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Health Library : Respiratory Disorders

 

Cystic Fibrosis Overview

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. However, due to improved treatments, most people with CF live into their late 30s, and many even into their 50s.

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.This includes sinuses and lungs.
  • Digestive.This includes pancreas, liver, gallbladder, and intestines.
  • Reproductive.This occurs more often in the male, where sperm-carrying ducts become clogged.
  • Sweat glands

There are about 30,000 people in the U.S. who are affected with the disease. It occurs mainly in whites who have a northern European heredity, although it also occurs in African-Americans, Asian Americans, and Native Americans.

More than 10 million people in the U.S. are carriers of the cystic fibrosis gene. These people are not affected by the disease, and usually do not know that they are carriers.

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Online Resources of Respiratory Disorders


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