Context: Guidelines for managing cystic fibrosis (CF) patients have been widely circulated.

Context: Guidelines for managing cystic fibrosis (CF) patients have been widely circulated, further 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 disposes 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 guidanceed using data from the Epidemiologic meditation of Cystic Fibrosis from 1995 within 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 common visit to a center during the 2-year consideration period were ranked on the basis of median values for FE[Vsub1] within each of three age assemblages (6 to 12 years, 13 to 17 years, and [greater than or equal to] 18 years).

Interventions: There were no prespecified interventions in this observational study

Main result 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 be attentive toed to be consistent across the three age clumps Patients who were treated at higher ranking sites had more every-day monitoring of their clinical status, measurements of lung function, and refinements for respiratory pathogens. These patients also received more interventions, particularly IV antibiotics for pulmonary exacerbations.

Conclusion: We establish substantial differences in lung health across different CF care sites. We establish that frequent monitoring and increased use of appropriate medications in the management of CF are associated with improved outcomes

elucidation words: cystic fibrosis; epidemiology; evaluation; monitoring; spirometry; respiratory tract culture; treatment

Abbreviations: CF = cystic fibrosis; ESCF = Epidemiologic subject of attention of Cystic Fibrosis

**********

The management of cystic fibrosis (CF) has improved dramatically in the 60 years since the early description of the disease by means of Anderson, (1) when the life expectancy of patients was < 1 year. commonly data from a number of registries estimate the median age of survival to be [greater than or equal to] 31 years. (23) This remarkable advance has been achieved [i]or[/i] part of to the other the establishment of specialized care facilities, improved pancreatic enzyme preparations, and the progressive growth of effective antibiotics with which to treat pulmonary exacerbations. (45) Guidelines disentangleed by the Cystic Fibrosis Foundation (6) have contributed to the standardization of CF management. These guidelines praise that patients have at least four clinical visits by year, that the measurement of lung function be performed each 6 months, and that agricultures of respiratory tract secretions be escorted annually.

In assessing issues for CF patients, the ideal measure is mortality. However, relatively hardly any patients die in the short expression Therefore, measurements of pulmonary function have become surrogate issues in most clinical trials, (7-10) as there is a robust association between lung function and mortality rates. (11-13)

The Epidemiologic meditation of Cystic Fibrosis (ESCF) is a multicenter, longitudinal, observational inquiry that prospectively collects detailed clinical, therapeutic, microbiological, and lung function data from a large number of CF treatment sites in the United States and Canada. (14-16) Initiated in December 1993 the meditation enrolled 18,411 patients by December 31 1995

The goal of this subject of attention was to identify whether differences in issues specifically lung health as ascertained using the surrogate marker of FE[Vsub1] existed between care sites. We sought to determine whether particular practice patterns at care sites were associated with better outcomes

In light of data linking improved survival to the usual use of IV antibiotics, (17) an additional goal of the investigation was to determine whether intensive treatment with IV antibiotics was associated with better outcomes

MATERIALS AND METHODS

The analysis examined ESCF data consider probableed during the 2-year period from 1995 by the and of 1996. Initiated in December 1993 the reflection had enrolled 18,411 patients by dint of 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 dint of the US Cystic Fibrosis Foundation. All Canadian and US sites were invited to participate if they had > 10 patients. Patients were listed during routine care visits. Institutional review boards for each site reviewed the inquiry Many did not require informed compliance as patient anonymity was maintained. Data were bring togethered prospectively at each visit or hospitalization using study-specific case report forms. each effort was made to make secure adherence to data collection at providing patient-specific data to the sites for source verification. In addition, data comparing the site to regional and national data were provided to investigators for external validation. Data-checking programs were hie by the statistical coordinating center (ClinTrials Research, Inc; Lexington, KY) and queries were sent to the sites in order to decipher discrepancies and outliers. The data included findings for each spirometry test (designated as having been obtained when "sick" or "stable"), the originates of every culture, and each antipseudomonal antibiotic intervention and/or hospitalization. Routine therapies were recorded at each visit, including the use of airway clearance techniques, oral bronchodilators, inhaled bronchodilators, oral corticosteroids, inhaled corticosteroids, inhaled cromolyn or nedocromil, inhaled dornase alfa, nonsteroidal antiinflammatory agents, insulin or oral hypoglycemic agents, nutritional supplys pancreatic enzymes, and oral antibiotics. The start and stop dates of therapy with oral quinolones, inhaled antibiotics, and IV antibiotics also were recorded. All of these variables were examined in the analysis. A ranking was used to evaluate the use of antibiotics in the same manner that if inhaled and IV antibiotics were used simultaneously, the patient cut down into the IV antibiotic group

...