibiotics all or most of the time.

11

12

CF and You

How are antibiotics taken? Antibiotics can be taken orally (e.g., in pills or liquids) or intravenously, or they can be inhaled. Antibiotics given intravenously can be more effective than oral ones because they are taken directly into the bloodstream and do not have to be absorbed through the small intestine. Antibiotics given intravenously reach the site of infection more quickly and in higher concentration. Some antibiotics are available only in an intravenous form. If you require intravenous antibiotics, you may need to be hospitalized for a short time, although you may also have an intravenous catheter, which allows you to take these antibiotics at home. Ask your CF clinic nurse or doctor if this is a possibility for you. Inhaled antibiotics (or aerosols) can also be effective because they are taken directly into the lungs, where the bacteria are. Only a small number of antibiotics are available in aerosol form. Aerosols are mists made from solutions of medication. A special mask and compressor are needed to produce an antibiotic mist that is fine enough to get down into the lungs. Aerosol antibiotics may be taken along with intravenous antibiotics. Your doctor or pharmacist will tell you how best to use antibiotics. Remember that you should never start or stop taking your medication without checking with your doctor or clinic nurse. If you need to get a prescription from a doctor who is not on your CF team, you should make sure that she or he knows that you have CF, and you should ask whether the medication is suitable for people with CF. If you have any doubts, check with your CF doctor or pharmacist. Anti-inflammatory agents Anti-inflammatory agents help to combat the natural but negative effects of the body's response to germs in the lungs. Inflammation of the lung tissue is the body's way of helping to fight germs in the lungs, but it can also cause lung damage such as fibrosis or scarring. Drugs such as ibuprofen and inhaled steroids can decrease inflammation and slow the damage due to infection and inflammation. New anti-inflammatory agents are being developed.

Physical Aspects of Cystic Fibrosis

Drugs to "thin" lung mucus Some people with CF find that inhalation of a drug called DNAse helps to thin the mucus in the lungs, which makes it easier to cough up secretions. In addition, inhaling special solutions of salt water (saline) may also help some CF patients to cough up secretions more easily. Say NO to cough medicines! Cough medicines and/or cough suppressants should never be taken by someone with CF, because coughing is a natural way of helping to clear the lungs of excess mucus and germs. Similarly, most decongestants are not recommended because they dry up secretions, which may make the secretions in people with CF thicker and even harder to cough up. It is a good idea to check with your CF doctor or pharmacist before taking any over-the-counter medication. Organ transplants Lung transplants and heart—lung transplants are done in patients with severe, end-stage CF. Donor (or transplanted) lungs do not create the thick and sticky secretions that are characteristic of the lungs of people with CF The success rate of these operations is quite high, but not all CF patients are suitable for transplant, and there is a shortage of donors.

13

14

CF and You

THERAPIES FOR THE DIGESTIVE SYSTEM Introduction

Digestion is the process whereby food is broken down into particles small enough to pass through the intestinal cells, where they are taken up by the bloodstream and then delivered to individual cells in the body. Normally, when food is eaten, it activates oesophagus the pancreas and causes it to secrete digestive enzymes into the small intestine. Once the food reaches the small intestine these enzymes liver stomach begin to break down the food into particles small enough to be absorbed and used by the cells of the body. In 85% of patients with CF, the secretions gall bladder that carry the digestive enzymes are so thick pancreas and sticky that they block the ducts of the pancreas. This prevents the digestive enzymes appendix from reaching the small intestine. As a result, large intestine a large amount of undigested food remains in small intestine the small intestine, moves into the large intesrectum tine, and is later expelled in the form of large, Fig. 6 The digestive system smelly stools. Since food is not as well absorbed in patients with CF, the body lacks the proper amounts of essential nutrients and energy needed for normal growth and maintenance of weight. Inadequate nutrition can result in failure to grow, and adolescents with CF are sometimes smaller and thinner than their classmates. Poor nutrition can also result in delayed sexual maturation and can cause lung damage to progress more rapidly. Enzyme replacement (see below) can minimize these problems by helping the body to maintain normal growth and nutrition. Maintaining your growth and nutrition will result in less lung damage and will allow you to have Μ r. and Mr`s. N. ΖΥΜΕ a more normal quality of life.

Physical Aspects of Cystic Fibrosis Enzyme Replacement

Enzymes help to break down food into particles small enough to be absorbed by the cells in the body. The enzymes you take are made from extracts of beef or pork pancreas. They perform the same role as enzymes produced by your own pancreas. When should /take the enzymes?

In normal digestion, the enzymes are already present when the food reaches the small intestine. Therefore, it is best to take the enzymes before you eat so that they are present at the same time as the food. Sometimes a meal is spread out over a long period of time (e.g., at restaurants you may have to wait a long time between courses). In this case, it may be best to take some enzymes when you start eating and some more during the meal. Enzymes are also needed when you have a snack. Why is the dosage of enzymes different from person to person?

The dosage of enzymes needed by each person depends on the degree of pancreatic function (the amount of digestive enzymes naturally secreted by the pancreas). This varies from person to person. In fact, 15% of people with CF don't need any enzyme replacement at all. The dosage of enzymes will also vary according to the amount of food eaten. You will need to learn to judge the number of enzyme capsules that you need for snacks, as well as for larger meals.

15

16

CF and You

What happens if i don't take the enzymes and 1 need them? Initially, you may experience bloating, cramps, diarrhoea, and/or large smelly stools. Eventually, you will lose weight and become malnourished. You may also experience intestinal obstruction, which is very painful and can be dangerous if not treated quickly.

What happens if 1 take too many enzymes? Too many enzymes taken for a long period of time (i.e., months) can cause damage to the walls of the large intestine, which makes it stiff. This can lead to permanent obstruction of the large intestine, which requires surgery. The CF clinic staff can help you determine the right amount of enzymes to take. Sometimes the addition of other drugs such as ranitidine, which change the acidity of the stomach, can make the enzymes function more effectively so that fewer are needed.

What if 1 feel uncomfortable taking enzymes in public? Some people prefer not to take their enzymes in public. Sometimes this is because they have been teased about being a "druggie." If this happens, it is best to laugh at the joke and then explain the real reason why you take the capsules. You may prefer to take your medication in the washroom or in some other private place before you begin your meal. If you decide to take your enzymes in public, you may need to explain to others why you are taking them. If you are having problems explaining this, you might say something like: "1 have a medical condition that causes some digestive problems. Without these pills, 1 can't digest my food properly." OR: "These pills are for a medical condition that 1 have. Don't worry; it's not catching!"

Physical Aspects of Cystic Fibrosis

17

In addition to your regular dosage of enzymes, your clinic doctor or nurse may advise you to take multi-vitamins every day. This will provide you with the fat-soluble vitamins that your body needs but which may not be completely absorbed from the food you have eaten. Movement of food through the large intestine (GI motility)

Sometimes the movement of food through the bowel is too slow, and you may experience crampy pain and problems having a bowel movement. Gastrointestinal (GI) motility drugs are used to relieve this problem. Cisapride will help improve the muscle action of the bowel and move food faster. Acetylcysteine (Mucοmysϊrm) is also used to help break up mucus in the bowel and prevent plugging or blocks in the intestine. CF and weight control What sort of diet will help me gain weight?

