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Nongenetic influences on cystic fibrosis outcomes

Schechter, Michael S.a,b,c

Current Opinion in Pulmonary Medicine: November 2011 - Volume 17 - Issue 6 - p 448–454
doi: 10.1097/MCP.0b013e32834ba899
Cystic fibrosis: Edited by Michael Boyle

Purpose of review Despite dramatic improvements over the last several decades, tremendous variation exists in cystic fibrosis (CF) health outcomes. Genetic explanations for variations in disease progression are a promising source of information regarding disease biology and pathophysiology, but an understanding of nongenetic factors provides more immediate tools for improving disease outcomes.

Recent findings The clinical course of CF for any individual with CF is determined by the interaction of genetic endowment (including CFTR and modifier genes), the physical environment (including environmental tobacco smoke, outdoor pollution, and pathogenic microorganisms), the sociodemographic, cultural, and family context (which mediates stress, social support, and disease self-management skills), and variations in delivery and acceptance of healthcare treatments. The evidence linking socioeconomic status (SES) with CF outcomes continues to be strong, and appears to be primarily due to associations with environmental and sociocultural stressors and disease-management behaviors rather than barriers in access to healthcare.

Summary Attempts to eliminate detrimental environmental exposures, improve disease self-management skills, and ensure consistently optimal healthcare practices will likely decrease variations in outcomes including SES-related disparities. Low SES is an important marker of adverse exposures and outcomes which are present throughout the CF population.

aDivision of Pulmonary, Allergy/Immunology, Cystic Fibrosis and Sleep, Department of Pediatrics, Emory University School of Medicine

bDepartment of Environmental Health, Rollins School of Public Health

cSection of Pulmonology, Children's Healthcare of Atlanta, Atlanta, Georgia, USA

Correspondence to Michael S. Schechter, MD, MPH, Division of Pulmonary, Allergy/Immunology, Cystic Fibrosis and Sleep, Department of Pediatrics, Emory University School of Medicine, 1547 Clifton Road, Atlanta, GA 30322, USA Tel: +1 404 727 0710; fax: +1 404 727 4828; e-mail:

© 2011 Lippincott Williams & Wilkins, Inc.