6500 Screening Rates in Emerging Adults With Cystic Fibrosis for Cystic Fibrosis-Related Bone Disease: A Retrospective Cohort Study

Abstract Disclosure: C. Cobb: None. A. Stecenko: None. W.R. Hunt: None. R. Linnemann: None. T. Daley: None. V. Tangpricha: None. INTRODUCTION Cystic fibrosis bone disease (CFBD) is a common endocrinopathy in patients with cystic fibrosis (CF). Poor bone health is associated with increased risk of fractures, potentially leading to decreased pulmonary function and worse health outcomes. The transition period from adolescent to adult care is a particularly vulnerable period in terms of bone health. It is recommended that patients with CF have bone density screening starting at age 18 and every 1-2 years. The purpose of this study was to examine the frequency of bone density screening in adolescents and young adults with CF followed at a single accredited CF center. Methods This was a retrospective chart review of patients with CF between ages 18 and 30 who had transitioned to adult care between 2013 and 2023 at our CF clinic. Frequency of bone density screening was assessed by completion of dual-energy x-ray absorptiometry (DXA) and included the last pediatric Z-scores, if available, and adult Z-scores up to 10 years after transition. Height adjusted Z-scores were used when appropriate for pediatric values. Transplant patients and patients who did not attend at least one year of adult clinic were excluded. Results Ninety-five patients were identified. Seven patients did not have documentation of a DXA scan, while 88 had at least one DXA scan as emerging adults. Ten out of 88 adults received a repeat DXA within 2 years of their prior scan. 42 out of 88 (48%) of patients were at risk for CFBD with at least one Z-score ≤ -1. Of these at risk patients only 5 got a repeat DXA within two years. None of the patients undertook routine DXA exams consistently every 2 years regardless of risk. CONCLUSION Our study suggests that emerging adults with CF and decreased bone mineral density are being under screened for progression of CFBD. Evaluating the barriers to bone density screening will be important to optimize bone health and improve outcomes. In the era of CFTR modulator therapies, more attention to bone health is required especially with the increased life expectancy of people living with CF. Strategies focused on increased bone density screening, promotion of healthy lifestyle, optimizing nutritional status, and addressing hypogonadism appear to be critical in this transition period for young adults with CF. Presentation: 6/3/2024

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Journal
Journal of the Endocrine Society
Published
2024-10-01
DOI
https://doi.org/10.1210/jendso/bvae163.495
Primary Topic
Cystic Fibrosis Research Advances
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article
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article

6500 Screening Rates in Emerging Adults With Cystic Fibrosis for Cystic Fibrosis-Related Bone Disease: A Retrospective Cohort Study

William R. Hunt, Tanicia Daley, Rachel W. Linnemann, Vin Tangpricha et al.
Journal of the Endocrine Society
Cystic Fibrosis Research Advances
article

6500 Screening Rates in Emerging Adults With Cystic Fibrosis for Cystic Fibrosis-Related Bone Disease: A Retrospective Cohort Study

William R. Hunt, Tanicia Daley, Rachel W. Linnemann, Vin Tangpricha, Arlene A. Stecenko, Crystal Cobb
article en

Abstract

Abstract Disclosure: C. Cobb: None. A. Stecenko: None. W.R. Hunt: None. R. Linnemann: None. T. Daley: None. V. Tangpricha: None. INTRODUCTION Cystic fibrosis bone disease (CFBD) is a common endocrinopathy in patients with cystic fibrosis (CF). Poor bone health is associated with increased risk of fractures, potentially leading to decreased pulmonary function and worse health outcomes. The transition period from adolescent to adult care is a particularly vulnerable period in terms of bone health. It is recommended that patients with CF have bone density screening starting at age 18 and every 1-2 years. The purpose of this study was to examine the frequency of bone density screening in adolescents and young adults with CF followed at a single accredited CF center. Methods This was a retrospective chart review of patients with CF between ages 18 and 30 who had transitioned to adult care between 2013 and 2023 at our CF clinic. Frequency of bone density screening was assessed by completion of dual-energy x-ray absorptiometry (DXA) and included the last pediatric Z-scores, if available, and adult Z-scores up to 10 years after transition. Height adjusted Z-scores were used when appropriate for pediatric values. Transplant patients and patients who did not attend at least one year of adult clinic were excluded. Results Ninety-five patients were identified. Seven patients did not have documentation of a DXA scan, while 88 had at least one DXA scan as emerging adults. Ten out of 88 adults received a repeat DXA within 2 years of their prior scan. 42 out of 88 (48%) of patients were at risk for CFBD with at least one Z-score ≤ -1. Of these at risk patients only 5 got a repeat DXA within two years. None of the patients undertook routine DXA exams consistently every 2 years regardless of risk. CONCLUSION Our study suggests that emerging adults with CF and decreased bone mineral density are being under screened for progression of CFBD. Evaluating the barriers to bone density screening will be important to optimize bone health and improve outcomes. In the era of CFTR modulator therapies, more attention to bone health is required especially with the increased life expectancy of people living with CF. Strategies focused on increased bone density screening, promotion of healthy lifestyle, optimizing nutritional status, and addressing hypogonadism appear to be critical in this transition period for young adults with CF. Presentation: 6/3/2024

Journal of the Endocrine SocietyVol. 8(Supplement_1)
Emory University (US)
Openalex Percentile: Top 68%
Cystic Fibrosis Research Advances
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