Cystic fibrosis
From Activating Evolution
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Cystic fibrosis (CF) is caused by a defective gene which tells the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and the pancreas, the organ that helps to break down and absorb food.
This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system.
Millions of Americans carry the defective CF gene, but do not have any symptoms. That's because a person with CF must inherit two defective CF genes -- one from each parent. An estimated 1 in 29 Caucasian Americans have the CF gene. The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It's more common among those of Northern or Central European descent.
Most children are diagnosed with CF by their 2nd birthday. A small number, however, are not diagnosed until age 18 or older. These patients usually have a milder form of the disease.
[edit] Treatment
Treatment includes:
- Antibiotics for respiratory infections.
- Pancreatic enzymes to replace those that are missing.
- Vitamin supplements, especially vitamins A, D, E, and K.
- Inhaled medicines to help open the airways.
- DNAse enzyme replacement therapy to thin the mucus and makes it easier to cough up.
- Pain relievers. Research has shown that the pain reliever ibuprofen may slow lung deterioration in some children with cystic fibrosis. The results were most dramatic in children ages 5 to 13.
- Postural drainage and chest percussion.
- Lung transplant may be considered in some cases.
[edit] Prognosis
Disease registries now show that 40% of patients with cystic fibrosis are over age 18.
Today, the average life span for those who live to adulthood is approximately 35 years, a dramatic increase over the last three decades.
Death is usually caused by lung complications.
[edit] In Memory Of
In Memory of Ralph Schults, lost to CF at the age of 22.
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