What is Cystic Fibrosis
(CF)?
Cystic fibrosis (CF) is a genetic disease affecting approximately
30,000 children and adults in the United States. A defective gene causes
the body to produce an abnormally thick, sticky mucus that clogs the lungs
and leads to life-threatening lung infections. These thick secretions also
obstruct the pancreas, preventing digestive enzymes from reaching the
intestines to help break down and absorb food.
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 treatment of CF depends upon the stage 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. Other types of treatments include TOBIŽ (tobramycin solution for inhalation), an aerosolized antibiotic used to treat lung infections; PulmozymeŽ, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function; and azithromycin, an antibiotic recently proven to be effective in people with CF whose lungs are chronically infected with the common Pseudomonas aeruginosa bacteria.
According to the CF
Foundation's National Patient Registry, the median age of survival for
a person with CF is 36.8 years. As more advances have been made in the
treatment of CF, the number of adults with CF has steadily grown. Today,
nearly 40 percent of the CF population is age 18 and older. Adults,
however, may experience additional health challenges including CF-related
diabetes and osteoporosis. CF also can cause reproductive problems - more
than 95 percent of men with CF are sterile. But, with new technologies,
some are becoming fathers. Although many women with CF are able to
conceive, limited lung function and other health factors may make it
difficult to carry a child to term.
The above information was taken directly from the Cystic Fibrosis
Foundation website. For more information visit www.cff.org