I N F O R M A T I O N

A guide for

children

with CF

aged 8-12

years

CYSTIC FIBROSIS & YOU

Cystic Fibrosis Trust. Registered Charity No. 1079049. Registered Company No. 3880213.

Published by theCystic Fibrosis Trustwith a grant fromMarks & Spencer.

November 2000

© Cystic Fibrosis Trust 2000.Thisbooklet may be copied in wholeor in part without priorpermission being sought from thecopyright holders, provided thepurpose of copying is not forcommercial gain and provided dueacknowledgement is given.

The Cystic Fibrosis Trustfunds medical andscientific research aimedat understanding, treatingand curing Cystic Fibrosis.It also aims to ensure thatpeople with Cystic Fibrosisreceive the best possiblecare and support in allaspects of their lives.

Contact details forinformation and otherliterature published by theCystic Fibrosis Trust aregiven on the back cover.

This booklet has answers to someof your questions about CysticFibrosis, or for short “CF” – we useboth names in this booklet. It isbased on one written by the CysticFibrosis Association of New SouthWales (Australia) for children withCF aged between 8 and 12 and theirparents.

When you were young, yourparents had to take charge offinding out about CF and givingyou the right treatment. Now youare older, you will want to find outfor yourself and be responsible foryour own treatment. CysticFibrosis is your illness and youhave a right to know as much aspossible about it, what treatment isavailable, why you need it and whathappens if you do not have it.

It is not possible to answer allyour questions. These are some ofthe people you could contact if youwant to find out more –

● From your CF Clinic: your doctor, nurse, socialworker, physiotherapist ordietitian.

● From the Cystic Fibrosis Trust:Support Service. Our addressand telephone is given at theback of this booklet.

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CONTENTS1 Introduction2 What is Cystic Fibrosis (CF)?

Why do I have CF?3 How can I be sure I have CF?

Can CF be cured?Can I lead a normal life?Why do I need chest physio?

4 Why do I need antibiotics?Why do I have to take enzymes?What happens if I don’t take my enzymes?Can I eat a normal diet?Do I need extra salt?

5 Why do I need regular check-ups?Why might I need to go into hospital?Why do I get tired easily at times?Why do I need exercise?

6 Will I miss much school?What can I do if people make rude comments?Should I tell other people that I have got CF?

7 Why do I sometimes feel angry or depressed?What about death?

8 Some questions for the future9 Further information

What is Cystic Fibrosis (CF)?CF is a disease which affects howyour lungs and digestive systemworks.

Why do I have CF?You were born with CF. By chance,you received two copies of the ‘CFgene’ from your parents – one copyfrom your mother and one fromyour father.

Genes are chemical instructionspassed onto us by our parents. Theycontrol how our bodies develop.Some genes control whether wehave blue eyes, some whether wehave brown hair and some whetherwe have certain diseases – like CF.People who have only one copy ofthe ‘CF gene’ are called carriers.They do not have the disease.

This family diagram shows the

chances of being born with CF.Both the mother and the father arecarriers of CF with one copy eachof the ‘CF gene’.

Their genes are passed onto theirchildren by chance. One child didnot get any copies. Two children gotone copy each and are carriers liketheir parents. The other child gottwo copies and has Cystic Fibrosis.

In every pregnancy where bothparents are carriers of the ‘CF gene’there is a one-in-four chance thattheir child will be born with Cystic Fibrosis.

In the UK, about one person in25 carries the CF gene. Scientistsnow know what the gene looks like,so people can be tested to see if theyare carrying it.

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How can I be sure that I have CF?Have you noticed how your skintastes salty, especially when youhave been working hard? Well, thisis normal for people with CF. Theyhave more salt in their sweat thanother people.

Your doctor will have given youa special test. This measures theamount of salt in your sweat and iscalled the SWEAT TEST. The sweattest shows whether people have CF.You may also have had a test to findout what sort of CF gene you have.

Can CF be cured?Unfortunately, not at the moment.But doctors all over the world areworking on ways to find a cure andon better treatments to fight CysticFibrosis.

Your doctor will know what thebest treatment is for you. Everyoneis different, so your treatment maynot be the same as someone else’s. Itmight also have to change from timeto time as you change.

