Cholangitis: definition
What Is Cystic Fibrosis?
[dropcap]C ystic fibrosis is a genetic disease of the exocrine glands which causes the body to produce abnormally thick and sticky mucus and results in progressive damage to the respiratory system and digestive problems.
Severity of disease depends on the particular genetic mutation of affected individuals.
Q: How does the abnormal mucus damage the lungs and digestive tract?
A: In all cases of cystic fibrosis, sticky mucus build-up in the bronchioles (small breathing passages) of the lungs causes COPD (chronic obstructive pulmonary disease) that results in life-threatening lung infections.
In the pancreas, the abnormal mucus damages its ability to produce required enzymes to digest food, causing failure to adequately digest fats, carbohydrates and protein which are vital for life.
Other tissues that may be involved are sweat glands, sinuses, and the male reproductive system, causing infertility.
Millions of Americans carry the defective cystic fibrosis gene, but do not have any symptoms. That is because cystic fibrosis requires the inheritance of two defective cystic fibrosis genes — one from each parent who carries it. An estimated 1 in 29 Caucasian Americans have the cystic fibrosis gene.
The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It is more common among those of Northern or Central European descent. About 30,000 children and adults in the United States have cystic fibrosis while more than 10 million people carry the gene.1
There is no cure yet for cystic fibrosis, however, treatments have greatly improved in recent years. The goals of treatment include:
- Preventing and controlling lung infections.
- Loosening and removing thick, sticky mucus from the lungs.
- Preventing or treating blockages in the intestines.
- Providing enough nutrition.
- Preventing dehydration (a lack of fluid in the body).2
