Cystic fibrosis (CF)
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
- 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
- frequent greasy, bulky stools or difficulty in bowel movements
Our quality and outcomes
BMI is a measure of a person's size based on their weight and height. If the number is too high or too low there may be health problems. The Cystic Fibrosis Foundation Patient Registry shows a strong association between a higher BMI percentile and better lung function in children with CF. Gaining weight and growing at a normal rate can be hard for children with CF. Therefore, with CF, a high BMI percentile is better.
Regular check-ups help people with CF be healthy and find complications early. The Cystic Fibrosis Foundation's Clinical Practice Guidelines recommend that every calendar year people with CF have at least four CF clinic visits, two lung function tests and one sputum or throat culture (a test to find out what germs are growing in the lungs).
This information has been adapted from the CF Foundation website.