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Information about Cystic Fibrosis

What is Cystic Fibrosis?

Cystic fibrosis is a chronic, non contagious, fatal genetic disorder that affects over 30,000 Americans. Cystic fibrosis is the most common inherited disease affecting children and young adults. Cystic fibrosis is marked by chronic infections, clogged airways, and digestive and reproductive problems.

Cystic fibrosis affects tissues that produce mucus secretions, such as the airway, the gastrointestinal tract, the ducts of the pancreas, the bile ducts of the liver and the male urogenital tract. Cystic fibrosis affects the body's epithelial cells. Epithelial cells make up the lining of the lungs, pancreas, liver, digestive tract and reproductive system, and are also found in the sweat glands and sinuses.

In cystic fibrosis patients, the mucus is very thick. Mucus usually protects tissues from harm, but abnormally thick mucus obstructs the ducts and airways, causing damage to the tissues. It affects different tissues different ways. In the lungs the thick mucus clogs the respiratory system and allows bacteria to grow in it. The thick mucus also impairs the pancreas by preventing enzymes from reaching the intestines to digest food. The bile ducts in the liver may be affected, causing biliary cirrhosis in a small percentage of patients. Salt absorption in the sweat ducts is also impaired, and cystic fibrosis patients produce extremely salty sweat.

Cystic fibrosis also affects the reproductive organs, causing infertility in nearly all men and some women who have the disease. Men with cystic fibrosis may become infertile because the tubules, called the vas deferens, that transport sperm from the testes are absent or undeveloped. Infertility may occur in women because of abnormal cervical mucus or menstrual irregularity.

Cystic fibrosis is also called CF.

Symptoms of Cystic Fibrosis

The most characteristic symptom of cystic fibrosis is the excessive production of thick, sticky mucus in the airways. Another characteristic is very salty sweat.

How is Cystic Fibrosis Spread?

In order to contract cystic fibrosis, you must inherit a defective copy of the cystic fibrosis gene from each parent. The disease is recessive, so you will not have it if only one of your parents gives you the gene. You cannot "catch" cystic fibrosis from touching someone who has it.

Cystic Fibrosis Statistics

  • Cystic fibrosis occurs in roughly one of every 3,000 live births.
  • Cystic Fibrosis is the #1 genetic killer of children and young adults in the United States.
  • One in 20 Americans - more than 12 million - is an unknowing, symptomless carrier of the disease.
  • Males and females are equally likely to inherit cystic fibrosis.
  • Worldwide, about 70,000 people have cystic fibrosis. Ninety percent are diagnosed before the age of 17.

Can Cystic Fibrosis be Cured?

Currently cystic fibrosis cannot be cure. However, researchers are currently working on finding a cure.

Can Cystic Fibrosis Be Treated?

Cystic fibrosis treatments vary. Cystic fibrosis is treated with a combination of strategies that includes medication, airway clearance therapies and aggressive nutrition.

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