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Bone mineral density screening by DXA for people with cystic fibrosis: A registry analysis of patient and program factors influencing rates of screening

Published:January 24, 2022DOI:https://doi.org/10.1016/j.jcf.2022.01.011

      Highlights

      • As treatments for cystic fibrosis (CF) improve life expectancy, complications that increase in prevalence with age such as CF-related bone disease (CFBD) require more attention and investigation.
      • Demineralization of the bones can have detrimental consequences on the lives of people with CF, including rib and spinal fractures, chronic pain, and potentially barriers to transplantation.
      • Despite guidelines on bone mineral density screening published by organizations such as the CF Foundation in the United States, systematic screening practices across care centers has not been universally adapted.
      • This evaluation of bone density screening practices reported in the United States CF Foundation patient registry showed that in individuals with CF with less severe disease, such as higher lung function, younger age, and pancreatic sufficiency, screened was less likely than those with more traditional risks for bone disease.
      • This study highlights the need for CF care centers to create processes to ensure adherence to bone density screening recommendations.

      Abstract

      Background: Current guidelines recommend screening for Cystic Fibrosis (CF) related bone disease (CFBD) using dual-energy x-ray absorptiometry (DXA) for all people ≥ 18 years of age and select people ≥ 8 years of age. However, adherence to these guidelines is variable. This study aims to evaluate screening practices among adult programs in the US and identify patient and program-based characteristics which may influence screening.
      Methods: The CF Foundation Patient Registry (CFFPR) was used to identify all people over the age of 18 who were seen at adult CF programs and received screening for CFBD using DXA at least once between 2014 and 2018. Associations with patient and program level characteristics were assessed using the Chi Square test. Patient level variables were also examined using standardized difference to assess for meaningful clinical differences in rates of screening.
      Results: From 2014 to 2018, a total of 15,134 people over the age of 18 were identified in the CFFPR. Of these people, 9,023 (60%) received a DXA during the time period. The median rate of screening by program was 66% and programs in the highest quartile of screening obtained DXAs on >76% of their population. At the program level, larger size and increased adherence to other guideline practices such as OGTT screening and 4 visits, 4 cultures in a year correlated with higher rates of screening for CFBD. At the patient-level, people with lower lung function (FEV1 <90%) and those with CF related diabetes were more likely to be screened. People without health insurance were less likely to receive recommended screening.
      Conclusion: Screening practices for CFBD vary widely across adult programs in the US despite recommendations to screen all people over the age of 18. Factors identified in this analysis may be used to identify those people at highest risk of missing appropriate screening. CFBD has significant implications for our patients and therefore routine screening should be emphasized as part of standard care moving forward.

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