Supporting young people and adultsborn with a heart condition

Pulmonary arterial hypertension with

congenital heart disease / Eisenmenger’s

syndrome

What it means for you

The production of this booklet was sponsored by Actelion Pharmaceuticals UK Ltd. with no influence on the content.

Date of preparation: October 2014; PAH 13/0024

IntroductionThis booklet provides information to help you understand pulmonary arterial hypertension (PAH) with congenital heart disease (CHD). This includes how the conditions are linked and their effect on you.

In this booklet, you can find out about:

� How the heart and lungs work together

� Pulmonary arterial hypertension with congenital heart disease (PAH–CHD)

� Diagnosing and managing PAH–CHD

� Practical advice for lifestyle, travel and work

By knowing more about your condition, you can work together with your medical team to help manage your condition and reduce the impact that it has on your life.

Contents

We are grateful to our committee of experts who helped write this booklet:

� Kostas Dimopoulos, Consultant Cardiologist, Royal Brompton, London

� Linda Griffiths, Adult Congenital Heart Specialist Nurse, Manchester Royal Infirmary

� Carl Harries, Pulmonary Hypertension Specialist Nurse, Royal Brompton, London

We would also like to thank the people with PAH–CHD who shared their experiences. Their insights supported the development of this booklet.

How do the heart and lungs work? 3

What is congenital heart disease? 3

What is pulmonary arterial hypertension? 3

Why does CHD sometimes cause PAH? 4

Diagnosing and managing PAH–CHD 6

Living with PAH–CHD 9

Working and PAH–CHD 11

Travelling and PAH–CHD 12

Relationships and PAH–CHD 13

Getting support 14

What to do in an emergency 15

2

How do the heart and the lungs work?To understand how pulmonary arterial hypertension with congenital heart disease (PAH–CHD) affects you, it is useful to know how the heart and lungs work together to supply your body with oxygen.

Your body needs oxygen to work properly and carry out essential functions. You get oxygen from the air you breathe:

� You breathe air into your lungs and oxygen is absorbed into your bloodstream

� The heart is then responsible for pumping this oxygen-rich blood back to the body where the oxygen is delivered to all your tissues

� Once the oxygen has been delivered to your tissues, the blood returns to your heart where it is pumped back to the lungs to collect more oxygen

The normal heartThe heart has four chambers:

� Two collecting chambers at the top – called the right atrium and the left atrium

� Two pumping chambers at the bottom – called the right ventricle and the left ventricle

The right side of the heart pumps blood that has used up its oxygen (de-oxygenated or ‘blue’ blood) back to the lungs where it collects a fresh supply of oxygen. This oxygen-rich blood (oxygenated or ‘red’ blood) returns to the left side of the heart, so that it can be pumped back to the body.

The right side of the heart only has to pump the blood to the lungs (low resistance circuit) and therefore pumps under low pressure. The left side of the heart has to pump blood all around the body (high resistance circuit) and therefore pumps at a higher pressure.

In the normal heart, the blue and red blood never mix.

Arteries

High pressureLow pressure

Pulmonary artery

Lungs

Heart

Veins

LEFT SIDERIGHT SIDE

Aorta

The heart pumps blood through to the lungs and to the rest of the body.

3

What is congenital heart disease?Congenital heart disease (CHD) is a ‘structural’ heart problem that some people are born with. Congenital means it is present at birth.

What is pulmonary arterial hypertension?Pulmonary arterial hypertension (PAH) is a condition in which the blood pressure in the lungs is high. This high pressure makes it difficult for the blood to be pumped to the lungs to get more oxygen.

Blood frombody

Blood frombody

Blood from lungs

Blood to lungs (low pressure)

Blood to body (high pressure)

RIGHT SIDE LEFT SIDE

A normal heart

Other types of congenital heart disease that may lead to PAHCHD can sometimes be more complex than just a hole in the heart. For example:

� Your congenital heart problem may have been repaired quite late and, despite a good repair, your lung arteries were exposed to high pressures leading to PAH developing immediately, or many years later

� Pressures in your lungs may have increased after an operation aimed at bringing more blood to the lungs, or you may have segmental (patchy) pulmonary hypertension if blood flow to the lungs is ‘abnormal’

� You may have been left with a residual hole in your heart after your operation

� You may only have a ‘small’ hole in your heart but still develop PAH – the reasons for this are often not clear

4

“When I was diagnosed, I just wanted to know the meaning of Eisenmenger’s syndrome...”

