The CFRI CF Community Voices Podcast

About Cystic Fibrosis

January 15, 2017

Cystic fibrosis (CF) is an inherited chronic life-threatening disease that affects the respiratory, digestive and reproductive systems. Mutations of a specific gene (the CFTR) affect the transfer of salts and chlorides into and out of cells that line the organs of these three systems. This defective gene causes the production of unusually thick, sticky mucus, which builds up and clogs the small airways and passageways of the respiratory, digestive, and reproductive systems. Rather than carry harmful bacteria out of the body, the mucus traps bacteria, which leads to serious infections. While new therapies have improved the quality of life and extended life expectancy to approximately 40 years, the median age of death in 2015 was still only 29 years. Those with CF spend hours each day following a complex medical regimen to slow the progression of the disease, which currently has no cure.

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