PAH with CHD/Eisenmenger`s syndrome PDF

Supporting young people and adults
born 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
Introduction
This 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.
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.
Contents
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:
The heart pumps blood through to the lungs and to the
rest of the body.
RIGHT SIDE
„„ You breathe air into your lungs and oxygen is absorbed
into your bloodstream
„„ The heart is then responsible for pumping this oxygenrich 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
LEFT SIDE
Lungs
Low pressure
High pressure
Pulmonary artery
Aorta
Heart
The normal heart
Veins
The heart has four chambers:
Arteries
„„ 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.
A normal heart
RIGHT SIDE
Blood from
body
LEFT SIDE
Blood to body
(high pressure)
Blood to lungs
(low pressure)
Blood from lungs
Blood from
body
In the normal heart, the blue and red blood never mix.
What is congenital
heart disease?
What is pulmonary
arterial hypertension?
Congenital heart disease (CHD) is a ‘structural’ heart
problem that some people are born with. Congenital means
it is present at birth.
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.
3
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?
Other types of congenital heart disease that
may lead to PAH
As the blood pressure in your lungs increases it also causes
the pressure on the right side of your heart to increase. This CHD can sometimes be more complex than just a hole in the
allows the blue blood from the right side of your heart to heart. For example:
‘shunt’ to the left and mix with red blood. This is referred to
as a ‘right-to-left’ shunt.
„„ Your congenital heart problem may have been repaired
quite late and, despite a good repair, your lung arteries
Over time the right side of the heart has to work harder to
were exposed to high pressures leading to PAH
pump blood to the lungs. The hole then becomes beneficial
developing immediately, or many years later
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.
“When I was diagnosed, I just wanted to know
the meaning of Eisenmenger’s syndrome...”
„„ 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
A left-to-right shunt
A right-to-left shunt
The blood shunts from the left side of the heart to
the right.
The blood shunts from the right side of the heart to
the left.
RIGHT SIDE
4
„„ 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’
LEFT SIDE
RIGHT SIDE
LEFT SIDE
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:
„„ 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
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.
5
Diagnosing and
managing PAH–CHD
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.
Many 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.
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.
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.
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.
To find specialist centres near you, visit:
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.
„„ 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 treated?
The different medications can be taken in the form of:
„„ Tablets
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.
„„ 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
How often should I see my doctor?
What will happen during a clinic visit?
Everyone with PAH should see a specialist approximately
every 3–6 months. This is especially important if you are
taking medication.
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.
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.
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.
7
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
8
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.
Living with PAH–CHD
It’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.
“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...”
Understanding the condition
Day-to-day life with PAH–CHD
PAH–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.
Life 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.
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).
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.
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 disabilities
People 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.
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.
Aspects of your diet to consider
Do talk to your PAH and ACHD healthcare teams about your
diet. Some helpful suggestions to build into your daily diet
and shown below.
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
Smoking and PAH-CHD
It 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.
Am I at risk of developing a heart infection?
You should take steps to reduce your risk of developing
endocarditis:
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.
10
„„ 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
Working and
PAH–CHD
Most 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/lookingfor-a-job
„„ www.dls.org.uk
11
Travelling and
PAH–CHD
Most 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–CHD
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:
People with PAH–CHD should not necessarily feel that they
cannot use commercial flights.
„„ Pull over, stand up or move around about every
1–2 hours
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.
„„ 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)
12
„„ There are also leg exercises you can do in your seat to
reduce the risk of DVT
Obtaining oxygen while on holiday
If 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.
Relationships and
PAH–CHD
Just 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 contraception
Pregnancy 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 family
Caring 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
Getting support
There 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 support
There 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.org
OR apply for benefits online at: www.nidirect.gov.uk/index/
information-and-services/people-with-disabilities/healthand-support.htm
Travel support for hospital appointments
There 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.aspx
OR 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.uk
Tel: 0800 854759
Email: [email protected]
Facebook: www.facebook.com/thesomervillefoundation
Twitter: @_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 761450
Email: [email protected]
Twitter: @PHA_UK
Pulmonary
Hypertension
Association UK forum
Online forum for people with pulmonary
hypertension and their family and
friends
An association for people living in the
Republic of Ireland who have pulmonary
hypertension. Organises an annual
conference and has a newsletter
Pulmonary
Hypertension
Association Ireland
www.phassociation.uk.com/forum/default.asp
www.pulmonaryhypertension.ie
Tel: (01) 8034420/4423
Email: [email protected]
Twitter: @pha_ireland
www.dhg.org.uk
Tel: 0844 288 4800
Email: [email protected]
Facebook: www.facebook.com/groups/downsheartgroup
Down’s Heart Group
Offers support to people with Down
syndrome who have heart conditions
and their families
British Heart
Foundation
Help and advice on heart disease and an Tel: 0300 330 3322
Email: [email protected]
online forum to share experiences
Yheart
Information, games and advice for
young people with heart conditions
www.bhf.org.uk
Facebook: www.facebook.com/bhf
Provides information and advocacy for
Irish Heart Foundation people with heart disease and their
families
14
www.yheart.net
Facebook: www.facebook.com/yheartfan
Twitter: @Yheart_Tweets
www.irishheart.ie
Tel: 1890 432 787
Facebook: www.facebook.com/irishheartfoundation
Twitter: @Irishheart_ie
“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...”
What to do in an
emergency
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).
Before 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.
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.
Your emergency contact numbers
Make a note of your emergency contact numbers below.
Emergency pulmonary arterial
hypertension (PAH) contact details
Name:
Clinic:
Phone number:
Emergency congenital heart disease
(CHD) contact details
Name:
Clinic:
Phone number:
GP Name:
General practitioner (GP) contact details Clinic:
Phone number:
15
Helpline: 0800 854759
or [email protected]
www.thesf.org.uk
The Somerville Foundation
Saracens House, 25 St Margarets Green
Ipswich IP4 2BN
01473 252007
Registered Charity No: 1138088