Cystic Fibrosis Overview

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disease of the glands that make mucus and sweat. It is a long-term (chronic), progressive disease. But improved treatments have made it possible for people with this disease to live longer lives. 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 70s. 

People with CF have an abnormality in the function of a cell protein called CFTR (cystic fibrosis transmembrane regulator). 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. CF makes sweat very salty. When people with CF sweat, they lose large amounts of salt. This upsets their body’s mineral balance and leads to other health problems.

About 30,000 people in the U.S. are affected with the disease. CF affects both males and females from all racial and ethnic groups. But it is more common in whites whose families are from northern Europe. It is also common among Latinos and American Indians. It is 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.