fibrosis is a fatal genetic disorder 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.
occurs in roughly one of every 3,000 live births. To contract
the disease, a child must inherit a defective copy of the CF gene
from each parent. One in 20 Americans - more than 12 million -
is an unknowing, symptomless carrier of the disease.
Each time two
CF gene carriers conceive a child, there is a 25-percent chance
the child will be born with the disease, a 50-percent chance that
the child will be a carrier, and a 25-percent chance that the
child will be a non-carrier.
fibrosis is not yet curable, the good news is 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. These treatments include the use of tobramycin - a drug
therapy for CF approved by the Food and Drug Administration in
1997 - and experimental gene therapy treatment.
is encouraging: in the 1960s, children struck by the disease were
not expected to reach their eighth birthday; today, most have
an average lifespan of 30 years.
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. Other common symptoms of CF include chronic
cough and difficulty breathing, frequent sinus and respiratory
infections, nasal polyps, excessive production of mucus, recurrent
pneumonia, frequent foul smelling stools and diarrhea, enlarged
fingertips, and sterility in males.
The daily rigors
of living with cystic fibrosis is tough both on the children afflicted
with the disease and on their families. 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.
about Cystic Fibrosis is complements of the Boomer
Esiason Foundation web page. His son currently suffers from