Cystic fibrosis

Cystic fibrosis (CF) is a multi-system disorder inherited in an autosomal recessive fashion with a carrier rate of 1 in 25 in the UK Caucasian population. The expected survival of a child born today in the UK with CF is at least 50 years. The commonest cause of death is respiratory failure arising from lung damage caused by persistent uncontrolled airway infection. The commonest infection in adults is Pseudomonas aeruginosa. Treatment of the disease is aimed at preventing obstruction of the airways by viscid secretions and prevention or control of airway infection. The outcome for patients with CF has improved with introduction of therapies targeted at each step of the disease pathway and careful attention to detail applied by multidisciplinary teams in CF centres. Thus, over half of the CF patients in the UK are now adults and over half of these are in full-time education or employment.