Children's Hospital Colorado
Cystic Fibrosis

Cystic Fibrosis

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What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disorder that affects the lungs, digestive system and other organs in the body. CF disrupts the normal function of epithelial cells, which line passageways in the respiratory tract, digestive system, sweat glands and reproductive system.

The epithelial cells produce mucus, digestive enzymes and sweat. Typically, those fluids are thin and move easily through the body. But in people with CF, the fluids are thick and sticky. Instead of lubricating passageways, they build up and cause blockages that interfere with breathing, digestion and other essential functions.

What causes cystic fibrosis?

Mutations (changes) in a gene that produces the cystic fibrosis transmembrane conductance regulator (CFTR) protein cause CF. A child needs to inherit two copies of the defective CF gene (one from each parent) in order to get cystic fibrosis.

People with cystic fibrosis don’t produce enough CFTR protein — or the protein doesn’t work the right way. That means the epithelial cells can’t control the way salt passes across cell membranes. Without the proper balance of salt and water in your cells, the body can’t produce the thin coating of fluid and mucus necessary inside the lungs, pancreas, liver and other organs.

How does cystic fibrosis affect the body?

Because cystic fibrosis impacts so many organs, it can disrupt many of the body’s essential functions.

Having CF affects everyone differently, but most changes are related to the disorder’s effects on these organs:

  • Lungs: Thick, sticky mucus builds up in the lungs. The mucus clogs the airways. It also traps bacteria, leading to lung infections, swelling and airway damage.
  • Pancreas: The buildup of mucus in the pancreas stops the release of digestive enzymes that help the body digest and process key nutrients. Children with CF often have problems gaining weight, even when they are eating a healthy diet.
  • Liver: Thick mucus in the liver can block the bile ducts, increasing the risk of liver disease.

Next steps

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Get to know our pediatric experts.

Gwendolyn Kerby, MD

Gwendolyn Kerby, MD

Pulmonology - Pediatric

Benjamin Wisniewski, MD

Benjamin Wisniewski, MD

Pulmonology - Pediatric, Pediatrics, Sleep Medicine

Trish Eells, CPNP-PC

Trish Eells, CPNP-PC

Certified Pediatric Nurse Practitioner

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