Cystic Fibrosis (CF) is an inherited 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 to break down and absorb food. People with CF tend to have a lot of sinus problems.
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People with CF can have a variety of symptoms including:
- Very salty-tasting skin
- Persistent coughing (at times with phlegm)
- Frequent lung infections
- Shortness of breath
- Poor growth/weight gain in spite of a good appetite
- Frequent greasy bulky stools
- Difficulty in bowel movements
- Sinus issues
A sweat test is the most common test used to diagnose cystic fibrosis. A small electrode is placed on the skin (usually on the arm) to stimulate the sweat glands. Sweat is then collected and the amount of chloride, a component of salt in the sweat, is measured.
In a genetic test, a blood sample or cells from the inside of the cheek is taken and sent to a laboratory to see if any of the various mutations of the CF gene are found. A genetic test is often used if the results from a sweat test are unclear.
Pat Pfahler, RN, BSN
Adult Cystic Fibrosis Coordinator
Julie Siegel, MSW
Master's of Social Work
Patricia Dummer, RRT
Registered Respiratory Therapist
Michelle McDonagh, RD, CD