Children's Health and Wellness

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. While it is a chronic, progressive disease improved treatments have significantly extended life expectancy.  Most people with CF live into their late 30s, and many even into their 50s or longer. Some CF patients now live into their 70's. 

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. Both men and women may experience reproductive problems

  • Sweat glands

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

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.

Online Source: Cystic Fibrosis Foundationhttp://www.cff.org/aboutcf/
Online Source: Cystic Fibrosis Foundationhttp://www.cff.org/aboutcf/testing/
Online Editor: Geller, Arlene
Online Medical Reviewer: Bass, Pat F. III, MD, MPH
Online Medical Reviewer: Grantham, Paula, RN, BSN
Date Last Reviewed: 7/30/2012
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