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

Abstract

ObjectivesMachado-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.DesignScoping review.Data sourcesSearches were conducted in July 2018 in MEDLINE, EMBASE, CINAHL, PsychINFO and Cochrane Databases.Eligibility criteria for selecting studiesPeer-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.ResultsThirty 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’.ConclusionsEvidence 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.

Stroke survivors' perspectives on recovering in rural and remote Australia: A systematic mixed studies review

“…Data were firstly mapped (Carr et al, 2019) to the categories of the Living My Life framework (Jackson et al, 2021): Recovering is about living my life, as it evolves ; by endeavouring to recover my way ; and by navigating my recovery in my world . A convergent qualitative synthesis (Pluye & Hong, 2014) was used to integrate, organise and review findings according to each category, then presented in a narrative form (SJ).…”

Staying Strong Toolbox: Co-design of a physical activity and lifestyle program for Aboriginal families with Machado-Joseph disease in the Top End of Australia

“…An invited non-Aboriginal researcher (JC), an experienced physiotherapist in neurological rehabilitation, worked alongside families to conduct this research (supported by RB). JC was well known to families with MJD on Groote Eylandt and in Ngukurr through the two earlier studies on which this current study was based [12,33]. JC partnered with JL, GL, GwL and BD in study preparation, data collection, analysis and manuscript preparation.…”

“…The Co-design process is outlined in Fig 2 . Two earlier studies formed the foundation for the co-design process [12,33]. Study 1 involved interviews with individuals and families with MJD to explore 'what is important' and 'what works best' to keep people with MJD walking and moving around [12].…”

“…In Study 2, the Staying Strong Framework provided a lens for a scoping review of peer reviewed literature worldwide to map interventions that promote walking and moving for individuals with MJD [33]. Most studies focussed on 'exercising your body' (e.g., walking training, balance training or task-specific training) with positive findings when exercise occurred for at least 50 minutes in duration, two to three times each week, for approximately 4 weeks.…”

“…Families with MJD from Groote Eylandt and Ngukurr, supported by the MJD Foundation, recently partnered with university researchers to explore the best ways to keep walking and moving around [12]. The objective of the partnership was to bring together the knowledge of families with MJD [12], with what is known from MJD research from around the world [33], to develop a physical activity program [33]. Based on the findings of two earlier studies [12,33], the aim of this current study was to co-design a meaningful physical activity and lifestyle program tailored to the priorities of Aboriginal families with MJD in the Top End of Australia.…”