Cardiomyopathy in children and young people

A guide for families

The Cardiomyopathy Association is a registered charity (no: 803262) that provides information and support to families affected by the heart muscle disease cardiomyopathy www.cardiomyopathy.org

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Types of cardiomyopathy ……………………………………………………… Causes of cardiomyopathy……………………………………………………… Symptoms………………………………………………………………………… Diagnosis…………………………………………………………………………. Tests……………………………………………………………………………….. Managing the condition………………………………………………………….. Growing up with cardiomyopathy………………………………………………. Eating and nutrition Immunisation Going to school Taking part in sport Career plans & Going on holiday Treatments………………………………………………………………………... Lifestyle……………………………………………………………………………. Drinking alcohol Substance abuse & Piercings and tattoos Effect on the family……………………………………………………………… Research………………………………………………………………………….. Common questions………………………………………………………………. More information………………………………………………………………….

P3-4 P4-5 P5 P6–8 P6–14 P8-9 P10–19 P13, 14 P14 P15–17 P17–18 P19 P15-24 P20-21 P20 P21 P22-23 P24 P25-27 P25-27

At the start

treatments available and how children can live with the condition.

When children and young people are first diagnosed with cardiomyopathy, families can be overwhelmed by uncertainty.

Having more information reduces the uncertainties and helps families deal more confidently with their situation.

This booklet is designed to provide parents, carers, children and young people with more information about the condition and how it will affect their lives.

A child’s experience of cardiomyopathy will depend on many things, including type of cardiomyopathy, symptoms, severity of disease, family history and the age of the child when the condition appears.

It aims to improve understanding of the condition, the diagnosis, the

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Cardiomyopathy, a disease of the heart muscle, has only been recognised by doctors in the last 50 years. So knowledge about its causes, development, diagnosis and treatment has changed quickly. There are different types of cardiomyopathy: Hypertrophic cardiomyopathy (HCM): the heart muscle is thicker.

Dilated cardiomyopathy (DCM): the heart muscle is thinner and the heart does not pump as well as it should. It is the most common type of cardiomyopathy in children. Arrhythmogenic right ventricular cardiomyopathy (ARVC): areas of the heart muscle are replaced by fat cells and scar tissue. Usually the heart’s right pumping chamber (the right ventricle) is affected and this can cause heart rhythm problems.

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Causes of cardiomyopathy Cardiomyopathy often runs in families and so is called an inherited disease.

infection or a virus triggering an underlying inherited disease. There are other causes of DCM such as chemotherapy or the result of another health problem.

One parent might be affected and not know. In some cases families only become aware when a young person or a parent has died suddenly and unexpectedly. When the condition is found in a family it is important that close family members have heart checks to ensure that, if necessary, they can get prompt treatment. Early diagnosis and treatment are very important. The seriousness of the condition can vary in all types of cardiomyopathy, with some children only slightly affected, while others are severely affected. Severe and mild forms can exist in the same family. Dilated cardiomyopathy (DCM) in children can be the result of a viral

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Restrictive cardiomyopathy (RCM): the heart muscle is stiffer and its abilities to relax and refill with blood between beats are reduced. There are also other conditions, such as left ventricular noncompaction (LVNC) where parts of the heart muscle look like a sponge. It appears that when the heart develops in the womb, the muscle

does not “compact” into one. For more details about the individual conditions, see the Cardiomyopathy Association’s website www.cardiomyopathy.org The charity also publishes booklets on dilated cardiomyopathy, hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. For a copy, call freephone 0800 0181 024 or email [email protected]

Hypertrophic cardiomyopathy (HCM) and arrhythogenic right ventricular cardiomyopathy (ARVC) usually run in families.

Both have a ‘dominant’ pattern of inheritance, which means that only one parent needs to be affected for a child to be at risk of inheriting it. A child of an affected parent has a one in two chance of inheriting the same condition. Sometimes HCM will happen sporadically with no other family members affected. HCM can also be associated with syndromes that affect other organs in the body. There are some conditions that look like HCM but are other diseases, such as Fabry’s disease. Then the heart enlarges because substances such as toxins, proteins and iron stay in the heart muscle. Some of these diseases can run in families too. Restrictive cardiomyopathy (RCM) is the rarest of the main types of cardiomyopathy. It can also run in families and be the result of more severe diseases affecting many organs.

