Cystic fibrosis (CF) is a chronic disease caused by mutations in the CFTR gene, which provides instructions to make a protein that channels salts across cell membranes. CFTR mutations cause the protein to be made incorrectly, leading to inefficient salt transport over the cell membrane that results in the buildup of thick and sticky mucus in different organs and tissues.
CF in the U.S.
The U.S. is among countries with the highest incidence of CF, with about 30,000 people currently living with the disease. Around 1 in 2,500 to 3,500 Caucasian babies are diagnosed with CF in the U.S. This ratio is much lower among African Americans at 1 in 17,000 births, and even lower for Asian Americans at 1 in 31,000. Approximately 1,000 new cases of CF are diagnosed each year in the U.S., with more than 75 percent being diagnosed by age 2.
Life expectancy for patients with CF has greatly increased in the past few years. The median age of survival for a person with CF is around 47 years (based on 2016 Registry data), but many patients are living well into their 50s or 60s.
CF around the world
It is estimated that more than 70,000 people worldwide are living with CF, but the incidence of the disease varies greatly across the globe.
Incidence rates for CF in Canada and the U.K. are similar to those seen in the U.S. Across the European Union, about 1 in 2,000 to 3,000 babies are diagnosed with CF at birth, slightly higher than the U.S.
It is thought that CF is under-diagnosed in Asia and Africa CF. In many countries, infants are not tested for CF at birth and national registries (if they exist) are lacking in data. In countries without a national CF registry, most studies are conducted by individuals rather than national organizations, making it difficult to establish a reliable prevalence of the disease.
A trend toward increasing lifespan for CF patients is also present in the global population. However, patients in developing countries generally have a much lower life expectancy, with many dying in their teens.
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