Actelion PAH

Are you ready to get a glimpse of what it is like to live with PAH?

Introduction
Living with Pulmonary Arterial Hypertension

Being diagnosed with pulmonary arterial hypertension (PAH) may be hard to accept. It can cause a variety of feelings, from shock and disbelief, to anger, fear, or sadness. It may also be a relief to leave uncertainty behind, to put a name to this condition, and learn how it can be managed.

Understanding PAH

Our bodies are amazing at doing what we want them to. When we run for a bus, or reach for our phones, our bodies instantly translate the oxygen we breathe and our energy reserves into coordinated movement, and we don’t have to give it a second’s thought.

It may be hard to believe, but for people living with PAH simple everyday tasks that you and I take for granted can feel as tough as climbing the summit of Everest.

What is Pulmonary Arterial Hypertension (PAH)?

PAH is a serious condition that affects the blood vessels that carry blood from the heart to the lungs. To understand more about PAH, first it helps to understand how the heart works.

“It’s like climbing a mountain... Like something is pushing you back all the time.”

Patient with PAH

Signs and symptoms of PAH

The first symptoms are often noticed when undertaking physical activities, such as climbing up stairs, running, walking uphill, or carrying bags.

As PAH progresses, these signs and symptoms may be experienced more often and may start to impact the patient’s ability to undertake everyday activities.

  • Chest pain
  • Limited excercise capacity
  • Fainting
  • Chest tightness
  • Abdominal bloating
  • Breathlessness
  • Swollen ankles and legs
  • Palpitations
  • Fatigue
  • Angina
  • Weakness

Who gets PAH and what causes it?

“We know a lot more now than we used to about how the disease develops, and it could be anyone who finds they are diagnosed with PAH.”

  • Q: How common is PAH?
    • PAH is a rare condition that can affect women, men, and children of any age or ethnic background.
    • Only 15 to 50 people in a million are affected by PAH. This is why many people do not know about PAH.
  • Q: What causes PAH?

    We still do not fully understand PAH. But while the exact cause of PAH is unknown, some of the disease mechanisms that lead to its development are known.

Types of PAH

There are many types of PAH. Often it is idiopathic, which means it is not linked to any other condition. Rarely it can run in families.

In some cases, PAH may be associated with other conditions, such as congenital heart disease or connective tissue disease. Some of the causes are known and some have yet to be identified. A PAH doctor can help people with PAH understand what type of PAH they have. Regardless of what type of PAH they have, the treatment options remain the same.

Diagnosis and treatment of PAH

PAH is a progressive disease and the rate at which it worsens varies depending on the type of PAH.

Early diagnosis is important as it has been shown to be linked with better outcomes. However, PAH is difficult to diagnose. A key problem is that the early symptoms (breathlessness, dizziness and fatigue) are not very specific and can be easily confused with many other, more common conditions such as asthma, chronic obstructive pulmonary disease (COPD) or sleep apnea.

Progression of PAH

Functional Class is a common term used in PAH. It is a critical element of the assessment of patients with PAH and determining treatment planning.

Class 1

Physical activity is not limited. Ordinary physical activity does not cause extra tiredness, rapid heartbeat, or shortness of breath.

Class 2

Physical activity is slightly limited. Comfortable at rest, but ordinary physical activity results in tiredness, rapid heartbeat, or shortness of breath.

Class 3

Greater limitation of physical activity. Comfortable at rest, but less than ordinary activity causes tiredness, rapid heartbeat, or shortness of breath.

Class 4

Unable to do any physical activity without discomfort. Symptoms of heart problems at rest. Any physical activity increases discomfort.

PAH diagnostic tests

“There is no single test to diagnose PAH”

Due to its complexity, PAH is usually diagnosed at specialist centres and involves a series of tests to rule out other possible conditions, and confirm diagnosis.

  • Electrocardiogram (ECG)

    This test uses electrodes which are attached to the skin to detect and record the electrical activity of the heart over time. It shows heartbeats and provides important information about the heart chamber size and position and tissue damage.

