Cystic fibrosis and anaesthesia

Abstract

Cystic fibrosis (CF) was first identified in 1938 by Dorothy Hansine Anderson, who initially called it ‘cystic fibrosis of the pancreas’. It is a multisystem autosomal recessive disease and the most common lethal genetic disease in Caucasians. CF is particularly common in Western Europe. The Republic of Ireland has the highest incidence in the world, at one in 1461 live births with a carrier rate for the genetic mutation of one in 19. In the UK, the carrier rate is one in 25 with an incidence of one in 2500 live births. In 2008, there were 8513 patients with CF on the UK Register. In Ireland in 2007, there were 1170 CF patients. CF is best considered as a disease spectrum rather than a distinct single clinical entity due to the heterogeneity of genotypic and phenotypic manifestations.