Cystic Fibrosis Overview
What is cystic fibrosis?
Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the glands that make mucus and sweat. 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 people with CF now live into their 70's.
People 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
About 30,000 people in the U.S. are affected with the disease. CF affects both male and female from all racial and ethnic groups, however it is more common in Caucasians who have a northern European heredity. It is also common among Latinos and American Indians and less common among African Americans and Asian 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.
- MMI board-certified, academically affiliated clinician
- Sather, Rita, RN