  • Echocardiogram

    This test uses ultrasound to image the heart and can help diagnose, manage, and follow-up patients with any suspected or known heart disease. Among many things, it shows the size and shape of the heart chambers, the thickness of the muscular walls, the heart’s pumping ability, and how well the heart relaxes. It can also provide an estimation of the pressure in the heart and the pulmonary arteries.

  • Ventilation/perfusion scan (V/Q SCAN)

    This test uses ultrasound to image the heart and can help diagnose, manage, and follow-up patients with any suspected or known heart disease. Among many things, it shows the size and shape of the heart chambers, the thickness of the muscular walls, the heart’s pumping ability, and how well the heart relaxes. It can also provide an estimation of the pressure in the heart and the pulmonary arteries.

  • Pulmonary function tests (PFT)

    This test measures how well air moves in and out of the lungs, the volume of air that can be held and how effectively oxygen and carbon dioxide is transferred.

  • Right heart catheterization

    Diagnosis of PAH is confirmed using a technique called right-heart catheterisation. A very thin, flexible tube to measure the blood pressure, oxygen content and cardiac output in the chambers of the heart and arteries is introduced through the skin into blood vessels and from there to the heart.

  • 6-minute walk test

    Following the diagnosis, the 6-minute walk test is done to better define the clinical status of the patient. This test records the best distance walked in 6 minutes. It gives an impression about how well a patient is coping with everyday tasks.

PAH therapy options

It is important to start treating PAH as early as possible.

Treating PAH as directed by a PAH doctor can help keep PAH from getting worse. While there is no cure for PAH yet, there are treatments that can help people manage their PAH.

  • More than one medicine

    To help people with PAH get the best quality of life possible, PAH doctors may give them one, or more than one, medicine at a time. The PAH doctor will choose the medicine (or medicines) that will best help each person with PAH do things today and help them continue to reach future goals. Adjustments in PAH treatment may not necessarily mean something is wrong, it may just be required to optimize the treatment.

  • Lung transplantation

    In very severe cases of PAH, when PAH medications are not successful, lung transplantation (or rarely heart and lung transplantation) may be considered by the PAH doctor.

Burden of PAH

“IT’S NOT A GOOD FEELING – IT'S BAD TO BE DEPENDENT ON OTHERS. IT'S BAD TO ASK OTHER PEOPLE TO HELP YOU. YOU FEEL BAD. YOU FEEL INFERIOR – LIKE A SECOND CLASS CITIZEN”

PATIENT WITH PAH

Whether walking to the local shops, picking up the children from school, meeting friends for a coffee, planning a holiday or the future, PAH is there at the forefront of their lives.

The international PAH patient and carer survey, supported by Actelion, revealed that the impact of PAH on those living with the condition extends far beyond the established physical symptoms of the disease itself.

Impact of PAH on
the patient

“IT TOTALLY CHANGES YOUR LIFE – YOU CAN’T IGNORE IT.”

PATIENT WITH PAH

PAH affects many aspects of daily life and it can take some time to adjust to living with the condition. It is important to know that many patients share similar experiences as they adapt to life with PAH, as uncovered by the international survey.

  • Emotional wellbeing

    Both living with the physical symptoms of PAH and making lifestyle changes as a result, can have an emotional impact on the patient. Many patients are treated for depression, or regularly visit the doctor due to it. Many more, however, experience three or more depressive symptoms, without acknowledging that they have depression. It is therefore important for patients to talk to their doctors about how they are feeling beyond their physical symptoms.

  • Friends and family

    'MY FRIEND SAID, "I WISH I HAD YOUR KIND OF HYPERTENSION" AND I WAS SO UPSET BY THAT. SHE THINKS I AM AT HOME AND JUST SITTING AROUND BEING LAZY. THEY DON’T UNDERSTAND I CAN’T DO IT, NO MATTER HOW MUCH I WANT TO.'