The best type of diet for gaining weight is a diet high in fats, calories, and proteins. Fats should not be avoided, as they are a good source of calories. Proteins are found in foods such as meat, chicken, fish, eggs, and peanut butter. The dietician at your clinic can help you learn how to plan a well-balanced diet and give you some recipes for high-fat and high-calorie meals and snacks. If eating a high-fat, high-calorie diet is not working, you may require enteral feeding. These may be given through a tube placed in the nose and threaded into the stomach (a naysogsstric tube). Supplemental nourishment may also be given through a tube surgically placed directly through the abdominal wall and into the stomach (a gastronomy tube) or directly into the small bowel (a jejunostomy tube, see below). During a respiratory infection, more energy is expended because breathing is more difficult. You may also find that you are less hungry than usual. As a result, you may lose weight at these times. Early treatment of these infections can help to minimize weight loss.

4

18

CF ρηά Υoει

1 think I'm overweight. Should 1 go on a weight-loss diet? For the past several years, it has been fashionable to be extremely thin. As a result, many people of normal weight, especially young girls, think of themselves as overweight. Dieting in order to achieve this extreme thinness is dangerous for everyone. For someone with CF, it is especially harmful. Maintaining an optimum weight will help you fight off infections and stay healthy. Your CF doctor or CF clinic nurse will help you determine what your optimum weight should be. What are jejunostomy and gastronomy tubes? Jejunostomy and gastronomy tubes are used when someone has had trouble maintaining his or her weight despite a high-fat, high-calorie diet and other nutritional supplements. The procedure for both tubes involves surgically implanting a small tube into the stomach (gastronomy tube) or the small intestine (jejunostomy tube). Supplemental feedings are then taken through this tube while you sleep at night in order nit to interfere with everyday activities. The tube is secured to the body by a piece of tape. Will havmg a jejunostomy or gastronomy tube interfere with any of my activities? No. The tube that is used is quite small (about the size of a piece of spaghetti). It should pose no problem for participating in day-to-day activities as well as most sports (including swimming). The feedings may actually allow you to participate in more activities because you will have more energy once your weight is in the normal range. Wearing a T-shirt is a good way to cover your tube while swimming.

Physical Aspects of Cystic Fibrosis

What are the pros and cons of having a jejunostomy or gastronomy tube? There are both advantages and disadvantages to having a jejunostomy or gastronomy tube. On the pro side, the extra feedings taken through the tube will probably make you feel better and allow you to participate more fully in activities. It is likely that you will also experience increased growth and weight gain. In addition, keeping yourself at your optimal weight helps in maintaining lung function. Also, if you have a chest infection and don't feel like eating, you can still get much-needed calories into your body. On the con side, having the tube means having an operation. It also requires a fair bit of work on your part, mixing and hooking up the feedings. U addition, you may also have some reservations about how the tube will affect your day-to-day life and how others will react to the tube. Most people who have had a jejunostomy or gastronomy tube are pleased with the results.

19

20

CF and You

NEW THERAPIES In addition to new drugs, developments are ongoing in the area of gene therapy. Gene therapy involves the placement of normal genes into tissue that is affected by the disease, such as the pancreas or the lungs. For example, researchers are experimenting with ways to inhale healthy versions of the CF gene into the lungs inside special transport molecules called liposomes or inside genetically engineered viruses. The goal is to stimulate the development of normal cells in order to prevent the effects of CF. Research using gene therapy with mice and rats has been encouraging, but it is still in an early stage. Should gene therapy become a reality in the future, it is important to understand that it cannot reverse damage that has already been done to your body. In the meantime, you must continue to take good care of yourself.

Physical Aspects of Cystic Fibrosis OTHER COMPLICATIONS ASSOCIATED WITH CF Intestinal obstruction The digestive tract is lined with millions of tiny mucus-secreting glands. The mucus lubricates the digestive tract and allows food and wastes to move smoothly. If too many of these glands become blocked with sticky mucus, wastes are no longer able to move smoothly and, as a consequence, tend to form a hard plug. This condition is known as intestinal obstruction. Symptoms include severe cramps and nausea. Intestinal obstruction can be treated with medicine usually given by nasogastric tube, which breaks up the obstructing material. It can also be treated with enemas, which are liquid injections into the large intestine through the rectum. The best way to avoid intestinal obstruction is to take your enzymes and the other medications that your doctor has prescribed. Diabetes Approximately 10% of people with CF develop diabetes, because the endocrine cells in the pancreas are destroyed by the fibrosis of CF. The most common time for diabetes to develop is during adulthood. It can sometimes be controlled by oral medication, but insulin may be necessary. Liver Disease Approximately 10% of people with CF develop fibrosis of the liver, leading to cirrhosis of the liver. This usually occurs in adulthood, and in most peopie it does not progress. In a small number of people, the cirrhosis can become quite severe. Research has shown that ursodeoxycholic acid (UDCA) may help to slow down the rate of damage in some patients with liver disease. Liver transplants have been successful in helping some people who have had severe liver disease.

21

22

CF and You

Heat prostration (heat collapse)

You are particularly prone to heat prostration during very hot weather or when engaging in vigorous exercise. The best way to avoid heat prostration is to drink lots of fluids, including water, juice, and sports drinks at frequent intervals during exercise. Because people with CF lose a lot of salt in their sweat, you may not feel thirsty even when you are becoming dehydrated. During sports, have your own large bottle of water and make sure you drink all of it by the end of the game. It is recommended that you drink about one glass (8 oz) of liquid every half hour when you are outdoors in the summer. You should also increase your salt intake at these times by eating salty foods or adding salt to your snacks and meals. Be on the look-out for symptoms such as dizziness, blurred vision, nausea, and headache, as these precede heat prostration. Finger clubbing

Finger clubbing occurs when the ends of the fingers (underneath the nails) become enlarged. The reason for the occurrence of this phenomenon is not known, but it is related to the presence of chronic lung infection. By itself, finger clubbing does not pose a problem, but it is an indication that damage due to lung infection has occurred. Barrel chest

This condition occurs when the lung becomes over-inflated because air is trapped in the small airways. This causes the chest to remain expanded and, as a consequence, it appears more rounded and barrel-like. By itself, having a barrel chest does not pose a problem, although it is a reflection of more serious lung damage.

Physical Aspects of Cystic Fibrosis

Pneumothorax This occurs when air leaks out of the lung due to a hole in the lung tissue. The symptoms of pneumothorax are shortness of breath and sudden sharp chest pains, and it usually occurs only during a chest infection. If you experience these symptoms, you should contact your doctor immediately or go to a hospital emergency department.

