What Is Cystic Fibrosis?
Cystic fibrosis (CF) is an inherited disease of your
mucus and sweat glands. It affects mostly your lungs, pancreas, liver,
intestines, sinuses, and sex organs.
Normally, mucus is watery. It keeps the linings of
certain organs moist and prevents them from drying out or getting infected. But
in CF, an abnormal gene causes mucus to become thick and sticky.
The 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
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 enzymes help break down
the food that you eat. Without them, your intestines cannot absorb fats and
As a result:
- Nutrients leave your body unused, and you can
- Your stools become bulky.
- You may not get enough vitamins A, D, E, and
- You may have intestinal gas, a swollen belly, and
pain or discomfort.
The abnormal gene also causes your sweat to become
extremely salty. As a result, when you perspire, your body loses large amounts
of salt. This can upset the balance of minerals in your blood. The imbalance
may cause you to have a heat emergency.
CF can also cause infertility (mostly in men).
The symptoms and severity of CF vary from person to
person. Some people with CF have serious lung and digestive problems. Other
people have more mild disease that doesn't show up until they are adolescents
or young adults.
Respiratory failure is the most common cause of
death in people with CF.
Until the 1980s, most deaths from CF occurred in
children and teenagers. Today, with improved treatments, people with CF live,
on average, to be more than 35 years old. Research continues to look for: