Medical University of South Carolina Hospital logo
Home |  Video Library | Podcast Library | e-Newsletters | Classes & Events | About Us | News Blog | University & Colleges 
Contact Us | 843-792-1414
  

Patients & Visitors

Medical Services

Maps & Parking

Health Library

Physician Portal

Careers

Online Services
Health Library
Health Topics A to Z
Clinical Trials & Research
Drug Information
Tests & Procedures
Lab Tests & Results
Health Assessment Tools
Treatment Options
Symptom Checker
Health e-Newsletters
Podcast Library
Video Library
Health Library
Bookmark Page icon Bookmark |

Print this page icon

|

E-mail icon

Health Library : Respiratory Disorders

 

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. Due to improved treatments, most people with CF live into their late 30s, and many even into their 50s.

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 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


 Sources & References

OUR SERVICES

 Treatment at MUSC:
 »Cystic Fibrosis Center

 

RELATED INFORMATION

 Interactive Tools:
 »Childhood Asthma Quiz
 »Child's Sleep Safety Quiz

 Tests & Procedures:
 »Bronchography
 »Bronchoscopy
 »Chest Fluoroscopy
 » View All 20

About This Site   |   Disclaimer   |  Privacy   |   Accessibility   |   Donations   |   Site Map
171 Ashley Avenue, Charleston, SC 29403 1.843.792.1414 | © 2014 Medical University of South Carolina

mobile web site iconrss feed iconText Messaging iconPodcast Library