Hemoptysis (blood-streaked sputum) The presence of a small amount of blood in the sputum (heamoptysis or hemoptysis) is fairly common in people with CF who have lung damage. There is usually little need to worry. If you are concerned about the amount of blood in your sputum, contact your doctor or clinic nurse.

Cor pulmonale Cor pulmonale is when the right side of the heart becomes thicker and larger than normal. It is caused by extensive lung damage, which reduces gas exchange in the lungs. Less oxygen gets to the blood, and the muscles of the right side of the heart must pump harder to push the blood through the damaged lungs, so this muscle becomes thicker. This occurs only in CF patients with severe lung disease.

Heartburn If you have a burning or painful feeling in your chest or upper abdomen, this may be from inflammation of the stomach or oώsορhagus (or esophagus). Some people don't feel a lot of pain, but experience nausea and a lack of appetite. Heartburn occurs when stomach acid gets into the oesophagus and damages it. This can lead to bleeding or narrowing of the cesophagus. If you have heartburn, you should speak to your CF doctor. Medications such as ranitidine, cimetidine, or omeprazole may be prescribed to prevent pain and damage.

23

24

CF and You

HOSPITALIZATION AND CF When does the doctor hospitalize someone with CF?

You should always feel free to ask your doctor why you are being hospitalized, but here are some common reasons for hospitalization: • Serious infection preventing participation in day-to-day activities. • Other problems, such as weight loss or a decrease in lung function, which might indicate a change in health. • Intestinal obstruction. The length of hospitalization varies depending upon the nature of the problem and your rate of recovery. For lung problems, you may be hospitalized for a week or two, although problems like intestinal obstruction usually only require a day or two of hospitalization. Depending on your condition and the reason for which you are hospitalized, the doctor may allow you to leave the hospital for a few hours at a time during your hospital stay. Sometimes your course of therapy can be completed at home. Infection control and cepacia

Cepacia (or Burkholderia cepacia) is a germ that CF patients can easily acquire, most often from being in close contact with another CF patient who already has it. For this reason, there are no longer summer camps for CF patients, and, when CF patients are hospitalized, they do not share rooms or equipment. CF patients who live together (e.g., brothers and sisters) usually share the same germs. Good hand washing and taking care of respiratory equipment are helpful in decreasing the spread of germs.

Physical Aspects of Cystic Fibrosis

25

COMMON TESTS The tests you are given allow the CF team to determine your condition and decide on the best course of treatment for you. Weight and height: The percentiles of weight for height are calculated to determine how close your growth and body weight are to normal values. This helps to determine your nutritional status. Skin folds: The percentage of body fat can be calculated using the thickness of the skin folds. This information is used in assessing nutritional status. CT (CAT) scan (of the lungs): A series of special X-rays that look at cross sections of the lungs in small slices (i.e., a bit at a time) from the top to the bottom. Pulmonary function tests: Detect changes in the lungs, such as narrowing of the breathing tubes. They are done both with and without bronchodilators (e.g., Vent°linΤm). Pulmonary function tests determine how well your lungs are working compared with those of a person without CF. Oximetry (or O2 Sat): Detects the amount of oxygen in the blood. This is done using a sensor that records the amount of oxygen through your finger, toe, or ear. This is painless and also provides information on how well your lungs are working. X-Rays: Allow the lungs to be seen directly in order to assess their condition. X-rays are also used to diagnose pneumothorax. Sometimes X-rays of the abdomen are also done if intestinal obstruction is suspected. Ultrasound: Similar to an X-ray, but it does not involve any radiation and is often used to look at the abdomen if intestinal blockage or liver disease is suspected.

~ ι

26

CF and You Urine test: A screening test that measures glucose (sugar) in the urine and determines whether you have or are prone to developing diabetes. Blood Gases: This test measures the amount of carbon dioxide and oxygen in the blood. The test can be done by taking either a sample of blood from the finger οr•frοm an artery in the wrist. The finger method provides only a good estimate of blood gas levels. Although the wrist method is more uncomfortable than the finger method, it gives the best test of blood gas levels. Since it is uncomfortable, the doctor will order it only if very accurate results are needed. When this test is done, your wrist is frozen to lessen discomfort. Blood samples: These tests measure: • The blood's capacity to carry oxygen to the tissues and to remove carbon dioxide from the body. • The body's ability to fight off infections—the two tests used to determine this are: (a) sedimentation (sed) rate: an indirect measure of the amount of inflammation (infection) in the body. (b) white count: measures the number of cells in the blood that are able to fight off infection. • The blood glucose (sugar) levels, which determine whether you have diabetes. • Liver and pancreatic function. • Nutritional status. • Antibiotic levels: with antibiotics (e.g., intravenous tolbramycin), it is important to measure the level of the antibiotic in your blood to determine that there is enough but not too much of the antibiotic present in your system.

Physical Aspects of Cystic Fibrosis

WHAT HAS RESEARCH DISCOVERED ABOUT CF? When CF was first described in 1938, it was thought to affect only the pancreas. Babies born with CF usually died before they were a year old. Since then, research has greatly increased what is known about CF. During the 1940s and 1950s, it was found that CF is an inherited disease affecting not only the pancreas but also the lungs and the sweat glands. The sweat test was designed to aid in the diagnosis of CF. Pancreatic enzymes began to be ‚"90 used routinely, and babies survived into early childhood. In the 1960s, it was found that 15% of people with CF have normal '¶60 pancreatic function, individuals with CF have reproductive problems, and liver 197° disease affects a small percentage of people with CF. During the 1970s, the factors responsible for the faulty clearance of 'qόc the lungs were discovered and described. Drugs to control pseudomonas infection and better pancreatic enzymes were 1950 formulated. The benefits of exercise in helping breathing were recognized, and new tests were designed to monitor lung function. In addition, new airway clearance techniques, which allowed greater independence of people with CF, were developed. In the 1980s, nutritional status was recognized as a major factor in the survival of CF patients. In 1989, Canadian and American researchers discovered the gene responsible for the development of CF. This made it possible to identify many carriers of the CF gene. Animal models (experiments using genetically modified animals that have diseases similar to CF) are being used to better understand how CF affects the cells of the body. In the 1990s, research involving animal models and gene therapy are ongoing. Organ transplants are also being done more often and with greater success. Researchers continue to develop and test more effective antibiotics and newer methods for antibiotic delivery and use. The search for vaccines to prevent lung infection is also under way. New ACTs are being explored. In addition, reproductive therapies to increase the fertility of males with CF who wish to have children are also being developed.

27

28

CF and You

The life span of people with CF is steadily increasing, thanks to the ongoing efforts of patients, researchers, and health care professionals in developing new treatments and therapies. The vast majority of children born with CF now live to be adults instead of dying in their teens. With hard work and a little good luck, some CF patients have almost normal lung functioning as they reach adulthood. Improved nutrition, physiotherapy, and antibiotics have all contributed to this success story.

