Survival Effect of Lung Transplantation Among Patients With Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is an autosomal recessive, multisystem disease leading to significant morbidities and early death. Treatments for pancreatic and pulmonary manifestations have improved median survival in the United States from less than 6 months to about 32 years in 1998.¹ Severe pulmonary disease is the primary cause of mortality in CF, underlying 76.4% of deaths in 1998.¹