15 years ago The Pulmonary Hypertension Association (PHA UK) was set up to support those living with the rare condition. The initial focus of the charity was to provide peer-to-peer support for those living with PAH. A lot has changed since then and the charity’s chairman, Iain Armstrong, who was one of the nurses who started the charity, describes the UK’s response to PAH as the “envy of the world.”

Back in 2000 there was no cure or licensed treatment for PAH and life expectancy was just two to three years. Very little was known about PAH, which occurs when the walls of the pulmonary arteries are thick and stiff or blocked by blood clots, making it difficult for them to expand and allow blood through, placing greater pressure on the right hand side of the heart.

Today, whilst there is no cure, there is treatment, investment in research and, with a life expectancy of six to seven years, PAH has been reclassified as a chronic rather than an acute disease. There is even a national audit so everyone diagnosed with the condition, which currently stands at around 7,000 individuals in the UK, is identified and can access specialist care.

“So much has changed over the past 15 years,” recounts Iain, “But the two major breakthroughs have been the licensing of a specific treatment and the development of specialist centres to care for those with PAH.” Those diagnosed can now receive medication that helps to relax the arteries, allowing blood to flow more easily, and get support from one of eight specialist centres around the country. The breakthroughs in treatment have also stimulated further industry research.

 

Risk of misdiagnosis

 

Whilst progress has been great, there’s still a lot to do. PAH can affect anyone, of any age and with unknown causes and vague symptoms, it can take up to two and a half years to diagnose. “The symptoms are so similar to those of asthma or chronic obstructive pulmonary disease, that people are often misdiagnosed,” says Iain. However, he is confident that the UK is in a better position than ever to continue advancing the care, research and general awareness of PAH.

“In terms of awareness, there isn’t the same public narrative that there is around, say, some forms of cancer, even though the outcome is just as bad, or even worse,” explains Iain. “This places a huge stress on individuals when claiming benefits, talking to employers or even their friends about the condition.”

Perhaps the most neglected area that comes with any rare condition is the wider on the general wellbeing of a patient. PHA UK continue to lead the way here, providing resources and connecting patients and families to ensure that those living with PAH have, as Iain says, “the right tools at the right time to make the right decision.”