Use of State Administrative Data Sources to Study Adolescents and Young Adults with Rare Conditions

Abstract

BACKGROUND: Effective care of young people with rare conditions requires ongoing coordinated medical treatment as well as educational and social support services. However, information on treatment is often lacking due to limited data. South Carolina has a repository of comprehensive health and human service data with which individuals may be tracked across the data systems of multiple state agencies and organizations. OBJECTIVE: To develop a method for studying health care of young persons with rare conditions using this repository. METHODS: We identified individuals aged 15 to 24 years diagnosed during 2000-2010 with Fragile X syndrome (FXS), spina bifida (SB), or muscular dystrophy (MD) using a series of algorithms. ICD-9-CM codes were used to initially identify the cohort from medical billing data. Demographics, medical care, employment, education, and socioeconomic status data were then extracted from linked administrative sources. RESULTS: We identified 1,040 individuals with these rare conditions: 125 with FXS, 695 with SB, and 220 with MD. The vast majority of the cases (95 %) were identified in the Medicaid database. Half of the cohort was male, with a higher percentage in the FXS and MD groups. Sixty-two percent of the cohort was enrolled in the last year of high school. Over half of the cohort received support services from the state's disability and special-needs agency; 16 % received food assistance. Thirty-eight percent were employed at some point during the study period. Forty-nine individuals with SB and 56 with MD died during the study period. CONCLUSIONS: We used a linked statewide data system to study rare conditions. Strengths include the diversity of information, rigorous identification strategies, and access to longitudinal data. Despite limitations inherent to administrative data, we found that linked state data systems are valuable resources for investigating important public health questions on rare conditions.

Skin Ulcers and Mortality Among Adolescents and Young Adults With Spina Bifida in South Carolina During 2000-2010

“…[16][17][18][19] 13% had first skin ulcer during adolescence, and 8% had first skin ulcer during young adulthood. It is noted that among 55 patients having their first skin ulcer during young adulthood, 37 of them entered the study when they were adolescents but had their first skin ulcer during young adulthood (20)(21)(22)(23)(24), while 18 of them entered the study and had their first skin ulcer at young adulthood. Table 3 and Table 4 provide the frequency of key variables according to skin ulcer and vital status, respectively, among patients with spina bifida.…”

“…This study is part of a larger project investigating the transition from pediatric to adult services for adolescents and young adults with rare health conditions. 21 The authors used 3 health databases for case ascertainment-Medicaid, State Health Plan and Uniform-billing Hospital Discharge Data, with a unique ID as a linkage for each patient. Vital records death certificate data were extracted using each case's unique ID.…”

Identification of people with acquired hemophilia in a large electronic health record database

“…Clinicians also demonstrated unique patterns of ICD-9-CM code use, with some codes being used more commonly than others in certain specialties and practices. 26 …”

Development of a tool to describe overall health, social independence and activity limitation of adolescents and young adults with disability

“…We asked residents of South Carolina who had FXS, SB, or MD and were 15–24 years old to sign an informed consent form giving us permission to link their survey answers to data compiled as part of a larger study investigating the transition from adolescent to adult services for people with rare health conditions in South Carolina; details of which can be found in a methods paper (Royer et al, 2014). The data for this larger project including Medicaid and State Health Plan medical claims data is housed at the South Carolina Revenue and Fiscal Affairs Office, Health and Demographics (H&D).…”