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Progressive Decline in Daily and Social Activities: A 9-year Longitudinal Study of Participation in Myotonic Dystrophy Type 1

  • Kateri Raymond
    Correspondence
    Corresponding author Kateri Raymond, MOT, Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, 2230, rue de l’Hôpital, C.P. 1200, Jonquière (Québec) G7X 7X2, Canada.
    Affiliations
    School of Rehabilitation, Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, Québec, Canada

    Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Neuromuscular Clinic, Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Jonquière, Québec, Canada

    Research Centre on Aging, Centre intégré universitaire de santé et de services sociaux de l'Estrie–Centre hospitalier universitaire de Sherbrooke, Sherbrooke, Québec, Canada

    Centre de recherche Charles-Le Moyne - Saguenay–Lac-Saint-Jean sur les innovations en santé (CR-CSIS), Centre intégré universitaire de santé et de services sociaux du Saguenay–Lac-St-Jean, Chicoutimi, Québec, Canada
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  • Mélanie Levasseur
    Affiliations
    School of Rehabilitation, Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, Québec, Canada

    Research Centre on Aging, Centre intégré universitaire de santé et de services sociaux de l'Estrie–Centre hospitalier universitaire de Sherbrooke, Sherbrooke, Québec, Canada
    Search for articles by this author
  • Jean Mathieu
    Affiliations
    School of Rehabilitation, Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, Québec, Canada

    Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Neuromuscular Clinic, Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Jonquière, Québec, Canada

    Centre de recherche Charles-Le Moyne - Saguenay–Lac-Saint-Jean sur les innovations en santé (CR-CSIS), Centre intégré universitaire de santé et de services sociaux du Saguenay–Lac-St-Jean, Chicoutimi, Québec, Canada
    Search for articles by this author
  • Cynthia Gagnon
    Affiliations
    School of Rehabilitation, Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, Québec, Canada

    Groupe de recherche interdisciplinaire sur les maladies neuromusculaires (GRIMN), Neuromuscular Clinic, Centre intégré universitaire de santé et de services sociaux du Saguenay-Lac-St-Jean, Jonquière, Québec, Canada

    Centre de recherche Charles-Le Moyne - Saguenay–Lac-Saint-Jean sur les innovations en santé (CR-CSIS), Centre intégré universitaire de santé et de services sociaux du Saguenay–Lac-St-Jean, Chicoutimi, Québec, Canada
    Search for articles by this author
Published:March 02, 2019DOI:https://doi.org/10.1016/j.apmr.2019.01.022

      Abstract

      Objective

      To describe and compare changes in participation over a 9-year period in women and men with myotonic dystrophy type 1 (DM1). To compare participation restrictions with available reference values from a typical aging population living in the community.

      Design

      Descriptive longitudinal design comparing data from baseline (2002) with data from follow-up (2011).

      Setting

      Neuromuscular clinic and participant’s home.

      Participants

      Adults with DM1 participated in the follow-up study (N=115).

      Interventions

      Not applicable.

      Main Outcome Measure

      The Assessment of Life Habits measured participation in 10 domains of daily and social activities. The minimal clinically important difference is 0.5 on a 10-point scale for participation accomplishment level.

      Results

      A total of 62% of participants were women, and the mean age was 52.3±10.3 years. A decline (P<.01) was observed with increasing difficulty and assistance required in global participation (mean ± SD, −0.5±0.9), social activities subscore (−0.6±1.2), nutrition (−0.7±1.4), fitness (−1.0±1.6), personal care (−0.7±1.2), mobility (−0.5±1.9), community life (−0.8±1.9), and recreation (−1.5±3.0). More life areas are disrupted over time: 8 domains were below reference values from a population aged 55-64 years at follow-up compared with 2 domains at baseline. Satisfaction with participation remains high and stable over time.

      Conclusion

      As disease duration increases, global participation and more daily and social domains were restricted with increasing difficulty and assistance required. Adults with DM1 showed not only age-associated but disease-specific changes in participation. Description over time of participation could improve clinical assessment and guide interdisciplinary management of DM1, leading to higher rehabilitation success. Further investigation of the factors influencing changes in participation is required to support disease management and services planning.

      Keywords

      List of abbreviations:

      CTG (cytosine-thymine-guanine), DM1 (myotonic dystrophy type 1), HDM-DCP (Human Development Model–Disability Creation Process), ICC (intraclass correlation coefficient), LIFE-H (Assessment of Life Habits), MCID (minimal clinically important difference), MIRS (Muscular Impairment Rating Scale), RM-ANOVA (repeated measures analysis of variance)
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