    The lack of awareness of PAH impacts on the wellbeing of over half of all patients, many patients report feelings of isolation, often caused by their PAH not always being physically ‘visible’. More than 40% of patients also reported experiencing loss of libido/interest in sex.

  • Home, work and income

    Almost half of patients said they were unable to work or needed help to work as a result of their condition. Nearly three quarters of patients who had to stop or change their work due to PAH said this had affected their overall household income. Day-to-day chores can prove to be a significant challenge, 83% of patients find their condition negatively impacts their ability to do their domestic housework.

  • Leisure activities

    'PAH CAN BE ISOLATING, SOMETIMES I HAVE NO DESIRE TO SOCIALIZE – SOMETIMES I DON'T WANT TO GO OUT AND TALK TO PEOPLE.'

    Almost all of patients found their ability to take part in sports or exercise was affected and over half said their leisure and cultural activities were affected.

Impact of PAH on
the carer

“LIVING WITH SOMEONE WITH PAH IS LIKE NOT KNOWING WHAT TO EXPECT, I DON'T KNOW HOW IT WILL DEVELOP.”

CARER

PAH affects many aspects of daily life and it can take some time to adjust to living with the condition. It is important to know that many carers share similar experiences as they adapt to looking after someone with PAH, as uncovered by the international survey.

  • Emotional wellbeing

    Two out of three of carers report feeling afraid of what the future holds for their relative, and 21% worry that they ‘should be doing more’.

  • Friends and family

    It was reported that 30% of carers said they felt isolated or excluded as a result of their role. Carers see the overall impact of their relatives’ PAH on their daily lives as very high. Almost three-quarters of partners of PAH patients reported a decrease in sexual relations and intimacy, due to the carer’s fear of making the person they care for more ill.

  • Home, work and income

    Just under a third of carers reported stopping work or changing their working conditions as a result of caring for a person with PAH, with 13% stopping work completely. 45% of carers are now involved in the tasks their relative used to manage, with 43% reporting they feel exhausted more frequently due to the extra responsibility. If you are a carer, naturally the burden falls on you to do more and more.

  • Leisure activities

    Half of carers’ time is spent caring for someone with PAH. One third of carers felt socially isolated or excluded and, of these, 61% found this had a significant impact on their daily life.

PAH practical guidance

EVERYONE WILL EXPERIENCE THEIR OWN REALITY OF LIVING WITH PAH.

If you are a patient with PAH, this section has been developed to provide you with additional information on what to expect when living with PAH and practical guidance to help you manage your feelings, manage your well-being, and move forward with a life beyond PAH.

Living with and managing PAH symptoms can have a significant emotional impact. It is important to know that you are not alone. These feelings are very common among people with PAH. Your family, friends and other important people in your life, together with your PAH doctor and your patient associations, are there to support you when you need it.

How to explain PAH to your family and friends

  • Share your thoughts and feelings

    People who do not know about PAH may not fully understand how you are feeling. To them, you may look and seem healthy in terms of your appearance. But underneath, you may be experiencing a range of feelings or limitations. You may wish to share some information about how PAH affects you. This can help others understand why you cannot always do what you used to or want to do.

  • Talk about your condition

    Although, at first, you may feel embarrassed to talk openly about PAH and its effects on your quality of life, talking to someone can be helpful. You may find comfort in sharing your thoughts, feelings, and experiences with a friend or relative and with your PAH doctor or nurse. A good relationship with others allows you to discuss your concerns more easily. The more your support network knows and understands about you, the better they can help.

  • Talking to people who know PAH

    Sometimes it is difficult to talk to your family or friends about certain topics. In this case, ask your PAH doctor for help in finding someone in your area who you can talk to, like a social worker, counsellor, or psychologist. Patient associations can also be a great source of support and comfort in helping you with all aspects of day-to-day living with PAH.

  • Learn from others’ experiences

    Speaking with other patients that are doing well with PAH can be a great way of learning how to manage your own condition. In this video, Hall Skaara, President of the Norwegian patient association, discusses his experience of diagnosis and living well with PAH.

