Cystic Fibrosis–related Diabetes: A First Canadian Clinical Practice Guideline

Abstract

CF is an autosomal recessive disease due to dysfunction or absence of the CF transmembrane conductance regulator (CFTR) channel. CF affects >4,000 Canadians, and has a major effect on the lungs, the gastrointestinal system, quality of life, and lifespan [1]. Innovations in nutrition, physiotherapy, and respiratory medicine have revolutionized CF care and increased life expectancy. The current median survival is 59.9 years [1]. New therapies (highly effective modulators) are expected to further improve lifespan.