Child Health A-Z

Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the body's salt, water- and mucus-making cells. It is chronic, progressive, and is usually fatal. In general, children with CF live into their 30s.

Children with CF have an abnormality in the function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and certain salts in and out of the body's cells. As the movement of salt and water in and out of cells is altered, mucus becomes thickened. The thickened mucus can affect many organs and body systems including:

respiratory - sinuses and lungs
digestive - pancreas, liver, gallbladder, intestines
reproductive - more so in the male, where sperm-carrying ducts become clogged
sweat glands

There are about 30,000 people in the US who are affected with the disease, and about 1,000 babies are diagnosed with it each year. It occurs mainly in Caucasians who have a northern European heredity, although it also occurs in African-Americans, Asian Americans, and Native Americans.

Approximately one in 31 people in the US are carriers of the cystic fibrosis gene. These people are not affected by the disease, and usually do not know that they are carriers.

Cystic fibrosis is a complicated illness that requires clinical care by a physician or other healthcare professional. Listed in the directory below you will find additional information about cystic fibrosis, for which we have provided a brief overview. Multidisciplinary teams organized into CF centers are recommended for care of CF and have been shown to result in less illness and longer lives for CF patients.

Last Update
February 3, 2008