About CF

About Cystic Fibrosis

 

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 1,200 people in Ireland (70,000 worldwide). There is a high prevalence of CF in Europe, with the highest worldwide prevalence in Ireland, which is three times the average rate in other EU countries and the United States.

 

Cystic Fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein which affects the regulation of the absorption and secretion of salt and water in various parts of the body, including the lungs, sweat glands, pancreas, and gastrointestinal tracts.
In people without CF this protein controls the flow of salt and water through the body’s cells, keeping mucus on the inside of the body’s organs thin and watery.
People with CF produce large volumes of thick, sticky mucus, which;

 

 Clogs airways and lead to life-threatening lung infections

 Obstructs the pancreas and stop natural enzymes from helping the body break down and absorb food.

 

Historically Cystic Fibrosis was labelled a ‘childhood illness’, with few children living to primary school age. 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.