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What is Cystic Fibrosis?

Cystic fibrosis

is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. In people with CF, a defective gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.

People with cystic fibrosis are at greater risk of getting lung infections because thick, sticky mucus builds up in their lungs, allowing germs to thrive and multiply. Lung infections, caused mostly by bacteria, are a serious and chronic problem for many people living with the disease.

The buildup of mucus in the pancreas can also stop the absorption of food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.

Quick Facts:

  • More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
  • Approximately 1,000 new cases of CF are diagnosed each year.
  • More than 75 percent of people with CF are diagnosed by age 2.
  • More than half of the CF population is age 18 or older.

Treatments:

Each day, people with CF typically complete a combination of the following daily therapies:

  • Airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs. Some airway clearance techniques require help from family members, friends, or respiratory therapists. Many people with CF use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus.
  • Inhaled medicines to open the airways or thin the mucus. These are liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer. These medicines include antibiotics to fight lung infections and therapies to help keep the airways clear.
  • Pancreatic enzyme supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF also usually take multivitamins.

In addition to daily treatments, people with CF must attend frequent doctor visits and undergo IV antibiotic therapy for lung infections.

(Credit to and for more information visit the Cystic Fibrosis Foundation’s website at cff.org)

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