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What is the best way to keep walking and moving around for individuals with Machado-Joseph disease? A scoping review through the lens of Aboriginal families with Machado-Joseph disease in the Top End of Australia
  1. Jennifer J Carr1,
  2. Joyce Lalara2,
  3. Gayangwa Lalara2,
  4. Moira Smith3,
  5. Jennifer Quaill3,
  6. Alan R Clough4,
  7. Anne Lowell5,
  8. Ruth N Barker1
  1. 1James Cook University, College of Healthcare Sciences, Cairns, Queensland, Australia
  2. 2Machado-Joseph Disease Foundation, Alyangula, Northern Territory, Australia
  3. 3James Cook University, College of Healthcare Sciences, Townsville, Queensland, Australia
  4. 4School of Public Health, Tropical Medicine and Rehabilitation Sciences, James Cook University, Cairns, Queensland, Australia
  5. 5Northern Institute, Charles Darwin University, Darwin, Northern Territory, Australia
  1. Correspondence to Jennifer J Carr; jennifer.carr2{at}my.jcu.edu.au

Abstract

Objectives Machado-Joseph disease (MJD) is the most common spinocerebellar ataxia worldwide. Prevalence is highest in affected remote Aboriginal communities of the Top End of Australia. Aboriginal families with MJD from Groote Eylandt believe ‘staying strong on the inside and outside’ works best to keep them walking and moving around, in accordance with six key domains that form the ‘Staying Strong’ Framework. The aim of this current study was to review the literature to: (1) map the range of interventions/strategies that have been explored to promote walking and moving around (functional mobility) for individuals with MJD and; (2) align these interventions to the ‘Staying Strong’ Framework described by Aboriginal families with MJD.

Design Scoping review.

Data sources Searches were conducted in July 2018 in MEDLINE, EMBASE, CINAHL, PsychINFO and Cochrane Databases.

Eligibility criteria for selecting studies Peer-reviewed studies that (1) included adolescents/adults with MJD, (2) explored the effects of any intervention on mobility and (3) included a measure of mobility, function and/or ataxia were included in the review.

Results Thirty studies were included. Few studies involved participants with MJD alone (12/30). Most studies explored interventions that aligned with two ‘Staying Strong’ Framework domains, ‘exercising your body’ (n=13) and ‘searching for good medicine’ (n=17). Few studies aligned with the domains having ‘something important to do’ (n=2) or ‘keeping yourself happy’ (n=2). No studies aligned with the domains ‘going country’ or ‘families helping each other’.

Conclusions Evidence for interventions to promote mobility that align with the ‘Staying Strong’ Framework were focused on staying strong on the outside (physically) with little reflection on staying strong on the inside (emotionally, mentally and spiritually). Findings suggest future research is required to investigate the benefits of lifestyle activity programmes that address both physical and psychosocial well-being for families with MJD.

  • Machado Joseph disease
  • spinocerebellar ataxia
  • walk
  • mobility
  • physical

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

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Footnotes

  • Contributors Authors JJC, RNB, AL, ARC designed the study. JJC, JQ, MS and RNB were involved in study selection, quality assessment and data extraction. JJC, JL, GL and RNB collaborated on data analysis and interpretation. The manuscript was drafted by JJC, RNB, AL and ARC. All authors approved the final version of the manuscript.

  • Funding The authors would like to thank the MJD Foundation, Anindilyakwa Land Council and Lowitja Institute Aboriginal and Torres Strait Islander Health CRC (Lowitja Institute CRC) (grant ID: 017-SF-005) (https://www.lowitja.org.au) for funding this work.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.