The association of pediatric cystic fibrosis-related diabetes screening on clinical outcomes by center: A CF patient registry study

Abstract

Background: Cystic fibrosis related diabetes (CFRD) has been associated with pulmonary function decline, nutritional status decline and increased mortality. In 2010, the CFRD Clinical Care Guideline were updated, recommending all patients with CF begin CFRD screening at 10 years old. This study uses CF Foundation Patient Registry to examine the impact of screening practices at centers across the United States from 2008 to 2015. We examined the association of screening practices and CFRD diagnosis at individual centers and trends in ppFEV1 and BMI percentile prior to and after diagnosis.

Methods: The cohort was defined as patients with CF and without CFRD who turned 10 years old from 2009 to 2015. Centers were classified based on their CFRD screening rates. Kaplan-Meier curves summarized the distribution of age at CFRD diagnosis. Among patients diagnosed with CFRD, we examined differences in ppFEV1 and BMI percentile two years prior to diagnosis and two years post-diagnosis by clinic screening rate.

Results: Of 3553 patients, 445 (13%) were diagnosed with CFRD. The average age of diagnosis was 13 years old. The screening rate of the patients' clinic was significantly associated with time to diagnosis (pvalue=0.0001). Among patients diagnosed with CFRD, clinics with lower screening rates of CFRD had steeper rates of pulmonary decline two years prior to diagnosis.

Conclusion: Centers that screen for CFRD more tend to diagnose a larger percent of patients with CFRD and at a younger age. Additionally, patients at centers with lower screening rates have faster rates of pulmonary decline prior to CFRD diagnosis.

Keywords: BMI; CFRD; Care guidelines; Cystic fibrosis; Diabetes; Registry; Screening; ppFEV1.