RIGHT SIDE LEFT SIDE

A left-to-right shuntThe blood shunts from the left side of the heart to the right.

Why does CHD sometimes cause PAH?Not all congenital heart disease (CHD) will result in pulmonary arterial hypertension (PAH). The most common reason for PAH to develop in patients with congenital heart disease is a ‘hole in the heart’ that has not been repaired. This allows too much blood to go the lungs.

To begin with, the blood shunts from the left side (high pressure) of your heart to the right side (low pressure) of your heart and then into your lungs. This is referred to as a ‘left-to-right’ shunt. The blood vessels in your lungs react to the high pressure by becoming thicker and narrower. Over time the blood pressure in your lung arteries rises.

What is Eisenmenger’s syndrome?As the blood pressure in your lungs increases it also causes the pressure on the right side of your heart to increase. This allows the blue blood from the right side of your heart to ‘shunt’ to the left and mix with red blood. This is referred to as a ‘right-to-left’ shunt.

Over time the right side of the heart has to work harder to pump blood to the lungs. The hole then becomes beneficial and acts like a ‘relief valve’ by allowing the blue blood to shunt into the left side of the heart and be pumped round the body.

This may allow the right side of the heart to cope for many years with the high pressure before it begins to struggle. The downside is that you can become progressively more blue over time. Once your blood shunts ‘right to left’ your condition is known as Eisenmenger’s syndrome.

RIGHT SIDE LEFT SIDE

A right-to-left shuntThe blood shunts from the right side of the heart to the left.

5

What is cyanosis?Not everyone who has cyanosis has PAH or Eisenmenger’s syndrome. Cyanosis is a condition in which your oxygen levels are lower than normal. This can be caused by blood shunting from the right side of your heart to the left side (right-to-left shunt).

How does cyanosis affect me? � Your body makes more red blood cells to try and carry more oxygen. This makes your blood thicker than normal

� You may notice a blue colour to your skin, particularly your fingers, toes and lips

� You may notice that your fingers and toes develop a ‘clubbed’ appearance

� You may be more prone to headaches

� You may be prone to becoming anaemic and iron deficient, which can make you feel even more tired

If you are cyanosed you are likely to have a high level of haemoglobin – this carries the oxygen in your blood. This is an expected way for your body to compensate for your lower oxygen levels. We no longer recommend the routine use of removing blood to thin your blood (known as venesection). The effects are temporary and it may result in you becoming anaemic and iron deficient.

Routine use of oxygen is not proven to make much difference to individuals with Eisenmenger’s syndrome. Many people do not use oxygen at home at all. There is certainly no need to have oxygen for a prescribed number of hours a day or night, although some people find it useful to have it available when they feel they need it. Oxygen can be helpful when your disease progresses.

� You have gradually become more tired, breathless and less able to do things that you used to be able to do easily

� You may develop cyanosis (see box below to find out more about cyanosis)

� You may start feeling dizzy when you exert yourself

� You may experience some chest pain or discomfort, especially when you are more active

� You may feel your heart racing, going too slow or beating quite irregularly – this may make you feel dizzy and lightheaded

� You may notice a build-up of fluid around your stomach and ankles

The four groups of PAH–CHD

A Eisenmenger's syndrome � Very high blood pressure in the lungs � Right-to-left shunt: blue blood flowing through to the left side of the heart,

leading to cyanosis at rest and/or when you exert yourself

B PAH with left-to-right shunt

� Mild-to-moderate increase in blood pressure in the lungs � Left-to-right shunt: red blood flowing through to the right side of the heart � No cyanosis

C PAH with small ‘hole’ � High blood pressure in the lungs, not clearly due to the ‘hole’ � May develop cyanosis

D PAH after closing a ‘hole’ � Blood pressure in the lungs remaining high or increasing after closure of a ‘hole’ � Usually no cyanosis at rest, unless the ‘hole’ persists and pressures are high in

the lungs

Different types of PAH–CHD can have different treatments. Please speak to your doctor treating your PAH and/or CHD about which group you belong to and what this means for you.

