New Clinical Guidelines for Treating Exacerbations in Cystic Fibrosis

Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States, according to the CF Foundation. Although the disease affects many organs, 85% of deaths in CF results from lung disease. CF is associated with progressive decline of lung function. Episodes of acute worsening of respiratory symptoms—or pulmonary exacerbations—frequently occur, and there are various clinical features associated with exacerbations (Table 1). “Pulmonary exacerbations have an adverse impact on patients’ quality of life and overall cost of care,” says Patrick A. Flume, MD. “They’re also the most common reason for hospitalization of CF patients. Identifying optimal treatment methods for pulmonary exacerbations in CF could lead to significant improvements in quality and length of life for patients living with the disease.” In the November 1, 2009 American Journal of Respiratory and Critical Care Medicine and available for free online at, guidelines for the treatment of pulmonary exacerbations in CF were published. Dr. Flume and members of the CF Foundation’s Pulmonary Therapies Committee collaborated to evaluate published results of controlled trials of common treatment methods for exacerbations in an effort to identify best treatment practices. “The intent of this research was to evaluate the evidence supporting therapies and approaches for the management of health decline,” says Dr. Flume. “Our systematic review allowed the committee to make specific treatment recommendations [Table 2] as well as determine areas that require further study. The guidelines are designed for general use in most patients with CF, but they should be adapted to meet specific needs as determined by the patient, their...