What Is Cystic Fibrosis (CF)?

Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the glands that produce sweat and mucus. Due to improved treatments, 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:

• Respiratory system
• Digestive system
• Reproductive system

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 Native Americans.

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 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 cystic fibrosis 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 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.