The General Approach to Cystic Fibrosis-Related Pulmonary Infection in the United States

Summary

The principal cause of morbidity and mortality in patients with cystic fibrosis remains pulmonary infection, resulting in progressive airway destruction, bronchiectasis, and pulmonary failure [1]. Endobronchial infection including pneumonia, with or without an appropriate inflammatory response, is nearly universal in cystic fibrosis patients. Our success at controlling albeit not curing, the pulmonary infection in cystic fibrosis has undoubtedly been a major cause of a remarkable improvement in median survival rates and quality of life seen in the U.S. over the past 20 years (Figure 1). This is also clearly related to coordinated management strategies disseminated by the U.S. CF Foundation, through their accredited clinical centers. This paper will focus on several aspects of the management of pulmonary infections that have been prioritized in the United States. First among these has been the development of new antibiotics and new antibiotic strategies to control acute bronchopulmonary exacerbations in cystic fibrosis. Second has been the development of appropriate strategies that may be useful in preventing progressive pulmonary infection. Third is the development of non-antimicrobial strategies. such as pulmonary clearance methods and anti-inflammatory therapies that may also prove useful in the control of progressive bronchopulmonary disease.