We need greater awareness of pulmonary arterial hypertension to improve patient support.
On her 53rd birthday, Julie Royle was told that she had stage three pulmonary arterial hypertension (PAH). The words might not mean much to most people – but Julie remembers, “I walked out of the hospital feeling like a bomb had gone off in my life. People talk about cancer and heart disease, but no one talks about PAH.”
PAH is extremely rare and there is no cure, so a diagnosis has life-changing consequences for the patient and their family.
Living with PAH is a 24-hour, mental and physical challenge and my only advice to others is to listen to your body, to take all the advice and support from the specialist centres and from PHA UK and to try and stay positive.
By the time Julie was diagnosed, she already had an advanced case of the condition, which causes a narrowing of the blood vessels that lead from the heart to the lungs. As a keen jogger and fitness instructor – Julie was extremely healthy with no underlying health complaints to indicate that anything was wrong.
When she started experiencing breathlessness, her GP initially prescribed an inhaler. This did little to help and Julie found that she was getting more exhausted at work, where she was a secondary school cookery teacher. After several more trips to the GP, she was eventually sent for an echocardiogram, which was the first step towards her diagnosis. “For every second of every minute and every hour of my day, I focus on my inability to breathe normally. It’s exhausting.”
“I started having blackouts”
In one sense, it was a relief to finally receive a diagnosis and start treatment, but there is little anyone can do to prepare for the reality of living with PAH. “I started treatment and immediately felt better, but then I started to have blackouts,” says Julie.
“Medical staff had no knowledge of my condition”
The horror of her first blackout was further compounded by the fact that the medical staff treating Julie at her local accident and emergency unit had no knowledge of PAH at all. Julie felt vulnerable and isolated. “The specialist centres are just amazing and the support from the charity PHA UK has been a lifeline, but the problem is that even people within the medical profession don’t know about it. I have to carry information around with me all the time,” continues Julie.
“Getting out of bed and walking is exhausting”
While Julie may not look physically unwell, her condition has transformed her life beyond recognition. She is unable to work and finds that simply getting out of bed and walking around the house can be as exhausting as a workout. Many patients require an intravenous line to be permanently attached to them, which delivers vital therapy on a 24-hour basis. This requires the constant attention of a PAH specialist nurse. Julie feels fortunate that, while she did have an IV line for three years, and could need one again, she has now been transferred onto a different therapy, which does not require one.
“I’m staying positive and I hope for a cure”
Julie is grateful to her specialist centre and team at Sheffield Royal Hallamshire for getting her to where she is now. She says “They are an extraordinary NHS team who treat us all like family.” Julie is hopeful that one day there may be a cure, but in the meantime she is focused on living positively. “Living with PAH is a 24-hour, mental and physical challenge and my only advice to others is to listen to your body, to take all the advice and support from the specialist centres and from PHA UK and to try and stay positive,” she concludes.
September 2018 | NOP 18/0423f