Cystic Fibrosis

Also known as: CF

What is cystic fibrosis?

Cystic fibrosis is a genetic disorder in which a child inherits two defective cystic fibrosis genes (one gene from each parent). The defective genes result in the buildup of thick, sticky mucus in the lungs, pancreas and other organs. In the lungs, mucus blocks airways and traps bacteria, leading to infections, extensive lung damage and, eventually, respiratory failure. In the pancreas, the mucus interferes with the release of enzymes that allow the digestive system to break down food, interfering with absorption of vital nutrients.

 

What causes cystic fibrosis?

Cystic fibrosis is an inherited genetic disorder that is passed along from both parents to their children. It is one of the commonest genetic disorders in white children in the USA, affecting more than 30,000 people. 


What are the symptoms of cystic fibrosis?

Children with cystic fibrosis can have a variety of symptoms, including:
  • Very salty-tasting skin
  • Persistent coughing, at times with phlegm
  • Frequent lung infections including pneumonia or bronchitis
  • Wheezing or shortness of breath
  • Poor growth or weight gain in spite of a good appetite
  • Frequent greasy, bulky stools or difficulty with bowel movements
 


What are cystic fibrosis care options?

Cystic fibrosis is a complex disease and the severity of symptoms can vary from person to person. Typically, patients with cystic fibrosis are treated with a combination of therapies involving prescription medications and medical devices that can be used at home. These treatments and medications are used to keep the lungs clear of mucus thus preventing infections and may be administered orally, nebulized or intravenously through a central line if needed.

Pancreatic enzymes are necessary in many patients to help absorb the nutrients in the food. Patients with cystic fibrosis may have trouble achieving normal height and weight due to nutritional challenges. A registered dietitian can help support patients in optimizing nutrition.
Although there is no cure for cystic fibrosis, new treatments are currently being studied. Having a well-rounded care team can greatly improve the quality of life of children with CF. The Nicklaus Children's Cystic Fibrosis Center offers comprehensive family-centered care for children and adults with cystic fibrosis since 1993. 


Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 8/13/2018 10:35:40 AM