How does CF affect the respiratory system?
The basis for the problem with CF lies in an abnormal gene. The result of this gene defect is an atypical electrolyte transport system within the cells of the body. The abnormal transport system causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in our lungs to become very thick and hard to remove. These thick secretions put the child with CF at risk for constant infection. The high risk of infection in the respiratory system leads to damage in the lungs, lungs that do not work properly, and eventually death of the cells in the lungs. The most common causes for infection in the lungs of the CF patient are the following bacteria: • Staphylococcus aureus • Haemophilus influenzae • Pseudomonas aeruginosa (PA) Over a period of time, PA becomes the most common bacteria that causes infection and becomes difficult to fight. A large percentage of respiratory infections in the CF patient are due to PA.