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Characterization of individuals with selected muscular dystrophies from the expanded pilot of the Muscular Dystrophy Surveillance, Tracking and Research Network ( MD STARnet ) in the United States
Journal article   Peer reviewed

Characterization of individuals with selected muscular dystrophies from the expanded pilot of the Muscular Dystrophy Surveillance, Tracking and Research Network ( MD STARnet ) in the United States

Bailey Wallace, K. Tiffany Smith, Shiny Thomas, Kristin M. Conway, Christina Westfield, Jennifer G. Andrews, Richard O. Weinert, Thuy Quynh N. Do, Natalie Street and Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)
Birth defects research, Vol.113(7), pp.560-569
04/15/2021
DOI: 10.1002/bdr2.1764
PMCID: PMC8114775
PMID: 32710484

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Abstract

Abstract Introduction Data on muscular dystrophies (MDs), a heterogeneous group of heritable diseases hallmarked by progressive muscle deterioration, are scarce. Objective We describe cross‐sectional sociodemographic and clinical characteristics of individuals with congenital, distal, Emery‐Dreifuss, facioscapulohumeral, limb‐girdle, myotonic, or oculopharyngeal MD. Methods The study was conducted in four sites (Arizona, Colorado, Iowa, and 12 western New York counties) as a pilot expansion of the Muscular Dystrophy Surveillance, Tracking and Research Network, funded by the Centers for Disease Control and Prevention. MDs were detected in healthcare facilities and administrative data sources using International Classification of Disease codes. Our sample contains 1,723 individuals with a MD diagnosis and a healthcare encounter between January 1, 2007 and December 31, 2011. Results and Conclusions Individuals were mostly non‐Hispanic and white. Median ages ranged from 9.2 to 66.0 years. Most (98%) had health insurance. The proportion of individuals who were disabled or unable to work increased with age (range: 8.6–46.4%). People with limb‐girdle MD aged ≥18 years were more likely to be nonambulatory (range: 24.5–44.7%). The percentages of individuals with documented clinical interventions during the surveillance period were low. The most common cause of death was respiratory causes (46.3–57.1%); an ICD‐10 code for MD (G71.1 or G71.0) was reported for nearly one‐half. Our findings show wide variability in sociodemographic and clinical characteristics across MDs.

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