Introduction

Cystic fibrosis (CF) is a multisystem disease. The basic genetic defect affects epithelial cells lining ductal systems. Consequently, significant pathology occurs in the bronchial and biliary trees, the gastrointestinal tract, the pancreatic ducts and the vas deferens. However, chronic pulmonary infection is responsible for almost the total mortality of patients with cystic fibrosis. Currently, medical management is palliative rather than curative. Patient care may be shared but should primarily be located in centres of expertise. Treatment of pulmonary infection is directed at delaying and diminishing the chronic pulmonary sepsis which leads to lung destruction and terminal respiratory failure. Death can be predicted to occur within two years for half of those patients whose lung function has fallen to a third of predicted values.

Improved management of CF lung disease has produced survival into adulthood (16 years old) for 90% of paediatric patients; a profound change from three decades ago when the majority of patients died in early childhood. It has been predicted that better care will increase median survival into the fourth decade of life by the year 2000. However, current medical treatment is not curative. Our current knowledge of the pathogenesis of CF pulmonary infection coupled with aggressive treatment is insufficient to halt the relentless advance of lung sepsis. Recent medical advances and scientific breakthroughs need to be translated into therapy to produce survival to a pensionable age.