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      ‘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

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          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.

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          Most cited references 36

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          Determining Validity in Qualitative Inquiry

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            Indigenous health part 2: the underlying causes of the health gap.

            In this Review we delve into the underlying causes of health disparities between Indigenous and non-Indigenous people and provide an Indigenous perspective to understanding these inequalities. We are able to present only a snapshot of the many research publications about Indigenous health. Our aim is to provide clinicians with a framework to better understand such matters. Applying this lens, placed in context for each patient, will promote more culturally appropriate ways to interact with, to assess, and to treat Indigenous peoples. The topics covered include Indigenous notions of health and identity; mental health and addictions; urbanisation and environmental stresses; whole health and healing; and reconciliation.
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              Consensus paper: management of degenerative cerebellar disorders.

               W A Ilg,  A Bastian,  S Boesch (2014)
              Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult. Yet there are recent developments that are likely to lead to significant improvements in the future. Most desirable would be a causative treatment of the underlying cerebellar disease. This is currently available only for a very small subset of cerebellar ataxias with known metabolic dysfunction. However, increasing knowledge of the pathophysiology of hereditary ataxia should lead to an increasing number of medically sensible drug trials. In this paper, data from recent drug trials in patients with recessive and dominant cerebellar ataxias will be summarized. There is consensus that up to date, no medication has been proven effective. Aminopyridines and acetazolamide are the only exception, which are beneficial in patients with episodic ataxia type 2. Aminopyridines are also effective in a subset of patients presenting with downbeat nystagmus. As such, all authors agreed that the mainstays of treatment of degenerative cerebellar ataxia are currently physiotherapy, occupational therapy, and speech therapy. For many years, well-controlled rehabilitation studies in patients with cerebellar ataxia were lacking. Data of recently published studies show that coordinative training improves motor function in both adult and juvenile patients with cerebellar degeneration. Given the well-known contribution of the cerebellum to motor learning, possible mechanisms underlying improvement will be outlined. There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed. Future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administrationRole: ValidationRole: VisualizationRole: Writing – original draftRole: Writing – review & editing
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: ValidationRole: VisualizationRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: InvestigationRole: MethodologyRole: ResourcesRole: ValidationRole: VisualizationRole: Writing – review & editing
                Role: Data curationRole: InvestigationRole: MethodologyRole: Resources
                Role: ConceptualizationRole: MethodologyRole: ResourcesRole: Writing – review & editing
                Role: ConceptualizationRole: ResourcesRole: Writing – review & editing
                Role: Project administrationRole: ResourcesRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: MethodologyRole: SupervisionRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: MethodologyRole: SupervisionRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: MethodologyRole: ResourcesRole: SupervisionRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                11 March 2019
                2019
                : 14
                : 3
                Affiliations
                [1 ] College of Healthcare Sciences, James Cook University, Cairns, Queensland, Australia
                [2 ] Machado Joseph Disease Foundation, Alyangula, Northern Territory, Australia
                [3 ] Community-based Health Promotion and Prevention Studies Group, College of Public Health, Medical and Veterinary Sciences and Australian Institute of Tropical Health and Medicine, James Cook University Cairns, Australia
                [4 ] Northern Institute, Charles Darwin University, Darwin, Northern Territory, Australia
                Western Sydney University, AUSTRALIA
                Author notes

                Competing Interests: This study received in-kind support from the MJD Foundation ( http://mjd.org.au), Anindilyakwa Land Council ( https://www.anindilyakwa.com.au) (flights (JC)) and Groote Eylandt and Bickerton Island Enterprises (GEBIE) (accommodation (JC)) ( http://www.gebie.com.au). GL, NK, LM and KP received salary from MJD Foundation. This study received funding support from the Lowitja Institute Aboriginal and Torres Strait Islander Health CRC (Lowitja Institute CRC) (Grant ID: 017-SF-005) ( https://www.lowitja.org.au). JL received salary from the Lowitja Institute CRC. Community research partners JL, GL and associate investigators LM, NK, KP are employees of the MJD Foundation. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

                Article
                PONE-D-18-30372
                10.1371/journal.pone.0212953
                6411155
                30856185
                © 2019 Carr et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                Page count
                Figures: 2, Tables: 2, Pages: 17
                Product
                Funding
                This study received in-kind support from the MJD Foundation ( http://mjd.org.au), Anindilyakwa Land Council ( https://www.anindilyakwa.com.au) (flights (JC)) and Groote Eylandt and Bickerton Island Enterprises (GEBIE) (accommodation (JC)) ( http://www.gebie.com.au). GL, NK, LM and KP received salary from MJD Foundation. This study received funding support from the Lowitja Institute Aboriginal and Torres Strait Islander Health CRC (Lowitja Institute CRC) (Grant ID: 017-SF-005) ( https://www.lowitja.org.au). JL received salary from the Lowitja Institute CRC. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Social Sciences
                Sociology
                Human Families
                Biology and Life Sciences
                Physiology
                Biological Locomotion
                Walking
                Medicine and Health Sciences
                Physiology
                Biological Locomotion
                Walking
                Medicine and Health Sciences
                Neurology
                Neurodegenerative Diseases
                Movement Disorders
                Ataxia
                Social Sciences
                Sociology
                Culture
                People and Places
                Geographical Locations
                Oceania
                Australia
                Medicine and Health Sciences
                Health Care
                Psychological and Psychosocial Issues
                Biology and Life Sciences
                Bioengineering
                Biotechnology
                Medical Devices and Equipment
                Assistive Technologies
                Wheelchairs
                Engineering and Technology
                Bioengineering
                Biotechnology
                Medical Devices and Equipment
                Assistive Technologies
                Wheelchairs
                Medicine and Health Sciences
                Medical Devices and Equipment
                Assistive Technologies
                Wheelchairs
                Biology and Life Sciences
                Psychology
                Emotions
                Social Sciences
                Psychology
                Emotions
                Custom metadata
                The study data cannot be made publicly available as it contains potentially identifying participant information (imposed by our approving ethics committees: Human Research Ethics Committee (HREC) of the Northern Territory (NT) Department of Health and Menzies School of Health Research (HREC 2016-2672), email: ethics@ 123456menzies.edu.au ; James Cook University HREC (H6860)), email: ethics@ 123456jcu.edu.au .

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