Administrative data identify sickle cell disease: A critical review of approaches in U.S. health services research

Abstract

To identify people living with sickle cell disease (SCD) and study their healthcare utilization, researchers can either use clinical records linked to administrative data or use billing diagnosis codes in stand-alone administrative databases. Correct identification of individuals clinically managed for SCD using diagnosis codes in claims databases is limited by the accuracy of billing codes in outpatient encounters. In this critical review, we assess the strengths and limitations of claims-based SCD case-finding algorithms in stand-alone administrative databases that contain both inpatient and outpatient records. Validation studies conducted using clinical records and newborn screening for confirmation of SCD case status have found that algorithms that require three or more nonpharmacy claims or one inpatient claim plus two or more outpatient claims with SCD codes show acceptable accuracy (positive predictive value and sensitivity) in children and adolescents. Future studies might seek to assess the accuracy of case-finding algorithms over the lifespan.

Evaluation of Stillbirth Among Pregnant People With Sickle Cell Trait

“…Billing codes are imperfect in their ability to capture clinical conditions; for sickle cell status, there is potential to underestimate the number of patients or overestimate them because of false-positive diagnoses. 31 However, misclassification owing to the latter is unlikely in this case because we identified patients with sickle cell over a period of approximately 7 years. Another related limitation is that we did not have data on hemoglobin variants, as available in other studies.…”

“…Evaluation of Stillbirth Among Pregnant People With Sickle Cell Trait false-positive diagnoses. 31 However, misclassification owing to the latter is unlikely in this case because we identified patients with sickle cell over a period of approximately 7 years. Another related limitation is that we did not have data on hemoglobin variants, as available in other studies.…”

“… 26 , 27 , 28 , 29 Our study explores these interactions by assessing the association of sickle cell status and race and ethnicity on the risk of pregnancy-related complications by leveraging electronic health records (EHRs) within a large hospital system. Electronic health records contain rich information on patient medical history, including records detailing sickle cell encounters, 30 , 31 , 32 and can be used to study associations between SCT and pregnancy-related complications such as stillbirth.…”

Validation of Discharge Diagnosis of Sickle Cell Disease Vaso-Occlusive Episodes in the French Hospital Electronic Database

“… 6 In 2020, Grosse et al reviewed all SCD identification algorithms used in USA insurance databases, with heterogeneous results. 7 However, all these algorithms used ICD-9 diagnosis classification, preventing from any comparison with our findings.…”

Geographic Barriers Drive Disparities in Specialty Center Access for Older Adults with Huntington’s Disease

“…The validity of HD codes in administrative health care databases is not yet known [61,62], but genetic diseases, especially fatal ones, in general have good validity or acceptable accuracy [63][64][65][66]. Our cross-sectional design cannot account for individuals moving closer or away from COEs nor provide evidence that proximity to a COE associates with health care use, quality or outcomes; these relationships will be the focus of future longitudinal analyses.…”