Chest
Volume 123, Issue 1, January 2003, Pages 20-27
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Clinical Investigations
Cystic Fibrosis
Factors Influencing Outcomes in Cystic Fibrosis: A Center-Based Analysis

https://doi.org/10.1378/chest.123.1.20Get rights and content

Context

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.

Design

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

Setting

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

Participants

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 FEV1 within each of three age groups (6 to 12 years, 13 to 17 years, and ≥ 18 years).

Interventions

There were no prespecified interventions in this observational study.

Main outcome measures

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.

Conclusion

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.

Section snippets

Materials and Methods

The analysis examined ESCF data collected during the 2-year period from 1995 through 1996. Initiated in December 1993, the study had enrolled 18,411 patients by December 1995,14 which was estimated to include > 80% of the population of CF patients in the United States and approximately 10% of that in Canada. The 194 sites included > 90% of those accredited by the US Cystic Fibrosis Foundation. All Canadian and US sites were invited to participate if they had > 10 patients. Patients were

Results

Of the 194 participating sites in the ESCF, 132 with 8,125 patients met the eligibility criteria for this analysis. For the 6 to 12-year-old age group, 112 sites with 3,361 patients (mean, 30 patients per site; SD, 19 patients per site) were eligible. For the adolescent age group (13 to 17 years of age), 84 sites with 1,734 patients (mean, 21 patients per site; SD, 11 patients per site) were eligible. For the adult group (≥ 18 years of age), 103 sites with 3,030 patients (mean, 29 patients per

Discussion

This analysis takes advantage of the large number of sites and patients reporting to the ESCF. It presents an opportunity to study outcomes and practices in a comprehensive way. The approach was to compare CF care sites based on an important index of lung health (ie, the percentage of predicted FEV1 values). Large differences in lung health across sites were demonstrated. The sites reporting the highest values for median FEV1 monitored patients, obtained measurements of lung function, and

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  • Cited by (0)

    Drs. Johnson and Butler are employees of Genentech. Drs. Konstan, Morgan, and Wohl are consultants for the Epidemiologic Study of Cystic Fibrosis sponsored by Genentech. All sources of support for the Epidemiologic Study of Cystic Fibrosis in the form of grants, case report forms, and data analysis were provided by Genentech.

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