Inpatient care of the adult with an exacerbation of cystic fibrosis

Patients with cystic fibrosis (CF) are living longer, albeit with chronic and progressive lung disease. Pulmonary exacerbations in CF are characterized by an increase in respiratory symptoms and a decrease in pulmonary function and gas exchange. Exacerbations that do not respond to outpatient management require hospitalization. Complications of CF can be devastating and life-threatening. Care of these patients is challenging not only because of the scope and severity of their medical problems, but also because of the need to coordinate services and to maintain continuity during transitions between hospital and home.