Part Two PERSONAL ASPECTS OF CYSTIC FIBROSIS

Personal Aspects of Cystic Fibrosis

INTRODUCTION Let's be honest, CF is a serious disease. You will experience problems because of the time needed for therapy, hospitalizations, clinic visits, and medications. Sometimes you will feel quite different from other people your age. BUT people with CF are normal. They have friends. They have enemies. They go to school. They work. They go out. They have good times. They have bad times. They have disagreements with their parents. They share good times with their families...

RELATIONSHIPS Should 1 talk about CF? Some people are reluctant to discuss their illness, while others feel comfortable talking about CF. Many young people are afraid that telling others means that they will be treated differently. It is important to realize that some of your friends may already knοω that you have health problems, even if you have never discussed your health with them. You may find that discussing CF will help your friends to understand you better. The vast majority of your friends will be supportive. Α small number may not knοω how to deal with it at first. Remember that this is not a reflection on you but on their own ability to handle a new situation. Who should! te!!?

There is no need to explain CF to everyone. You will probably find it helpful to let your close friends knοω. This will help them understand certain things about you, such as the time you spend doing your physiotherapy, taking pills, eating a lot of snacks, and being in the hospital, as well as any limitations on your physical activities.

31

32

CF and You

When and how should I tell? The time and manner of telling will vary from person to person. The best time to disclose this information is when you feel comfortable that your friendship is well established. It is probably best not to wait too long to discuss CF. It is also possible that a friend may ask if you have a health problem. Should this happen, answer your friend honestly. You should be straightforward in your explanation. If you are having difficulties, you might begin with: "1 have an inherited disease called CF. Ιt can cause some problems with the lungs and the digestive system. You can't catch it." You could continue by explaining the treatments and the reasons for doing them. Be prepared to answer some questions, since most people know very little about CF. If you emphasize that CF is not the most important thing in your life, your friends will be less likely to make an issue of it. You will find that telling others about CF will become easier as you tell more people and you develop your own way of telling them.

Boyfriends/girlfriends It is not necessary to discuss CF when dating someone casually. You should consider telling your girlfriend/boyfriend when you sense that your relationship may be long term. Discussing CF with a boyfriend or girlfriend will become more important as you become more committed to a relationship. You should approach the topic as you would with a good friend. It is possible that your partner may find it difficult to cope with your illness, perhaps because he or she is afraid of losing you one day. On the other hand, your partner may also be supportive and understanding about your illness, and you may become closer.

Personal Aspects of Cystic Fibrosis

33

Relatives In some families, CF is discussed openly and easily. Occasionally, parents are not willing to acknowledge to their relatives that their child has CF. If you find that this is a problem for you in your family, you should discuss it with your parents. You might want to ask your parents why they are reluctant to let others in the family know that you have CF. You should also discuss how this makes you feel. If you are anxious about this, a nurse or social worker at the CF clinic may be able to help you figure out the best strategies for discussing CF with your parents and relatives. Impact of CF on parents Having a child with a chronic illness places more responsibility and demands on parents and their families. Most parents can cope and do cope quite well. Occasionally, parents and families break up. When this happens, children and adolescents often feel responsible for the ° difficulties that their parents are having. It is important to realize that you are not responsible for your parents' difficulties and that o many families who do not have a child with a chronic illness also break up. When family problems arise, it is important to have people outside the family on whom you can rely for support. Having a close friend to talk to can help you get through rough times. If you feel that you need additional support, however, speak to someone you trust at the CF clinic. That person may be able to help you directly or may be able to help you find someone who can. On the other hand, many families report that a problem like CF has brought them closer together. These families work together and fight CF as a team. They learn about one another's strengths and weaknesses. They celebrate the successes and support one another when there are setbacks.

~ ι

34

CF and You

SCHOOL Should my teachers be told about CF?

M. D.ΤENs'οN

yes. It is a good idea for your teachers to knοω about CF. This will help them understand if you have a frequent cough and if you must be absent from school a lot. Covering up hospitalizations and appointments is not a good idea. Your teachers will think that you do not take your school work seriously and, as a consequence, will give you a hard time. Teachers will be more helpful if you are honest with them about your absences from school.

Who should tell them? Some people find it easier to tell their teachers on their own, while others prefer to discuss this with the help of a parent, a CF social worker, or a CF nurse. Remember that the clinic staff is always available to help you deal with problems at school. What should I say?

You should describe what CF is, its effects, as well as its treatment. If there is a possibility that you may be hospitalized during the school year, you should — discuss this with your teachers. It may also be helpful to make an -', arrangement whereby you can leave the room without the teacher's permission, when you need to cough or go to the washroom. I'm afraid of getting behind in my school work if I'm hospitalized

Most people find hospitalizations stressful. One of the reasons for this is the fear that they will get behind in their work or at school. It is a good idea to warn your teacher if you knοω in advance that you will be hospitalized. This will give your teacher extra time to organize your work. You may also

Personal Aspects of Cystic Fibrosis

35

want to let your teachers know your routine while in the hospital. This will help them decide how much work you can realistically accomplish during your stay there. If you are in high school, you may have as many as eight different teachers. You may also find that your work load is heavier than it was in elementary school. Your guidance counsellor may be able to help you if you are having problems getting all your courses organized. I'm nervous about going to high school, Is this normal?

The transition from elementary school to high school is stressful for many adolescents both with and without CF. In elementary school, you probably knew almost everyone in your school, whereas at the beginning of high school, you may knοω only a few people in each of your classes. The greater number of students in high school means there is more competition in athletics and academics. There is also usually more peer pressure to contend with. All these changes will take time to become accustomed to. For an adolescent with CF, there may be additional changes to deal with. In elementary school, you were probably in a class where ///' ni everyone knew you had CF. When you begin high school, it is likely that very few people will knοω that you have CF. This may make you feel uncomfortable about taking your pills in the cafeteria and having physiotherapy at school. It is not unusual to feel uneasy and self-conscious in these situations. These situations will become easier as you develop a new circle of friends with whom you feel comfortable. High schools usually offer a wide variety of activities. Joining in on activities that you enjoy will make it easier to meet people who share your interests.

1

Can I participate in sports?

Sports are an important part of school life for many adolescents. Many children and adolescents with CF are active and are able to compete with others their age. Due to a decline in lung function, however, some adolescents may

11\\~

®

36

CF and You find it harder to keep up with their peers. These changes may be difficult to accept, but it is important not to blame yourself. If you are finding it difficult to keep up with your peers, there are several ways to cope with the situation. Some adolescents prefer to play sports in a less competitive league (intramurals) or to remain in competitive sports but play for shorter shifts. Others prefer to be active in individual sports such as swimming, running, cycling, skating, or skiing, where the emphasis is on competing with yourself. Still others stay involved with their own team in another capacity, such as assistant coach, trainer, or manager.

