The clinical approach to lung disease in patients with cystic fibrosis.
There is strong evidence that early, aggressive therapy of lung disease leads to improved quality and quantity of life for patients with cystic fibrosis (CF). The treatment of pulmonary disease associated with CF is multifactorial, encompassing prophylaxis, aggressive treatment of infection, use of antiinflammatory agents, and treatment of severe complications. Chest physiotherapy on a regular basis, perhaps using new modalities that allow patient autonomy, is also crucial. This review covers the pathogenesis of CF lung disease and current approaches to therapy, highlighting guidelines recently published by the Cystic Fibrosis Foundation. Clinicians caring for patients with CF should maximize current therapies with the goal of preserving lung function until the time a more definitive curative or controller medication is developed. Empowering patients in the process of providing their own care is a key to achieving this goal.
O'Sullivan
Brian P
BP
Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA. osullivb@ummhc.org
Flume
Patrick
P
D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D026684:Personal Autonomy; D017410:Practice Guidelines as Topic; D011788:Quality of Life; D012129:Respiratory Function Tests; D012138:Respiratory Therapy / Q000379:methods; D012141:Respiratory Tract Infections / Q000188:drug therapy* / Q000209:etiology / Q000382:microbiology