Fears after 30 cystic fibrosis deaths

THIRTY people with cystic fibrosis (CF) died last year, the highest death toll from the disease in 14 years.

Chief executive of the Cystic Fibrosis Association of Ireland (CFAI), Godfrey Fletcher, said they were hugely concerned by the record number of deaths.

There were 16 CF-related deaths in 1994 while in 1999, the second highest year over the period, 26 people died. He wondered if last year’s escalation in deaths was just a hiccup or a sign of things to come.

“We do not want to be scare-mongering. We just want to highlight our concern and hope and pray the that last year’s figure is just a blip,” he said yesterday.

CF is Ireland’s most common life-threatening inherited disease. About one in 19 people are carriers of the CF gene and where two carriers parent a child, there is a 25% chance of the baby being born with the disease.

Around 1,200 people in Ireland are living with CF, that affects the glands. It causes respiratory and digestive problems, diabetes and other complications.

The Health Service Executive (HSE) said a number of improvements had been made in the standard of care for CF patients. The HSE said €6.8 million was invested in dedicated CF services over the last two years and 80 additional specialist staff were recruited. Also, a major investment was planned for the national CF referral centre — St Vincent’s Hospital in Dublin — to provide single en suite units for CF patients.

But Mr Fletcher said: “There are plans there for the recruitment of 80 additional staff but while the commitments are there since 2006, there are not all in place yet. While we do appreciate all that has been done with regard to staffing, the provision of facilities for CF patients is still the major issue.”

Mr Fletcher said planning permission had been sought last year to develop a 102-bed unit for immunocompromised patients at St Vincent’s. The unit would, however, take two years to build once approved and a number of months before it is staffed and equipped.

Currently, CF patients at the hospital are at high risk of infection because they are sharing rooms and facilities with other patients.

Mr Fletcher said people with CF and their families were “extraordinarily frustrated” at the lack of facilities for CF sufferers.

There was some light at the end of the tunnel, said Mr Fletcher, because the HSE planned to introduce neo-natal screening for CF later this year: “To identify children who have CF at an early stage means they can be treated sooner and have a brighter future as adults.”