Tuesday 25 October 2016

Living with Cystic Fibrosis: 'We desperately need additional specialised units for adults in hospitals'

Having a chronic disease like cystic fibrosis is extremely challenging. Grainne Fahey tells how she is now hopeful that innovative ways of treating the condition will bring a new lease of life to many

Joy Orpen

Published 18/04/2016 | 02:30

Grainne Fahey: 'These fantastic, ground-breaking drugs mean some adults' lives are likely to be much, much longer'. Photo: Colin O'Riordan.
Grainne Fahey: 'These fantastic, ground-breaking drugs mean some adults' lives are likely to be much, much longer'. Photo: Colin O'Riordan.

In the early 1960s, almost half of the children living with cystic fibrosis (CF) didn't make it to their sixth birthday. Today, most people with CF will live well into their 30s, while many, most likely, will survive for much, much longer.

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The reason for the uncertainty is the fact that the world of CF is rapidly changing. Excellent new drugs and revolutionary treatments are already on the horizon. And given that this country has the highest incidence of CF in the world, it is not surprising that Irish doctors are at the vanguard of efforts to understand and treat the effects of this chronic, debilitating and most serious disease.

Someone who is vociferously pushing for improvements in health care for people living with CF is Grainne Fahey (24), from Borrisoleigh, Co Tipperary. When she was three, her nine-week-old baby brother was diagnosed with CF following a bout of pneumonia. And because CF is a genetically inherited disease, Grainne was also tested, and she was found to be suffering from the condition.

Grainne grew up on a sheep and cattle farm, and consequently spent a good deal of time playing outdoors with her much-loved brother. They went to the village school, which only had two teachers and less than 30 pupils. Grainne says it was a gentle and nurturing environment. For example, the principal often took them on spur-of-the-moment nature walks. "We had a bit of a charmed life at primary school and on the farm," she says.

Grainne's first real encounter with ill health only happened when she was already 14 years old, and visiting family friends in Dublin. "I got an awful pain in my chest, so I was taken to Temple Street [Children's University Hospital]," she explains. Following chest X-rays and blood tests, pneumonia was diagnosed. When her mother got the news, by phone, that Grainne had been hospitalised, she immediately assumed her daughter had broken a leg or an arm. "CF never even crossed mam's mind," says Grainne. "I'd been so well until then."

Grainne was then transferred to University Hospital Limerick. She explains that CF causes secretions in the body to become thick and sticky. "The lungs get blocked, which makes it difficult to breathe, while the pancreas and digestive system are unable to release enough enzymes to break down and absorb food."

Nonetheless, over the next couple of years, she did well at the Ursuline Convent secondary school in Thurles. "I loved every minute," she enthuses. Back then, Grainne was working and playing hard. "I became vice-chair of the school council. I was involved in the Green School initiative, which promotes environmental issues, and I was swimming." She was also studying hard.

All this activity put so much pressure on her health, Grainne was off school for almost three months in total. "I got one thing after another and was convinced I'd have to repeat the Leaving," she says. In the end, she got a fantastic 535 points and was so surprised, she thought she'd been given someone else's results. Grainne then did one year of a teacher training course, before switching to radiography at UCD. "I'm in the third year now, and really liking it," she says.

Getting to her final year wasn't without glitches. In second year, Grainne became profoundly fatigued. "I had never understood what it felt like to be really, really tired until then," she says. "I thought, 'I must be going crazy'."

Grainne had CT and MRI scans, blood tests, a lumbar puncture and so on, and was eventually diagnosed, by a process of elimination, with chronic fatigue syndrome (CFS). This condition is also known as myalgic encephalomyelitis (ME), or 'yuppie flu'. "I might wake up at 9am and be back in bed by 10am," she explains. "I tried everything, including diet and exercise programmes, but there was no magic cure." In the end, Grainne had to take 18 months off college, to rest at home.

She says having no energy also had a bad impact on her CF, because she couldn't exercise properly to clear her lungs. But, fortunately, she was able to return to college and is again working hard to complete her degree.

Grainne is hopeful that the huge strides currently being made in the treatment of CF will bring much longer lives for those living with the condition. According to Philip Watt, CEO of Cystic Fibrosis Ireland (CFI), two relatively new drugs are providing much hope for patients. "These fantastic, ground-breaking drugs mean some adults with CF can now work and become productive members of society, while their lives are likely to be much, much longer."

He says CFI has invested €8.5m in specialised CF centres and in research into the condition. The organisation will continue to campaign vigorously for improvements in health-care facilities, and for research into the disease.

"There is a great sense of hope," Philip says. Grainne concurs, while pointing out that even though some of these new drugs are very costly, the long-term benefits may outstrip those costs.

"Some people with CF are in and out of hospital all the time. They have to be kept in private rooms to avoid cross-contamination. It's already expensive to treat their symptoms," she says. "Then there is the fact that many people with CF can't work and have to rely on social welfare, and don't get enough from the State to cover the cost of the good diet and supplements they require. Anyway, most of the people I know with CF would much prefer to work and be able to contribute to society."

She says CFI has done much to help its members.

"Our facilities for CF in Limerick were poor," says Grainne. "So TLC4CF [Tipperary, Limerick & Clare CF] helped fund a special unit in the city. Because people with CF are living so much longer, we need additional specialised units for adults in hospitals." She says that recent improvements in treating CF mean increases in lung function, resulting in better oxygenation of the blood and improved BMI, while overall, patients taking the new drugs enjoy a longer, and much better quality of life.

That is why Grainne will be taking part in CFI's 'One in 1,000' campaign, which hopes to recruit 1,000 women to take part in the VHI Women's Mini Marathon on bank holiday Monday, June 6. Funds from this fun event will do much to help find ways to improve the lives of all those people living with CF.

For more information about Cystic Fibrosis Ireland, and for details about how to participate in the One in 1,000 campaign, tel: (1890) 311-211 or see cfireland.ie

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