What are the symptoms of PAH in patients with CHD?Many adults with PAH-CHD can remain well for many years. The onset of symptoms tends to be gradual and can vary from person to person. You may notice the following:

Diagnosing and managing PAH–CHDMany patients who have pulmonary arterial hypertension with congenital heart disease (PAH–CHD) are managed at specialist pulmonary hypertension centres. These centres may share a location or work closely together with adult congenital heart disease (ACHD) centres. You may have heard these referred to as grown-up congenital heart disease (GUCH) centres.

You may be cared for by a hospital with a shared care arrangement between the two centres, or be looked after by a specialist congenital heart disease team.

To find specialist centres near you, visit:

� ACHD centres: www.thesf.org.uk/help-advice/specialist-centres.aspx

� PAH centres: www.phassociation.uk.com/treatment_for_ph/ph_specialist_centres.php

How is PAH diagnosed?If you have congenital heart disease (CHD), your doctor will routinely carry out tests to see if you have PAH. Your doctor will assess the function of your heart using an:

� Electrocardiogram (ECG) – this measures the electrical activity of the heart

� Echocardiogram (heart ultrasound) – this measures the shape and size of the heart and can provide information on PAH

Your doctor might suggest cardiac catheterisation. During this procedure, a very thin, soft tube called a ‘catheter’ is used to measure the blood pressures in the heart and lungs. This procedure is usually done under a local anaesthetic. The catheter is guided into the heart through a blood vessel in the leg, neck or arm.

Cardiac catheterisation is the most reliable way of finding out whether someone has PAH. It can also measure how severe PAH is and which treatments might be effective.

How is PAH assessed?To help make the best treatment decisions for you, you and your doctor will need to think about how much PAH affects your everyday life.

The World Health Organization (WHO) has developed a system to grade PAH. Your doctor will use it to assess your condition and the effect that it has on your life. You may find that your Class changes over time. See the table on Page 7 for more information on Classes.

How is PAH treated?You may be given treatment to lower the blood pressure in your lungs. This can improve your ability to be active without becoming very breathless or tired.

There are different types of medication. You should discuss with your consultant or specialist nurse which treatment is likely to work well for you and any potential side effects that it may cause.

The different medications can be taken in the form of:

� Tablets

� By inhaling medication from a special piece of equipment (inhaled nebuliser)

� A mini-pump to administer the medicine straight into the bloodstream (intravenous infusion)

You may also need extra oxygen, especially if your condition has progressed

The types of medication that may be prescribed to treat PAH in the UK are:

� Phosphodiesterase type 5 (PDE5) inhibitors (tablets): sildenafil or tadalafil

� Endothelin receptor antagonists (ERA) (tablets): ambrisentan, bosentan or macitentan

� Prostanoids (inhaled or delivered into the bloodstream): epoprostenol (intravenous), iloprost (nebulised)

� Soluble guanylate cyclase (sGC) stimulator: riociguat

Treatment usually starts with a tablet or a combination of tablets. Over time, you may need additional tablets or to add inhaled or injectable therapy to control your condition.

6

Functional Classification of PAH from the WHO

WHO Class I

� There are no symptoms of PAH with exercise or at rest � You do not notice breathlessness or chest pain, or feel like you are going to

faint, when you undertake ordinary physical activities (such as walking, simple household chores, shopping etc)

WHO Class II � Your physical activity is slightly limited � Ordinary physical activity makes you feel breathless, fatigued, chest pain or like

you are going to faint, but you feel comfortable at rest

WHO Class III � Your physical activity is noticeably limited � Less than ordinary activity causes breathlessness, or fatigue, chest pain or

near-fainting, but you feel comfortable at rest

WHO Class IV

� You cannot undertake any physical activity without experiencing symptoms � There are signs of right heart failure � You may feel breathlessness and/or fatigued even at rest � Your discomfort is increased by any physical activity

7

How often should I see my doctor?Everyone with PAH should see a specialist approximately every 3–6 months. This is especially important if you are taking medication.

You should stay in close contact with your healthcare team between your clinic appointments. Contact your specialist nurse if you have any concerns. If your condition worsens, contact your specialist nurse immediately.

How can I prepare for a clinic visit?It can be helpful to keep a diary. You can keep track of the changes in your symptoms and the amount of activity you can do.

“I write everything down in a diary and I also write how I feel at the end

of every day. I mention anything that stands out at my appointments when my doctor

says ‘How are you?’”

A diary can help you check how your symptoms have changed over time. This will help you to identify changes in how you feel and what you can do over a period of weeks and months. Keep track of any differences between the amount of activity you can do compared with your friends or family and how this has changed over time.

