Cystic Fibrosis Overview
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. 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.
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 whites 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.
More than 10 million people in the U.S. are carriers of the cystic fibrosis gene. They don't have symptoms. Often they don't know that they are carriers.
Online Medical Reviewer: Alan J Blaivas DODaphne Pierce-Smith RN MSN CCRCJohn Hanrahan MD
Date Last Reviewed: 3/1/2019
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- Autosomal Recessive: Cystic Fibrosis, Sickle Cell Anemia, Tay Sachs Disease
- Chronic Respiratory Disorders
- Cystic Fibrosis and the Digestive System
- Cystic Fibrosis and the Reproductive System
- Cystic Fibrosis and the Respiratory System
- Cystic Fibrosis in Children
- Diagnosis of Cystic Fibrosis
- Digestive Disorders
- Feeding Your Child with Cystic Fibrosis
- High-Risk Newborn Blood Disorders
- Medical Genetics: DNA Studies for Single Gene Defects
- Medical Genetics: Types of Genetic Changes
- Single Gene Defects
- Symptoms of Cystic Fibrosis
- Testing for Birth Defects
- The Genetics of Cystic Fibrosis
- Treatment for Cystic Fibrosis
- Acetylcysteine inhalation solution
- Acetylcysteine solution for injection
- Dornase Alfa inhalation solution
- Pancrelipase capsules
- Pancrelipase powder
- Pancrelipase tablets
- Tobramycin eye ointment
- Tobramycin eye solution
- Tobramycin injection
- Tobramycin powder for inhalation
- Tobramycin solution for inhalation