Factors influencing outcomes in cystic fibrosis: a center-based analysis.
BACKGROUND
Guidelines for managing cystic fibrosis (CF) patients have been widely circulated, but little is known about the variations in practice between sites and their association with outcomes.


OBJECTIVE
To determine whether differences in lung health existed between groups of patients attending different CF care sites and to determine whether these differences are associated with differences in monitoring and intervention.


METHODS
The analysis was conducted using data from the Epidemiologic Study of Cystic Fibrosis from 1995 through 1996.


METHODS
This was an observational database collecting prospective information from a large number of CF patients undergoing routine care in North America.


METHODS
Participating sites that had at least 50 CF patients who had each made at least one visit to a center during the 2-year study period were ranked on the basis of median values for FEV(1) within each of three age groups (6 to 12 years, 13 to 17 years, and >or= 18 years).


METHODS
There were no prespecified interventions in this observational study.


METHODS
The frequency of patient monitoring and the use of therapeutic interventions were compared between sites in the upper and lower quartiles after stratification within the site for disease severity.


RESULTS
Within-site rankings tended to be consistent across the three age groups. Patients who were treated at higher ranking sites had more frequent monitoring of their clinical status, measurements of lung function, and cultures for respiratory pathogens. These patients also received more interventions, particularly IV antibiotics for pulmonary exacerbations.


CONCLUSIONS
We found substantial differences in lung health across different CF care sites. We found that frequent monitoring and increased use of appropriate medications in the management of CF are associated with improved outcomes.
Johnson
Charles
C
Genentech, 1 DNA Way, Mail Stop 59, South San Francisco, CA 94080, USA. johnson.charles@gene.com
Butler
Steven M
SM
Konstan
Michael W
MW
Morgan
Wayne
W
Wohl
Mary Ellen B
ME
D000293:Adolescent; D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D002648:Child; D003550:Cystic Fibrosis / Q000188:drug therapy*; D006801:Humans; D011446:Prospective Studies; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents