About Cystic Fibrosis

What is Cystic Fibrosis?

Cystic Fibrosis, or CF as it is commonly called, is a disease caused by an inherited genetic defect. As such it is not contagious and there is no risk of "catching" CF from another person with CF. About 1 in 23 people in the United States carry at least one defective gene, which makes it the most common genetic defect of its severity in the United States.

For many years the causes of Cystic Fibrosis were a mystery. Today, recent advances in biology have made the cause more clear. Humans have a gene encoded in their DNA which manufactures a special protein called CFTR. This protein controls the flow of chloride ions across the cell membrane. Each gene is made up of two alleles; a single correctly encoded allele is adequate for normal CFTR production. Thus it is only when a person has two defective CFTR alleles that they actually have Cystic Fibrosis. Those with a single defective allele are called carriers, and those with two defective alleles have Cystic Fibrosis.

People with Cystic Fibrosis suffer from chronic lung problems and digestive disorders. The lungs of people with Cystic Fibrosis become covered with a sticky mucus which is hard to remove and promotes infection by bacteria. Many people with CF require frequent hospitalizations and continuous use of antibiotics, enzyme supplements, and other medications. The life expectancy of people with Cystic Fibrosis used to be very short; 30 years ago the median life expectancy was about 8 years. Today, thanks to medical advances, the median life expectancy is just under 30 years and increasing.

CF used to be known as a children's disease, and to some major fund raising organizations maybe it still is. But as medical advances increase the life expectancy of people with Cystic Fibrosis, they face a new set of problems--going to college, getting a job, finding health insurance, building permanent relationships--all while keeping up the physical therapy and medications. The primary goal of this list is to help people with those new set of problems.

There are approximately 40,000 people in the United States with Cystic Fibrosis.


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