Cystic Fibrosis

What is Cystic Fibrosis?

Cystic Fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system of about 1,300 children and adults in the Ireland (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

· clogs the lungs and leads to life-threatening lung infections; and

· obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend primary school. Today, advances in research and medical treatments, including in Ireland, have further enhanced and extended life for children and adults with CF. Many people with the disease in Ireland can now expect to live into their 30s, 40s and beyond. People with CF in Ireland are increasingly going on to attend third level colleges, accessing employment, and living more independent lives, with the support of family and friends.

STATISTICS

· About 25 new cases of cystic fibrosis in Ireland are diagnosed each year.

· Because of new born screening, most babies with CF should now be diagnosed quickly

· Around 55% of the CF patient population in Ireland is aged 18 or older.

· The predicted median age of survival for a person with CF is in the early and mid-30’s in Ireland.

· For the most up-to-date statistics on CF in Ireland please see the Cystic Fibrosis Registry of Ireland (CFRI) Annual Report

ADVANCES IN CARE AND THERAPIES IN IRELAND

· There are specialized CF centers of expertise in Ireland

· Each center has a multidisciplinary team, including for example a consultant, CF nurse, Dietitian and physiotherapist

· There are new exciting therapies being developed that will further improve survival ages and quality of life in Ireland