Pulmonary Arterial Hypertension (PAH)
Imagine that simple, everyday tasks like running for a bus, meeting friends for coffee or reaching for your phone feel like climbing the summit of a mountain. Small actions, which we perform without giving them a second’s thought, suddenly become a huge challenge, making you feel exhausted and breathless. This is the reality for people diagnosed with Pulmonary Arterial Hypertension (PAH), a life-changing condition, which doesn’t just present physical challenges, but also affects the social and emotional wellbeing of both patients and their caregivers.1
Feeling out of breath and tired after intense physical exercise is a normal reaction of the human body, especially when it is not used to regular workouts. But feeling like this while undertaking mundane activities, such as carrying your shopping bags, is not normal.
Pulmonary Arterial Hypertension (PAH), a type of Pulmonary Hypertension, is a progressive disorder associated with a number of structural changes to the pulmonary arteries.2 In simpler terms, it means that the blood vessels that carry blood from the heart to the lungs become thickened and narrowed and restrict the blood flow, which raises the blood pressure in the lungs. As a result, the heart has to work harder to pump blood to the lungs, and it can become tired and overworked.3 Because less blood circulates through the lungs, less oxygen is picked up, resulting in the patient feeling tired and out of breath.
undefined component is not yet defined.
What are the symptoms of Pulmonary Arterial Hypertension?
PAH is a progressive disease, which is often difficult to diagnose at an early stage as initial symptoms such as shortness of breath and fatigue can often be mistaken for other common lung conditions such as asthma and sleep apnoea.4 When left untreated, PAH can cause the heart to overwork and weaken and eventually fail. An early diagnosis can make a significant difference in the patient’s prognosis.
More severe symptoms include chest pain, a racing heartbeat, and leg and ankle swelling.5 It is important to understand that each person experiences PAH differently and the condition doesn’t follow a strict sequence of symptoms.
Once your doctor suspects your symptoms are caused by PAH, they will refer you to a specialist centre where a series of tests will be conducted to confirm the diagnosis and exclude any other possible causes. Common tests2 are:
- Electrocardiogram (ECG)
Electrodes attached to the skin detect the heart’s electrical activity, providing important information about the size of the heart chamber and possible tissue damage. - Echocardiography
An ultrasound which gives detailed insight into the state of the heart, such as the heart’s pumping abilities. - Ventilation / perfusion lung scan
A scan that looks for blood clots in the lungs.
- Pulmonary function tests (PFT)
A test that measures the volume of air and how well it moves in and out of the lungs.
- Right-heart catheterisation6
The only conclusive test to diagnose PAH; all other tests may increase the suspicion of PAH and are used to evaluate possible causes. A very thin and flexible tube is inserted into the right side of the heart and into the pulmonary artery to measure the blood pressure in the heart and lungs, and how well the heart pumps per minute. Patients are awake but sedated to avoid any discomfort.
- 6-minute walk test
A test that records the patient’s physical performance while walking to better understand health status.
How common is Pulmonary Arterial Hypertension?
With only 15 to 60 people in a million affected, awareness about PAH is still very minimal. It can affect people of every age, sex and ethnic background, but is found to be most common among women aged between 50 and 65 years.7
While it is still not clear what exactly causes PAH, studies have identified several of the mechanisms behind the disease. There are different possible types,8 which include:8
IdiopathicPulmonaryArterialHypertension | Heritable Pulmonary Arterial Hypertension | AssociatedPulmonaryArterialHypertension |
|---|---|---|
IdiopathicPulmonaryArterialHypertension No obvious cause can be identified.8 | Heritable Pulmonary Arterial Hypertension A form of PAH that can be identified in patients who have a family history of the disease; or carry a mutation in a gene associated with PAH.98 | AssociatedPulmonaryArterialHypertension When PAH occurs in patients with other conditions such as connective tissue disease, congenital heart disease, HIV or liver diseases. Drug abuse and certain medications, such as diet pills, are also known to cause the development of PAH.8 |
9: https://oxfordmedicine.com/view/10.1093/med/9780198784906.001.0001/med-9780198784906-chapter-590
Treatment and Care
Unfortunately, no cure for PAH has been found yet, but in the past decade, several studies have triggered great improvements in treatments that can help slow down the disease’s progress and improve a patient’s quality of life significantly. An early diagnosis can change the outcome drastically.
