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Cystic Fibrosis

more about Cystic Fibrosis

  • 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.

  • Constant Lung Infections
  • Pancreatic problems
  • Infertility
  • Cough
  • Fatigue on exercise
  • Greasy stools

  • Examination:
    1. Clubbed fingers
    2. Chest is widened front-to-back
    3. Lung exam has crackles
    4. Nasal polyps
    5. Tenderness in right upper abdomen
  • Laboratory findings:
    1. Arterial blood gas shows decreased oxygenation
    2. Pulmonary function test shows abnormal
    3. Sweat test shows elevated Sodium chloride
  • Imaging:
    1. Chest X-Rays reveal increased interstitial markings, Atelectasis
    2. CT scan may be abnormal

  • Cystic fibrosis centers and specialists are needed for treatment.
  • Antibiotics for infections (may have complicated infections)
    1. Inhaled aerosol (saline) solutions
    2. Bronchodilators such as Albuterol
    3. Prednisone may be used
    4. 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

more about Cystic Fibrosis

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