Word On Pulmonary Arterial Hypertension (PAH)

About Pulmonary Arterial Hypertension (PAH or PH)  PAH is a disease in which the blood pressure in the arteries in the lungs elevates putting pressure on the heart and reducing the amount of oxygen that is able to reach the tissues of the body causing breathlessness and exhaustion.  This can significantly impact the sufferer’s ability to lead a normal life.

PAH is often mistakenly diagnosed by health professionals due to its similarities to the more common asthma and research shows almost three-quarters of PAH sufferers have had to see more than three doctors and just over a quarter have had to visit over five before being correctly diagnosed.

People with PAH experience a range of symptoms, the most common of which are breathlessness, dizziness, fainting, chest pain, palpitations, and increased lethargy. These symptoms are described as "non-specific" which means it is quite difficult to diagnose.

PAH can often lead to heart failure and can be a standalone condition or associated with other diseases.

Untreated, PAH has a worse prognosis than many forms of cancer. 

PAH affects people of all ages (including young children).  It is twice as common in women as men and the commonest age at presentation is 40 - 50 years old.

While there are currently approximately 4,000 diagnosed PAH sufferers across the UK, the lack of awareness amongst healthcare professionals and frequent misdiagnosis make the actual number of sufferers harder to estimate.

PAH affects people of all ages (including young children).  It is twice as common in women as men and the commonest age at presentation is 40 - 50 years old.

While there are currently approximately 4,000 diagnosed PAH sufferers across the UK, the lack of awareness amongst healthcare professionals and frequent misdiagnosis make the actual number of sufferers harder to estimate.

Although there is no cure, there are effective and relatively new treatments that can help sufferers if they are diagnosed early in the disease progression.

With correct treatment and faster diagnosis, almost two-thirds of PAH patients will survive longer than 5 years, however, if not treated, approximately half of PAH patients die within two years.

There are 8 PAH specialist centres in the UK, (4 in London including Great Ormond Street with the remainder situated in Glasgow, Sheffield, Newcastle, and Cambridgeshire) which provide expert diagnosis, treatment, and management for PH patients.  Given the lack of understanding within the healthcare community in general on this very complex disease, the specialist centres provide beacons of information and support for patients and families affected by PAH.

Thanks to recent advances in medical understanding and treatments the future for patients with PAH is getting increasingly brighter.  However, there is still some way to go before experts fully understand the causes of PAH and formulate drugs to combat or even prevent the disease from developing.

Transplantation: Heart-lung transplantation is a procedure offered to patients when all possible medical treatments have proved to be ineffective.  Therefore patients who receive transplants are very ill and have a very poor quality of life. This treatment is limited by the availability of organs that are good enough to use.  Sadly this means that some patients die whilst waiting for suitable organs.

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