Cystic Fibrosis (CF)
Cystic fibrosis (CF) is an inherited disease that causes glands throughout the body to make abnormally thick, sticky mucus.
The most frequently affected organs include the:
- lungs
- pancreas
- sweat glands
- intestines
The liver and reproductive organs are also commonly affected.
CF is caused by inherited mutations in the cystic fibrosis transmembrane regulator (CFTR) gene. The CFTR normally functions to hydrate the airway and facilitate the clearance of mucus from the lungs. In CF, airway hydration is decreased and mucus is poorly cleared from the lungs due to abnormal CFTR function.
The impairment in mucus clearance predisposes people with CF to chronic airway infections and obstructive lung disease.
At the Adult Cystic Fibrosis Care Program at UPMC, quality improvement is part of our mission to fight CF. We encourage our patients to be active participants in their health care and in our research and quality improvement initiatives.
The Antonio J. and Janet Palumbo Cystic Fibrosis Center (CF Center) at Children's Hospital of Pittsburgh of UPMC is also dedicated to providing care to patients and families with cystic fibrosis.
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