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- Cystic fibrosis is a genetic disease
that causes mucous-secreting glands to produce abnormal
secretions, resulting in improper lung tissue lubrication
and subsequent damage.
- The mucous in patients with cystic fibrosis tends to be very thick, making it impossible for the lungs to be cleared properly of bacteria. This invites multiple infections (bronchitis, Pneumonia, Bronchiectasis), Atelectasis (collapse
of lung tissue), and lung scarring.
- Cystic fibrosis is a very serious
illness in which only about half of those affected live past
20 years of age. It is the most commonly inherited disease
leading to death among the white population of the U.S.
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- Constant Lung Infections
- Pancreatic problems
- Infertility
- Cough
- Fatigue on exercise
- Greasy
stools
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- Clubbed fingers
- Chest is widened front-to-back
- Lung exam has crackles
- Nasal polyps
- Tenderness in right upper
abdomen
- Arterial blood gas shows decreased
oxygenation
- Pulmonary function test shows
abnormal
- Sweat test shows elevated Sodium
chloride
- Chest X-Rays reveal increased interstitial markings, Atelectasis
- CT scan may be
abnormal
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- Cystic fibrosis centers and
specialists are needed for treatment.
- Antibiotics for infections (may have
complicated infections)
- Inhaled aerosol (saline) solutions
- Bronchodilators such as Albuterol
- Prednisone may be used
- Recombinant human
deoxyribonuclease thin secretions
- Chest physiotherapy (chest
"pounding" and massage to promote clearing secretions)
- Pneumonia vaccine
every five years
- Influenza vaccine
yearly
- Proper nutrition
- Genetic counseling
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