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.
- About 1,000 new cases of cystic fibrosis are diagnosed each year.
- More than 70% of patients are diagnosed by age two.
- More than 45% of the CF patient population is age 18 or older.
- The predicted median age of survival for a person with CF is in the late 30s.
Testing for Cystic Fibrosis
How can you know for sure if you or your child has cystic fibrosis? If someone in your family has the disease, could you pass it down to your children?
There are several ways to help answer these important questions.
Sweat Test — If a person shows symptoms of CF or if a baby has a positive newborn screen for CF, a doctor may order a sweat test. This simple, painless test is the best way to diagnose CF. It measures the concentration of salt in a person’s sweat. A high salt level indicates CF.
Sweat tests should be done at a Cystic Fibrosis Foundation-accredited care center where strict guidelines help ensure accurate results.
Click here to learn more about the sweat test.
Newborn Screening — Newborns screened for cystic fibrosis can benefit from early diagnosis and treatment, which can:
- Improve growth;
- Help keep lungs healthy;
- Reduce hospital stays; and
- Add years to life.
While newborn screening is not a definitive diagnostic test for cystic fibrosis, it may lead to tests that can rule out or confirm a CF diagnosis. All states in the U.S. screen newborns for cystic fibrosis.