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Symptoms of serious heart problems include:

Young children rarely complain about palpitations.

♥ fainting

Usually they say they are in pain, (which can be chest pain or palpitations), or that they are tired.

♥ chest pain caused by activity or exercise ♥ chest pain with a cold sweat ♥ paleness ♥ shortness of breath ♥ palpitations

Heart failure in small children can initially look like a chest infection that does not get better after a week or two. Children under one may not put on weight. 5

Diagnosis Cardiomyopathy is diagnosed when a child shows a number of features of the condition. But diagnosis may not be straightforward. So experts in cardiomyopathy need to be involved.

Some isolated features of a normal heart can resemble those of a heart with cardiomyopathy. So seeing them does not necessarily mean a child has the disease. Also, some abnormalities are found in more than one heart condition Because of the complexity, all these features need to be carefully considered before a diagnosis can be made. In children, diagnosis can be even harder because their hearts are changing as they grow. The first step in diagnosis is for the hospital team to take details of the child’s medical and family history (including drawing up a family tree). A heart specialist (cardiologist) will then arrange for tests to be done. This is usually all done in one visit. If a child has typical features of cardiomyopathy, diagnosis may be possible after only basic tests. If the changes are less typical, the diagnosis can be quite difficult.

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A variety of tests are carried out on children being checked for cardiomyopathy. The great majority of the tests do not hurt but the child needs to co-operate. Some of the tests will be done before the child sees the doctor. With the results in hand, the doctor will meet the child and the parent or carer for the first time and take the child’s medical history, physically examine him or her and

look at and explain the test results. In some cases more tests may be needed later. One of the simplest tests is an ECG (a 12 lead electrocardiogram). It is done by a cardiac physiologist and looks at the heart’s electrical activity. With the child lying flat on a bed, the leads are attached to the chest and the arms and legs using skinfriendly stickers.

Then subtle findings may need to be considered and more complex tests done (see Tests on Pages 6-14). What the tests involve and why they are being done should always be explained in detail and in simple words to the child and parent or carer. For certain tests signed consent is needed from an adult. Particularly when diagnosis is difficult, tests have to be planned and guided by the doctor.

of the condition in the child and other family members. Sometimes doctors need information from past events or medical conditions that have affected the child or other members of the family. If there has been a death in the family that might be connected, a post mortem may be needed or results looked at (after the appropriate consent is obtained).

Not all tests are needed for every child. The doctor is also very important in interpreting the results as some may not be straightforward. As cardiomyopathy is often inherited, finding one family member affected can help in diagnosing other family members. This is why looking at the family tree is important. And even when a child has been diagnosed with cardiomyopathy, continuing investigations may be needed for a better understanding

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An ECG takes a few minutes and causes no discomfort. A graph of the heart’s electrical activity is printed on a piece of paper while the test is running. With very young children who will not lie still, an ECG can be difficult. Usually distraction helps. An ECG is a sensitive test and can almost always rule out heart disease when none is present. But it is not good at identifying

specific heart diseases. Interpreting a child’s ECG is different to an adult one as the doctor has to take into account changes caused by the heart’s development. Some features seen on an ECG have different significance depending on the disease being looked for. So the result must be looked at by a doctor with the right experience.

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When a child cannot be given a diagnosis at the first appointment, he or she will be given treatment for the most likely condition.

Managing the condition

This will help keep the child safe and ensure follow-up care. The child may get a proper diagnosis at the next appointment or after other family members have heart checks.

Caring for children with cardiomyopathy involves giving the right treatments for the child’s symptoms and looking at what course the illness might take. In rare cases cardiomyopathy can lead to sudden death.

Often when a final diagnosis is made, close family members are advised to have heart checks.

So doctors will assess if your child is at risk and, if so, take action to reduce any risk. Children will need to stay under the care of a cardiologist. That care will be tailored to the child’s individual situation. Adolescence can be a particularly vulnerable period for some children. Their bodies change a lot in a short time and this can affect the development of the condition. So some children will need to be checked more often during this time. Children will not need the same tests at every hospital appointment, but an echo and an ECG will be repeated regularly.

