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Standardization of Coding Definitions for Sickle Cell Disease Complications: A Systematic Literature Review
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  • Paulette Negron Ericksen,
  • Firas Dabbous,
  • Rajrupa Ghosh,
  • Surbhi Shah,
  • Xunming Sun,
  • Emily Riehm Meier,
  • Carmine Colavecchia
Paulette Negron Ericksen
Pfizer Inc

Corresponding Author:[email protected]

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Firas Dabbous
Evidera Inc
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Rajrupa Ghosh
Evidera Inc
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Surbhi Shah
Evidera Inc
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Xunming Sun
Pfizer Inc
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Emily Riehm Meier
Pfizer Inc
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Carmine Colavecchia
Pfizer Inc
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Abstract

Purpose: Sickle cell disease (SCD) affects all organ systems and is characterized by numerous acute and chronic complications and comorbidities. Standardized codes are needed for complications/comorbidities used in real-world evidence (RWE) studies that rely on administrative and medical coding. This systematic literature review was conducted to produce a comprehensive list of complications/comorbidities associated with SCD, along with their diagnosis codes used in RWE studies. Methods: A search in MEDLINE and Embase identified studies published from 2016-2023. Studies were included if they were conducted in US SCD populations and reported complications/comorbidities and respective International Classification of Diseases, Clinical Modification (ICD-CM) codes. All identified complications/comorbidities and codes were reviewed by a certified medical coding expert and hematologist. Results: Of 1,851 identified studies, 40 were included. The most reported complications/comorbidities were stroke, acute chest syndrome, pulmonary embolism, venous thromboembolism, vaso-occlusive crisis, and priapism. Most of the studies used ICD-9-CM codes (n=21), while some studies used ICD-10-CM codes (n=3) or both (n=16), depending on the study period. Most codes reported in literature were heterogeneous across complications/comorbidities. The medical coding expert recommended modifications for several conditions. Conclusion: While many studies we identified did not report their codes and were excluded from this review, the studies with codes exhibited diverse coding definitions. By providing a standardized set of diagnosis codes that were reported by studies and reviewed by a coding expert and hematologist, our review can serve as a foundation for accurately identifying complications/comorbidities in future research, and may reduce heterogeneity, enhance transparency, and improve reproducibility.
Submitted to Pharmacoepidemiology and Drug Safety
01 Feb 20241st Revision Received
01 Feb 2024Submission Checks Completed
01 Feb 2024Assigned to Editor
01 Feb 2024Review(s) Completed, Editorial Evaluation Pending
02 Feb 2024Editorial Decision: Accept