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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 CF, on average, live into their mid to late 30s.
There are about 30,000 people in the US 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 US are carriers of the cystic fibrosis gene. These people are not affected by the disease and usually do not know that they are carriers.
The organ primarily affected is the pancreas, which secretes substances that aid digestion and help control blood-glucose levels.
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 more than 40 percent develop the disease after 30 years of age.
The symptoms that may be present due to the involvement with the GI tract include the following:
The liver may also be affected. A small number of patients may actually develop liver disease. Symptoms of liver disease may include:
Specific treatment for cystic fibrosis will be determined by your physician based on: