‘Staying strong on the inside and outside’ to keep walking and moving around: Perspectives from Aboriginal people with Machado Joseph Disease and their families from the Groote Eylandt Archipelago, Australia

Abstract

Machado Joseph Disease (MJD) (spinocerebellar ataxia 3) is a hereditary neurodegenerative disease causing progressive ataxia and loss of mobility. It is the most common spinocerebellar ataxia worldwide. Among Aboriginal families of Groote Eylandt and related communities across Australia’s Top End, MJD is estimated to be more prevalent than anywhere else in the world. This study explored lived experiences of individuals and families with MJD to determine what is important and what works best to keep walking and moving around. A collaborative qualitative exploratory study, drawing from constructivist grounded theory methods, was undertaken for data collection and analysis. Semi-structured in-depth interviews were conducted with individuals with MJD (n = 8) and their family members (n = 4) from the Groote Eylandt Archipelago where ~1500 Aboriginal people (Warnumamalya) live. Interviews were led by Warnumamalya community research partners in participants’ preferred language(s). Participants described their experience of living with MJD, from ‘knowing about MJD’, ‘protecting yourself from MJD’ and ‘adjusting to life with MJD’. While the specific importance of walking and moving around differed widely between participants, all perceived that walking and moving around enabled them to do what mattered most to them in life. ‘Staying strong on the inside and outside’ (physically, mentally, emotionally, spiritually) was perceived to work best to keep walking and moving around as long as possible. A framework that included personal and environmental strategies for staying strong emerged: ‘Exercising your body’, ‘having something important to do’, ‘keeping yourself happy’, ‘searching for good medicine’, ‘families helping each other’ and ‘going country’. This study, the first to explore lived experiences of MJD in Australia, highlights the importance of maintaining mobility as long as possible. Strategies perceived to work best address physical and psychosocial needs in an integrated manner. Services supporting families with MJD need flexibility to provide individualised, responsive and holistic care.

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

“…Interventions designed to promote mobility for Aboriginal families with MJD from the Top End of Australia, whose culture and lifestyle are uniquely different to those with MJD in other parts of the world, have not been investigated 18. Importantly, these interventions are unlikely to be effective if they do not incorporate Indigenous views and concepts of physical activity and lifestyle in line with cultural and traditional practices 18–20…”

“…Aboriginal families with MJD from the Groote Eylandt Archipelago have experienced the impact of MJD on their families for generations 18. In a recent study,21 these families shared their perspectives on what is important and what works best to keep walking and moving around 18.…”

“…Aboriginal families with MJD from the Groote Eylandt Archipelago have experienced the impact of MJD on their families for generations 18. In a recent study,21 these families shared their perspectives on what is important and what works best to keep walking and moving around 18. Participants emphasised the importance of ‘staying strong on the inside and outside’ (physically, mentally, emotionally and spiritually) through ‘exercising your body’, ‘keeping yourself happy’, ‘going country’, ‘searching for good medicine’, ‘families helping each other’ and having ‘something important to do’ 18.…”

“…In a recent study,21 these families shared their perspectives on what is important and what works best to keep walking and moving around 18. Participants emphasised the importance of ‘staying strong on the inside and outside’ (physically, mentally, emotionally and spiritually) through ‘exercising your body’, ‘keeping yourself happy’, ‘going country’, ‘searching for good medicine’, ‘families helping each other’ and having ‘something important to do’ 18. These domains formed the ‘Staying Strong’ Framework to keep walking and moving around; a framework driven by community and culturally founded needs (table 1).…”

“…These domains formed the ‘Staying Strong’ Framework to keep walking and moving around; a framework driven by community and culturally founded needs (table 1). 18 This review set out to explore: (1) What interventions/strategies have been explored to promote walking and moving around for people with MJD (2); How the findings of these explorations align with the perspectives of families with MJD from Groote Eylandt, according to the domains of the ‘Staying Strong’ Framework 18…”