Overview

What is cystic fibrosis?

Cystic fibrosis (CF) is a progressive, genetically-inherited disease that leads to a decrease in lung function and increased frequency of lung infections. CF also causes decreased pancreatic function, which can affect the patient’s ability to absorb food.

CF is caused by a gene mutation that increases the amount of mucus in the body, as well as increasing its viscosity (thickness) and stickiness. The mucus can obstruct organs like the lungs, pancreas, and liver. ¹ In the lungs, it enables bacteria to stick and multiply more easily, increasing the risk of infection. It obstructs the lungs, making breathing more difficult. In the pancreas, the mucus prevents pancreatic enzymes from digesting food, leading to possible malabsorption and fatty stools. The mucus in the liver can block the bile duct, which can result in liver disease. Increased mucus in the reproductive system can lower fertility in women, and can cause infertility in men.²

Although there is no cure for CF, recent advances in research have greatly improved quality of life, and the average lifespan has been steadily increasing. Children with CF can now go to college, get married, and even have children.

Treatment of cystic fibrosis includes multiple inhaled and oral medications. Airway clearance, nutrition, and psychosocial support are all integral components of a patient’s care plan.

How common is cystic fibrosis?

CF is most common in Caucasian patients, with 1 out of every 3,000 births having CF. Latin Americans have a rate of 1 out of 4,000 to 10,000, and African Americans have a rate of 1 out of 15,000 to 20,000. About 1,000 children are born with CF every year in the United States. ³

The Cystic Fibrosis Foundation has 29,497 people with CF in the United States in their registry as of 2016. The worldwide total of people with CF is estimated to be around 70,000 people. ³

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1 “Cystic Fibrosis.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 13 Oct. 2016, www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700.

2 “Cystic Fibrosis (CF).” American Lung Association, 2018, www.lung.org/lung-health-and-diseases/lung-disease-lookup/cystic-fibrosis/.

3 Cystic Fibrosis Foundation Patient Registry. 2016 Annual Data Report. Bethesda, Maryland. ©2017 Cystic Fibrosis Foundation. Accessed February 19, 2018.