Case Ascertainment of Sickle Cell Disease Using Surveillance or Single Administrative Database Case Definitions

Abstract

Objective: In the absence of access to surveillance system data, single-source administrative databases are often used to study health care utilization and health outcomes among people with sickle cell disease (SCD). We compared the case definitions from single-source administrative databases with a surveillance case definition to identify people with SCD. Materials and Methods: We used data from Sickle Cell Data Collection programs in California and Georgia (2016-2018). The surveillance case definition for SCD developed for the Sickle Cell Data Collection programs uses multiple databases, including newborn screening, discharge databases, state Medicaid programs, vital records, and clinic data. Case definitions for SCD in single-source administrative databases varied by database (Medicaid and discharge) and years of data (1, 2, and 3 years). We calculated the proportion of people meeting the surveillance case definition for SCD that was captured by each single administrative database case definition for SCD, by birth cohort, sex, and Medicaid enrollment. Results: In California, 7117 people met the surveillance case definition of SCD from 2016 through 2018; 48% of this group was captured by the Medicaid case definition and 41% by the discharge case definition. In Georgia, 10 448 people met the surveillance case definition of SCD from 2016 through 2018; 45% of this group was captured by the Medicaid case definition and 51% by the discharge case definition. These proportions differed by years of data, birth cohort, and length of Medicaid enrollment. Practice Implications: The surveillance case definition identified twice as many people with SCD as the single-source administrative database definitions during the same period, but trade-offs exist in using single administrative databases for decisions on policy and program expansion for SCD.

Characteristics of Emergency Department Visits Made by Individuals With Sickle Cell Disease in the U.S., 1999–2020

“…The main limitation of this study is the reliance on single-source and single-encounter methods for identifying ED visits associated with SCD, which have been shown to underestimate the true population size. 60 However, with only 11 states currently implementing population-level surveillance systems for SCD 11 and the absence of a national longitudinal registry, 61 data sources such as NHAMCS still provide useful national-level estimates, especially over long periods of time as in this study. Nonetheless, when stratifying certain characteristics by age, sample sizes became too small to produce reliable and precise estimates, and these results should be interpreted with caution.…”

Self-reported pain levels for emergency department visits associated with sickle cell disease in the United States

“…Namely, we relied on ICD codes in a limited set of diagnosis fields to identify ED visits associated with SCD, which is known to underestimate the SCD population. 23 Because NHAMCS data were nonidentifiable, we were also unable to track individuals at the patient-level to confirm the validity of the SCD diagnoses. There was also a modest amount of missing data that was addressed using standard imputation procedures.…”