What is Cystic Fibrosis?

Cystic fibrosis is a fatal genetic disorder that affects over 30,000 Americans. This disease is marked by chronic infections, clogged airways, and digestive and reproductive problems.

In cystic fibrosis patients, problems occur because the mucus in their lungs is very thick and sticky. Instead of serving as a lubricant, it clogs the respiratory system and allows bacteria to grow within it, impairing the body's natural defenses. The thick mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to digest food.

Cystic fibrosis occurs in roughly one of every 3,000 live births. To contract the disease, a child must inherit a defective copy of the CF gene from each parent. One in 20 Americans - more than 12 million - is an unknowing, symptomless carrier of the disease.

Each time two CF gene carriers conceive a child, there is a 25-percent chance the child will be born with the disease, a 50-percent chance that the child will be a carrier, and a 25-percent chance that the child will be a non-carrier.

Although cystic fibrosis is not yet curable, the good news is that the situation is not hopeless. Each day since the cystic fibrosis gene was discovered in 1989, the pace of CF research has greatly accelerated, and scientists continue to find ways to help those who live with the disease. These treatments include the use of tobramycin - a drug therapy for CF approved by the Food and Drug Administration in 1997 - and experimental gene therapy treatment.

The prognosis is encouraging: in the 1960s, children struck by the disease were not expected to reach their eighth birthday; today, most have an average lifespan of 30 years.

The disease, which is not contagious, usually is diagnosed in early childhood. The most reliable diagnostic test is the "sweat test," aptly named because most cystic fibrosis patients have skin that is salty to the taste. Other common symptoms of CF include chronic cough and difficulty breathing, frequent sinus and respiratory infections, nasal polyps, excessive production of mucus, recurrent pneumonia, frequent foul smelling stools and diarrhea, enlarged fingertips, and sterility in males.

The daily rigors of living with cystic fibrosis is tough both on the children afflicted with the disease and on their families. Fortunately, a combination of therapy and pharmaceuticals can help keep the symptoms under control and enhance the child's quality of life. The long-term treatment process includes: enzymes that help in digestion; high doses of antibiotics during bacterial infections; daily therapy sessions that include special exercises to loosen and promote drainage of the mucus; other types of exercise to strengthen breathing and cardiovascular function; proper nutrition and vitamins; and several inhalation treatments each day to cut through the mucus.

This information about Cystic Fibrosis is complements of the Boomer Esiason Foundation web page. His son currently suffers from CF.

PLEASE Help Cure CF - Heidi
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