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Sometimes, a signal averaged ECG is performed. Similar to the standard 12 lead ECG, it takes longer as many more heart beats are recorded. This test is mainly used for diagnosing ARVC and looks for irregularities in the heart’s electricity. Although not a key test, it can be helpful in some cases. An echocardiogram (echo) scan is an easy and painless way of

looking at the shape of the heart. It is done by a cardiac physiologist with the child lying flat on his or her side on a couch. A small amount of gel is put on the child’s chest and a probe (transducer) is run over the chest, sending and receiving harmless ultrasound waves. It is similar to the ultrasound scan that pregnant women have when their babies are checked.

With some forms of HCM (HCM with obstruction), children may need open heart surgery. This is done when the thickened muscle obstructs blood flow in the heart causing symptoms. If drugs do not control the symptoms, an operation can be done to reduce the thickening and symptoms. Some children with DCM and restrictive cardiomyopathy might need follow-up tests such as catheterisation (a test that measures the blood pressures inside the heart). A tube (catheter) is fed into the heart by a vein, usually at the top of the leg. In most cases children are given a general anaesthetic and need a short stay in hospital. The doctor may continue to ask about the health of other family members as this may affect the child’s treatment. In some cases genetic testing will be offered.

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An echocardiogram (echo) is very important in diagnosing cardiomyopathy and can be done many times without any danger to the child.

rule out birth defects. Then the doctor will look at the heart muscle’s thickness, its ability to pump and relax, and the heart valves.

So it is widely used for diagnosis and for follow-up checks.

The data from the echo is stored digitally so it can be compared with later readings.

During the echo, detailed pictures of the heart are taken. In young babies, the doctor has to 4

In growing children, their age and size is taken into account when considering heart measurements. 9

Growing up with cardiomyopathy Children with cardiomyopathy face many challenges as they grow up. These include diagnosis, living with day-to-day limitations, hospital visits, medication and coping with a change in their health. This is on top of coping with all the normal things such as school, friendships and becoming independent.

'caught' cardiomyopathy or it is a punishment. Their understanding changes as they get older. So it is important that adults check what they understand as they grow up. For adolescents, a diagnosis of cardiomyopathy is a real challenge. They are forming their identity and have to include their new diagnosis into their view of themselves.

Their first challenge is understanding the condition and how it affects them. Cardiomyopathy is very complex. Children tend to remember little of what they hear. So it is important to try different ways of explaining it to them, such as pictures, drawings, talking to people who have the condition, and checking with them what they understand. Adults assume children understand what they have said but often this is not the case. Young children may think they have

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Occasionally, a different type of echo called a transoesophageal echocardiogram may be needed. The child is sedated and a thin tube is gently placed down the throat. When the tube lies immediately behind the heart, close-up pictures are taken of the heart’s structures. These close-ups give a clearer and more revealing picture than a usual echo.

An exercise test looks at the heart’s performance. The test is used on children from their early teens. It can be done on a treadmill or a bicycle. By measuring the amount of oxygen used, the tests give a good indication of the child’s fitness. Heart rate, ECG and blood pressure are monitored throughout the test.

They could see themselves as someone with a heart condition or someone with an extra aspect to themselves. Their career plans could also be affected (see Career plans on Page 19). It can take a year for children to come to terms with their diagnosis.

Older children will be concerned about not being like their class mates. New limitations on their activities (because of their condition and symptoms) and extra adult interference may not go down well. At a time of expecting more privacy and independence, they may find hospital staff and family members repeatedly asking about their symptoms and limiting their independence. This can feel very intrusive and grossly unfair. Disruption to daily life caused by having to take medicine or missing school for hospital appointments can make a child resentful. It is important to talk openly to children about their condition and their feelings as this will help them to adjust. Sometimes children find talking to a family member difficult because they do not want to upset them. If your child does not want to talk to you, arrange for them to talk to their doctor, nurse or another adult such as a school counsellor or a teacher.

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When irregular heart rhythms are suspected, a Holter monitor is used to record the child’s heartbeat over one or two days at home. These small devices with digital tapes are attached to the child’s chest (usually with three leads). The child can carry on life as usual, but any symptoms should be written down with the time they

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are felt. The tape is then analysed on a computer and compared to any symptoms the child has had. This test may be repeated. Blood tests are regularly needed. Before these are done a local pain-killing gel is put on the skin to reduce discomfort.

