Cystic fibrosis: a decade of progress

Cystic fibrosis (CF) is an autosomal recessive, multisystem disorder which greatly reduces the lifespan of its victims, largely by gradual, infection-driven destruction of the lungs. Advances in therapy to date have improved median survival from less than 10 years to over 30 years of age. New scientific advances in the past 10 years, notably discovery of the CF gene and increasing understanding of its protein product, hold forth hope of even further therapeutic advances. This review will highlight the clinical characteristics of CF, current standard therapies and directions of ongoing research.