Improving an Administrative Case Definition for Longitudinal Surveillance of Sickle Cell Disease

Several states are building infrastructure and data collection methods for longitudinal, population-based surveillance systems for selected hemoglobinopathies. The objective of our study was to improve an administrative case definition for sickle cell disease (SCD) to aid in longitudinal surveillance. We collected data from 3 administrative data sets (2004-2008) on 1998 patients aged 0-21 in Georgia who had ≥1 encounter in which an SCD International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code was recorded, and we compared these data with data from a laboratory and medical record review. We assessed performance (sensitivity, specificity, positive predictive value [PPV], and negative predictive value [NPV]) of case definitions that differed by number and type of SCD-coded encounters; addition of SCD-associated treatments, procedures, and complications; and length of surveillance (1 vs 5 years). We identified correct diagnoses for patients who were incorrectly coded as having SCD. The SCD case definition of ≥3 SCD-coded encounters in 5 years simplified and substantially improved the sensitivity (96.0% vs 85.8%) and NPV (68.2% vs 38.2%) of the original administrative case definition developed for 5-year, state-based surveillance (≥2 encounters in 5 years and ≥1 encounter for an SCD-related treatment, procedure, or complication), while maintaining a similar PPV (97.4% vs 97.4%) and specificity (76.5% vs 79.0%). This study supports an administrative case definition that specifies ≥3 ICD-9-CM–coded encounters to identify SCD with a high degree of accuracy in pediatric patients. This case definition can be used to help establish longitudinal SCD surveillance systems