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Hospital visits can be stressful for everyone because of the journey, disruption to family life, fear of needles and other medical procedures and worry about the results of tests. Fear is a normal response to threat (hospital is threatening because it is unpredictable and outside your child's control). So it is not surprising children become upset about having tests. The family can help by preparing children for medical procedures beforehand by drawing pictures and practising procedures on toys. Every child is different and while the majority cope best by looking away, others feel more in control by watching the procedure. You can tell your child it is ok to make a noise or cry if that helps them cope. Adults can help children feel more in control by explaining what will happen, how long each test will take and giving them some choice about how the test happens.

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An MRI (magnetic resonance imaging) is being used more and more on young people when more detailed heart images are needed. It is pain-free, does not involve radiation and so can be used whenever necessary. The young person lies flat on a trolley that goes into a tunnelshaped magnetic scanner. A child may need to be sedated during this test.

For example, where possible, encourage the child to discuss who should be in the room, who sits where, which arm the blood will be taken from and give the child control over when the procedure will start, within a time frame. Remember that avoiding procedures increases anxiety in the long run. So it is not possible for children to have a choice about whether the procedure should take place. Reward schemes for co-operating with tests are also useful. Rewards should be given to children as soon as possible after the procedure and can be anything from a sticker to chocolate or a trip to the cinema. A cardiac CT is another imaging technique that may be required. It is also pain-free but involves radiation. Invasive tests are occasionally needed too. These include studies of the heart’s electricity or small arteries. Wires and catheters (small tubes) are threaded into the heart through blood vessels in the arm or top of the leg. If these tests are needed, the doctor will explain them in more detail.

Eating and nutrition Children with heart disease (unlike adults) may need a high calorie diet. Because their hearts are working much harder, children with cardiomyopathy will burn off more calories than other children. Without a high enough calorie intake they will fail to thrive and will put on weight slowly.

Adults should give the child lots of praise for trying, even if he or she does not quite manage it the first time. Adults should try to relax and keep their own anxiety at bay. Sounding cross can make things worse. If adults cannot stay calm, staff will not mind if they do not go in for the procedure with the child.

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Smaller and more frequent meals may be needed for babies and children who tend to get a full-up or bloated feeling. Children taking diuretics benefit from having potassium-rich foods such as dried fruit and bananas. It can be helpful to see the hospital dietician for advice and support and, if problems arise, the paediatric gastro-enterologist.

Gene testing can also be very helpful.

associated with both DCM and ARVC have also been found.

Looking at families affected by HCM led to the discovery that it is caused by mutations in the genes affecting heart muscle proteins. The mutations can be thought of as spelling mistakes in the genes.

We know that in the same family the same “spelling mistake” of the genetic code can occur – although members of the same family might be affected very differently.

The first mutation was found in 1989. Since then, gene mutations 4

Supplements such as Maxiful or Duocal can be added to babies’ formula milk or expressed breast milk.

Gene testing is done to look for disease causing gene mutations in a family and the results can take many months.

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Sometimes children may need to be fed by a nose or stomach tube. If children are prone to bringing up their food, antacids and anti-sickness treatments, such as domperidone, ranitidine, and ondansetron, can be used.

Immunisation Children with cardiomyopathy should be kept up-to-date with childhood immunisations wherever possible including those for flu, pneumonia and chicken pox. Talk to your child’s doctor every year about this.

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Gene testing is also done when a gene mutation has been found in a family and other family members are being checked to see if they have inherited the mutation. Those getting a positive result will know they are at risk of developing the condition and need regular checks. Those who have not inherited the mutation do not need further heart checks.

Gene testing is done from blood samples. In some cases, finding the gene mutation can help the doctors decide the best treatment and after-care for the child. Before genetic testing is done, the doctor and a genetic counsellor will discuss important issues with the family.

Going to school Children should continue to go to school because it is important for their social and emotional development as well as helping them lead lives that are as normal as possible. But managing school and friendships can be tricky for newly-diagnosed children, especially if they have missed a lot of school. Good communication with the school is vital. Ask your child’s doctor or nurse to send an information pack to the school explaining your child’s medication and the need for hospital appointments and stays and also what the school should do in cases of illness or an emergency. It is important to meet regularly with the teachers to talk about your child's health and how they are coping. If your child is seriously affected, talk to the school about whether he or she can receive any individual support or might qualify for a statement of special educational needs.

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Not all patients with cardiomyopathy need the same drugs, and particular medications may be used for different reasons. The main ones include: ACE inhibitors (captopril, enalapril, lisinopril) These drugs widen the blood vessels, allowing more blood to pass through.

They are very important when the heart has pumping problems. The drugs reduce the heart’s workload, allowing the heart to recover. The drugs are started at a low dose and gradually increased. Blood pressure and kidney function will need to be checked, particularly when the drugs are first taken. 15

Sometimes schools can overreact and limit your child more than they need to, which makes children feel stifled and highlights that they are different from their classmates. However, if children feel they are not being listened to at school, they can find themselves pushed into activities they feel unable to do and they feel unsafe. Older children also find it hard to talk to their friends and can feel left out. Some schools help by pairing children so that a child will not be the only one not doing an activity.

into the school for you. Older children probably won't want you to talk in detail to their classmates. So rehearse with your child a sentence or two that explains the condition. They can then trot this out whenever anyone asks about it. Act out situations with children asking questions so your child can practise replying and feel more confident about doing so.

A child’s experience will also depend on their symptoms and how their condition limits their activities. It can be very useful for adults to go into school and explain the child's condition to the teacher or class and answer questions. This helps relieve the burden on the child too. Your nurse might be happy to go

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Betablockers (atenolol, bisoprolol and carvedilol) When the heart is not working well, the body produces a substance called adrenaline which tries to make the heart work harder. This can cause further damage to the heart. Betablockers block or reduce the effects of adrenaline on the heart. They make it work more slowly, reducing the heart rate. The heart

then has more time to relax and it uses less energy. These drugs help to reduce chest pain on exertion, palpitations and blood pressure. Betablockers are also started at a low dose and gradually increased. Calcium channel blockers (verapamil or diltiazem) These drugs improve the heart’s ability to relax and reduce the heart rate. They are generally very good at reducing chest pain.

Many children worry that if they answer one question their friends will ask many more. Usually, however, children ask a question or two and then move on. But you might like to practise phrases such as “I've answered your questions, can we talk about something else now?” in case they need them.

Taking part in sport Lack of exercise can cause many health problems such as heart disease, osteoporosis, stroke, diabetes, high blood pressure, back pain and obesity, and this is also true for children with cardiomyopathy. By being active, the risk of developing these problems in later life is reduced. Cardiomyopathy can limit the amount of exercise children can do. However they should lead as normal a life as possible and try to have a healthy lifestyle. So do ask your child’s cardiologist about sporting activities your child can do. Your child may have to avoid competitive sports, including crosscountry running, rugby and athletics. But many are able to enjoy PE, break-time activities and after school clubs, but may have to have regular rests.

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Sartans (losartan, candesartan, ibesartan)

of HCM with obstruction. It is rarely used on its own.

From the same family as the ACE inhibitors, they work in a similar way. Sometimes they are used when ACE inhibitors cannot be tolerated and sometimes they are used with ACE inhibitors. Blood tests may be needed sometimes.

Amiodarone controls rhythm problems. It is long-acting, and blood tests may be required. It often causes skin sensitivity. So complete sun block is needed.

Antiarrhythmic drugs Disopyromide is often used with betablockers to reduce symptoms

Digoxin is used to help those who have a type of irregular heart rhythm called atrial fibrillation. But it may also be given to help the 17 heart work more efficiently.

They can, for example, take a less active part, such as playing football in goal instead of on the pitch. Older children can get involved in coaching their favourite sports. It is essential that your child’s nursery, school or college is kept up-to-date about your child’s heart condition and are involved in all discussions about exercise and sport.

feel unwell while exercising they should stop at once. If they continue to feel unwell, parents or carers should be contacted for advice. These symptoms should be taken seriously and assessed by a teacher immediately. If a child collapses an ambulance should be called for.

