Performance of ICD-10-CM diagnosis codes for identifying children with Sickle Cell Anemia

Health Serv Res. 2020 Apr;55(2):310-317. doi: 10.1111/1475-6773.13257. Epub 2020 Jan 9.


Objective: To develop, test, and validate the performance of ICD-10-CM claims-based case definitions for identifying children with sickle cell anemia (SCA).

Data sources: Medicaid administrative claims (2016) for children <18 years with potential SCA (any D57x diagnosis code) and newborn screening records from Michigan and New York State.

Study design: This study is a secondary data analysis.

Data collection/extraction methods: Using specific SCA-related (D5700, D5701, and D5702) and nonspecific (D571) diagnosis codes, 23 SCA case definitions were applied to Michigan Medicaid claims (2016) to identify children with SCA. Measures of performance (sensitivity, specificity, area under the ROC curve) were calculated using newborn screening results as the gold standard. A parallel analysis was conducted using New York State Medicaid claims and newborn screening data.

Principal findings: In Michigan Medicaid, 1597 children had ≥1 D57x claim; 280 (18 percent) were diagnosed with SCA. Measures of performance varied, with sensitivities from 0.02 to 0.97 and specificities from 0.88 to 1.0. The case definition of ≥1 outpatient visit with a SCA-related or D571 code had the highest area under the ROC curve, with a sensitivity of 95 percent and specificity of 92 percent. The same definition also had the highest performance in New York Medicaid (n = 2454), with a sensitivity of 94 percent and specificity of 86 percent.

Conclusions: Children with SCA can be accurately identified in administrative claims using this straightforward case definition. This methodology can be used to monitor trends and use of health services after transition to ICD-10-CM.

Keywords: ICD-10-CM; administrative claims; sickle cell anemia.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / classification*
  • Anemia, Sickle Cell / diagnosis*
  • Anemia, Sickle Cell / epidemiology
  • Child
  • Child, Preschool
  • Female
  • Guidelines as Topic*
  • Humans
  • Infant
  • Infant, Newborn
  • International Classification of Diseases / standards*
  • Male
  • Medicaid / standards*
  • Michigan / epidemiology
  • New York / epidemiology
  • Reproducibility of Results
  • Socioeconomic Factors
  • United States