CF affects approximately 30,000 children and young adults. It occurs
in approximately one of every 3,200 live Caucasian births (in one of every
3,900 live births of all Americans.) There are about 1,000 new cases
of CF diagnosed each year. Most individuals are diagnosed by the age
of three, however, nearly eight percent of all newly diagnosed cases are 18
or older. According to the CF Foundation's National Patient Registry,
one half of all individuals with CF live to the age of 31, one half do not.
One in 31 Americans (one in 28 Caucasians) - more than 10 million people -
is an unknowing, symptomless carrier of the defective gene.
An individual must inherit a defective copy of the CF gene - one from each
parent - to have cystic fibrosis. Each time two carriers conceive a
child, there is a 25 percent chance that the child will have CF; a 50
percent chance that the child will be a carrier, and a 25 percent chance
that the child will be a non-carrier.
CF has a variety of symptoms. The most common are: very salty-tasting
skin; persistent coughing, wheezing or pneumonia; excessive appetite but
poor weight gain; and bulky stools.
The basic defect in CF cells is the faulty transport of sodium and chloride
(salt) within epithelial cells - which line organs such as the lungs and
pancreas - to their outer surfaces.
CF causes the body to produce an abnormally thick, sticky mucus. This
abnormal mucus clogs the lungs and leads to fatal infections. The
thick CF mucus also obstructs the pancreas, preventing enzymes from reaching
the intestines to digest food.
The treatment of CF depends upon the stage of the disease and which
organs are involved. One means of treatment, postural drainage (also
called chest physical therapy [CPT]), requires vigorous percussion (by using
cupped hands) on the back and chest to dislodge the thick mucus from the
lungs. Antibiotics are also used to treat lung infections and are
administered intravenously, via pills, and/or medicated vapors which are
inhaled to open up clogged airways. When CF affects the digestive
system, the body does not absorb enough nutrients. Therefore, people
with CF may need to eat an enriched diet and take both replacement vitamins
and enzymes.
