Cystic Fibrosis 

 

 

Considerations

 

  • High risk for perioperative pulmonary complications

  • Pathophysiologic sequelae:

    • Pulmonary: mucous plugging, chronic pneumonia, bronchiectasis & hemoptysis, bronchospasm, pneumothorax, mixed restrictive & obstructive lung physiology, bullous disease, hypoxemia/hypercarbia

    • Cardiovascular: pulmonary hypertension, cor pulmonale

  • Extra-pulmonary disease:

    • Anemia of chronic disease

    • GERD, sinusitis

    • Hepatic: abnormal transaminases, cirrhosis, portal hypertension, coagulopathy

    • Pancreatic insufficiency & diabetes 

    • Chronic malnutrition, cachexia, deconditioning

  • CF-related medications: oxygen, bronchodilators, mucolytics, antibiotics, steroids, insulin, pancreatic enzymes

 

 

Goals & Conflicts

 

  • Preoperative optimization in collaboration with respiratory medicine

  • Avoidance of GA if feasible

  • Intraoperative management principles:

    • Lung protective ventilation

    • Aggressive pulmonary toilet, bronchodilation, hydration

    • Avoid prolonged ventilation 

    • Multimodal analgesia with limited sedating analgesics

    • Avoid exacerbation of pulmonary hypertension

  • Postoperative high acuity setting with aggressive respiratory therapy

  • Verify normal coagulation parameters prior to neuraxial/regional

 

 

Pregnancy

 

  • Very high risk patient & ↑ risk of low birth weight babies & pre-term delivery:

    • Vaginal delivery:

      • Ensure monitored setting, consider invasive monitoring if significant cardiorespiratory dysfunction 

      • Epidural is an excellent choice to reduce hyperventilation & stress, but titrate carefully to T10 to prevent respiratory muscle weakness 

    • Cesarean delivery:

      • Epidural preferred:

        • Careful titration of epidural to avoid high block 

      • GA is acceptable but remember goals: 

        • Prevent perioperative bronchospasm

        • Frequent suctioning for pulmonary toilet

        • Appropriate ventilatory settings, especially to avoid air trapping/pneumothorax

      • Post-op monitoring in HAU/ICU, chest physiotherapy, pulmonary optimization 

      • NIPPV to treat respiratory failure 

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