Enhancing Function, Fun and Participation with Assistive Devices, Adaptive Positioning, and Augmented Mobility for Young Children with Infantile-Onset Spinal Muscular Atrophy: A Scoping Review and Illustrative Case Report

Abstract

Recent advances in medical interventions have changed the prognosis for children with infantile-onset spinal muscular atrophy (SMA-1); however, little has been published regarding rehabilitation management. A rapid scoping review was conducted in November 2020 using Medline and CINAHL databases. Evidence supporting use of assistive devices and equipment to enhance participation, mobility, function, and posture in lying, sitting, and standing positions was sought. From 239 articles, only five studies (describing use of augmentative communication, manual and power mobility, supported standing and orthotic devices) met inclusion criteria. Results are presented alongside a case report of a 5-year-old boy (treated with Nusinersen since 7 months-of-age) who uses a variety of devices to enhance his activity and participation in family life. While reclined and tilted sitting positions as well as power mobility were previously considered for children with SMA-1, this child has progressed to supported upright standing, self-propelling a lightweight manual wheelchair indoors, communicating using multiple methods and taking steps in a dynamic mobility device. Power mobility was introduced in a switch-adapted cart at 11 months and he was independently exploring indoors and outside in his power wheelchair before 20 months. Research evidence is limited, but alongside the case report highlights the importance of a comprehensive and proactive approach to enhancing function, fun and participation with family and friends through adaptive equipment for children with significant and life-limiting disabilities.

AMESobreRuedas: early power mobility for increasing participation in children with Spinal Muscular Atrophy type I. Protocol of a randomized controlled trial

“…The assessment of the impact of interventions on participation is highly relevant because through it, children form friendships, acquire knowledge, learn skills, and express creativity, all of which promote their cognitive, perceptual, social, and motor development (25). Furthermore, participation-based interventions can also enhance functional activity and bodily functions overall, as well as motivation to explore and interact with the environment (11,36). This project could be of signi cant interest in this area, as it is expected to have a direct impact on evidence-based clinical practice, contributing to providing recommendations on the early use of low-cost motorized mobility devices in natural environments for children with SMA type I.…”

“…As far as the authors know, there is no consensus to guide recommendations on interventions based on participation and early use of powered mobility devices for children with SMA, since the evidence remains unclear (11). Therefore, the aim of this randomized clinical trial (RCT) is to evaluate whether an early power mobility intervention is effective for increasing participation, functional ability, independence and quality of life of young children diagnosed with SMA type I.…”

Diving into progress: a review on current therapeutic advancements in spinal muscular atrophy

“…The use of assistive devices and technology plays a significant role in SMA management. These devices help individuals with SMA lead more independent and fulfilling lives (112). Examples include: wheelchairs, scooters, and other mobility aids provide individuals with the freedom to move and explore their environments (113).…”

Community mobility and participation assessment of manual wheelchair users: a review of current techniques and challenges

“…In 2021, a critical review by Livingstone and Paleg (2021) examined over 200 studies for children with mobility disorders and found that intervention and rehabilitation, like strength training, stretching programs, and wheelchair use training, could improve the MWU's participation. In Hossain et al (2023) conducted a cross-sectional survey of 90 MWU to see the effect of wheelchair skills training.…”