Feasibility of creating a National ALS Registry using administrative data in the United States

Abstract

Uncertainty about the incidence and prevalence of amyotrophic lateral sclerosis (ALS), as well as the role of the environment in the etiology of ALS, supports the need for a surveillance system/registry for this disease. Our aim was to evaluate the feasibility of using existing administrative data to identify cases of ALS. The Agency for Toxic Substances and Disease Registry (ATSDR) funded four pilot projects at tertiary care facilities for ALS, HMOs, and state based organizations. Data from Medicare, Medicaid, the Veterans Health Administration, and Veterans Benefits Administration were matched to data available from site-specific administrative and clinical databases for a five-year time-period (1 January 2001–31 December 2005). Review of information in the medical records by a neurologist was considered the gold standard for determining an ALS case. We developed an algorithm using variables from the administrative data that identified true cases of ALS (verified by a neurologist). Individuals could be categorized into ALS, possible ALS, and not ALS. The best algorithm had sensitivity of 87% and specificity of 85%. We concluded that administrative data can be used to develop a surveillance system/ registry for ALS. These methods can be explored for creating surveillance systems for other neurodegenerative diseases.

Amyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics

“…A death certificate specifying motor neuron disease was a core component of the algorithm developed by Kaye et al, but that level of specificity was not available from the death notifications The numbers below the confidence intervals reflect the total number of patients by category with ALS in the database/total number of patients in the same category in the database. from CMS, which was the source of mortality information in this study (28). Given this difference, our ALS prevalence may have been overestimated as ALS classification was based on a combination of an ALS diagnosis and notification of death.…”

Prevalence of Amyotrophic Lateral Sclerosis — United States, 2012–2013

“…The algorithm used was developed initially during the pilot projects and categorized cases as "definite ALS," "possible ALS," or "not ALS," with a sensitivity of 87% and a specificity of 85% (24). On the basis of knowledge of ALS and findings from other studies (25)(26)(27), individual and combined variables were entered into the algorithm.…”

“…First, because ALS is not a notifiable disease, ensuring that all newly diagnosed and prevalent ALS cases in the United States are captured in the Registry is challenging and therefore the possibility of under-ascertainment exists. However, the large administrative database methodology that ATSDR is using was vetted through a pilot effort and is expected to identify most of the ALS cases in the United States, given its high sensitivity and specificity (24). In addition, ATSDR is partnering with national stakeholders to promote the Registry to persons with the disease so they can self-enroll though the Registry's web portal.…”

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

“…32 Higher positive predictive values for identifying cases of amyotrophic lateral sclerosis for the National Amyotrophic Lateral Sclerosis Registry were obtained when criteria for case identification included multiple occurrences of the diagnosis code, searching for diagnosis codes in multiple years, and linking to additional sources for case confirmation. 33,34 37 Additional studies are under development.…”