Tess Jewson often gets odd stares when she parks her car and displays her disability badge. She just doesn’t look ill a lot of the time.
But appearances can be deceptive, as Tess knows only too well.
In fact, she has a rare heart and lung condition, so serious that when it was diagnosed at the age of just 17 she was advised to bring forward her 18th birthday celebrations, originally planned for nine months later.
“They just didn’t know what my prognosis was,” says Tess, “only that I was really, really ill.”
Tess, now 27, has pulmonary hypertension (PH), a life-limiting illness that causes high blood pressure in the blood vessels connecting the heart and lungs.
She is among sufferers who are calling for more awareness and understanding of the disease following the results of a survey by the Pulmonary Hypertension Association (PHA UK), which shows PH has a major impact on people’s quality of life.
Tess, who lives in Chelmsford and works as a music therapist, was studying for A-levels when her condition was diagnosed. She was originally told by doctors that her symptoms were down to asthma. It was only when she collapsed with chest pains that she was taken to hospital and tests that revealed the truth.
“Not many people know what pulmonary hypertension is,” she says. “And when you do explain it to them, they think it’s just high blood pressure. As well as it being rare, it’s also invisible, and that’s what I find tough. I have a disability badge for my car, and I get a lot of looks because when I go out I slap on the make-up so I can look my best, and not look ill.”
Pulmonary hypertension is very rare, affecting just 7,000 of the UK population. PHA UK’s Living with PH Survey, which looked at all aspects of life with PH, discovered that emotional and mental wellbeing and relationships with family and friends are severely affected by the condition. It also revealed that 48% of patients waited over a year after first experiencing symptoms before being diagnosed, and 40% had to see four or more doctors.
Tess, who is treated at the Royal Brompton Hospital in London, says: “I was originally told my symptoms of breathlessness were down to asthma and I tried every inhaler going. No-one realised it was a symptom of pulmonary hypertension.
“(When I was diagnosed) I didn’t have a clue what pulmonary hypertension was. I was totally lost.
“There are times when I do think about what’s ahead of me and I feel sad. I know things will get bad but until that time comes, I’m going to continue living the life I’m leading and think of the now, not the future.”
Almost 600 patients responded to the Living with Pulmonary Hypertension survey, which reveals that concerns about life expectancy have the biggest impact on quality of life - with over 50% reporting a ‘major impact’. Almost two thirds (63%) said that financial worries had an impact on their lives.
PHA UK is using the results of its survey to campaign for public policy changes that will improve quality of life for people with PH. Its advocacy programme, PHocus2021, is aiming to reduce the time to diagnosis, ensure access to treatments and reduce financial hardship incurred by living with the disease.
Iain Armstrong, chair of PHA UK, says: “Everyday life can be very challenging for people with pulmonary hypertension and the results of this survey show just how much the condition impacts upon quality of life for patients and their loved ones. This was vital research into what it means to have PH in the UK today and it provides concrete evidence which can be used to address the crucial need for targeted treatment and specialist care.”
Symptoms of PH typically involve breathlessness, fatigue, black-outs and swelling around the ankles, arms and stomach. PH affects the ability to carry out basic tasks and get around.
Adult patients with PH are treated at seven specialist centres across the UK, and children with the disease are treated at Great Ormond Street Children’s Hospital in London.
To see a video of Tess talking about life with pulmonary hypertension go to https://www.youtube.com/watch?v=5tmdnSQi1S8.
An illness that can affect anyone
People can be born with PH or develop it at any time and it can affect anyone, regardless of age or ethnic background, although it does affect more women than men.
There are seven specialist adult PH centres in the UK, including Papworth Hospital NHS Trust in Cambridgeshire, Hammersmith, Royal Brompton and Royal Free hospitals in London.
Thanks to a range of highly advanced treatments developed over the last 15 years people with PH have an improved quality of life and life expectancy has doubled to around six to seven years on average, with many living longer. There is, however, no cure, although a few people with PH undergo heart and lung transplants.
For more information about pulmonary hypertension visit www.phauk.org, call the PHA UK office on 01709 761450 or email office@phauk.org.
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