'My rare lung condition is still not understood'

Paula Hartley was diagnosed with pulmonary hypertension in 2007 and given two to four years to live

Paula Hartley does not look unwell - but in reality, she is carrying a bag with a device that pumps medication into her heart 24 hours a day to keep her alive.

The former pub landlady from Grenoside, Sheffield, was told she had two to four years to live when she was diagnosed with a rare condition, pulmonary hypertension, which sees high blood pressure in the blood vessels that supply the lungs.

Now, 18 years later, she says many people including hospital staff still do not know much about the illness.

She says she was "devastated" when she received her diagnosis at the age of 37 but finds that the majority of people have "never heard of it".

Pulmonary hypertension is a condition that sees the walls of the arteries that supply the lungs become thick and stiff, meaning they cannot expand well to let blood through, according to the NHS.

This makes it harder for the for the heart to pump blood through the body and can gradually weaken the muscle, which can lead to heart failure.

Before her diagnosis, mother-of-three Ms Hartley says her life was "very hectic" and she worked up to 18 hours a day as a pub landlady alongside her husband, Ian.

However, she started to notice she was becoming breathless during normal daily activities such as climbing the stairs.

After being diagnosed with the condition at Sheffield's Royal Hallamshire Hospital, she was told she could die within four years.

"It just knocked me off my feet, I was 37 and I thought I'd never see my kids grow up," she says.

"The first thing I did was buy birthday cards and wedding day cards for all three of them so they could remember me if I wasn't there."

She says her symptoms mean that some days she is unable to leave the house, but she tries to "do as much as I can, when I can".

"My ethos is to try and live it, not let it live me."

Dr Iain Armstrong says the condition is not widely understood

Dr Iain Armstrong, a specialist in pulmonary hypertension at the Hallamshire Hospital, says the condition can affect anybody of any age - including children - although it was more common in women.

Alongside breathlessness and fatigue, symptoms can include chest pain, dizziness, and swelling of the legs, ankles, feet or abdomen.

Only 8,000 people in the UK are diagnosed with pulmonary hypertension.

Although it can be connected to some other diseases, many people - like Ms Hartley - develop it "out of nowhere", he says.

New treatments have improved the life expectancy of patients but they are expensive, he adds.

Patients are often challenged when applying for benefits because the impact of the disease is not widely understood, Dr Armstrong claims.

He says it is important to raise awareness of the condition.

"If it was cancer, people would understand. It's actually worst than most types of cancer these days, because we can't cure pulmonary hypertension, we're just trying to manage it."

'Grateful for life'

Ms Hartley is now backing an awareness day by Sheffield charity the Pulmonary Hypertension Association to highlight the difficulties of living with hidden disabilities and health conditions.

She says a lack of understanding of her condition makes her life more difficult, such as when she has to go to non-specialist hospital departments and staff have not heard of it.

For example, she says on one occasion healthcare workers did not know how to change the device which pumps the intravenous medication into her chest.

She has also lost friendships because "people get fed up of you letting them down, as they see it, and saying no all the time".

Ms Hartley says having pulmonary hypertension will eventually shorten her life - she says she is not upset by this.

"Being ill has made me learn what's most important to me in my life, which is my family and my children and my grandchildren," she says.

"I'm grateful for being ill, because it's taught me that."

Listen to highlights from South Yorkshire on BBC Sounds or catch up with the latest episode of Look North.