Can I lead a normal life?Yes. You should be able to do whatmost other children your age can do.

You just have to remember three things:● Physiotherapy and exercise● Enzymes● Antibiotics

Why do I need chest physio?People with CF have extra thickand sticky mucus. You need to keepyour lungs clear of mucus to stopinfections.

The best ways to keep yourlungs clear and healthy are:● Daily physiotherapy such as

breathing exercises and huffingto help clear mucus.

● Inhalations to help open the tinyair passages in the lungs and toloosen mucus.

● Exercising as much as possiblesuch as playing sport, ridingyour bicycle.

● Staying away from smoky anddusty places and unnecessaryexposure to “colds”.If you would like to know more

about your lungs, or different waysof clearing the mucus, ask yourphysiotherapist at the CF clinic.

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Why do I need antibiotics?The sticky mucus in the air passagesin the chest are easily infected withgerms. People with CF havedifficulty clearing these infectionswithout the help of antibiotics tokill the germs and physiotherapy toclear the mucus.

Why do I have to take enzymes?An enzyme is a chemical whichbreaks down food into the fuel yourbody needs for growth and energy.The enzymes which help break downfats, proteins and carbohydrates areproduced in the PANCREAS.

When you have CF, the tinytubes in the pancreas can get blockedwith mucus. This stops the enzymesgetting to where they are needed.

The enzymes you take ascapsules during meals make up forthis. They make sure your bodyuses the food you eat to help yougrow, give you energy, keep you fitand reduce “tummy aches”.

What happens if I don’t take myenzymes?● Your body won’t be able to use

the food you eat; meaning youwill not be properly nourished.

● You will not have the energy todo the things you want to do.

● You will get a pain in yourstomach.

● You will have to go to the toileta lot.

Can I eat a normal diet?Yes. You should be able to eat thesame food as anyone else, althoughyou will need to eat more. That’sbecause even with the enzymesyour food isn’t being digested asefficiently as theirs and you willprobably need more energy thanthey do. Just remember, the morefatty food you eat,the more enzymesyou may need. Sotalk to yourdoctor anddietitian atyour CFclinicabout thebest dietfor youand exactlyhow many enzymes you need.

Do I need extra salt?When it is very hot, you may losemore salt in your sweat than otherpeople. Salt tablets are not usuallynecessary but are advised in hotclimates or if you are undertakingsevere exercise in hot weather inthis country. Your doctor willadvise the dose.

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Why do I need regular check-ups?To make sure you are well. Itis easier to treat an infectionwhen it begins rather thanto wait until it starts tomake you ill.

When you have acheck-up, you will have athroat swab culture toidentify any infection, alung function test(“blows”) or a sputum test.

Lung-function testscheck how well your lungsare working. If they are notworking properly, your doctor willtell you what you can do to helpthem work better.

Throat cultures and specimens(your “spit”) are tested for germsthat cause infection. This helps yourdoctor know what antibiotictreatment you need to clear thegerms.

Why might I need to go intohospital?Sometimes your doctor will decidethat you need special treatment thatcan only be given in hospital.

Why do I get tired easily at times?Your body has to work harder thanother people’s bodies to keep youfeeling healthy and fight offinfections.

Sometimes it cannot keep upwith this work and you will feeltired. This might happen if you:● have an infection● are not digesting enough food● are very busy● are doing a lot of exercise

Why do I need exercise?You are more likely to get lunginfections than other people. Tomake sure you have a good supplyof oxygen to keep you healthy, youneed to do exercises which get yourlungs and heart working.

Good exercises are running,skipping, swimming, cycling…Choose those that suit you best.

By being fitter, you will feel greatwhen you are well and your bodywill cope better when you are sick.

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Will I miss much school?No, you should not.

If you know you are going to bein hospital, it is a good idea to talkto your teacher about it so you cantake work with you.

If you are away from school fora while, there are teachers inhospital and even some who canhelp you at home.

What can I do if people make rudecomments?Some people do not try tounderstand. They might call younames or give you a hard timebecause you often have a bad coughor have to take pills.

