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DOI: 10.1055/s-0029-1238909
The Clinical Approach to Lung Disease in Patients with Cystic Fibrosis
Publication History
Publication Date:
16 September 2009 (online)
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ABSTRACT
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.
KEYWORDS
Cystic fibrosis - inflammation - antibiotic therapy - treatment - prophylaxis - pathogenesis
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Brian P O'SullivanM.D.
Department of Pediatrics, University of Massachusetts Medical School, UMass Memorial Medical Center–University Campus
55 Lake Ave. North, Worcester, MA 01655
Email: osullivb@ummhc.org