title: 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.
authors.affiliation: Division of Research and Statistics, South Carolina Budget and Control Board, 1919 Blanding Street, Columbia, SC, 29201, USA, Julie.Royer@ors.sc.gov.
authors.forename: J A
authors.initials: JA
authors.lastname: Royer
authors.forename: J W
authors.initials: JW
authors.lastname: Hardin
authors.forename: S
authors.initials: S
authors.lastname: McDermott
authors.forename: L
authors.initials: L
authors.lastname: Ouyang
authors.forename: J R
authors.initials: JR
authors.lastname: Mann
authors.forename: O D
authors.initials: OD
authors.lastname: Ozturk
authors.forename: J
authors.initials: J
authors.lastname: Bolen
mesh_terms: D000293:Adolescent; D005260:Female; D005600:Fragile X Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D006077:Government Agencies; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D035583:Rare Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D012042:Registries; D013022:South Carolina / Q000453:epidemiology; D016135:Spinal Dysraphism / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D013219:State Government; D055815:Young Adult