II. Epidemiology

  1. No increase in fetal malformations noted

III. Management

  1. Pregnancy
    1. Most antibiotics may be used
      1. Avoid Tetracyclines
      2. Quinolones are not Teratogenic
    2. Beta Adrenergic Agonists may be used
  2. Delivery
    1. Avoid general Anesthesia and Narcotics
    2. Epidural Anesthesia is preferred for analgesia
    3. Avoid prolonged labor (maternal exhaustion)
  3. Lactation
    1. Healthy mothers with Cystic Fibrosis may Breast feed
    2. Breast Milk does not contain excess Sodium
    3. Essential Fatty Acids and fat content is lower

IV. Complications

  1. Low Weight Gain in Pregnancy
  2. Cystic Fibrosis exacerbation
  3. Preterm delivery rate: 6 to 35%

V. Prognosis

  1. Factors associated with best pregnancy outcomes
    1. FEV1 >80%
    2. No Burkholderia cepacia colonization
  2. Factors predicting pregnancy complications
    1. Pulmonary Hypertension
    2. Cor Pulmonale
    3. FEV1 <50%

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