Related Experiment Video
Updated: Dec 31, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Performance of ICD-10-CM diagnosis codes for identifying children with Sickle Cell Anemia
Sarah L Reeves1,2, Brian Madden1, Meng Wu3
1Susan B Meister Child Health Evaluation and Research Center, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan.
Insights
A new case definition accurately identifies children with sickle cell anemia (SCA) using ICD-10-CM claims data. This method aids in monitoring healthcare trends for SCA patients.
Area of Science:
- Pediatric Hematology
- Health Services Research
- Medical Informatics
Background:
- Accurate identification of pediatric sickle cell anemia (SCA) is crucial for effective healthcare management and research.
- Transitioning to the International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) necessitates validated methods for case ascertainment.
- Existing methods for identifying SCA in administrative data may require refinement for optimal performance.
Purpose of the Study:
- To develop, test, and validate ICD-10-CM claims-based case definitions for identifying children with sickle cell anemia (SCA).
- To assess the performance of various case definitions using sensitivity, specificity, and area under the ROC curve.
- To establish a reliable methodology for monitoring SCA patient populations in administrative datasets.
Main Methods:
- Secondary data analysis of Medicaid administrative claims (2016) and newborn screening records from Michigan and New York.
- Application of 23 distinct case definitions using specific and nonspecific ICD-10-CM diagnosis codes (D57x).
- Performance evaluation of case definitions against newborn screening results as the gold standard.
Main Results:
- A case definition requiring at least one outpatient visit with an SCA-related or D571 code demonstrated the highest performance.
- This optimal definition achieved a 95% sensitivity and 92% specificity in Michigan Medicaid data.
- The same definition showed high performance in New York Medicaid data with 94% sensitivity and 86% specificity.
Conclusions:
- A straightforward case definition utilizing ICD-10-CM codes can accurately identify children with SCA in administrative claims.
- This validated methodology supports the monitoring of trends and healthcare service utilization in pediatric SCA populations.
- The findings facilitate improved public health surveillance and research following the adoption of ICD-10-CM coding.
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.
Related Concept Videos
Classification of Illness
An illness is a response to a disease in which the person's level of functioning is changed compared with a previous level. The general classification of illness includes acute and chronic.
Acute illness is severe...
Documentation of Nursing Diagnosis
In some settings, data-driven computerized decision support systems are in place, allowing for more accurate nursing diagnoses. The database within one of these systems includes diagnostic labels defining characteristics, activities, and indicators for nursing. A nurse enters...
Nursing Interventions II: Selecting and Classifying the Nursing Interventions

