Benefits of powered standing wheelchair devices for adolescents with Duchenne muscular dystrophy in the first year of use

Abstract

Aim Poorer physical and mental health often accompany loss of walking in Duchenne muscular dystrophy. This study assessed the impacts of powered wheelchair standing device (PWSD) use on muscle and joint pain, joint angles when standing and mental health in adolescents with Duchenne muscular dystrophy. Methods Fourteen adolescents and parents participated in a stepped wedge design study over 12 months. During a baseline and intervention period, adolescents described pain and mental health, and parents reported their child's mental health. Video data were collected to measure hip, knee and ankle joint angles in the preferred standing position. Results Compared with baseline and adjusting for covariates, standing wheelchair use was associated with no change in muscle or joint pain or videoed joint angles in standing. Child‐reported Strengths and Difficulties total scores decreased (coefficient −3.1, 95% confidence interval −4.6, −1.5); and parent‐reported Personal Adjustment and Role Skills Scale total scores increased (coefficient 7.9, 95% confidence interval 3.3–12.5). Conclusions PWSD use was associated with maintenance of musculoskeletal status and advantages to mental health. Long‐term observations are necessary to improve understanding of how to support wellbeing in adolescents with Duchenne muscular dystrophy.

Everyday Life Participation Using Powered Wheelchair Standing Devices by Boys With DMD

“…Identifying the subset of “high frequency users” is also important for researchers. Recruiting successful users for future studies on DMD and PWSDs would power studies more effectively and strengthen any conclusions made on the outcomes of the PWSD intervention, especially in view of inconclusive evidence on physiological changes such as bone mineral density and range of motion (Bayley et al, 2020; Pedlow et al, 2019; Townsend et al, 2016).…”

“…However, the desire for social engagement appeared an important motivator for supported standing in the ambulatory boys to “keep up” with peers in active physical activities. With disease progression, supported standing in PWSDs for non-ambulatory boys might allow equality and connection to peers, as indicated by comments from P3 such as, “ I feel I am less invisible when I can stand” and, “Standing makes people want to talk more to me.” Social skills, social maturity and change in adolescents’ interests may also alter perceptions and motivators about belonging and participation (Egilson & Traustadóttir, 2009) and use of PWSDs may confer mental health benefits (Bayley et al, 2020).…”

“…It is important to understand the use of the supported standing feature and prevent its abandonment since, without it, the PWSD merely performs the function of a seated PWC which could have been provided at lower cost. Supported standing in PWSDs by boys with DMD has potential to enable participation in everyday activities (Vorster et al, 2019), maintain musculoskeletal status and mental health benefits (Bayley et al, 2020). According to consensus guidelines on prescription of PWSDs for boys with DMD (Schofield et al, 2020), therapists and users agree on necessary considerations.…”

What is the effect of assistive devices for standing in the rehabilitation of patients with Duchenne muscular dystrophy? A Cochrane Review summary with commentary

“…Loss of ambulation usually occurs during adolescence. 7 The appearance and progression of musculoskeletal impairments and other complications such as respiratory and cardiac involvement lead to limitation in the activities of daily living and early death. 8 In general, the use of standing devices and orthoses is important to maintain ambulation and prevent musculoskeletal problems such as contractures.…”

“…DMD is a genetic condition that is characterized by muscle damage and progressive loss of muscle function in males. Loss of ambulation usually occurs during adolescence 7 . The appearance and progression of musculoskeletal impairments and other complications such as respiratory and cardiac involvement lead to limitation in the activities of daily living and early death 8 .…”