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Being diagnosed with PAH may be hard to accept. It could cause a variety of feelings, from shock and disbelief, to anger, fear, or sadness. However, it may also be a relief for someone to leave uncertainty behind, to have a name for their condition, and find out that it can be managed with the optimal treatment.
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.
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.
Adapted from Galiè N, et al. Eur Heart J 2010; 31:2080-6.
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.
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.
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.
"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.
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.
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.
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.
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.
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.
Regardless of the fact that you have just learned you have PAH, or have been living with PAH for a while, you are still you. Making adjustments to your everyday life, based on how you are feeling and what you want to do, will help you stay in control of your life.