If you are having trouble – andthere is no reason why you shouldbe – talk it over with your family orfriends or someone else that you feel

comfortablewith.

Should I tell other people that Ihave got CF?Everyone is different, so this has tobe up to you. Some people find ithelps to talk to one or two closefriends about their Cystic Fibrosis.

If you are not sure, talk about itfirst with your:● Family● Doctor● CF Nurse● Social Worker● Teacher● CF Trust Support Service, where

there are staff you can talkthings over with in private.Maybe you could give your

friends this book to read.

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Why do I sometimes feel angry ordepressed?It is normal to feel depressed orangry from time to time. With CF,what sometimes gets you down is:● thinking that people are rejecting

you because of your CF; youfeel alone and even sometimesthat you do not need friends

● being completely fed up withhaving to do your treatmentevery day and sick and tired ofyour parents nagging you to do it

● feeling there is no point inmaking plans for the future

● a really bad spell of being ill● when you want to do something

special and having CF gets in theway.You will not be the only one

feeling down, but if it keeps on, tryfinding someone you can talk to. Itcould be your parents, a brother orsister, a close friend, staff at the CFClinic or at the CF Trust’s Support

Service, or someoneat school.

What about death?Cystic Fibrosis is one of thosesubjects that makes a good newsstory, often with pretty dramaticheadlines. When these stories comeout and other kids who know youhave got CF read them or see themon television, there are bound to becomments. So what are you to makeof it?

Remember, news stories have tobe unusual or they would not benews. CF affects people in a lot ofdifferent ways. Some have itseverely, but many have it mildly ormoderately but the papers do notwrite about them. No one can tellyou how long you are going to live.Treatment is getting better andpeople are living longer and longer.

Do not, whatever you do, thinkto yourself, “Oh well, I will not bemaking it to 40, 50, 60, or 70, sowhat is the use of bothering…”

If this really does bug you, findsomeone to talk to NOW!

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Some questions for the future.

Can I smoke?Are you serious?! Smoking is badfor everyone. For someone withlung problems, the answer isdefinitely NO!

What about drinking alcohol?Drinking too much alcohol is badfor anyone. For people with CF,there is also the matter of your liverand how well it is working. Somepeople with CF have liverproblems. Others could be takingmedicines that should not be mixedwith alcohol – always check withyour doctor.

Can I take any job?Within reason. Whatis important is tomake sure you arenot workingwhere, forinstance, there isa lot of airpollution fromdust and smoke.To be safe, talk itover with staff at theCF clinic.

Will I be able to have children?Fertility varies from person toperson, as it does for everyone else.

Women with CF may be slightlyless fertile. However, several havehad children. It is important thatwomen with CF thinking of havinga baby should check that they arehealthy enough to do so. Yourdoctor will be able to give youadvice when the time comes.

Men with CF are often notfertile. Sadly, the tubes that the

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sperm goes down are not formedproperly. This does not meanproblems with having sex, buthaving children can be difficult formany. However, there are newtechniques being developed toallow some men with CF to havechildren.

Will my children have CF?They will carry the ‘CF gene’.Only if your partner also carriesthe ‘CF gene’ will your childrenhave a very high chance (1 in 2) ofhaving CF.

Before having a baby yourpartner should be checked to see ifhe or she is a carrier.

Further informationIn this booklet we have been able toanswer only some of the questionspeople often ask. However if youfeel you would like to talk withsomeone or that you still have someunanswered questions you canspeak to staff, in private, at the CFTrust’s Support Service bytelephoning 020 8464 7211.

The CF Trust Support Servicehas a range of information leafletson Cystic Fibrosis and their trainedstaff will be pleased to hear fromyou about anything you may beunsure of.

Staff at your CF Clinic will beable to offer you support and help –so why not make a note now ofyour contacts, either at the CFTrust or your CF Clinic.

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I N F O R M A T I O N

Cystic Fibrosis Trust11 London RoadBromleyKent BR1 1BY

Tel: 020 8464 7211Fax: 020 8313 0472

enquiries@cftrust.org.uk

www.cftrust.org.uk

For further information and literaturepublished by the CF Trust, or how tomake a donation, please contact: