Cystic fibrosis (CF) is an inherited disease characterized by an
abnormality in the glands that produce sweat and mucus. Due to
people with cystic fibrosis, on average, live into their mid to late 30s.
Cystic fibrosis affects various systems in children and young
adults, including the following:
There are about 30,000 people in the U.S. who are affected with the
disease, and about 1,000 babies are diagnosed with it each year. It
occurs mainly in Caucasians, who have a northern European heredity,
although it also occurs in African-Americans, Asian Americans, and
Approximately one in 31 people in the U.S. are carriers of the
cystic fibrosis gene. These people are not affected by the disease and
usually do not know that they are cystic fibrosis carriers.
How does cystic fibrosis affect the respiratory system?
The basis for the problem with cystic fibrosis 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 cystic fibrosis at risk for
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 cystic fibrosis patient are the following bacteria:
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 cystic fibrosis patient are due to PA.
As a result of the high rate of infection in the lower respiratory
tract, people with cystic fibrosis may develop a chronic cough, blood
in the sputum, and sometimes can even have a collapsed lung. The cough
is usually worse in the morning or after activity.
People with cystic fibrosis also have involvement of the upper
respiratory tract. Some patients 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 also have a high
rate of sinus infections.