Treatment Strategies for Maintaining Pulmonary Health in Cystic Fibrosis

I. Introduction Progressive lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). To address this, significant effort has been put forth in understanding the pathophysiology of the disease and in developing therapies. In this chapter, a conceptual framework for maintenance treatment of CF pulmonary disease is presented. This framework includes both models of the progression of CF lung disease and strategies to maintain and improve lung health. Specific therapies are discussed, but the reader is encouraged to recognize that therapies for CF lung disease are constantly evolving.