A condition which affects 7,000 people in the UK is causing misery for sufferers – both physical and emotional.
Catherine Scott reports.
Just over ten years ago Paula Hartley was the life and soul of the party and running her own pubs.
Now at just 49 years old she cannot work, needs a mobility scooter to get around and has to have drugs pumped directly into her heart 24 hours a day.
Paula from Grenoside, Sheffield suffers from pulmonary hypertension and is backing a national awareness week to help more people understand her condition.
The life-shortening disease, which affects just 7000 people in the UK, causes high pressure in the blood vessels connecting the heart and lungs.
Symptoms include severe breathlessness, fatigue, blackouts and swelling around the ankles, arms and stomach. There is no cure.
“Every part of my daily life is affected in some way by PH,” says Paula.
“I struggle with breathlessness and tiredness even when making a cup of tea, or trying to get down the stairs, and I need help to get dressed.
“These are all things that people take for granted, but PH stops me from being ‘normal’. More people need to understand what the disease is, and how it makes people feel.”
Paula was diagnosed when she was just 38 and her life changed forever.
“I had started to feel a bit breathless going up and downstairs – but I was juggling running a pub with my husband Ian and three kids so life was busy.”
But eventually she was admitted to the Northern General Hospital with very high blood pressure where she stayed for 26 days.
“There was a locum doctor who thought he knew what was wrong with me and contacted the special unit at the Royal Hallamshire. They diagnosed me as having Pulmonary Hypertension. They told me not to look on the internet when I got home but of course I did.”
Paula was shocked by that she found.
“It said that life expectancy was two to four years. At the time my children were only eight, ten and 12. You just start to plan not just for the time you might have left but for after you’ve gone.
“I even went out and bought birthday cards and wedding cards for the children including their 21sts, 30th and 40th. It seems ridiculous looking back now but that was just what I felt I had to do.”
That we nearly 12 years ago and although Paula’s health has deteriorated and her and Ian have had to give up the pubs with Ian becoming her carer, she has seen milestones she though she might never see, including the birth of her first grandchild.
A survey by the Sheffield-based Pulmonary Hypertension Association (PHA UK), shows 92 per cent of those with PH feel their emotional wellbeing has been affected.
The research, released for PH Awareness Week (November 4-10), shows 53 per cent have experienced or been diagnosed with anxiety or depression, and 43 per cent feel lonely.
Over half find socialising difficult and 70 per cent say the condition has lowered their self-confidence.
“I feel very differently about myself now,”says Paula.
“I feel I am not the full person I was before, and I feel very lonely. I’ve had counselling, which did help. And my one-year-old granddaughter really helps my mental health too.
“She doesn’t know anything about my condition, she just wants her Nanan. And while I know that spending a day with her will wipe me out the following day I will still do otherwise you aren’t living you are just existing and I don’t want that.
“It does get me down sometimes and you start to think what’s the point?’ But then you look at your family and realise that’s the point.”
Paula receives treatment at the Sheffield Pulmonary Vascular Disease Unit (SPVDU), based within the Royal Hallamshire Hospital. It is one of only nine specialist PH treatment centres in the UK.
Ian Briggs, 45, lives in Sherburn in Elmet near Selby and has also been treated in Sheffield following his diagnosis in 2015.
He has received counselling for Post-Traumatic Stress Disorder and continues to struggle with anxiety.
“Through the therapy I realised I was grieving, for my life before and everything I could do,” says Ian.
“My whole world had been turned upside down. Having PH has left me disappointed more than anything. I feel cheated out of my life and disappointed with how things have turned out.”
Iain Armstrong, nurse consultant at the SPVDU and chair of the PHA UK, said: “Pulmonary hypertension is an extremely serious condition that has a huge impact on people’s lives. You often can’t tell someone has PH just by looking at them – so encouraging understanding of this rare, devastating condition is vital.”
The Pulmonary Hypertension Association UK (PHA UK) is the only charity in the UK dedicated to supporting the PH community made up of patients, their family and carers and NHS professionals too.
Paula adds: “What I would really like is greater understanding, not just from ordinary people but from the medical profession and also the benefits agencies. A lot don’t hear the word pulmonary and think it is just high blood pressure when it is far more serious than that.”
To find out more about pulmonary hypertension or get involved with PH Awareness Week visit www.phauk.org. Search #PHWeek19 and #TogetherForPH on social media.
People can be born with Pulmonary Hypertension or develop it at any time,.
It can affect anyone, regardless of age or ethnic background.
It affects more women than men.
A range of highly advanced treatments developed over the last 17 years has seen quality of life improve and life expectancy double to around six to seven years on average, with many sufferers living longer.
A few people with PH undergo heart and lung transplants.There are nine NHS specialist PH centres in the UK, based within hospitals in Sheffield, London, Glasgow, Cambridge and Newcastle.