The Clinical Approach to Lung Disease in Patients with Cystic Fibrosis

Brian P O'Sullivan; Patrick Flume

doi:10.1055/s-0029-1238909

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2009; 30(5): 505-513
DOI: 10.1055/s-0029-1238909

The Clinical Approach to Lung Disease in Patients with Cystic Fibrosis

Brian P. O'Sullivan¹ , Patrick Flume²

¹Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts
²Department of Medicine, Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, Medical College of South Carolina, Charleston, South Carolina

Abstract

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

Full Text

References

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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