Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system of about 1200 people Ireland, 45% of whom are under 18.
About fifty new cases are diagnosed in Ireland each year and because of newborn screening, most babies with CF are now generally diagnosed quickly.
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 into 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.
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.
What is it?
CF is a defective gene and its protein product causes the body to produce unusually thick, sticky mucus that:
1. clogs the lungs and leads to life-threatening lung infections;
and;
2. obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
What are the symptoms?
People with CF can have a variety of symptoms, including:
1. Very salty-tasting skin
2. Persistent coughing, at times with phlegm;
3. Frequent lung infections;
4. Wheezing or shortness of breath;
5. Poor growth/weight gain in spite of a good appetite
6. Frequent greasy, bulky stools or difficulty in bowel movements.
(Photo via Wikipedia)
How is it treated?
There is still no known cure for Cystic Fibrosis. However, treatments have improved in recent years.
The goals of treatment are as follows:
1. Preventing and controlling lung infections
2. Loosening and removing thick, sticky mucus from the lungs
3. Preventing or treating blockages in the intestines
4. Providing enough nutrition
5. Preventing dehydration (a lack of fluid in the body)
Depending on the severity of CF, you or your child may be treated in a hospital.
**If you are ever in any doubt when it comes to the health of your family, please visit your local GP immediately**
For further information on Cystic Fibrosis or to join the support network, see Cystic Fibrosis Ireland
