Feasibility and impact of a physical activity and lifestyle program for Aboriginal families with Machado-Joseph disease in the Top End of Australia.

INTRODUCTION: Physical activity and lifestyle programs are scarce for people with hereditary ataxias and neurodegenerative diseases. Aboriginal families in the Top End of Australia who have lived with Machado-Joseph disease (MJD) for generations co-designed a physical activity and lifestyle program called the Staying Strong Toolbox. The aim of the present study was to explore feasibility and impact of the program on walking and moving around. METHODS: A mixed-methods, multiple case study design was used to pilot the Staying Strong Toolbox. Eight individuals with MJD participated in the program for 4 weeks. Participants tailored their own program using the Toolbox workbook. Families, support workers and researchers facilitated each individual's program. Feasibility was determined through program participation, adherence, coinciding or serious adverse events, participant acceptability and cost. Impact was determined through measures of mobility, ataxia, steps, quality of life, wellbeing and goal attainment, assessed before and after the program. RESULTS: All participants completed the program, averaging five activity sessions per week, 66 minutes per session, of walking (63.5%), strengthening/balance-based activities (16%), cycling (11.4%) and activities of daily living, cultural and lifestyle activities (10.5%). Seven participants were assessed on all measures on three occasions (baseline, pre-program and post-program), while one participant could not complete post-program measures due to ceremonial responsibilities. All had significant improvements in mobility, steps taken and ataxia severity (p<0.05) after the program. Quality of life and wellbeing were maintained. CONCLUSION: The program helped participants remain 'strong on the inside and outside'. Participants recommended implementation in 4-week blocks and for the program to be shared internationally. The Staying Strong Toolbox program was feasible for families with MJD. The program had a positive impact on walking and moving around, with participants feeling stronger on the outside (physically) and inside (emotionally, spiritually, psychosocially). The program could be adapted for use by other families with MJD.

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.

Physical activity has positive health implications for individuals living with neurodegenerative diseases. The success of physical activity programs, particularly in culturally and linguistically diverse populations, is typically dependent on their alignment with the culture, lifestyle and environmental context of those involved. Aboriginal families living in remote communities in the Top End of Australia invited researchers to collaborate with them to co-design a physical activity and lifestyle program to keep individuals with Machado-Joseph disease (MJD) walking and moving around. The knowledge of Aboriginal families living with MJD, combined with findings from worldwide MJD research, formed the foundation for the co-design. An experience-based co-design (EBCD) approach, drawing from Indigenous and Participatory methodologies, was used. An expert panel of individuals with lived experience of MJD participated in a series of co-design phases. Prearranged and spontaneous co-design meetings were led by local community researchers within each phase. Data was collected using a culturally responsive ethnographic approach and analysed thematically. Sixteen panel members worked to develop the 'Staying Strong Toolbox' to cater for individuals with MJD who are 'walking strong'; or 'wobbly'; or 'in a wheelchair'. Based on the 'Staying Strong Framework', the Toolbox was developed as a spiral bound A3 book designed to guide the user to select from a range of activities to keep them walking and moving around and to identify those activities most important to them to work on. The 'Staying Strong Toolbox' is a community driven, evidence based resource for a physical activity and lifestyle program for Aboriginal families with MJD. The Toolbox provides a guide for health professionals and support workers to deliver person-centred support to Aboriginal families with MJD, and that can be modified for use by other families with MJD or people with other forms of ataxia around the world.