What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disease of the glands that make mucus and sweat. People with CF get a defective gene from both parents. People who have one defective gene from one parent are called carriers. They don't have the disease.

CF is a long-term (chronic) disease that gets worse over time. It is a life-threatening condition. But better treatments have made it possible for people with this disease to live longer lives. 

CF affects 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 changed, the mucus that many cells normally make gets thicker. This thicker 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 have 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.

More than 30,000 people in the U.S. have the disease. More than half of those with CF in the U.S. are age 18 or older. In the U.S., CF affects both males and females from all racial and ethnic groups. But it is more common in patients whose families are from northern Europe. It is less common among African-Americans, Hispanic-Americans, and Asian-Americans. In rare cases it is seen in people from the Far East and in Native Americans.

Driscoll Medical Reviewer: Roberts, Jon, MD, FCCP; Reviewed on 10/13/2020