SOCIAL ACTIVITIES Participating in social activities at school or with friends is important. Some of these activities have a tendency to finish quite late. You may find staying up late quite tiring. It is often helpful to rest or sleep during the day if you plan to stay out late. If you are hospitalized during a time when a special social activity has been planned, discuss this with your doctor. It may be possible for you to arrange to leave the hospital in order to attend the event.

O Are cigarettes and marijuana harmful? There is often pressure on young adults to try smoking cigarettes or marijuana (pot). Research has clearly shown that cigarette and marijuana smoking are harmful for everyone. For someone with CF, smoke and other fumes are particularly harmful. They irritate delicate membranes in the lungs and can cause more rapid lung damage. Your friends will understand if you decide not to smoke, especially if you explain to them the serious negative effects of smoke on the lungs of people with CF. Also remember that second-hand smoke (i.e., being around others who are smoking) also damages the lungs.

Personal Aspects of Cystic Fibrosis

Will alcohol affect someone with CF differently?

37

*

Alcohol may be absorbed differently in people with CF and may change the effectiveness of the medication you are taking. Ask your doctor or pharmacist whether any of the medications you are taking have side effects when ,combined with alcohol. Will my doctor tell my parents if 1 ask him/her about things like drugs and alcohol? The CF clinic doctors know that keeping private information confidential is important to teens, just as it is important to adults. When you discuss very personal things with your doctor, you should make it clear that you would prefer the conversation to remain confidential. There are certain circumstances, however, under which your doctor must discuss your private conversations with other people. Find out from your CF doctor what kind of information cannot remain confidential. Can someone with CF travel?

O

r.

Many schools and groups organize trips for adolescents. It is possible for someone with CF to go on these trips. If you are planning a trip, you should discuss it with your doctor at the CF clinic. The doctor will advise you on the medications that you will need to bring with you and on the health care routine you should follow while you are away. Being able to do ACBTs on your own can make you more independent with your physiotherapy program while travelling.

38

CF and You

Can someone with CF get a driver's licence? Yes. There is no reason why a person with CF should not learn how to drive.

How do I explain my cough? Sometimes it may seem necessary to explain your cough if it is particularly persistent. If you are having trouble explaining it, you may want to say something like this: "This cough 1 have is due to a chronic lung problem (or medical condition). You can't catch it." You should not feel obliged to mention that you have CF unless you feel comfortable doing so. Sometimes doing physiotherapy just before you go out can clear mucus from your lungs and lessen your cough while in public.

SELF-IMAGE It is common for adolescents both with and without CF to be dissatisfied about some aspect of their appearance. Adolescents who are smaller and thinner than others due to poor nutrition may feel self-conscious about their appearance. These adolescents may also experience delays in sexual maturation. Girls may have delayed breast development and onset of menstrual periods. For boys, delayed growth and development of pubic and facial hair may occur. The delays tend to be more pronounced in people who have repeated lung infections and poor nutritional status throughout adolescence. As a result, some adolescents become self-conscious of their bodies. It is important to remember that all teenagers mature at a different rate. Some people mature in their early teens, while others do not mature until they are almost twenty. Adolescents with CF, although they may be delayed in developing, usually fall into the normal range of development. In addition, adolescents with CF may feel self-conscious about having a barrel chest or clubbed fingers.

Personal Aspects of Cystic Fibrosis Although many things about one's appearance cannot be changed, developing your own sense of style will help you become more confident about the way you look. Ask someone whose style you admire where they get their hair cut or where they shop for clothes. You may find it useful to have a look at some fashion magazines or to take a course at a department store or community centre in order to learn how to highlight your best features. It may also be useful to discuss your feelings about your appearance with someone at the CF clinic.

SEXUALITY AND REPRODUCTION As you get older, you may find that sexuality becomes more of a concern. Many of these concerns are shared by others. For the adolescent with CF, there are some special points to consider.

Males Cystic Fibrosis does not affect the level of sex hormones or the development of the penis and testes directly. However, approximately 95% of men with CF cannot father children. This is due to the blockage of the vas deferens. The vas deferens is the tube that carries the sperm from the testes to the penis. Although semen is produced and intercourse can take place normally, conception usually cannot occur because the semen contains no sperm. The blockage of the vas deferens is believed to occur before birth. A test can be done to determine whether sperm are present in the semen. For adult men with CF who are infertile but wish to have children, there are other Fig. 7 The male reproductive system options. Aside from adoption or foster care, a

39

40

CF and You

man with CF may want to try microscopic epididymal sperm aspiration (MESA). MESA involves removing mature sperm from the epididymis (in the scrotum) and combining the sperm with a partner's egg in a laboratory dish. The fertilized egg is then implanted into the female partner, a process called in vitro fertilization. A man with CF who is thinking of starting a family should be aware that there is an increased risk of his child having CF.

Females There are no abnormalities in the reproductive organs of females with CF. In fact, more and more women with CF are choosing to have babies. It is believed, however, that women with CF may be somewhat less fertile than other women. There are two reasons for this: • The cervical secretions of women with CF are likely to be stickier than normal, making it more difficult for the sperm to reach the eggs for fertilization. • Women with CF may not ovulate regularly. Irregular ovulation has a tendency to happen during bouts of respiratory infection. If you are sexually active and do not want to be pregnant, you should definitely be using some form of birth control. A woman with CF has an increased risk of giving birth to a child with CF. Furthermore, there are increased health risks for the woman herself. Many questions concerning sexuality are personal. They are best dealt with on an individual basis. If you have any questions, do not hesitate to speak to someone you feel Fig. 8 The female reproductive system comfortable with at your CF clinic.

Personal Aspects of Cystic Fibrosis

Birth control and sexually transmitted diseases

Some researchers have found that teenagers with CF have less knowledge about reproductive health issues and sexually transmitted diseases (STDs) than teenagers without CF. If you plan to become sexually active, it is important to take responsibility for learning about these issues, including how they relate to CF. For females, the most effective method of contraception is the birth control pill, which can be prescribed by your doctor. The pill will not affect lung functioning. It is also okay to take the pill with enzymes. Some antibiotics can reduce the effectiveness of the pill, however, and your doctor should be told if you are taking oral contraceptives and need antibiotics. Even though most males with CF are infertile, unless you know for certain that this is the case, you should still use birth control. Latex condoms are an effective means of birth control and are the only way of preventing the spread of STDs such as chlamydia, syphilis, and AIDS if you are having sexual intercourse. Even if you and your partner use another form of contraception, you should still use condoms. Sexual desire and deciding when to have sex

Even though CF affects some aspects of sexual functioning, you probably already know that it does not affect your sexual desire! If you are thinking about having sex, there are a few things that you should know. Deciding on the right time to have sex is difficult for all teenagers. Some people feel a lot of pressure to have sex before they're ready. Nevertheless, you need to decide when it feels right for you. Part of growing up and becoming an adult is learning to be responsible for your sexual feelings and making sure that you do what's right for you and your partner. There is, unfortunately, no magic formula to determine whether or not you're ready to have sex. If, however, you're not sure if you're ready, you should wait until you feel more sure. If you and your partner are both ready to have sex, then you should also be ready to discuss it openly and honestly with each other, including issues such as the prevention of STDs, birth control, and how CF affects reproduction.

