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Cystic fibrosis
(CF)
Cystic fibrosis (CF) - a life-threatening
disorder that causes severe lung damage and
nutritional deficiencies - used to be a genetic
mystery, and most people with the disease didn't
live beyond their teens. But researchers have made
progress in unraveling the genetic basis of CF,
which has led to earlier detection. In addition,
improved and more consistent treatments now allow
people with CF to live into their 30s and 40s and
even beyond, and to have fuller, more comfortable
lives.
Treatment
Many
treatments exist for the symptoms and complications of CF. The main goal
is to prevent infections, reduce the amount and thickness of secretions
in the lungs, improve airflow, and maintain adequate calories and
nutrition.
To
accomplish these objectives, treatments for CF may include:
-
Antibiotics.
Newer antibiotics may more effectively fight the bacteria that cause
lung infections in people with CF. Among these are aerosolized
antibiotics that send medication directly into airways. One of the
major drawbacks of long-term use of antibiotics is the development
of bacteria that are resistant to drug therapy.
-
Mucus-thinning drugs.
When your white blood cells attack bacteria in your airways, DNA in
the cells is released, making the mucus in your airways even
thicker. The aerosolized drug dornase alfa (Pulmozyme) is an enzyme
that fragments DNA, making mucus thinner and easier to cough up.
Side effects of Pulmozyme may include airway irritation and sore
throat.
-
Bronchodilators.
Use of medications such as albuterol (Proventil, Ventolin) may help
keep open the bronchial tubes by clearing thick secretions.
-
Bronchial
airway drainage.
People with CF need a way to physically remove thick mucus from
their lungs. This is often done by manually clapping on the front
and back of the chest — a procedure that's best performed with the
person's head over the edge of the bed so that gravity helps clear
the secretions.
In some
cases an electric chest clapper, known as a mechanical percussor, is
used. An inflatable vest that vibrates at high frequency can also help
people with CF cough up secretions. Both adults and children with CF
need to have bronchial airway drainage at least twice a day for 20 to 30
minutes. Older children and adults can learn to do this themselves,
especially if they use mechanical aids such as vests and percussors.
Young children need the aid of parents, grandparents or older siblings.
-
Oral
enzymes and better nutrition.
CF can cause you to become malnourished because the pancreatic
enzymes needed for digestion don't reach your small intestine,
preventing food from being absorbed. As a result, you may need many
more calories than you otherwise would. Supplemental high-calorie
nutrition, special vitamins and enteric-coated oral pancreatic
enzymes can help you maintain or even gain weight.
-
Lung
transplantation.
Your doctor may suggest lung transplantation if you have severe
breathing problems, life-threatening pulmonary complications or
increasing resistance to antibiotics used to treat lung infections.
Whether you're a good candidate for the procedure depends on a
number of factors, including your overall health, certain lifestyle
factors and the availability of donor organs. Because both lungs are
affected by CF, both need to replaced. If your chest isn't large
enough to hold two adult donor lungs, your surgeon is likely to use
two lower lobes contributed by two living donors. However it's
performed, lung transplantation is a major operation and may lead to
serious complications, especially post-surgical infections. The
five-year survival rate has increased in the last 10 years, however.
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