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

  • Would you like to learn more about us?

    Learn more about the Mike McMorris Cystic Fibrosis Research and Care Center
  • Do you have questions about your child’s condition?

  • Are you ready to schedule an appointment?

    Schedule an appointment

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Get to know our pediatric experts.

Nicole Linehan, CPNP-AC

Nicole Linehan, CPNP-AC

Certified Pediatric Nurse Practitioner

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Gwendolyn Kerby, MD

Gwendolyn Kerby, MD

Pulmonology - Pediatric

Stephen Hawkins, MD

Stephen Hawkins, MD

Pulmonology - Pediatric, Pediatrics, Sleep Medicine

Christina Papantonakis, MD

Christina Papantonakis, MD

Pulmonology - Pediatric, Pediatrics

Children's Colorado in the news

  • Healio
    Late cystic fibrosis care negatively impacts infant weight, height
    August 22, 2023

    Delayed cystic fibrosis care contributed to reduced weight and height-for-age in infancy through age 5 years. Infants with cystic fibrosis who received care at 47 days old had decreased weight and height-for-age during infancy compared with infants who received care at 10 days old, according to results published in The Journal of Pediatrics.