Basic therapies in cystic fibrosis. Does standard therapy work?
Epidemiologic data demonstrate a dramatic improvement in survival for cystic fibrosis (CF) over the last few decades and projections suggest that trend will continue. Standard therapy works and should be aggressively applied to this patient population. Although the specific therapies have evolved over the years, the basic tenets of CF care remain unchanged and include antibiotics to control infection, airway clearance, and adequate nutrition. This article focuses on treatment of the pulmonary disease and includes a discussion of the following specific components of a standard therapeutic approach to CF: (1) antibiotics, (2) airway clearance and exercise, (3) mucolytics, (4) bronchodilators, (5) oxygen, (6) anti-inflammatory therapies, and (7) nutritional support. Judicious application of these therapies coupled with careful monitoring of pulmonary, nutritional, and metabolic parameters results in most CF patients surviving into adulthood with an acceptable quality of life.
Marshall
B C
BC
Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Intermountain Cystic Fibrosis Center, University of Utah School of Medicine, Salt Lake City, USA.
Samuelson
W M
WM
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001993:Bronchodilator Agents / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000382:microbiology / Q000503:physiopathology* / Q000628:therapy*; D005100:Expectorants / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D009747:Nutritional Physiological Phenomena; D012138:Respiratory Therapy
D000900:Anti-Bacterial Agents; D001993:Bronchodilator Agents; D005100:Expectorants; D005938:Glucocorticoids