Endocrine Care During Transition in Emerging Adults With Cystic Fibrosis: A Single Center Retrospective Study

Abstract

Objectives: Two common endocrine manifestations of cystic fibrosis (CF) are cystic fibrosis-related diabetes (CFRD) and cystic fibrosis-related bone disease (CFBD). Trends in CF endocrinopathies in emerging adults during health care transition have not been well studied. Our primary aim was to examine changes in glycemic control in participants with CFRD and bone density in all participants up to 10 years after transition to adult CF care. Secondary aims included analyzing rates of endocrine screening exams.

Methods: This was a retrospective chart review study that included participants ages 18-30 with CF who transitioned to a single center adult CF clinic between January 2013 and June 2023. The final cohort included 94 participants. Bone mineral density based on vitamin D status, CFRD diagnosis and highly effective modulator use was compared using two-sample t-tests, and linear mixed effects analysis was used to analyze hemoglobin A1c over time.

Results: Participants with CFRD on insulin demonstrated a mean increase of hemoglobin A1c by a geometric mean of 7.8% (62 mmol/mol) to 9.4% (79 mmol/mol) after 5 years of adult follow up (P = .003). Forty-seven percent of all emerging adults exhibited at least one bone mineral density Z-score < -1. Completion rates of oral glucose tolerance tests and dual-energy x-ray absorptiometry screenings were low.

Conclusions: Health outcome measures of CFRD and CFBD worsen during transition from pediatric to adult CF care at a single CF Care Center. Physicians should recognize this vulnerable period of transition and institute programs to increase screening of CFRD and CFBD during this period.

Keywords: adolescents; cystic fibrosis; cystic fibrosis-related bone disease; cystic fibrosis-related diabetes; health care transition.