As you fill in your diary, make a note of any questions you have. Your appointments are a good time for you to discuss any queries or concerns you may have about your condition or treatment with your doctor or nurse.

What will happen during a clinic visit?During your clinic visit, your doctor may perform some tests. This may include an echocardiogram (an ultrasound of the heart), electrocardiogram (ECG), blood tests and an exercise test. They will use the results to see if they need to change any aspect of your care.

Your doctor may also ask questions about your daily life and how you have been since your last visit. Even if you are asked a general question, try to give specific examples using every everyday activity, such as:

� The distance you are able to walk

� The number of stairs/steps you can climb

� Washing, cleaning, cooking, gardening etc

� Sports and exercise

� Playing with children

� Coping with work

� Shopping

Try using everyday activities and landmarks to check your progress. For example, “I was able to walk from the bus stop to home without stopping – now I have to stop halfway to rest”.

You can bring your diary with you to help you remember how your symptoms have changed since your last clinic visit.

How will I know if my treatment is working?Once treatment for PAH has been started, it is important that you take it regularly, as agreed with your healthcare team.

You may not notice any changes straight away. It can take up to 3–4 months before you know whether a treatment is working for you. At times, your family and friends may notice an improvement before you do. It is important to give your treatment time to have a noticeable effect.

Are everyday activities, such as walking, climbing stairs or shopping, becoming more difficult? If they are, perhaps your treatment is not working as well as it used to. Keep in mind that you may notice small changes in your symptoms during periods of intense cold or very warm weather. In addition, chest infections can make symptoms worse for a short or even a longer time, as people with PAH may find it more difficult to recover. Speak to your healthcare team about having an annual flu and pneumonia vaccination.

If you feel concerned about how well your treatment is working, or if you feel that your symptoms are getting

worse, talk with your PAH and ACHD specialist doctors, who will examine you, perform tests and talk with you about the best option. If your treatment is not working well, you may be able to change to a different treatment or add another treatment to your existing one (this is known as combination therapy).

It is important to fully understand any changes to your treatment, so don’t be afraid to ask questions and contact your team at any point.

You should not stop taking your treatment without first talking to your doctor or specialist nurse. If you are experiencing significant side effects, you should discuss this with your doctor or specialist nurse as soon as possible.

Will I eventually need transplantation?Some PAH–CHD patients may be referred for transplantation. The availability of effective drugs to treat PAH has delayed the need for transplantation. If transplantation is required, a heart–lung transplantation is often required for patients with PAH–CHD.

8

Living with PAH–CHDIt’s important for you and your family to understand your condition, so you can manage your health and its impact upon your life in the best way possible. In this section, we will discuss how pulmonary arterial hypertension with congenital heart disease (PAH–CHD) can affect your daily life, and ways that you can minimise its impact.

Understanding the conditionPAH–CHD is not a very common condition, and your family and friends may find it difficult to understand it. Some healthcare professionals outside of your specialist team may not be very familiar with your condition. You might find it useful to share this booklet with them.

It is important that you know the name of your condition, your treatment and who to contact in an emergency (see What to do in an emergency on Page 15).

It is useful to carry copies of up-to-date clinical letters with your diagnosis, current status and treatments. Some centres may provide a Patient Passport to hold important information about your condition. Alternatively, you may wish to use the Personal Health Passport also available on The Somerville Foundation website (www.thesf.org.uk).

PAH–CHD and learning disabilitiesPeople with Down’s syndrome are more likely to have complex cardiac problems, and so are at risk of developing PAH–CHD. If you are caring for someone with a learning disability, the healthcare team will be able to help you and the person you are caring for to understand the condition.

If treatment is recommended then the person with learning difficulties will be supported to help them make their own decision as far as possible – if they lack capacity to make this decision for themselves, then a ‘best interest’ decision will be made on their behalf.

“On a good day I can just do normal things...”

“On a bad day I just don’t feel right. I have no get up and go, whatsoever...”

Day-to-day life with PAH–CHDLife with PAH–CHD differs from person to person and from day to day. You will probably have days when you can carry out most everyday activities, such as going for a walk or shopping. At other times, you might have little energy or feel unwell.

Build in additional time to keep active when you can, and balance time for activities with time for rest. Your PAH therapies are there to improve your ability to cope with physical activity and so make it easier for you to perform ordinary activities.

