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 multiple organs such as the lungs, pancreas, and liver. In the lungs, it enables bacteria to adhere and multiply more easily, increasing the risk of infection. It also obstructs the lungs, making breathing more difficult. In the pancreas, the mucus prevents pancreatic enzymes secretions used to digest 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.

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. Due to these advancements, for the first time ever there are currently more adults living in the US today with CF than 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.¹

How common is cystic fibrosis

CF is most common in Caucasian patients, affecting 1 out of every 3,000 births. Latin Americans have a rate of 1 out of 4,000 to 10,000 births, and African Americans have a rate of 1 out of 15,000 to 20,000 births. About 1,000 children are born with CF every year in the United States.²

The Cystic Fibrosis Foundation displays 29,887 people with CF in the United States in their registry as of 2017. The worldwide total of people with CF is estimated to be around 70,000 people.²

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1. Cystic Fibrosis Foundation Patient Registry. 2016 Annual Data Report. Bethesda, Maryland. ©2017 Cystic Fibrosis Foundation. Accessed January 25, 2019.

2. “Cystic Fibrosis Foundation.” CF Foundation, Cystic Fibrosis Foundation, 2017, www.cff.org/What-is-CF/About-Cystic-Fibrosis/.