Pulmonary Hypertension is a very rare, very serious condition. There is an increase in pressure in the lungs and pulmonary artery, meaning blood cannot be oxygenated. The rest of the body doesn’t get a sufficient supply of oxygen-rich blood and the patient experiences symptoms such as breathlessness and extreme fatigue. Pressure builds in the heart, increasingly straining it as it develops, eventually leading to heart failure. There is no known cure.
Sarah has Idiopathic Pulmonary Arterial Hypertension, which is a vanishingly rare variant of the condition for which the cause is unknown. The vessels carrying blood from the heart to the lungs constrict and not enough blood can be pushed into the lungs to be oxygenated. When she was in the University Hospital in Oxford, she had a steady stream of Medical Students coming to listen to her heartbeat as it was something they may not come across again for many years. Due to its rarity there are only seven hospitals in the UK which have consultants experienced and able to deal with the condition.
The rareness and difficulty to detect mean the diagnosis for PH patients often takes many years. By that stage the condition is often in an advanced state and their heart is already not functioning properly. Sarah had been to different GPs several times over 8 years describing symptoms which, in hindsight, seem like PH. It was only picked up after 2 hospitalisations within a few months in 2013 and a barrage of tests and assessments.
The Pulmonary Hypertension Association works to improve the lives of people with PH by supporting research, raising awareness and helping with day-to-day life for people affected by PH.
For many people a combination of drugs and other treatments can manage the symptoms of PH. For some, however, their only option is a heart and/or lung transplant.