41

42

CF and You

Should someone with CF have a baby? As a teenager, you are probably not ready to have a baby. Nevertheless, you may have questions about whether or not an adult with CF should have children. When deciding whether or not to have children, there are several things to keep in mind. First, if you have CF, there is an increased risk of your children having CF. Second, the demands of your illness (e.g., daily physiotherapy, possible hospitalizations, etc.) may make parenting even more difficult than it usually is. Third, even though the life span of people with CF is steadily increasing, you will probably not live long enough to see your children grow up. Despite these barriers, however, parenthood also brings many rewards. When you are ready to think about these issues, you and your partner will have to decide together whether having children is right for you.

INDEPENDENCE An important part of growing up is becoming independent of your parents and family. It is normal for you to want to develop some distance between yourself and your parents. You may also find that you are starting to rely more on your friends for emotional support. Young people with CF sometimes find becoming independent more challenging than do some of their friends. While wanting to be more independent, you may still feel dependent on your family for your treatments and for support. It ís also important to realize that your parents may find it difficult to let you do things on your own because, until now, you have needed their help for many things. They may need some time to get accustomed to your growing independence. It is not uncommon for feelings of tension to arise between any adolescent and his/her family during this period. These tensions usually subside with time. Since good health care is vital to young people with CF, taking responsibility for your own health care offers an excellent way to gain independence. You might want to begin by preparing your own medications and doing some physiotherapy on your own. Eventually, you may want to begin making your

Personal Aspects of Cystic Fibrosis

own appointments at the CF clinic and going to these appointments on your own. If you feel unwell between scheduled visits to the CF clinic, you can call the clinic yourself to discuss your condition and decide whether you should see the doctor earlier. No one knows how you feel better than you do. Some adolescents with CF go through a period where they stop going to the clinic, taking medications, and doing treatments. Eventually, many of these adolescents become ill and return to treatment with the expectation that the physical damage can be reversed. Unfortunately, many times the damage cannot be reversed. Pretending that you don't have CF by not going to the clinic or not taking medications does not stop the effects of CF. The only way to slow the effects of CF is by taking care of your health. In addition, if you are having problems getting your parents to accept that you are responsible and independent, it is important that you show them that you are able to take care of your own health. HEALTH

Sometimes 1 find it hard to deal with the people at the CF clinic Some adolescents have difficulty dealing with the staff at the CF clinic. Often, adolescents feel that the staff repeats the same things over and over and nags them about doing physiotherapy, exercising, and eating more. Although you may not want to hear these things, it is important to 0,V remember that this is done only because the staff wants your health to be the best it can be. ΝΑG The key to having good relationships with the people at the CF clinic is good communication. It is not uncommon for adolescents, at one time or another, to feel that other people don't understand them. If you often forget the issues that you want to discuss during your appointments, it is helpful to make a list and bring it with you to your appointment. If you find it easier to express yourself in writing, you may want to write a letter to the clinic staff. Remember that no one can read your mind. The caregivers at the clinic can help you only if you let them know about the things that concern you. If you don't understand

43

~

•

44

CF and You

something, ask that person to explain it again until you do understand. If you feel a mistake has been made, don't hesitate to bring it up. The staff can and does make mistakes. Sometimes adolescents have difficulty with one particular member of the CF team. You can continue to have a successful relationship with the CF team without liking everyone. If you are having difficulties with a particular team member, it is sometimes helpful to discuss this with someone else on the team. If you have a problem with a particular member of the clinic staff but do not want to talk to someone else on the staff, speak to a trusted adult such as a parent or counsellor. They may be able to help you.

Transfer to adult care It is normal to feel worried about transferring to the adult clinic. You have probably been going to the same ρ udiatric (or pediatric) CF clinic for a long time and have become attached to the staff there. Remember that the staff at the clinic has become attached to you as well. Moving to the adult CF clinic doesn't mean you have to cut off all ties with them. You can still visit the staff at the pmdiatric clinic and keep them up to date on your progress. When you transfer to the adult CF clinic, your treatment regimen may also change. This is because the problems seen in adults with CF often differ from those seen in adolescents. Researchers have found that CF patients who feel that the regimen they followed at the pmdiatric clinic worked well sometimes do not follow their new adult treatment plan. If you are unhappy with your new treatment plan or with some other aspect of the adult CF clinic, you should talk it over with the staff at your adult CF clinic. Taking initiative with your treatment regimen will help you to successfully transfer to adult care and is an important part of becoming independent.

Personal Aspects of Cystic Fibrosis

Why should I go to the adult CF clinic? As more people with CF reach adulthood, more adult clinics are being planned in order to meet the needs of these individuals. There are many reasons in favour of adults being treated at the adult CF clinics. First, as an adult, you will face physical and social problems different from those of adolescents and children. These issues can best be dealt with by a doctor and staff who have been specifically trained in the treatment of adults with CF. Second, the transition is a normal part of the growing up process for everyone; adults with and without health problems do not usually visit doctors or clinics that specialize in dealing with children and adolescents. When will (transfer to the adult CF clinic? Preparation for the transfer to the adult clinic begins in early adolescence. At that time, the clinic staff emphasizes independence with respect to health care. The age of transfer varies from person to person, and transfer is usually done before the patient is 18 years of age. Health deterioration Deterioration in health is stressful for anyone. People with CF who have experienced serious health deterioration have emphasized the benefits of having goals to get through these difficult periods. Having things to look forward to, such as a friend's birthday, a trip, a party, or getting a driver's licence, will help keep your spirits up. It is important to try to focus on positive activities, thoughts, and feelings. Death and dying Many of us have questions and fears about death. You may have known someone at the adult or pædiatric CF clinic who has died. This may be difficult to accept, and it may increase your own fears. These feelings are normal. The

45

CF and You

46

clinic staff is sensitive to these issues and is there to help you deal with these problems and questions as they come up. If you have any concerns, feel free to approach the staff at the CF clinic. Some people find it helpful to speak to a priest, minister, rabbi, or other advisor.

PART-TIME JOBS AND CAREERS Working for a living may seem a long way off at the moment. But, like everyone else, it is important that you begin to think about what you would like to do. Many teenagers do take on part-time or summer employment to earn extra money or to save for college or university. This is an excellent way to become more independent. In addition, the experience you gain may help you with career choices. is there anything special I should consider when planning a career or taking a part-time job?

'4

Like everyone else, you should consider your interests and your talents. People with CF should also consider the following points: • Environment: It is best to choose an occupation where the work environment ? is free from fumes, dust, and smoke. These substances irritate the delicate lung tissues and may further damage the lungs. S~ieπΡti Q.,νok ! • Physical demands: Your health may not interfere with a physically demand? Lawyer ing occupation at the moment. In the future, however, your health may prevent you from meeting the demands of such an occupation. On the other hand, CF affects everyone differently. People with CF are successful sales7 ν . . Dicίσ r ? people, farmers, lifeguards, nurses, and doctors, all of which are physically ? demanding occupations. • Flexibility: An occupation with flexible hours may make it easier for you to schedule medical appointments and physiotherapy.

