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What Is Cystic Fibrosis?
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (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 elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
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.
Statistics
- About 1,000 new cases of cystic fibrosis are diagnosed each year.
- More than 70% of patients are diagnosed by age two.
- More than 40% of the CF patient population is age 18 or older.
- In 2006, the predicted median age of survival was 37 years.
Treatments
People living with cystic fibrosis must follow a regular treatment routine to stay healthy and maintain optimal lung function.
Most often, treatment for cystic fibrosis begins with techniques to trigger strong coughs that help loosen and clear thick mucus from the airways. Of course, airway clearance is just one part of the treatment routine. Nutrition and drug therapies are also important for children and adults with cystic fibrosis.
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