If you have had PAH since childhood, you might underestimate its impact on your life in terms of limiting your day-to-day activities. Discuss with your specialist doctor the different options available to help control your PAH symptoms and help you with your daily activities.

Spending time talking with friends and family can be an important part of feeling positive. Online patient support groups can be a good way to get in touch with other people with PAH–CHD or similar conditions, and to share experiences and support one another. You can find out about different patient support groups available on Page 14. Although these groups may be a good source of support, your healthcare team will be the most accurate source of information relating to your condition. Finding out about activities or groups in your local area could be a helpful option for you. When you go to your appointments, look out for information around the clinic, or just ask your PAH and ACHD team.

9

Do I have to change what I eat and drink?Eating a healthy balanced diet is a good idea for everybody, and even more so if you have PAH–CHD. What you eat can make a big difference to the health of your heart.

Do talk to your PAH and ACHD healthcare teams about your diet. Some helpful suggestions to build into your daily diet and shown below.

Aspects of your diet to consider Advice for eating well

Make sure you eat plenty of foods that are high in fibre

� Eat fresh vegetables and fruit � Choose wholegrain breads and cereals

Try to avoid fatty meats � Choose lean chicken, fish and pulses

Limit saturated fats � Drink skimmed or low-fat milk � Use vegetable oil instead of butter where you can

Avoid hydrogenated oils (trans fats) � Trans fats are found in margarine, chips and fast food

Be careful about how much salt you eat or put on your food

� Don’t add salt to your food during cooking � Smoked meats, snacks and processed foods can

contain high amounts of salt

Don’t drink too much coffee � Try drinks with little or no caffeine such as herbal tea

(although some herbal teas contain caffeine, so check the label)

Avoid or reduce alcohol intake � One or two alchoholic drinks are the current

recommended maximum daily intake � It is best not to binge drink

Am I at risk of developing a heart infection?Endocarditis is an infection of the inner lining of the heart. People with CHD are at risk of developing endocarditis, whether or not they have had an operation to repair their congenital heart defect. It is relatively rare but can have serious consequences if not treated as soon as possible. Please discuss endocarditis with your healthcare team during your visits.

Endocarditis can develop quite slowly over a number of weeks or months, or it can develop quite quickly. You may feel generally unwell, with night sweats, low-grade fever, general aches and pains, or lack of appetite. A fever develops in most cases. Please contact your team immediately if you experience any of the above.

You should take steps to reduce your risk of developing endocarditis:

� Make sure you keep your teeth and gums clean and healthy. Be careful not to eat too many sugary foods, which can lead to tooth decay. Visit your dentist for regular check-ups, and let your dentist know that you have PAH–CHD and may be at a greater risk of developing endocarditis, so that they can appropriately care for you dental health

� Alert any health professional who may need to give you treatment or perform a procedure

� There may be a risk of infection with body piercing or tattooing. Before deciding to get a piercing or tattoo, please speak to your healthcare team

10

Smoking and PAH-CHDIt is best not to start smoking or to give up as early as possible. Not smoking is particulary important if you are waiting for surgery, as it can increase your risks of complications.

Speak to your GP or practice nurse if you would like to find out about the support available to help you quit smoking.

Working and PAH–CHDMost people who have pulmonary arterial hypertension with congenital heart disease (PAH–CHD) are able to work, although they may not be able to carry out some jobs that require a lot of physical activity, such as heavy lifting. What you are able to do depends on the severity of your disease and level of fitness. If you are not sure, talk to your PAH and CHD healthcare team about what is manageable and safe.

“If I have a busy day, I just know that I need to get rest the day before – it is

critical...”

Your employer has a legal obligation to make reasonable adjustments to both your job role and your workplace. For example, they may need to provide special equipment, or adjust your working hours or duties.

You may find that larger employers are better able to support you with any changes you need. If you are a member of a trade union or trade association, they should be able to help with any issues. Always answer questions from your employer or prospective employer to the best of your knowledge.

Check if you have any legal cover through your household insurance, as some policies include cover for medical and employment issues. You can also contact The Somerville Foundation (www.thesf.org.uk) if you cannot obtain free legal advice through any other means.