Personal Aspects of Cystic Fibrosis

Do I have to mention CF at a job interview? If you are confident that CF will not interfere with your work in any way, whether or not you mention it is up to you. You are not obliged to discuss this unless you sign a legal contract. If you are asked direct questions with regards to your health, you should answer honestly. You may find it easier to discuss CF once you have had a chance to prove yourself on the job. If CF will affect your work in any way, it is best to discuss this ahead of time with a potential employer. What should I tell an employer? You should discuss the disease, the treatments, and why they are done. If you feel that CF will not affect your work, then emphasize this. There is a booklet designed to answer the questions of employers. It is entitled Information for Employers. Copies may be available at your CF clinic or can be obtained by writing or calling: In Canada: Canadian Cystic Fibrosis Foundation 2221 Yonge Street, Suite 601 Toronto, Ontario 14S 2Β4 (416) 485-9149

In USA: Cystic Fibrosis Foundation 6931 Arlington Road Bethesda, Maryland 20814 1-800-344-4823 or (301) 951-4422

You may fmd it helpful to leave a copy with prospective employers.

is there anyone who can help me with course choices or career plans? Your guidance counsellor at school is there to help you make these decisions. If you have any questions, make an appointment to see your counsellor. It is also helpful to ask your parents, their friends, and your relatives about their occupations.

47

48

CF and You

CONCLUSION Numerous physical, intellectual, social, and emotional changes occur during adolescence. These changes are experienced by adolescents with and without CF. Some of the bodily changes that you will experience are directly influenced by CF. CF may also indirectly influence how you feel about becoming independent, sexuality, career choices, and other personal issues. These feelings are normal. You may have questions about issues that have not been addressed in this booklet. The staff members at the CF clinic are sensitive to these issues, but it is up to you to let them know about the issues that concern you.

GLOSSARY ACBTs: see Active cycle of breathing techniques. Acetylcysteine: A drug that helps to break up the mucus in the gut to prevent blockage. Also called Mucοmystt►~t. Active cycle breathing techniques (ACBTs): A physiotherapy technique involving different kinds of breathing. This helps to remove mucus from the lungs. ACTS: see Airway clearance techniques. AD: see Autogenic drainage. Aerosols: Mists made from medicated solutions, which can be inhaled and deposited directly into the lungs. Airway clearance techniques (ACΊ ): Physiotherapy techniques such as PDVP and ACBTs which help to remove mucus from the lungs. Aiveoli: Tiny air sacs within the lungs into which the bronchioles terminate. In people with CF, the alveoli often become clogged with mucus, which interferes with the exchange of oxygen and carbon dioxide. Animal models: Experimentation with animals (e.g., mice and rats) that have diseases similar to CF. This is done in order to learn more about CF. Antibiotics: Drugs that destroy or prevent the growth of bacteria. Antibiotics are given to people with CF in order to fight lung infections. Anti-inflammatory agents: Drugs that act against inflammation or swelling. Autogenic drainage (AD): A physiotherapy technique involving controlled breathing, which is done in a seated position. Bacteria: One-celled organisms that can cause diseases and illnesses in humans. Barrel chest: Condition in which the rib cage is enlarged and, as a consequence, the chest appears more rounded. Blood gases: This test measures the amount of carbon dioxide and oxygen in the blood. The test can be done by taking a sample of blood either from the finger or from an artery in the wrist. The finger method provides only a good estimate of blood gas levels. Although the wrist method is more uncomfortable than the finger method, it gives the best test of blood gas levels. Since it is uncomfortable, the doctor will order it only if very accurate results are needed. When this test is done, your wrist is frozen to lessen discomfort. Bronchi: The pair of breathing tubes branching from the trachea into the kings, which subdivide into the bronchioles and terminate in the alveoli. Bronchioles: Breathing tubes that are a subdivision of the bronchi.

50

CF and You

Bronchodilators: Drugs that dilate or expand the bronchial tubes. Calorie: The unit used to describe the amount of energy contained in food. Carrier of the CF gene: A person who has the altered CF gene paired with a normal gene. This person does not have the disease or show any symptoms. Every parent of a child with CF is a carrier of the CF gene. Also, people with CF are carriers of two altered versions of the CF gene. Catheter: Small tube inserted into a vein so that intravenous antibiotics can be given. More permanent intravenous catheters are inserted into large veins and are called central lines. Surgery is required to insert one of these. Cepacia: A type of bacteria or germ known as Burkholdeńa cepacla, which CF patients can easily acquire. Cervix (adj. cervical): The narrow outer end of the uterus. CF gene: The gene that odes for the CFTR protein. Mutations of the CF gene result in a defective CFTR, which is responsible for the abnormally thick and sticky secretions typical of CF. CFTR: see Cystic Fibrosis Transmembrane Regulator Chromosomes: Microscopic structures found in each cell of the body. Chromosomes carry the genes. Normally, everyone has 23 pairs of chromosomes. Cimetidine: A drug that is used to treat the pain of heartburn and the damage caused by the presence of stomach acid in the oesophagus. Cirrhosis (of the liver): The hardening of the liver due to scarring. Císapride: A gastrointestinal motility drug that enhances the passage of contents in the bowel. Conception: The moment when pregnancy begins. Contraception: Method of avoiding pregnancy (e.g., the pill, condoms, etc.). Contract: A formal agreement between two or more people. Cor pulmonale: A thickening of the right side of the heart caused by extensive lung damage. CT (CAT) scan (of the lung): A series of X-rays of cross sections of the lungs, which are done from top to bottom. These pictures are taken rapidly in order to "scan" the lungs. Cystic Fibrosis Trransmembrane Regulator (CFTR): The abnormal protein produced by the CF gene. This protein forms a channel for the transport of chloride ions across cell membranes. In CF, this protein does not work properly and causes the thick mucous secretions seen in people with CF. Decongestants: Medications that dry up secretions in the nose, sinuses, and respiratory system. Dehydrated: Lacking essential body fluids as a result of heat prostration.

