What is Cystic Fibrosis?
Cystic Fibrosis is a fatal genetic disease 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 the CF gene from each parent. One in 20 Americans – more than 12 million is an unknowing, symptomless carrier of the disease.
People with CF have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm, wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene.
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.
A treatment of CF depends upon the state of the disease and the organs involved. Clearing mucus from the lungs is an important part of the daily CF treatment regimen. Chest physical therapy is a form of airway clearance done by vigorous clapping on the back and chest to dislodge the thick mucus from the lungs.
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.
Although Cystic Fibrosis is not yet curable, the good news in 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.
The prognosis is encouraging: in the 1960s, children struck by the disease were not expected to reach their 8th birthday; today, most have an average lifespan of 30 years.
