ANESTHESIA FOR ADULTS WITH CYSTIC-FIBROSIS

Cystic fibrosis is an autosomal recessive disorder which affects one i n 2500 live births. It is a multisystem disease and has a variety of p resentations. The major clinical features affect the gastrointestinal and respiratory tracts. Severe respiratory disease, diabetes and gastr oesophageal reflux are common features of concern to anaesthetists. Im proved care of young patients has allowed many to survive into adultho od. Lung transplantation has significantly improved the outlook for ma ny patients. At Alfred Hospital, 74 patients with cystic fibrosis unde rwent 149 procedures from January 1978 to January 1994, with a mortali ty of 0.6% (95% CI 0.4%-0.8%). This retrospective cohort study describ es the anaesthetic management and perioperative care of these patients . Most of the anaesthetics were for procedures related to cystic fibro sis but 12% were for unrelated conditions. Cystic fibrosis related pro cedures include diagnostic, venous access, enteral feeding procedures, treatment of complications of cystic fibrosis and lung transplantatio n. Despite extremely poor respiratory function, these patients can be managed with acceptably low postoperative mortality (1%). Pre- and pos toperative care must be directed towards optimal clearance of viscous respiratory secretions. Procedures need to be planned so that optimal care can be given by each member of the team caring for cystic fibrosi s patients.