There are several specific medicines2 that influence the biological processes that play a role in the development of PAH. These will help widen blood vessels and prevent the overgrowth of cells in the blood vessel walls, making it easier for the heart to pump blood to the lungs and provide more oxygen, slowing down the disease’s progress in time.
Several other treatments2 can also help with relieving symptoms:
- Blood thinners: prevents blood clots.
- Diuretics: reduces swelling in the legs and ankles.
- Digoxin: increases heart strength and slows down the heart rate.
- Oxygen therapy: provides supplementary oxygen to counter a deficiency.
Since everyone’s body is different, it can take some time to find a treatment that works. Be prepared for extensive follow-up care to make sure doctors find the best result for you.10
In very severe cases when the disease progresses despite the medication, a lung transplant may be considered.2
Living with Pulmonary Arterial Hypertension
Living with PAH can be an immense challenge, both physically and mentally. People with PAH may be heavily reliant on others to perform everyday tasks, which can severely affect their feeling of self-worth. It may become difficult to maintain social relations, leading to feelings of isolation. Misconceptions about PAH can exacerbate feelings of being misunderstood and marginalised.
It is important to know that you are not alone, and these feelings are very common. Be open about your condition and how it affects you. Share your feelings, thoughts and experiences with loved ones, friends or with your doctor or nurse.
Sometimes, it can be difficult to talk about certain topics with those close to you. Patient associations and social services can be a great source of support and comfort due to their vast experience with PAH. Reputable PAH-specific online support forums and associations can also be valuable should it be difficult to find local support groups for this rare disease.
Make sure to take care of your physical wellbeing as well. You can take control of PAH by making healthy choices. Cut back on salt and sodium, avoid stimulants such as coffee and alcohol, and include plenty of iron in your diet.11
What to ask your doctor?
- How advanced is the disease, and what can I expect?
- What would be the best treatment for my disease?
- Should I make any special accommodations for school, home, or my work?
- Should I get formal care?
- How will this affect my day-to-day life?
- Can I exercise? What forms of exercise are appropriate for me?
- Can any lifestyle changes positively affect my condition?
- Is there any medication that can make my condition worse?
- Will I need surgery? What does surgery entail?
- Can I be referred to patient support groups?
Janssen & Pulmonary Arterial Hypertension
PAH has recently become a new area of focus for Janssen, working to transform the disease into a long-term manageable condition, so that patients can live a normal life.
Driven by science and striving to develop and bring to market innovative treatments that improve PAH patients’ quality of life and increase their life expectancy,12 we are working to reach more patients and aim to help tackle the diagnosis gap that is delaying access to the care that patients need.
Clinical trial data is helping us find a new effective drug to target PAH, for which research at Janssen is currently underway.13
Glossary
- Angina: chest pain or pressure
- Cyanosis: bluish colour to skin or lips
- Dyspnoea: shortness of breath
- Oedema: swelling in the ankles
- Hypoxia: a low concentration of oxygen in the blood
- Syncope: dizziness or fainting spells
Patient advocacy groups and external sources
This website is developed exclusively by Janssen Pharmaceutica NV. Please note that the patient advocacy groups, and external sources listed below are an additional and independent source of information you might find useful. These groups and sources were not involved in the creation of this website and do not endorse its content in any way.
Every 5th of May, Pulmonary Hypertension groups and organisations around the world participate in activities to raise awareness of and pay tribute to people living with Pulmonary Hypertension (PH).
PHA Europe is an international umbrella organisation that identifies the needs of those living with Pulmonary Hypertension and recommends effective ways to address those needs.
The European Lung Foundation (ELF) was founded by the European Respiratory Society (ERS). It brings together medical professionals, and patients and their loved ones, to inform on lung health.