For more information regarding employee rights and preparing for job interviews, visit:

� www.gov.uk/rights-disabled-person/employment

� www.gov.uk/looking-for-work-if-disabled/looking-for-a-job

� www.dls.org.uk

11

Travelling and PAH–CHDMost people who have pulmonary arterial hypertension with congenital heart disease (PAH–CHD) are able to go on holiday in the UK or abroad when they are stable and well. Make sure you choose activities that are suitable for you.

You should get comprehensive medical insurance before travelling abroad. You will need to tell them about your condition to ensure that your insurance is valid. You may have to speak to several insurance providers to get the cover you need. Make sure you plan your insurance well in advance of your holiday.

If you are having trouble finding insurance, it may be helpful to discuss any difficulties on The Somerville Foundation, British Heart Foundation (BHF) or Pulmonary Hypertension Association UK (PHA UK) forums:

� www.thesf.org.uk/help-advice/resources

� www.bhf.org.uk

� www.phassociation.uk.com/forum

Flying and PAH–CHDPeople with PAH–CHD should not necessarily feel that they cannot use commercial flights.

If you use oxygen, you may also require it during the flight. This is quite easy to arrange through some airlines and may be free of charge. It is always best to check with your airline first. You may also need to check with your travel providers about permission to take your oxygen on trains, ships, coaches or aircraft, and there may be restrictions on the size of the cylinder you can take. Oxygen concentrators are smaller than oxygen cylinders, and may be a better option for travel.

It’s important to move around regularly and drink plenty of water to ensure that you remain well hydrated, both during flights and in warm weather.

Be careful in airports:

� If you need to walk long distances to your gate, you can ask for assistance (e.g. wheelchairs or other transport). Arrange this well in advance of your travel day

� Do not carry heavy luggage

� Remember to keep well hydrated in the airport – this is particularly important if you cannot take water with you through airport security

Some tips for taking medicines on airplanes:

� Carry medicines in their original containers

� Always carry your medicines in your hand luggage, rather than check-in luggage

� If you need to carry syringes, make sure you have a letter from your doctor

� In case of queries, it may be useful to take a copy of your prescription

Take care during long bus, train and car journeys:

� Pull over, stand up or move around about every 1–2 hours

� Ask your doctor about whether you need to take aspirin or another treatment to reduce the risk of deep vein thrombosis (DVT) for long journeys (i.e. over 3–5 hours)

� There are also leg exercises you can do in your seat to reduce the risk of DVT

12

Relationships and PAH–CHDJust because you have pulmonary arterial hypertension with congenital heart disease (PAH–CHD) doesn’t mean that you can’t have intimate relationships and a healthy love life. Through open communication with your partner about how you feel and what you desire, you can enhance your feelings of trust and confidence, and talk through any issues or worries you may have.

If breathlessness and fatigue are restricting your love life, try having sex when you’re feeling at your best – perhaps in the morning or early afternoon. You might consider finding time for sex shortly after taking your medication. This is when the positive effects of your treatment are most noticeable.

Some sexual positions may be difficult or uncomfortable for people with PAH–CHD. It is important that you avoid positions that put weight on your chest, so that you can breathe freely. Be open and honest with your partner, and you can find positions and techniques that you both enjoy.

Pregnancy and contraceptionPregnancy is dangerous for women with PAH–CHD. It can put strain on the heart and lungs, and some of the treatments you may be taking may be harmful to a developing baby. Most women with PAH are advised not to become pregnant.

The use of contraception is therefore very important, so ask your ACHD or PAH healthcare team where you can get appropriate contraception and which methods are suitable for you.

Looking after your familyCaring for children can be quite physically demanding and you may find it difficult to keep up with them. There are many people with PAH–CHD who manage to care for their family with few problems, providing they get enough support from friends and family and tailor activities to suit their abilities.

13

Obtaining oxygen while on holidayIf you use long-term oxygen and are travelling within the UK, you should be able to have your oxygen prescription available at your destination. Discuss your oxygen requirements with your oxygen supplier at least 2 weeks before you travel.

If you are travelling abroad, your oxygen requirements need to be organised separately, and you may be charged. Speak to your oxygen supplier at least 3 weeks before your travel.

Different oxygen suppliers have service teams available who may be able to help arrange oxygen at your destination. Contact your oxygen supplier to find out more.

Getting supportThere may be different ways you can obtain support, including financial and travel support, and support from other people with the same condition.

During your appointments, don’t forget to ask about local services and activities that are available in your area.

