No abstract

The annual economic cost of DMD was found to be high, reflecting a significant socioeconomic burden, especially in boys who reach adulthood, where household resource use and caregiving burden is highest. Muscle Nerve 53: 877-884, 2016.

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.

This paper is a description of the updated care considerations for Duchenne muscular dystrophy originally published in 2010. This paper is the second of three papers that address the shifts in care and understanding of the disease since the previous publication in order to understand the new and everchanging care considerations which acompany the longer lifespan of those with DMD. This paper contributed to the development of item 5 for Walking in Table 2 indicating that standing programs including use of wheelchairs with upright positioning should commence in the early non-ambulatory phase.