What is Cystic Fibrosis?

Cystic Fibrosis is a genetic disease that causes the production of thick, sticky mucus in the lungs, pancreas, and other organs. Inside the lungs, this mucus not only blocks the airways but also causes inflammation, leading to scarring of the lung tissues. In the pancreas, the mucus blocks the opening to the digestive system, interfering with the proper breaking down of food.

Cystic fibrosis is an inherited disease, its proliferation second only to sickle cell anemia in children. Estimations state that over 10,000,000 people in the United States carry the gene. About 30,000 people have the disease, with 1,000 more being diagnosed each year.

Cystic Fibrosis only occurs when a child gets the defective genes from both parents. 25% of children of parents that both have the gene will get the disease, while 50% will be carriers, and 25% will not even carry the gene.

Cystic Fibrosis is serious because the thick mucus makes an ideal breeding ground for bacteria, leading to deadly respiratory infections. Each infection leads to more damage in the lungs, eventually causing respiratory failure.

The life expectancy, although rising significantly, is still only about 37.4 years of age. In 1985, most patients with cystic fibrosis only lived to about 25 years old. Part of the reason is that the digestive issues can also lead to serious complications like diabetes and osteoporosis. Regular treatment and adherence to the plan developed by the child’s physician are imperative to ensure the highest quality of life possible.

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