Financial supportThere is a range of allowances available from the government to support people with health issues and their carers. Some people who have pulmonary arterial hypertension with congenital heart disease (PAH–CHD) may be eligible for this kind of financial support.

Find out more about UK disability benefits here: www.gov.uk/browse/benefits/disability OR you can talk to an adviser about which benefits you might be entitled to on 0800 882 200

OR you can fill in an online form to find out more about benefits here: www.gov.uk/benefits-adviser

In Northern Ireland you can get advice about benefits from Disability Action here: www.disabilityaction.orgOR apply for benefits online at: www.nidirect.gov.uk/index/information-and-services/people-with-disabilities/health-and-support.htm

Travel support for hospital appointmentsThere are travel cost support schemes run by healthcare services in Great Britain and Northern Ireland. There may be transport organised by your GP or hospital for outpatient appointments. Keep in mind that travel support for hospital appointments may not extend to companions.

For England, Scotland and Wales see: www.nhs.uk/nhsengland/healthcosts/pages/travelcosts.aspxOR telephone: 0300 330 1343

In Northern Ireland see:www.nidirect.gov.uk/hospital-travel-costs-scheme

Patient support groups

The Somerville Foundation

Supporting people with a congenital heart condition offering help and advice. Forum for people with a congenital heart condition

www.thesf.org.ukTel: 0800 854759Email: helpline@thesf.org.ukFacebook: www.facebook.com/thesomervillefoundationTwitter: @_thesf

Pulmonary Hypertension Association UK

UK charity for people with pulmonary hypertension. Provides information, organises events and funds research

www.phassociation.uk.com Tel: 01709 761450Email: office@phassociation.uk.comTwitter: @PHA_UK

Pulmonary Hypertension Association UK forum

Online forum for people with pulmonary hypertension and their family and friends

www.phassociation.uk.com/forum/default.asp

Pulmonary Hypertension Association Ireland

An association for people living in the Republic of Ireland who have pulmonary hypertension. Organises an annual conference and has a newsletter

www.pulmonaryhypertension.ieTel: (01) 8034420/4423Email: pha@mater.ieTwitter: @pha_ireland

Down’s Heart GroupOffers support to people with Down syndrome who have heart conditions and their families

www.dhg.org.ukTel: 0844 288 4800Email: info@dhg.org.ukFacebook: www.facebook.com/groups/downsheartgroup

British Heart Foundation

Help and advice on heart disease and an online forum to share experiences

www.bhf.org.ukTel: 0300 330 3322Email: supporterservices@bhf.org.ukFacebook: www.facebook.com/bhf

Yheart Information, games and advice for young people with heart conditions

www.yheart.netFacebook: www.facebook.com/yheartfanTwitter: @Yheart_Tweets

Irish Heart FoundationProvides information and advocacy for people with heart disease and their families

www.irishheart.ieTel: 1890 432 787Facebook: www.facebook.com/irishheartfoundationTwitter: @Irishheart_ie

14

What to do in an emergencyBefore any emergencies arise, make a clear plan with your team on exactly what you should do in an emergency. They will be able to tell you what to look out for, given your individual condition and the medication you are taking.

Keep a copy of the latest letter containing your clinical information with you, along with an up-to-date list of your current medication and contact details of your specialist teams.

You may also wish to carry your Patient Passport if you have been supplied one by your healthcare team, or a Personal Health Passport which is available on The Somerville Foundation website (www.thesf.org.uk).

In some areas of the country it is possible to register your details with your local ambulance service. This means that when they receive a 999 call from your address they know that you have a complex condition and that you are normally cared for by a specialist team at a specific hospital. The ambulance staff then have the option of taking you to that hospital rather than your local A&E. Please speak to your specialist team about this.

Emergency pulmonary arterial hypertension (PAH) contact details

Name:

Clinic:

Phone number:

Emergency congenital heart disease (CHD) contact details

Name:

Clinic:

Phone number:

General practitioner (GP) contact detailsGP Name:

Clinic:

Phone number:

“Because my condition is so rare, I can’t meet anyone with the same problem where I live so I asked on the forum if anyone had the same as me

and I now talk to the person I met regularly...”

Your emergency contact numbers Make a note of your emergency contact numbers below.

15

Helpline: 0800 854759 or helpline@thesf.org.uk

www.thesf.org.uk

The Somerville FoundationSaracens House, 25 St Margarets Green

Ipswich IP4 2BN

01473 252007Registered Charity No: 1138088