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What Is
Cystic Fibrosis?
Cystic fibrosis is an inherited chronic
disease that affects the lungs and digestive system of about
30,000 children and adults in the United States (70,000 worldwide).
A defective gene and its protein product cause the body to
produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening
lung infections; and
- obstructs the pancreas and stops
natural enzymes from helping the body break down and absorb
food.
In the 1950s, few children with cystic
fibrosis lived to attend elementary school. Today, advances
in research and medical treatments have further enhanced and
extended life for children and adults with CF. Many people
with the disease can now expect to live into their 30s, 40s
and beyond.
Symptoms
of Cystic Fibrosis
People with CF can have a variety of
symptoms, including:
- very salty-tasting skin;
- persistent coughing, at times with
phlegm;
- frequent lung infections;
- wheezing or shortness of breath;
- poor growth/weight gain in spite
of a good appetite; and
- frequent greasy, bulky stools or
difficulty in bowel movements.
Statistics
- About 1,000
new cases of cystic fibrosis are diagnosed each year.
- More than 70%
of patients are diagnosed by age two.
- More than 40%
of the CF patient population is age 18 or older.
- In 2006, the
predicted median age of survival was 37 years.
Treatments
People living with cystic fibrosis must follow a regular
treatment routine to stay healthy and maintain optimal lung
function.
Most often, treatment for cystic fibrosis begins with techniques
to trigger strong coughs that help loosen and clear thick
mucus from the airways. Of course, airway clearance is just
one part of the treatment routine. Nutrition and drug therapies
are also important for children and adults with cystic fibrosis.
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