Johns Hopkins Cystic Fibrosis Center

Johns Hopkins CF Center for Teens Site

 Search Chat Schedule an Appointment Contact Us Ways to Give
Site Tools Navigation

CF Main Site

CF for Member Families

CF for Kids

What is CF?

Living with CF

Game Room

Home

What is CF?

Overview
Science of CF
  - CFTR
        - Structure
        - Function
        - Cellular Processing
        - Mutations

   - Other Ion Channels
   - Lung Problems
        
Bacteria
        Inflammation
        Lung Damage

   - Gastrointestinal Tract 
      Problems

What's It All About?
Treatments
CF A to Z
Ask the Experts
Library

Science of CF: Lung Problems
Printer-Friendly Version

Although CF was originally described as cystic fibrosis of the pancreas, lung problems are the leading cause of troubles in the body. The lungs are injured by:

• Constantly recurring bacterial infections because thick mucus is not well removed from the lung. Changes in the lung airway surface liquid or ASL affect the body’s natural antibacterial defenses.

• Continuing inflammation that leads to scarring and loss of function. Because CF cells constantly produce chemicals that cause inflammation, even when there is no infection present, they cause more inflammation.

Think of your airway system as an upside-down tree. The trachea is a hollow trunk and the bronchioles, or small airways in the lungs, the smallest stems before the leaves. The outside of the "trunk" is made up of strong tissue and muscle, but is flexible to allow coughing. The inside of the trunk is covered by cells containing CFTR and other ion channels, cells with cilia and cells that make mucus and the ASL.

In CF, the ASL is abnormally thick and gooey because it contains thick mucus, bacteria, neutrophils -- white blood cells attempting to kill the bacteria -- and cell debris. These thick secretions block the smaller airways and make the airway swelling worse.

Select from the menu above to compare the normal lung aiway to the lung airway with CF and bacterial infection or with CF and inflammation.

Back to Top

Johns Hopkins Children's Center

Johns Hopkins Medicine

© 2006 Johns Hopkins Medicine  |  Terms & Privacy  |  About this Site