What is Cystic Fibrosis?
Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system of about 1200 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.
The impact of CF can vary from one person to another. There are some people with CF who live until their teens and there are others that live in to their 50’s. Ireland has among some of the most severe strains of CF and also has the highest incidence (per head of population) of CF in the world, with three times the rate of the United States and the rest of the European Union. However it is important that we now have a network of centres of expertise in place and dedicated multi-disciplinary teams lead by specialised CF consultants.
Symptoms of Cystic Fibrosis
People with CF can have a variety of symptoms, including:
- very salty-tasting skin
- persistent coughing, at times with phlegm;
- frequent lung infections;
- wheezing or shortness of breath;
- poor growth/weight gain in spite of a good appetite; and
- frequent greasy, bulky stools or difficulty in bowel movements.
- About 50 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 CF Registry of Ireland Report
Advances in Care and Therapies in Ireland
- There are specialised CF centres of expertise in Ireland
- Each centre 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
Cystic Fibrosis Ireland (CFI)
We are here to help. We were established by parents in 1963. We provide advice and other supports, we advocate to improve services and we support research. We can put you in touch with other parents and you can talk to our staff about any concerns. Three of our staff are people with CF.