Glossary Deoxyribonucleic acid: see DNA. Diabetes: A disorder that occurs when the pancreas does not secrete adequate amounts of insulin resulting in excessive amounts of sugar in the urine and the blood which cannot be used by the body. This can occur as a complication of CF. Diaphragm: A muscle used in breathing, which separates the chest from the stomach, intestines, and other internal organs. Digestive enzymes: Substances produced by the pancreas that break down food into particles small enough to be absorbed and used by the cells of the body. DNA: Deoxyribonucleic acid. The material that genes are made of. DNAse: An enzyme that may help to thin mucus in the lungs of people with CF. Ducts: A tube that carries secretions from the organ where they were produced. Endocrine glands: Glands that secrete the hormones responsible for growth απd sexual maturation. Enema: Liquid injections into the large intestine, which are used in treating intestinal obstruction. Enterai feedings: Supplemental feedings through nasogastric, jejunostomy, or gastronomy tubes. Enzyme replacement: Capsules that contain extracts of beef or pork pancreas. The capsules perform the same role as enzymes produced by the human pancreas. Enzymes: Proteins produced by living cells that speed up biochemical reactions. Exocrine glands: Glands that secrete their products into ducts. These are the glands directly affected by CF. The sweat, salivary, tear, and mucus glands are examples of exocrine glands. Fat: A greasy, soft, solid material found in animal tissues απd many plants. Foods containing fats (e.g., ice cream, potato chips, butter, etc.) help CF patients gain or maintain their weight. Fibrosis (of the liver): Formation of scar tissue due to damage caused by the abnormal accumulation of mucus. Finger clubbing: Enlargement of the ends of the fingers. Gastrointestinal (GI) motility drugs: Drugs that are used to speed up the movement of food through the large intestine. Gastronomy tube: A small tube surgically inserted into the stomach, which allows extra feedings to be taken at night. Genes: Minute structures that are found in chromosomes. They determine or influence a person's physical and chemical characteristics. Gene therapy: Au experimental technique involving the placement of cells that do not contain the CF gene into the lungs or pancreas of people with CF.

51

52

CF and You GI motility drugs: see Gastrointestinal motility drugs. Glands: Organs in the body that produce chemical secretions. Haemoptysis (or hemoptysis): Coughing up blood with sputum due to broken blood vessels in the lungs. Heartburn: A burning or painful sensation in the chest or upper abdomen due to stomach acid entering and damaging the oesophagus. Heat prostration: Collapsing/fainting due to excessive loss of fluids and salt during hot weather or vigorous exercise. Hormones: Chemical substances produced by the endocrine glands which circulate in the bloodstream and affect the activity of distant cells and organs. Humidify: To make moist/wet. Ibuprofen: An anti-inflammatory drug that is used to relieve pain and fever. Inflammation: Swelling and irritation of tissue (e.g., in the lungs). Inflammation is the result of the body's attempt to rid itself of infection. Insulin: A substance secreted by the pancreas, which allows sugar to be utilized by the cells of the body. Intestinal obstruction: Blockage of the gut with mucus, partially digested food, and waste. Intravenous: By way of the veins. In vitro fertilization: The implantation of a fertilized egg into a woman's uterus. Jejunostomy tube: A small tube that is implanted into the small intestine and which allows additional feedings to be taken at night while a person is sleeping. Large intestine: Organ that receives undigested food from the small intestine and in which stools are formed. Liposomes: Molecules used in gene therapy to transport genes that do not carry the CF mutation into the lungs of CF patients. Lobe: One of the five sections of the lungs. Lubricate: To make smooth or slippery. Malnourished: Having less than the minimum amount of food needed for proper growth and good health. MESA: see Microscopic epidídymal sperm aspiration. Microscopic epididymal sperm aspiration (MESA): The removal of mature sperm from the epididymís and combining them with a partner's egg in a laboratory dish. This procedure is followed by in vitro fertilization.

Glossary Μucοmysϊm: see Acetylcysteine. Mucus: A sticky, slippery secretion that is produced by mucous membranes, which serves to moisten and protect the membranes. Mutation: Genetic change. For example, people with CF have two altered or mutated versions of the CF gene. Nasogastríc tube: Tube inserted into the stomach through the nose in order to give supplemental nourishment. ίΕsορhagus (or esophagus): A tube joining the pharynx to the stomach, through which food passes. Omeprazole: A drug that is used to treat the pain of heartburn and the damage caused by the presence of stomach acid in the Øopphagus. Ovulate: Time during the menstrual cycle when eggs are released from the ovary and a woman can become pregnant. Oximetry (or 02Sat): Detection of the amount of oxygen in the blood. This is done using a sensor that records the amount of oxygen through your finger, toe, or ear. This is totally painless and provides information on how well your lungs are working. Paediatric (or pediatric): Pertaining to the medical treatment of children and adolescents. Pancreas: Gland located near the stomach, which produces digestive enzymes and insulin. PDVP: see Postural drainage, vibration, and percussion. PEP mask: see Positive expiratory pressure mask. Pneumothorax: Air leaking out of the lungs due to a small hole in the lung tissue. This is a serious complication of CF. Positive expiratory pressure mask (PEP mask): Physiotherapy method done in a seated position, which involves breathing through a small opening. Postural drainage, vibration, & percussion (PDVP): The traditional method of physiotherapy with CF patients. The goal is to remove mucus from the lungs and breathing passages. Protein: Large molecules made up of amino acids. Proteins are found in foods including meats, fish, vegetables, and fruit. Pseudomonas: Bacteria responsible for many of the chest infections in people with CF. Ranitidine: A drug that blocks gastric acid secretion and can help enzymes to work more effectively in some patients. Respiration: Process by which the body takes in oxygen and gets rid of carbon dioxide. Sex hormones: Hormones responsible for the development and functioning of the sex organs and the secondary sexual characteristics.

53

54

CF and You Sexually transmitted diseases (STD's): Diseases and infections that are mainly transmitted through close bodily contact, such as sexual intercourse. Sexual maturation: Development of the secondary sexual characteristics such as breasts, underarm hair, pubic hair, and facial hair. Small intestine: Organ in which the final phase of digestion occurs. Sputum: Mucus and other substances coughed up from the lungs. Staphylococcus: Bacteria responsible for many of the chest infections in people with CF. STD's: see Sexually transmitted diseases. Steroids: Drugs that can be used to decrease inflammation and slow damage due to infection and inflammation in the lungs. Sweat test: The standard test used for diagnosing CF. This measures the amount of sodium or chloride in a standard amount of sweat collected from the forearm. This test is not painful. Tobramycin: An antibiotic prescribed to fight lung infection. Trachea: Tube that leads air, taken in by the nose, into the lungs. Transport molecules: Molecules that transport healthy versions of CF genes into the affected organs of people with CF. UDCA: see Ursodeoxycholic acid. Ultrasound: A test thαt uses high-frequency or ultrasonic waves to examine organs. An ultrasound may be done on the abdomen to detect intestinal obstruction. Unlike an X-ray, this test does not involve radiation. Ursodeoxycholic acid (UDCA): A drug thαt alters liver function and may help to slow the rate of liver damage in CF patients with liver disease. Vaccine: A preparation of living or dead microorganisms, which is administered to increase immunity to (or decrease the likelihood of getting) a particular disease. Vas deferens: Tube that caries sperm from the testes where sperm are produced to the prostrate where sperm are stored. In 95% of men with CF, the vas deferens is blocked with mucus, resulting in sterility. X-ray: A "photograph" of a body part (e.g., the chest) taken using electromagnetic radiation to mark the film.

NOTES