Cystic Fibrosis

Cystic fibrosis is an inherited disease of the mucus and sweat glands. Cystic fibrosis mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. Until the 1980s, most deaths from cystic fibrosis occurred in children and teenagers. Today, with improved treatments, people with cystic fibrosis live, on average, to be more than 35 years old.

 

An Introduction to Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. Cystic fibrosis mainly affects the:
 
  • Lungs
  • Pancreas
  • Liver
  • Intestines
  • Sinuses
  • Sex organs.
     
In the United States, cystic fibrosis is a common genetic disease that mainly affects Caucasians (whites). Cystic fibrosis also affects other ethnic groups, but this is much less common.
 
Cystic fibrosis occurs in:
 
  • About 1 in 3,000 Caucasian births
  • About 1 in 15,000 African-American births
  • About 1 in 31,000 Asian-American births.
     

Cystic Fibrosis: Understanding Mucus and CF

Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, in cystic fibrosis, an abnormal gene, called the cystic fibrosis transmembrane conductance regulator (CFTR) gene, causes mucus to become thick and sticky. The thick, sticky mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs.
 
The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These digestive enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully.
 
As a result of cystic fibrosis:
 
  • Nutrients leave your body unused, and you can become malnourished
  • Your stools become bulky
  • You may not get enough vitamins A, D, E, and K
  • You may have intestinal gas, a swollen belly, and pain or discomfort.
     
(Cystic Fibrosis Continued: Page 2)
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Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD