Cystic fibrosis is a life-threatening, genetic disease that causes persistent lung infections and progressively limits the ability to breathe. In people with CF, a defective gene causes a thick, buildup of mucus in the lungs, pancreas and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients. Cystic fibrosis is caused by defects in the cystic fibrosis gene, which codes for a protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP). Mutations in the CFTR gene result in abnormalities of cAMP-regulated chloride transport across epithelial cells on mucosal surfaces.
The CF Foundation has carried these goals forward and is working tirelessly to find lifesaving new therapies, and one day, a lifelong cure for all people living with this devastating disease.
Ireland has highest incidence of Cystic Fibrosis in the world wide – approximately 1 in 19 Irish people are said to carry one copy of the altered gene that causes Cystic Fibrosis. About 50 new cases of cystic fibrosis in Ireland are diagnosed each year. Around 55% of the CF patient population in Ireland is aged 18 or older.
The incidence of CF has been previously estimated to be 1 in 4150 in France.