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Risk
Factors & Prevention
> Symptoms
& Treatment
Cystic
Fibrosis
Cystic fibrosis
is an inherited disease that affects the respiratory and
digestive systems. Cystic fibrosis affects the exocrine
(mucus and sweat) glands of the body and is caused by a
defective gene. Thick mucus is formed in the bronchial tree
which predisposes the person to chronic lung infections.
Many pancreatic enzymes involved in the breakdown and
absorption of fats in the intestine are absent causing
malabsorption (inadequate absorption of nutrients from the
intestinal tract) and malnutrition.
Risk
Factors & Prevention
About one in 2500 Caucasians
is affected and one in 25 is a carrier of the cystic
fibrosis gene. It is the most common cause of chronic lung
disease in children and young adults, and the most common
fatal hereditary disorder affecting Caucasians in the US.
Risk factors include a family history of cystic fibrosis or
unexplained infant death. The incidence in adults is 2 out
of 10,000 people.
Screening of family members of a cystic fibrosis patient may
detect the cystic fibrosis gene in up to 75% of carriers.
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Symptoms
& Treatment
Symptoms of cystic fibrosis
include:
- no meconium stool in first
24 hr. of life
- stools, pale or clay
colored and foul smelling
- stools - floating
- skin may taste salty
(infants)
- persistent respiratory
infections such as pneumonia
- coughing or wheezing
- weight loss
- clubbing of the fingers or
toes
- diarrhea
- delayed growth
- easy fatigue
- splenomegaly
Early recognition of cystic
fibrosis and a comprehensive, multidisciplinary treatment
program can lengthen survival time and improve the quality
of life. Specialty clinics for cystic fibrosis are present
in many communities.
Medications include antibiotics for respiratory infections
and pancreatic enzymes to replace the missing enzymes.
Mucomyst may be used on occasion to thin secretions.
Other treatments include postural drainage and chest
percussion, and other breathing treatments.
Lung transplant may be considered in some cases.
New treatments include replacement of the DNAase enzyme.
This is available as a medication called dornase. Genetic
research is ongoing in hopes of correcting the disease by
artificially inserting a "normal" gene into the
person. Using retroviruses to insert operating genes into
the lungs has proven effective. Research on possible methods
used to correct the disorder before birth also looks
promising.
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