Niyomwungere Ernest, Routhier François, Gagnon Cynthia, Kirby R Lee, Rodrigue Xavier, Lessard Isabelle, Lettre Josiane, Best Krista L
School of Rehabilitation Sciences, Université Laval, Quebec City, QC, Canada.
Centre for Interdisciplinary Research in Rehabilitation and Social Integration, Centre Intégré Universitaire de Santé et de Services Sociaux de la Capitale-Nationale, Quebec City, QC, Canada.
JMIR Res Protoc. 2025 Jul 31;14:e66974. doi: 10.2196/66974.
Mobility impairment and participation restrictions are commonly experienced by individuals with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) and myotonic dystrophy type 1 (MD1), 2 disorders that are highly prevalent in the province of Quebec, Canada. People with ARSACS and MD1 experience a progressive decline in mobility, which commonly results in the provision of manual or power wheelchairs. While wheelchairs can facilitate mobility and social participation, their provision alone does not guarantee safe and effective use. Wheelchair skills training has been shown to be effective for improving manual skills and confidence among adult users with various diagnoses, which may enhance self-directed mobility and participation and reduce the risk of chronic and acute injuries. However, manual wheelchair skills training for people with ARSACS and MD1 remains understudied.
The primary aim of this study is to evaluate the efficacy of manual wheelchair skills training for safely improving wheelchair performance in people with ARSACS and MD1. The secondary outcomes include exploring the influence of manual wheelchair skills training on skill capacity, use self-efficacy, mobility, and the retention of outcomes 3 months later. We will also qualitatively explore the manual wheelchair training experiences of people with ARSACS and MD1.
This study will use a sequential multimethods design, combining a waitlist randomized controlled trial and qualitative interviews. The participants will include adults who have a diagnosis of ARSACS or MD1 who use a manual wheelchair for mobility. Participants will be randomly assigned to the intervention or control group using a 1:1 allocation ratio. The intervention group will receive 5 manual wheelchair skills training sessions (1-2 sessions/week), while the control group will receive no training. Data will be collected at baseline (T1), after the 4-week intervention (or waiting period for the control group; T2), and 3 months after T2 to assess retention (T3). The primary outcome will be manual wheelchair skills performance. Secondary outcomes will include manual wheelchair skills capacity, self-efficacy, and mobility. Semistructured individual interviews will be conducted to explore participants' expectations regarding manual wheelchair use, past manual wheelchair experiences, and perceptions of manual wheelchair skills training. Quantitative data will be analyzed using analysis of covariance (ANCOVA), controlling for baseline scores, and qualitative data will be analyzed using reflexive thematic analysis.
This study received ethical approval (2025-3100) in July 2024. Recruitment started in January 2025. A graduate student, a research assistant, and a research coordinator have been recruited and trained.
The results of this randomized waitlist-controlled trial will confirm whether manual wheelchair skills training can improve self-directed mobility and related outcomes for people with ARSACS and MD1. The findings may help guide clinical practice toward manual wheelchair skills training for understanding potential influences on manual wheelchair mobility in people living with neuromuscular disorders.
ClinicalTrials.gov NCT06596850; https://clinicaltrials.gov/study/NCT06596850.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/66974.
患有常染色体隐性遗传性夏尔勒瓦-萨格奈痉挛性共济失调(ARSACS)和1型强直性肌营养不良(MD1)的个体通常会出现行动障碍和参与受限,这两种疾病在加拿大魁北克省极为常见。ARSACS和MD1患者的行动能力会逐渐下降,通常需要配备手动或电动轮椅。虽然轮椅可以促进行动能力和社会参与,但仅提供轮椅并不能保证其安全有效使用。轮椅技能培训已被证明对提高患有各种疾病的成年使用者的手动技能和信心有效,这可能会增强自主行动能力和参与度,并降低慢性和急性损伤的风险。然而,针对ARSACS和MD1患者的手动轮椅技能培训仍未得到充分研究。
本研究的主要目的是评估手动轮椅技能培训对安全改善ARSACS和MD1患者轮椅使用性能的效果。次要结果包括探讨手动轮椅技能培训对技能能力、使用自我效能感、行动能力以及3个月后结果保持情况的影响。我们还将定性探索ARSACS和MD1患者的手动轮椅培训经历。
本研究将采用序贯多方法设计,结合等待名单随机对照试验和定性访谈。参与者将包括已诊断为ARSACS或MD1且使用手动轮椅行动的成年人。参与者将以1:1的分配比例随机分配到干预组或对照组。干预组将接受5次手动轮椅技能培训课程(每周1 - 2次),而对照组将不接受培训。数据将在基线(T1)、4周干预后(或对照组的等待期;T2)以及T2后3个月收集,以评估结果保持情况(T3)。主要结果将是手动轮椅技能表现。次要结果将包括手动轮椅技能能力、自我效能感和行动能力。将进行半结构化个体访谈,以探索参与者对手动轮椅使用的期望、过去使用手动轮椅的经历以及对手动轮椅技能培训的看法。定量数据将使用协方差分析(ANCOVA)进行分析,并控制基线分数,定性数据将使用反思性主题分析进行分析。
本研究于2024年7月获得伦理批准(2025 - 3100)。招募工作于2025年1月开始。已招募并培训了一名研究生、一名研究助理和一名研究协调员。
这项随机等待名单对照试验的结果将证实手动轮椅技能培训是否能改善ARSACS和MD患者的自主行动能力及相关结果。这些发现可能有助于指导临床实践进行手动轮椅技能培训,以了解对神经肌肉疾病患者手动轮椅行动能力的潜在影响。
ClinicalTrials.gov NCT06596850;https://clinicaltrials.gov/study/NCT06596850。
国际注册报告识别码(IRRID):PRR1 - 10.2196/66974。