Initially the school may put restrictions on your child’s activities. However as teachers get to know your child and feel more confident about dealing with his or her health, they may allow more.

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Carers and teachers need information about the type of cardiomyopathy and the symptoms to be aware of when children are exercising, including shortness of breath, chest pain, palpitations, dizziness and fainting.

If the child is breathing and has a pulse, he or she should be put in the recovery position and monitored.

Children should avoid exercise if they are already feeling tired or unwell. If they have symptoms or

If there is no pulse or breathing is impaired, life support should be started.

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Diuretics (frusemide, bumetamide, spironolactone) There are different types of diuretics. These drugs, also known as water tablets, help the body get rid of any extra fluid caused by the heart’s pumping problems. They are quite powerful and can affect the kidneys. The effects on the kidneys have to be watched.

Others (warfarin, aspirin) When the heart’s pumping is severely affected, aspirin or warfarin may be prescribed to reduce the chances of a stroke. These drugs thin the blood and reduce the chances of a blood clot. But those taking warfarin need regular blood tests to check the clotting ability.

Career plans

Going on holiday

Children with cardiomyopathy can have a career in most occupations, but there are some exceptions.

Before going abroad with your child, discuss with your medical team where you plan to go.

Heavy manual jobs should be avoided and some careers, including airline pilots, lorry and bus drivers, and in the Armed Forces, have strict medical guidelines.

A trip to a country where hygiene standards are poor or where good medical treatment is unavailable is not advised. Flying may affect some children’s heart conditions and going to a hot country can dehydrate them and make them unwell. They may also need documents with them to allow them to avoid going through some security checks if they have a pacemaker or ICD. It’s very important you have travel insurance and you may be asked for a medical letter saying your child is fit to travel. You may want your child to be checked before travelling. So speak to your hospital team a few weeks before you are going. Extra vaccinations may also be needed.

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Medicine tips It is useful to have an up-to-date list of medicines your child is on with the dosages and keep it with you and your child at all times. Get to know a helpful pharmacist who understands your child’s illness and remember to order repeat prescriptions in good time.

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Lifestyle issues Young people with cardiomyopathy can live a long and full life, but there are some precautions they should take.

Drinking alcohol

to forget about their condition. It helps if they can be encouraged from a young age to take some responsibility for their health and be made aware of the extra dangers they face by drinking too much. They may also benefit from having strategies in hand to cope with peer pressure.

Drinking alcohol can worsen cardiomyopathy and cause Talking openly and nonsymptoms such as palpitations, fainting and dehydration. Sometimes judgementally to children about their choices helps them make sound it can interact with medication. decisions. When teenagers start drinking there Talking privately to a doctor, nurse is a temptation to binge drink at or counsellor specialised in dealing parties because this is what their with young people might also help. friends are doing. Others may do it

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Pacemakers Some children with cardiomyopathy may need a medical device, either to reduce the risk of sudden death or as a treatment in severe cases. A pacemaker is used when the heart’s electrical system is damaged and causing problems. It has a battery and programmer and acts like an electrical back-up system.

The child is usually given a general anaesthetic before the pacemaker is put in just under the skin below the collarbone on the front of the chest. The pacemaker, which also records details of rhythm problems, needs to be checked regularly by specialist technicians to find out what it has been doing and ensure the battery is working normally.

Substance abuse

Piercings and tattoos

Some teenagers experiment with drugs. Teenagers with cardiomyopathy tempted to do so may not take their prescribed drugs for fear of interactions or simply because the illegal drugs have affected their judgement.

Most young people with cardiomyopathy can have tattoos and body piercings but there are some exceptions.

Others may use drugs to help them fit in, to ease life’s pressures or to cope with anxiety or feelings of depression or anxiety.

Those with heart valve disease, a pacemaker or defibrillator, or taking drugs that thin the blood, such as warfarin, are advised not to have them because of possible infection.

Parents and carers need to be aware their children might take drugs. Do not assume that because they have a chronic condition, they will always make the right choices. Children with cardiomyopathy need to be aware that illegal drugs could increase side effects, introduce new ones, or increase or decrease the effect of their drugs. Cocaine for example can cause irregular heart rhythms, high blood pressure and inflammation of the heart.

