Cystic fibrosis: living better

Irish people with the condition are living longer, more active lives

Áine Hannafin (7), who has cystic fibrosis, and her brother Liam (3) at the launch of Cystic Fibrosis Ireland’s Roses Day fundraising appeal. Photograph: Tom Honan

An estimated 1,300 children and adults are affected by cystic fibrosis in this State. It is a relatively small number. But the incidence of this rare and inherited disease that causes life-threatening lung infections is three times higher here than in other European countries. Because of that, its treatment has received particular attention within the health services, and the benefits for patients and for society at large have been revealed by a recent survey.

Forty years ago, people with cystic fibrosis were not expected to live beyond childhood. Improved medical treatments and hospital care, however, have brought significant change in the past two decades. Surveys conducted in 1998 and again in 2007 by Cystic Fibrosis Ireland (CFI) paint dramatically different pictures. Because of specialist treatment centres, a rising incidence of double lung transplants and access to new drugs, average survival rates have risen by more than 70 per cent, to 30 years. Quality of life has also dramatically improved.

From a position where adults were not expected to work in 1998, more than half of those affected by the disease are now reported to be in full or part-time employment. Some 26 per cent of those surveyed were married, up from eight per cent in 1998, and half of these families had children of their own. Improvements were also evident in terms of education with some 49 per cent securing third level degrees.

Medical awareness and treatment has improved since 2011, when babies were first screened for cystic fibrosis. More recently, CFI has campaigned vigorously for better hospital services and the provision of high-tech drugs. Last year, an expensive new medicine was made available to people over 12 years of age. Then, some weeks ago, children from the age of six upwards were included in the scheme. A commitment in the Programme for Government to build a dedicated cystic fibrosis unit at Beaumont Hospital in Dublin has not yet, however, been honoured, while staff shortages are reported at specialist treatment centres.