Trends in Cystic Fibrosis-Related Diabetes Epidemiology Between 2003 and 2018 From the U.S. Cystic Fibrosis Foundation Patient Registry

Abstract

Objective: A number of disease-modifying therapies have been introduced for people with cystic fibrosis (CF) over the past two decades. The cumulative effects of this changing landscape on cystic fibrosis-related diabetes (CFRD) are unclear. We examined trends in CFRD epidemiology over time using data from the U.S. Cystic Fibrosis Foundation Patient Registry (CFFPR).

Research design and methods: CFFPR data from 2003 to 2018 were queried to determine annual screening, incidence, and prevalence rates of CFRD. Individuals with incident CFRD were compared with individuals without CFRD. Survival analyses were performed to estimate the cumulative hazard of CFRD given predictors of interest over the 15 years of study. Data were also grouped into three time periods (2003-2008, 2009-2013, and 2014-2018) to investigate whether the hazard of developing CFRD varied over time.

Results: CFRD screening rates increased from 2003 to 2018, particularly in 10- to 18-year-olds. Although screening rates increased in adults, overall rates remained low. In 10- to 18-year-olds, the incidence of CFRD was stable over time, while incident cases in adults steadily decreased, approaching incident rates in adolescents. Despite this, the prevalence of CFRD has gradually increased in adults, likely reflecting increased longevity. Age, female sex, Black race, severe mutation class, liver disease, poorer lung function, pancreatic insufficiency, enteric feeds, and low and high BMI were all risk factors associated with CFRD.

Conclusions: Findings support the need for the development of tailored CFRD screening algorithms and increased subspecialists to care for a growing population of adults with CF and CF-associated comorbidities.