JMIR Publications

ABSTRACT

Background:

Mobility impairment and participation restrictions are experienced by individuals with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) and myotonic dystrophy type 1 (MD1), two 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, provision alone does not guarantee safe and effective use. Wheelchair skills training has been shown effective for improving manual wheelchair (MWC) skills and confidence among adult MWC users with variable diagnoses, which may enhance self-directed mobility and participation and reduce the risk of chronic and acute injuries. However, MWC skills training for people with ARSACS and MD1 remains understudied.

Objective:

The primary objective of this study is to evaluate the efficacy of MWC skills training for safely improving MWC skills performance in people with ARSACS and MD1. Secondary outcomes include exploration of the influence of MWC skills training on MWC skills capacity, MWC use self-efficacy, MWC mobility, and retention of outcomes three months later. We will also qualitatively explore the MWC training experiences of people with ARSACS and MD1.

Methods:

This study will use a sequential multi-methods design, combining a waitlist randomized controlled trial and qualitative interviews. The participants will include adults who have a diagnosis of ARSACS or MD1, and who use a MWC 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 MWC 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 MWC skills performance. Secondary outcomes will include MWC skills capacity, MWC use self-efficacy, and MWC mobility. Semi-structured individual interviews will be conducted to explore participants’ expectations regarding MWC use, past MWC experiences and perceptions of MWC skills training. Quantitative data will be analysed using ANCOVA (controlling for baseline scores) and qualitative data using reflexive thematic analysis.

Results:

Research ethics was approved in July 2024. Study teams members have met to plan recruitment which will start in Fall 2024. A graduate student, research assistant, and research coordinator have been recruited and trained.

Conclusions:

MWC skills training may be one way to improve self-directed mobility and related outcomes for people with ARSACS and MD1. The results of this randomized wait-list controlled trial will help guide clinical practice toward MWC skills training for understanding potential influences on MWC mobility of people living with neuromuscular disorders. Clinical Trial: Clinicaltrials.gov NCT06596850