Cystic Fibrosis Pulmonary Exacerbation – Natural History, Causative Factors and Management

Abstract

Cystic Fibrosis (CF) is the most common life-threatening inherited disease in the United Kingdom, affecting over 9,000 people, 56% of which are 16 years or older (UK CF Registry Annual Data Report 2009). Life expectancy currently remains well below the general population, but with improvement of care most CF patients born after 1980 are expected to reach adulthood. Much of the morbidity and mortality associated with CF are related to the respiratory system (Goss & Burns, 2007). Recurrent acute infective pulmonary exacerbations are one of the most important features of CF. Pulmonary exacerbations are common events throughout the lifetime of a patient with CF. Frequent exacerbations are associated with impaired quality of life and accelerated de