Perioperative management of the adult with cystic fibrosis.

Abstract

Since cystic fibrosis (CF) was first differentiated from celiac disease in 1938, the medical care of patients with CF has substantially improved. These improvements have resulted in a significant increase in median survival and the quality of life experienced by patients. The resultant increase in survival has caused the "average" CF patient to be a young adult and not a child. The gene that causes CF was first identified in 1989 and is the first gene discovered by positional cloning. Unfortunately, gene therapy for CF has not been successful, although it continues to hold great promise for future patient care. Although pulmonary disease is responsible for more than 90% of the morbidity and mortality in patients with CF, they also experience pancreatic disease, including diabetes mellitus, bone disease, hepatobiliary disease, and genitourinary disease. The optimal perioperative management of patients with CF requires an understanding of the relevant pathophysiology and the unique challenges presented by these patients. We reviewed these concepts, including special considerations such as liver and lung transplantation and pregnancy.