Treatment of pulmonary exacerbations in adult cystic fibrosis patients: a review.
Cystic fibrosis (CF) is a complex multisystemic disease requiring specialized care. It is characterized by progressive pulmonary decline, with intermittent worsening of lung function, often called pulmonary exacerbations. Eighty-five percent of all deaths from CF are a result of pulmonary disease, and pulmonary exacerbations are associated with decline in lung function. Treating pulmonary exacerbations requires a multifaceted approach and includes a multidisciplinary team composed of a physician, nurse, respiratory therapist, physical therapist, and nutritionist. Recently, the Cystic Fibrosis Foundation published guidelines addressing the comprehensive treatment of pulmonary exacerbations. This article outlines the treatment goals established by these guidelines, including the evaluation and treatment of comorbid conditions (eg, CF-related diabetes) and serious complications associated with CF exacerbations (eg, pneumothorax). Proper treatment of pulmonary exacerbations in CF patients will continue to improve their health and quality of life.
Barto
Tara Lynn
TL
Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical University of South Carolina, Charleston, SC 29425, USA. barto@musc.edu
Flume
Patrick A
PA
D000208:Acute Disease; D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001229:Aspergillosis, Allergic Bronchopulmonary / Q000209:etiology; D002423:Cause of Death; D003550:Cystic Fibrosis / Q000150:complications*; D004334:Drug Administration Schedule; D006469:Hemoptysis / Q000209:etiology; D006801:Humans; D018529:Nutritional Support; D010348:Patient Care Team / Q000458:organization & administration; D011030:Pneumothorax / Q000209:etiology; D017410:Practice Guidelines as Topic; D012042:Registries; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D012138:Respiratory Therapy / Q000379:methods
D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal