Original Article

Summary of Selected Healthcare Encounters among a Selection of Patients with Myotonic Muscular Dystrophy

Authors: Kevin J. Bennett, PhD, Joshua R. Mann, MD, MPH, Lijing Ouyang, PhD


Objective: Research has not examined the use of health care by patients with myotonic muscular dystrophy (MMD), but it would provide insights into this population, which is prone to comorbidities and high service needs. This study is an analysis of this understudied subgroup, using a unique linked dataset to examine the characteristics and healthcare utilization patterns for people with MMD.

Methods: This analysis used 3 South Carolina datasets (2009–2014). The subjects included individuals with at least 1 encounter with an International Classification of Diseases, Ninth Revision, Clinical Modification code of 359.21. The variables included sex, race, visit type, payer, and diagnoses. The analyses examined characteristics and number of encounters.

Results: The subjects were predominately female, white, and 45 to 64 years old. A total of 44.6% of the study population had at least 1 inpatient visit, whereas 64.2% had at least 1 emergency department visit. A majority of the subjects had at least 1 office visit (55.0%), and most (85.3%) did not have a home health encounter.

Conclusions: Investigation of the reasons for these inpatient and emergency department encounters may be helpful in identifying ways to deliver high-quality care.
Posted in: Neurology6

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1. National Institute of Neurological Disorders and Stroke. Muscular dystrophy information page. https://www.ninds.nih.gov/disorders/all-disorders/muscular-dystrophy-information-page. Accessed February 2017.
2. Meola G, Cardani R. Myotonic dystrophy type 2 and modifier genes: an update on clinical and pathomolecular aspects. Neurol Sci 2017;38:535-546.
3. Smith CA, Gutmann L. Myotonic dystrophy type 1 management and therapeutics. Curr Treat Options Neurol 2016;18:52.
4. Mathieu J, Allard P, Potvin L, et al. A 10-year study of mortality in a cohort of patients with myotonic dystrophy. Neurology 1999;52:1658-1662.
5. Peric M, Peric S, Rapajic N, et al. Multidimensional aspects of pain in myotonic dystrophies. Acta Myol 2015;34:126-132.
6. Suokas KI, Haanpää M, Kautiainen H, et al. Pain in patients with myotonic dystrophy type 2: a postal survey in Finland. Muscle Nerve 2012;45:70-74.
7. Gadalla SM, Lund M, Pfeiffer RM, et al. Cancer risk among patients with myotonic muscular dystrophy. JAMA 2011;306:2480-2486.
8. Ouyang L, Grosse SD, Fox MH, et al. A national profile of health care and family impacts of children with muscular dystrophy and special care needs in the United States. J Child Neurol 2012;27:569-576.
9. Mann JR, Royer JA, Mcdermott S, et al. Hospitalizations and emergency room visits for adolescents and young adults with muscular dystrophy living in South Carolina. Muscle Nerve 2015;52:714-721.
10. Ozturk OD, McDermott S, Mann JR, et al. Disparities in health care utilization by race among teenagers and young adults with muscular dystrophy. Med Care 2014;52:S32-S39.
11. Kenneson A, Vatave A, Finkel R. Widening gap in age at muscular dystrophy-associated death between blacks and whites, 1986-2005. Neurology 2010;7:982-989.
12. Dogan C, De Antonio M, Hamroun D, et al. Gender as a modifying factor influencing myotonic dystrophy type 1 phenotype severity and mortality: a nationwide multiple databases cross-sectional observational study. PloS One 2016;11:e0148264.
13. Iezzoni LI, Frakt AB, Pizer SD. Uninsured persons with disability confront substantial barriers to health care services. Disability Health J 2011;4:238-244.
14. Mahmoudi E, Meade MA. Disparities in access to health care among adults with physical disabilities: analysis of a representative national sample for a ten-year period. Disability Health J 2015;8:182-190.
15. Scheer J, Kroll T, Neri MT, et al. Access barriers for persons with disabilities: the consumer’s perspective. J Disabil Policy Stud 2003;13:221-230.
16. Lishner DM, Richardson M, Levine P, et al. Access to primary health care among persons with disabilities in rural areas: a summary of the literature. J Rural Health 1996;12:45-53.
17. Gulley S, Rasch E, Chan L. Difference, disparity, and disability: a comparison of health, insurance coverage, and health service use on the basis of race/ethnicity among US adults with disabilities, 2006-2008. Med Care 2014;52(10 suppl 3):S9-S16.
18. Peterson-Besse J, Walsh E, Horner-Johnson W, et al. Barriers to health care among people with disabilities who are members of underserved racial/ethnic groups: a scoping review of the literature. Med Care 2014;52(10 suppl 3):S51-S63.
19. Horner-Johnson W, Konrad D. Usual source of care and unmet health care needs: interaction of disability with race and ethnicity. Med Care 2014;52(10 suppl 3):S40-S50.
20. Ouyang L, Grosse SD, Kenneson A. Health care utilization and expenditures for children and young adults with muscular dystrophy in a privately insured population. J Child Neurol 2008;23:883-888.
21. Royer JA, Hardin JW, McDermott S, et al. Use of state administrative data sources to study adolescents and young adults with rare conditions. J Gen Intern Med 2014;29:732-738.
22. Bennett KJ, Mann JR, Ouyang L. Utilizing combined claims and clinical datasets for research among potential cases of rare diseases. https://www.igi-global.com/article/utilizing-combined-claims-and-clinical-datasets-for-research-among-potential-cases-of-rare-diseases/198490. Published 2018. Accessed April 5, 2019.
23. Hahn C, Salajegheh MK. Myotonic disorders: a review article. Iran J Neurol 2016;15:46-53.
24. Clinical Classifications Software (CCS) for ICD-9-CM. https://www.hcup-us.ahrq.gov/toolssoftware/ccs/ccs.jsp. Accessed February 2017.
25. Montagnese F, Mondello S, Wenninger S, et al. Assessing the influence of age and gender on the phenotype of myotonic dystrophy type 2. J Neurol 2017;264:2472-2480.
26. Scott IA. Chronic disease management: a primer for physicians. Intern Med J 2008;38:427-437.
27. Adams SG, Smith PK, Allan PF, et al. Systematic review of the chronic care model in chronic obstructive pulmonary disease prevention and management. Arch Intern Med 2007;167:551-561.
28. Damery S, Flanagan S, Combes G. Does integrated care reduce hospital activity for patients with chronic diseases? An umbrella review of systematic reviews. BMJ Open 2016;6:e011952.