Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the glands that produce sweat and mucus. It is chronic, progressive, and is usually fatal. Due to improved treatments, people with CF, on average, live into their mid to late 30s.

Cystic fibrosis affects various systems in children and young adults, including the following:

1. Respiratory system

2. Digestive system

3. Reproductive system

About 30,000 people in the U.S. 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.


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.

Due to the high rate of infection in the lower respiratory tract, people with CF 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 CF 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.

How does CF affect the gastrointestinal (GI) system?


As a result of the abnormal electrolyte transport system in the cells, the secretions from the pancreas become thick and lead to an obstruction of the ducts of the pancreas. This obstruction then causes a decrease in the secretion of enzymes from the pancreas that normally help to digest food. A person with CF has difficulty absorbing proteins, fats, and vitamins A, D, E, and K.

The problems with the pancreas can become so severe that some of the cells in the pancreas can become destroyed. This may lead to glucose intolerance and insulin-dependent diabetes.

About 35 percent of CF patients develop this type of diabetes in their 20s, and 43 percent develop the disease after 30 years of age.

Symptoms that may be present due to the involvement with the GI tract include: bulky, greasy stools; rectal prolapsed (a condition in which the end part of the bowels comes out of the anus); delayed puberty, fat in the stools, stomach pain, bloody diarrhea.