Abstract

The life span of people with cystic fibrosis (CF) has increased dramatically over the past 50 years. Many factors have contributed to this improvement. Respiratory exacerbations of CF lung disease are associated with the need for hospitalisation and antibiotic treatment, reduction in the quality of life, fragmented sleep and mortality. A number of preventive treatment strategies have been developed to reduce the frequency and severity of respiratory exacerbations in CF including mucolytic agents, physiotherapy and exercise, antibiotics, nutritional strategies, anti-inflammatory treatments and vaccinations against common respiratory pathogens. The evidence for each of these treatments and their potential impact is discussed.