Cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it is unable to help move chloride, a component of salt, to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky. This can lead to a variety of symptoms, including:
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Lungs: The thick mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications.
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Pancreas: The mucus can block the ducts in the pancreas, causing problems with digesting food.
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Other organs: Cystic fibrosis can also affect the liver, sinus, intestines, and sex organs.
Cystic fibrosis is a chronic and progressive disease that requires daily care, but people with CF are usually able to attend school and work. Improvements in screening and treatments mean that people with CF now may live into their mid- to late 30s or 40s, and some are living into their 50s.