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Internal cardioverter defibrillators (ICDs) These devices are very similar to pacemakers but are used to prevent sudden death. As well as being able to pace the heart if it develops an abnormal rhythm, their main job is to shock the heart into a normal rhythm if a dangerous one occurs. ICDs also need to be regularly checked.

Biventricular ICD and pacemaker This device is similar to an ICD but has an extra wire fitted. It helps to regulate the heart’s rhythm and assists the heart’s different chambers to pump together. Only a small number of children have had this device fitted so far but the results are promising, showing it helps the recovery of children who have severe heart failure or are waiting for a heart transplant. 21

Effect on the family

blamed by partners or in-laws.

Families often find it difficult to adjust to their children having a condition that will affect them all their lives.

Or they might feel they have to take responsibility for looking after their sibling, becoming more like parents themselves. Be prepared and reassure them that they do not have to take responsibility.

They feel it like a bereavement. The future they had planned for their child might not work out. Some parents feel guilty for passing on the disease and some feel

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Other devices Sometimes a child’s cardiomyopathy can be very severe and intensive care may be needed. The heart can be so sick that it cannot sufficiently support vital organs or their work. In these cases and always for a limited time, the child may need breathing and heart pumping support or just pumping support.

Brothers and sisters may resent all the focus on the affected child.

You and the medical team have things under control and thank them for being concerned.

These support systems, called LVADs (left ventricular assist devices), can be used until either the heart recovers or it is replaced by a new heart from transplantation. Heart transplants A few children severely affected by cardiomyopathy may need a heart transplant. If this is the case, they will be referred to a specialist transplant centre.

Hospital appointments and stays can also disrupt family routines, causing a host of other problems. But things are likely to look very different after six months or a year. So don't despair. Make sure you have people to turn to for emotional and social support. This can be friends, family, your nurse or a patient support charity, such as the Cardiomyopathy Association.

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Surgery Patients with HCM and severe symptoms due to obstruction to blood flow in the heart may also need a type of surgery called a myectomy. A small piece of muscle that is obstructing blood flow is removed.

Try to help the whole family lead as normal a life as possible. Always be prepared to talk about things openly and honestly. This means no taboo subjects, and occasionally asking all your children how they are feeling. Allow everyone to show their anger, resentment and sadness, because this helps the family cope. These feelings are normal and discussion will help everyone find solutions. needed and can be done at the same time. For more detailed information about this procedure, see the Cardiomyopathy Association’s booklet on HCM or the charity’s website www.cardiomyopathy.org

Sometimes a repair to a heart valve (the mitral valve) may be 23 23

Research

shown to help the heart work better and improve life expectancy.

At the moment there is no cure for cardiomyopathy. So research is focused on understanding the causes of the disease, developing new treatments and improving life expectancy.

New advances and the use of more drugs have also improved the survival of those needing a heart transplant.

Not all the gene mutations causing cardiomyopathy have been found. New projects are looking for more mutations on more genes and what effects the gene changes have. Stem cell research is another area of interest. Stem cells can transform into different tissue. After birth the heart is not able to repair itself very well.

Devices such as internal cardioverter defibrillators (ICDs) and biventricular pacemakers are being used in younger children. In young people at risk of sudden death, ICDs are proving to be lifesaving. In cardiac resynchronisation therapy (CRT) a special pacemaker can improve heart pumping when needed and has been shown to help children with severe DCM.

Stem cells, however, can create new tissue and researchers are looking into using them to repair damaged heart tissue. Whether this work will help cardiomyopathy patients is currently unknown. In DCM, drug therapy has been

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Electrophysiology Sometimes children’s heart rhythm problems can remain despite drug treatments. They may then need an electrophysiological study (EP) to look at the electrical activity of the heart in more detail. A small wire, passed into the heart through a vein in the top of

the leg, can measure the electrical currents in the heart and find if there are any abnormalities. Some problems in the electrical pathways can be treated using radiofrequency waves. This is called radiofrequency ablation.

