Anaesthesia in patients with cystic fibrosis

Abstract

Cystic fibrosis is an autosomal-recessive disorder. In 1989 the gene mutation that causes cystic fibrosis was localized on the long arm of chromosome 7. Cystic fibrosis occurs in 1/2000 children and the majority now reach adulthood. In view of numerous clinical manifestations of cystic fibrosis, these patients frequently require surgery. Cystic fibrosis is therefore of increasing interest to anaesthesiologists. Preoperative assessment is reviewed. Pre-, intra- and postoperative care must be directed toward optimal clearance of viscous respiratory secretions, and should minimize the risk of postoperative respiratory complications. All procedures should be planned but it is very important to prepare patients for surgery, with daily physiotherapy, administration of therapeutic agents using aerosols, management of nutrition and pancreatic enzymes, and administration of vitamins and antibiotics if indicated. Currently, anaesthesia can safely be carried out in cystic fibrosis patients undergoing minor surgery, with very low incidence of postoperative respiratory complications. Finally, organ transplantation, and in particular lung transplantation, with all its attendant anaesthesiological implications, has improved the outcome for many patients with cystic fibrosis.