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American Family Children's Hospital
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Cystic Fibrosis (CF)

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(608) 263-5215

(608) 263-7203

 

Related Programs

Advanced Lung Disease Program

 

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Cystic Fibrosis Connections

Cystic fibrosis (CF) is a genetic disease characterized by production of abnormally thick mucus in the lungs. This is due to abnormal sodium and chloride transport in the lining cells of the lungs. According the Cystic Fibrosis Foundation, approximately 30,000 people in the United States have CF.

In cystic fibrosis, mucus clogs the lungs and leads to recurrent, life-threatening infections and destruction of the lungs. The secretions may also block the pancreas, preventing enzymes from absorbing nutrients from food, and block the bile duct in the liver, causing permanent liver damage.
 
Life expectancy for patients with cystic fibrosis, although improving, is about 30 years. End-stage lung failure is almost always the cause of death in CF patients.

Symptoms
 
Symptoms of cystic fibrosis include:
  • Shortness of breath
  • Persistent coughing with phlegm
  • Poor weight gain
  • Greasy stools
  • Salty skin

Health Information

Treatments

  • Cystic fibrosis is usually managed with medications. These medications include antibiotics and mucus thinners to prevent or clear up infection and improve lung function. Patients with CF also need to perform daily airway clearance exercises to dislodge mucus from the lungs.
  • Patients with severe cystic fibrosis may require advanced medical therapy or a Lung Transplant. During a lung transplant, the diseased lungs are replaced with donor lungs.