Common questions When and how much should I tell my child about his or her condition? Children adjust better to their condition if you are open and honest with them. But what you tell them needs to be guided by their

age and understanding. Tell them as early as you can in general conversation or as questions arise. You do not have to burden them with all the details at an early age. But it is important to encourage openness so they feel they can ask you anything at any time, particularly when they have problems. How can I tell if my child is not coping with the illness? Most children adjust well. However, keep an eye out for changes in behaviour such as separation anxiety, tantrums, attention-seeking behaviour, physical complaints, bed wetting, sleep problems, a decline in school work, becoming withdrawn from their friends, and problems getting them to take their medication. But don't be concerned if these problems only persist for a few days and you manage to sort them out. This is all part of normal growing up. However, if their new behaviour persists over weeks or months and your usual strategies do not work, then discuss it with your GP, your nurse or consultant who can give you advice on further support.

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Places to go for further information, include: Cardiomyopathy Association Freephone 0800 0181 024 www.cardiomyopathy.org Great Ormond Street Hospital 020 7405 9200 www.goshfamilies.nhs.uk www.childrenfirst.nhs.uk

British Heart Foundation 0300 330 3311 www.bhf.org.uk Genetic Information Service 0300 456 8383 Children’s Heart Federation 0808 808 5000 www.childrens-heart-fed.org.uk 25

What do we do when our child gets common illnesses? Most children with cardiomyopathy should be treated like other children when they get colds, flu, sickness and diarrhoea. But do take a few extra precautions. Be aware of your child’s normal breathing and heart rate so you know what is abnormal. Help your child stay hydrated by encouraging them to drink and checking babies’ nappies are regularly being made wet. Make sure your child has plenty of rest. If he or she develops a temperature, use paracetamol or ibuprofen syrup (headache and cold remedies are generally safe but always check with the pharmacist and your child’s cardiologist). If your child has been sick and you feel some medication needs to be repeated, get advice from your GP or nurse specialist. Give your child high calorie food as the heart will be working harder and using more energy. If you are concerned about your child’s condition, contact your GP or cardiac nurse. Sometimes antibiotics may be needed.

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How do I control the amount of exercise my young child does, especially when I am not with him or her? It is difficult to explain to young children, especially when they are playing with friends, that they need to stop and rest. Children will often feel breathless or tired and slow down and stop themselves. Give them regular drinks to keep them hydrated while playing or exercising.

Authors

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Written by Dr Maite Tome and Dr Antonis Pantazis, consultant cardiologists, the Heart Hospital, London; Dr Sara O’Curry, clinical psychologist, Great Ormond Street Hospital, London; and Sarah Mead-Regan, clinical nurse specialist, inherited cardiovascular disease, Great Ormond Street Hospital.

Funding for the booklet has been provided by the Wates Foundation, an independent grant-making family trust that aims to improve the quality of life of five to 25-year-olds through promoting physical, mental and spiritual welfare.

Can my child go on fairground rides? Most children with cardiomyopathy can go on fairground rides, but there are some exceptions. For example, those with pacemakers and internal defibrillators (ICDs) could dislodge some of the leads going into their hearts. So always discuss this with your child’s doctor. Is my child going to die? Cardiomyopathy is a serious heart condition and occasionally children will die from it. But drug treatments and medical devices are improving all the time and saving more and more lives and improving life expectancy (see More info on Page 25).

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The CMA provides information and support to families affected by cardiomyopathy. It publishes information booklets on the main types of cardiomyopathy— hypertrophic, dilated and arrhythmogenic right ventricular— and CD-Roms on hypertrophic and dilated cardiomyopathy. It has a freephone helpline (0800 0181 024)

How do we handle moving from children’s to adult health services? Young people need to become independent and less reliant on adults and the medical team as they transfer to adult services. They need to understand their condition, recognise symptoms and know what to do, and know where and how to find information or support when they need it. This should happen gradually so children can develop confidence in trying things out for themselves and slowly take more responsibility for their health.

It holds information days around the country for patients and families and has a network of volunteers who offer one-to-one support on the telephone and run support groups. The charity also raises awareness in the medical profession through annual medical conferences for doctors and nurses. It works closely with the Government to improve care for affected families.

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Published by the Cardiomyopathy Association, Unit 10, Chiltern Court, Asheridge Road, Chesham, Bucks, HP5 2PX Registered charity no: 803262 Freephone helpline: 0800 0181 024 Website: www.cardiomyopathy.org Email: [email protected]