Cystic Fibrosis
Considerations
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High risk for perioperative pulmonary complications
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Pathophysiologic sequelae:
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Pulmonary: mucous plugging, chronic pneumonia, bronchiectasis & hemoptysis, bronchospasm, pneumothorax, mixed restrictive & obstructive lung physiology, bullous disease, hypoxemia/hypercarbia
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Cardiovascular: pulmonary hypertension, cor pulmonale
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Extra-pulmonary disease:
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Anemia of chronic disease
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GERD, sinusitis
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Hepatic: abnormal transaminases, cirrhosis, portal hypertension, coagulopathy
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Pancreatic insufficiency & diabetes
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Chronic malnutrition, cachexia, deconditioning
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CF-related medications: oxygen, bronchodilators, mucolytics, antibiotics, steroids, insulin, pancreatic enzymes
Goals & Conflicts
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Preoperative optimization in collaboration with respiratory medicine
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Avoidance of GA if feasible
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Intraoperative management principles:
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Lung protective ventilation
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Aggressive pulmonary toilet, bronchodilation, hydration
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Avoid prolonged ventilation
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Multimodal analgesia with limited sedating analgesics
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Avoid exacerbation of pulmonary hypertension
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Postoperative high acuity setting with aggressive respiratory therapy
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Verify normal coagulation parameters prior to neuraxial/regional
Pregnancy
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Very high risk patient & ↑ risk of low birth weight babies & pre-term delivery:
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Vaginal delivery:
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Ensure monitored setting, consider invasive monitoring if significant cardiorespiratory dysfunction
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Epidural is an excellent choice to reduce hyperventilation & stress, but titrate carefully to T10 to prevent respiratory muscle weakness
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Cesarean delivery:
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Epidural preferred:
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Careful titration of epidural to avoid high block
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GA is acceptable but remember goals:
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Prevent perioperative bronchospasm
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Frequent suctioning for pulmonary toilet
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Appropriate ventilatory settings, especially to avoid air trapping/pneumothorax
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Post-op monitoring in HAU/ICU, chest physiotherapy, pulmonary optimization
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NIPPV to treat respiratory failure
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