Monday, May 2, 2011

What is Cystic Fibrosis?

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 Cystic Fibrosis

Cystic fibrosis (SIS-tik fi-BRO-sis), or CF, is an inherited disease of your secretory glands, including the glands that make mucus and sweat.
"Inherited" means that the disease is passed through the genes from parents to children. People who have cystic fibrosis inherit two faulty cystic fibrosis genes—one from each parent. The parents likely don't have the disease themselves.
Cystic fibrosis mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.
Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if you have cystic fibrosis, your mucus becomes thick and sticky. The mucus builds up in your lungs and blocks your airways—the tubes that carry air in and out of your lungs. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage your lungs.
The thick, sticky mucus also can block tubes, or ducts, in your pancreas. As a result, the digestive enzymes that your pancreas makes can't reach your small intestine.
These enzymes help break down the food that you eat. Without them, your intestines can't fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients leave your body unused. It also can cause bulky stools, intestinal gas, a swollen belly from severe constipation, and pain or discomfort.
Cystic fibrosis also causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can upset the balance of minerals in your blood and cause a number of health problems. Examples include dehydration (a condition in which your body doesn't have enough fluids), increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death.
If you or your child has cystic fibrosis, you're also at increased risk for diabetes or a bone-thinning condition called osteoporosis. Cystic fibrosis also causes infertility in men, and it can make it harder for women to get pregnant.

What are other names for cystic fibrosis?

  • CF
  • Cystic fibrosis of the pancreas
  • Fibrocystic disease of the pancreas
  • Mucoviscidosis (MU-ko-vis-i-DO-sis)
  • Mucoviscidosis of the pancreas
  • Pancreas fibrocystic disease
  • Pancreatic cystic fibrosis

What causes cystic fibrosis?

A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes a protein that controls the movement of salt and water in and out of your body's cells. In people who have cystic fibrosis, the gene makes a protein that doesn't work right. This causes thick, sticky mucus and very salty sweat.
Research suggests that the CFTR protein also affects the body in other ways. This may help explain other symptoms and complications of cystic fibrosis.
More than a thousand known defects can affect the CFTR gene. What type of defect you or your child has may influence how severe cystic fibrosis is. Other genes also may play a role in how severe the disease is.

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