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Cystic Fibrosis

Cystic fibrosis (CF) is one of the most common life-shortening genetic diseases. Ireland has the highest incidence of CF with an incidence 1 in 1,600 births. CF is a hereditary disease affecting the mucus glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.

Thick mucus production results in frequent lung infections. Diminished secretion of pancreatic enzymes is the main cause of poor growth, greasy stools, and deficiency in fat-soluble vitamins. Males can be infertile due to the condition congenital bilateral absence of the vas deferens.

Individuals with CF can be diagnosed prior to birth by genetic testing. Newborn screening tests are increasingly common and effective.

There is no known cure for CF, and most individuals with CF die young: many in their 20s and 30s from lung failure. The median age of survival for a person with CF is 37 years. Lung transplantation is often necessary as CF worsens. Living with CF means adhering to a regime of chest physiotherapy, tablets, frequent hospital admissions and never being on top form.

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