Cystic Fibrosis: More Than a Lung Disease
Cystic fibrosis (CF) is a chronic, life-shortening disease that occurs as a result of a genetic defect. The defective gene interferes with the body’s ability to transfer water and salt to and from cells. This causes secretions, which are normally thin and watery in healthy people, to become very thick and sticky. The thick secretions clog up organs and prevent them from working properly.
Cystic fibrosis causes problems in the lungs when the thick mucus builds up and gets stuck in the airways.
When this happens:
- Breathing passageways become blocked and air can’t get through.
- Bacteria grow in the mucus collections and causes infection in the lungs, nose, and sinuses.
- Nasal polyps may develop in some CF patients.
The pancreas is part of the digestive system. Its job is to secrete enzymes that are needed to digest food, and a hormone called insulin that controls blood sugar.
Cystic fibrosis also causes these secretions to become thick.
When this happens:
- The pancreatic ducts become clogged.
- Enzymes can’t get past the obstruction.
- Food is not digested properly and the body can’t absorb nutrients.
- Eventually, the obstruction of the pancreas may cause scarring that damages the insulin-producing cells and prevents them from producing insulin. Insulin is not available to the cells, which causes blood sugar levels to rise. This condition is called insulin-dependent diabetes, which happens in about 15% of all CF patients.
Liver and Gallbladder
Although it is not common, thickened secretions can also clog up the bile ducts of the liver and gallbladder and prevent them from working properly.
If blockage of ducts in the liver continues for long periods of time, the liver could become permanently damaged. If the ducts of the gallbladder become clogged, the gallbladder is usually removed.
Sometimes, the intestines are the first organ to be affected by cystic fibrosis. In about 20% of all newborns with CF, thick secretions form in the intestines causing a life-threatening blockage of the intestines known as a meconium ileus.
Cystic fibrosis affects the reproductive organs of men and women differently.
Men: are almost always infertile because the thick secretions in the sperm canal cause a blockage that prevents sperm from getting through.
Women: are often able to conceive but may have decreased fertility because of thick cervical mucus blocking the entry of sperm.