Cystic fibrosis is a genetic disease of the exocrine glands primarily affecting the pulmonary and gas-trointestinal systems. Abnormally thick and viscous secretions coupled with decreased ciliary activity lead to pneumonia, wheezing, and bronchiectasis. Pulmonary function studies reveal increased residual volume and airway resistance with decreased vital capacity and expiratory flow rate. Malabsorption syndrome may lead to dehydration and electrolyte abnormalities.
Premedication should not include respiratory depressants. Anticholinergic drugs have been used in large series without ill effects, and the choice either to use or not to use them appears to be incon-sequential. Induction with inhalational anesthetics may be prolonged in patients with severe pulmonary disease. Intubation should not be performed until the patient is deeply anesthetized to avoid coughing and stimulation of mucus secretions. The patient’s lungs should be suctioned during general anesthesia and before extubation to minimize the accumulation of secretions. Outcome is favorably influenced by preoperative and postoperative respiratory therapy that includes bronchodilators, incentive spirometry, postural drainage, and pathogen-specific antibiotic therapy.