Take care of your health

“BEFORE I DID RUNNING AND NOW I DO WALKING, I TRY TO FOCUS ON THE THINGS THAT I CAN DO.”

PATIENT WITH PAH
  • Set goals and make active choices

    There are many choices you can make to help maintain your health whilst living with the symptoms of PAH. Making these choices can help you to achieve the things you want. Eating healthily, being active, learning how to cope with tiredness, and sharing your feelings are just some of the things that can make a big difference to how you feel and how you adjust to living with PAH.

  • Talk about your condition

    Just like everyone else, you should aim to eat healthily and to achieve and maintain a healthy weight. If you notice any significant weight loss or gain, tell your PAH doctor. Speak with your doctor about additional considerations for eating well with PAH. Certain foods, especially those with a high salt content, can contribute to swelling or build-up of excess fluid in your body. Your doctor will also be able to advise you on any food or vitamins that may interact with your medicines and therefore should be avoided.

  • Talking to people who know PAH

    What is the right level of exercise for you? It may be hard to know how much or what type of exercise is possible with PAH and the protocol for exercise is individualized for each patient. With help from your PAH doctor, you can figure out the level of activity that is appropriate. Choose activities that you enjoy and that make you feel good.

    Here are some helpful tips:

    • Start out slowly and listen to your body
    • Have enough breath to be able to talk
    • Adjust your activity before you become tired
    • If you experience any symptoms that concern you, for example dizziness or chest pain, seek medical advice

    If you have any concerns or simply want to have reassurance that you are doing the right amount of activity, speak to your PAH doctor. Gym instructors, personal trainers, or people with similar jobs may not understand how PAH can affect an individual.

Additional areas to
think about

  • Join a patient association

    "THE PATIENT ORGANIZATION KEEPS YOU UP TO DATE WITH THINGS AND YOU'RE NOT ALONE"
    - PATIENT WITH PAH

    There are many patient associations across the world that are dedicated to supporting patients, carers and their families to live well with PAH. If you haven’t already joined a patient association or are unfamiliar with what they offer their members, patient associations can be invaluable in supporting patients and their families to learn more about living well with the condition, meet other people affected by PAH and share experiences, and provide advice on welfare and benefits you may be entitled to in your country of residence. To find a patient association close to you, click here.

  • Managing tiredness

    A common symptom of PAH can be extreme tiredness. It may feel different from feeling run down or tired from lack of sleep. Getting to know your body and understanding the signs it gives are important. This will enable you to rest when your body needs it.

    Keeping notes or a diary of when you have felt particularly tired will help you to notice, and then avoid, specific triggers. A diary can help you know the times of the day that you are at your best and which activities use the most energy. This can help you adapt to situations and plan for both your everyday activities and the bigger events in your life.

  • Starting a family

    Pregnancy is very risky and associated with an increased chance of severe complications for the mother and the unborn child. It is highly recommended to speak to your PAH doctor and seek medical advice if you plan to start a family or become pregnant.

  • Working

    Your ability to keep working is based on how you feel, the choices you make, and the type of job you have. Your employer may offer you some flexibility as well. The social worker in your PAH Center or your patient association may be able to provide information on laws relating to your rights in the workplace or what to do if you are self-employed.

  • Education

    For children still at school and people in higher education who have PAH, consider speaking to the appropriate person in authority at that institution so that they can provide support if practical changes are needed, for example rearranging a timetable, assignments or exams. Reach out to your PAH Center or your patient association for further information.

  • Social Services

    Ask at your PAH Center for advice on finding more information on support services that are available to you.

  • Travelling

    It is important that you talk with your doctor if you plan to travel, especially if you plan to take a flight or visit places at higher altitudes.

    Altitude may cause breathing difficulties, so you should check well in advance with your doctor before booking your flight. You may need supplemental oxygen on board or other additional assistance at the airport. It is also important to ensure that you are covered by the appropriate insurance (i.e. medical and/or travel insurance).