Cystic Fibrosis (CF)

Optimize the lungs first - Preoperative chest physiotherapy, bronchodilators, and treatment of any active infection; involve pulmonology. Assess baseline pulmonary function and oxygenation.

Manage secretions - Use humidified gases, adequate hydration, and frequent tracheal suctioning; thick secretions can obstruct the tube. Deep anesthesia at instrumentation helps blunt bronchospasm.

Reactive airway - Treat bronchospasm proactively with bronchodilators and ensure adequate depth before airway manipulation and extubation.

Protect respiratory function postoperatively - Minimize long-acting respiratory depressants, favor multimodal and regional analgesia, and plan early mobilization, chest physiotherapy, and monitored recovery.

Systemic disease - Screen for CF-related diabetes and manage glucose; note pneumothorax risk from bullae when using positive-pressure ventilation and PEEP.

Regional where feasible - Regional and neuraxial techniques that avoid airway instrumentation and preserve respiratory mechanics are attractive.

An autosomal recessive disease caused by mutations in the CFTR chloride channel, producing thick, viscous secretions that damage multiple organs. The lungs bear the brunt: bronchiectasis, chronic bacterial infection, airway obstruction with a reactive component, and progressive respiratory failure - the leading cause of death. Pancreatic exocrine insufficiency, cystic-fibrosis-related diabetes, sinus disease, and hepatobiliary involvement complete the picture. Anesthetic management centers on optimizing and protecting fragile, secretion-laden lungs.