Newborn screening for cystic fibrosis will result in better prognosis for long term for sufferers

The introduction of cystic fibrosis screening for newborn babies will result in earlier intervention and treatment and a much better prognosis long term for children with the condition, according to the chairperson of the Galway branch of the Cystic Fibrosis Association of Ireland.

Mary Lane Heneghan says nationwide screening - for this genetic disorder of the lungs and digestive system - which commenced at the beginning of the month, is a “most welcome” development and something which the national CF association has been campaigning for for years.

The introduction of CF screening in other countries has been shown to reduce hospital admissions and the need for invasive therapy and is cost effective. Screening has been available in the UK and Northern Ireland for the past 25 years. It is estimated that three babies a week will be diagnosed with the condition or will be diagnosed as being a healthy carrier of the altered gene that causes CF, due to the new screening programme.

“Galway is an identified centre for CF referrals in the west of Ireland and gets referrals from as far as Donegal,” explains Ms Lane Heneghan, a former national chairperson and director of the Cystic Fibrosis Association of Ireland. “This screening will be a huge relief for families if there is any history of CF in them. It is fantastic to know that there will be screening, diagnosis and treatment early on. The sooner treatment begins for children with cystic fibrosis the better the long term results.”

She says there are cases around the country of children with the condition who were not diagnosed until they were older, about 11 to 13 years.

“A lot have gone through school coughing and chesty with infection after infection. The fact that newborn screening has been introduced will mean early intervention and early treatment and the long term prognosis will be much better. This will prevent early damage to the lungs. Early intervention is the key so this new screening is most welcome.”

A steering group to advise on the implementation of the introduction of screening of newborn children for CF was established by the HSE under the chairpersonship of Professor Gerry Loftus of NUI, Galway.

Ireland has the highest incidence of cystic fibrosis in the world, with one in 20 people carrying the gene. Where two carriers parent a child there is a one in four chance of the baby being born with the condition. CF carriers are healthy and totally unaware that they are carriers until they have a child with CF.

The disease affects the glands, damaging many organs including the lungs, the pancreas and the digestive system. About 1,200 people in this country have the condition. More than 60 from Galway, Mayo and Roscommon attend Galway University Hospitals - half of these are adults. The number of sufferers are increasing with improvements in care.

There are three specialised in-patient rooms for patients at University Hospital Galway. These are single en suite rooms with climate control and air filtering systems to reduce the risk of cross infection. Additional in-patient facilities are also available at Merlin Park University Hospital..

Treatment is intensive and people with CF have a daily routine of drugs, enzymes, physiotherapy, exercise and a high fat diet. Oral, inhaled and intravenous antibiotics are crucial for controlling recurring chest infections. High-energy foods supplemented by enzymes combat digestive problems while physiotherapy and exercise are important for fitness and for keeping the lungs clear.

“For a considerable number of CF patients a lung transplant is often the only remaining option,” says Ms Lane Heneghan. “While therapeutic options have expanded in recent years, life expectancy and quality of life for people with CF can be significantly improved through a lung transplant.

“Now that the Mater Hospital’s lung transplant unit in Dublin is up and running we expect to see an increase in the number of CF patients receiving lung transplants. Welcome news was the recent announcement of the approval of a dedicated lung transplant surgeon there. This will mean that persons requiring a lung transplant will no longer have to travel to Newcastle in the UK.”

She says the life expectancy and quality of life for people with CF improves in direct proportion to the availability of proper medical care and facilities.

“The Galway Branch of the CF Association are continually fundraising to provide the basic facilities for the 60 plus patients with CF in the Galway area who attend UHG. It is critical that cystic fibrosis patients be treated in single rooms with en-suite facilities as the lack of segregation and isolation facilities poses a significant risk of cross-infection by virulent organisms.

“Galway was one of the first hospitals outside of Dublin to get dedicated facilities for persons with CF. In September 2009 three single en-suite rooms were opened in St Anthony’s Ward for adults (over 16s ) which cost over €300,000, paid for out of voluntary contributions. CF Galway also funded a lung function laboratory and other medical equipment in UHG.”

She says the Galway branch’s top three priorities are improved isolation en-suite facilities at UHG, the provision of additional specialist medical staff, and necessary medical equipment for patients.

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