OUR INSTITUTES: CANCER | HEART | NEUROSCIENCE | ORTHOPAEDICS
 
 

Pediatric Health Library :: Pediatric Health Library Topics
Share |
Printer Friendly
RESPIRATORY DISORDERS :: Cystic Fibrosis

Cystic Fibrosis and the Respiratory System

How does cystic fibrosis (CF) affect the respiratory system?

Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the body's mucus glands. It is chronic, progressive, and is usually fatal. In general, children with CF live into their 30s.

Children with CF have an abnormality in the function of a cell protein called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and certain salts in and out of the body's cells. As the movement of salt and water in and out of cells is altered, mucus becomes thickened.

In the respiratory system, mucus is normally thin and can easily be cleared by the airways. With CF, mucus becomes thickened and sticky and results in blocked airways. Eventually, larger airways can become plugged and cysts may develop.

Lung infections are very common in children with CF, because bacteria that are normally cleared, remain in the thickened mucus. Many of these infections are chronic. Pseudomonas aeruginosa (PA) is the most common bacteria that causes lung infections.

Children with CF also have involvement of the upper respiratory tract. Some individuals have nasal polyps that need surgical removal. Nasal polyps are small protrusions of tissue from the lining of the nose that go into the nasal cavity. Children with CF also have a high rate of sinus infections.

Symptoms that may be present due to the effects of CF on the respiratory system include the following:

  • chronic cough
  • coughing up blood
  • collapsed lung
  • clubbing (rounding and enlargement of the fingers and toes)
  • heart enlargement
  • nasal polyps (fleshy growths in the nose)
  • sinusitis (inflamed nasal sinuses)

Click here to view the
Online Resources of Respiratory Disorders

GR_ATP

It is important to remember the health information found on this website is for reference only not intended to replace the advice and guidance of your healthcare provider. Always seek the advice of your physician with any questions you may have regarding a medical condition. If you think you may have a medical emergency, call your physician or 911 immediately.
PEDIATRIC HEALTH LIBRARY
RESPIRATORY DISORDERS HOME
TOPIC INDEX
CONDITIONS
RESOURCES
GLOSSARY
RELATED SPECIALTIES:
ALLERGY / IMMUNOLOGY
PULMONOLOGY
RELATED LINKS:
FIND SPECIALISTS
ARTICLES
VIDEO
spacer

Facebook  Twitter  Pinterest  Instagram  Foursquare  LinkedIn  YouTube  RSS  CHOC Blog

US News     CAPE Award   Magnet      Beacon Award      Most Trusted Brand     Leapfrog

chocChildren's Hospital of Orange County | UCI University of California, Irvine

Children's Hospital of Orange County is affiliated with UC Irvine Healthcare and UC Irvine School of Medicine

CHOC Children's - 1201 W La Veta Ave, Orange, CA. Phone: 714-997-3000. .