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Medication adherence in patients with myotonic dystrophy and facioscapulohumeral muscular dystrophy

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Abstract

Myotonic dystrophy (DM) and facioscapulohumeral muscular dystrophy (FSHD) are the two most common adult muscular dystrophies and have progressive and often disabling manifestations. Higher levels of medication adherence lead to better health outcomes, especially important to patients with DM and FSHD because of their multisystem manifestations and complexity of care. However, medication adherence has not previously been studied in a large cohort of DM type 1 (DM1), DM type 2 (DM2), and FSHD patients. The purpose of our study was to survey medication adherence and disease manifestations in patients enrolled in the NIH-supported National DM and FSHD Registry. The study was completed by 110 DM1, 49 DM2, and 193 FSHD patients. Notable comorbidities were hypertension in FSHD (44 %) and DM2 (37 %), gastroesophageal reflux disease in DM1 (24 %) and DM2 (31 %) and arrhythmias (29 %) and thyroid disease (20 %) in DM1. Each group reported high levels of adherence based on regimen complexity, medication costs, health literacy, side effect profile, and their beliefs about treatment. Only dysphagia in DM1 was reported to significantly impact medication adherence. Approximately 35 % of study patients reported polypharmacy (taking 6 or more medications). Of the patients with polypharmacy, the DM1 cohort was significantly younger (mean 55.0 years) compared to DM2 (59.0 years) and FSHD (63.2 years), and had shorter disease duration (mean 26 years) compared to FSHD (26.8 years) and DM2 (34.8 years). Future research is needed to assess techniques to ease pill swallowing in DM1 and to monitor polypharmacy and potential drug interactions in DM and FSHD.

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Acknowledgments

The Registry is supported through the National Institute of Arthritis and Musculoskeletal and Skin Diseases and the National Institute of Neurological Disorders and Stroke (NIH Senator Paul D. Wellstone Muscular Dystrophy Cooperative Research Center Grant #U54-NS048843 and NIH contracts #N01-AR-5-2274 and #NO1-AR-0-2250).

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Authors and Affiliations

  1. Wegmans School of Pharmacy, St. John Fisher College, 3690 East Avenue, Rochester, NY, 14618, USA

    Bryan P. Fitzgerald, Kelly M. Conn, Joanne Smith, Andrew Walker & Amy L. Parkhill

  2. Department of Neurology, University of Rochester Medical Center, Rochester, NY, USA

    James E. Hilbert, Elizabeth A. Luebbe & Richard T. Moxley III

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  1. Bryan P. Fitzgerald
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  2. Kelly M. Conn
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  3. Joanne Smith
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  4. Andrew Walker
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  6. James E. Hilbert
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  7. Elizabeth A. Luebbe
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  8. Richard T. Moxley III
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Correspondence to Bryan P. Fitzgerald.

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This study was approved by the St. John Fisher College Institutional Review Board and performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Fitzgerald, B.P., Conn, K.M., Smith, J. et al. Medication adherence in patients with myotonic dystrophy and facioscapulohumeral muscular dystrophy. J Neurol 263, 2528–2537 (2016). https://doi.org/10.1007/s00415-016-8300-3

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  • DOI: https://doi.org/10.1007/s00415-016-8300-3

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