Supported Standing in Boys With Duchenne Muscular Dystrophy

Abstract

Purpose To evaluate the safety, tolerability, and efficacy of supported standing in a small sample of boys with Duchenne muscular dystrophy (DMD). Methods Four 12- to 15-year-old boys with DMD engaged in a home-based supported standing program for 6 to 12 months. A single-subject design was employed to examine muscle length. Bone mineral density was assessed at 4-month intervals using dual-energy x-ray absorptiometry. Results Upright, sustained supported standing was tolerated in 3 of the 4 boys. Mean weekly stand times ranged from 1.3 to 3.3 hours. Improved hip or knee flexor muscle length was seen in 3 of the 4 boys. No boys showed improved plantar flexor muscle length or increased lumbar bone mineral density. Conclusions Findings offer preliminary empirical evidence addressing the safety, tolerability, and efficacy of standing in boys with DMD. Additional research with an emphasis on better program adherence is indicated.

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

“…We did, however, observe knee flexion to be slightly more pronounced with time. Ankle angles were maintained, also consistent with previous observations following PWSD use 11 . A small raising of the heels in standing while in shoes may, however, not have been detected.…”

“…The participants appeared to avoid progression of joint deformity when using the PWSD over the 8‐month observation period, although we acknowledge that the video data did not directly assess the presence or absence of joint contracture which would have required direct assessment of full passive joint range. In standing, hips were more extended over time, consistent with slightly increased hip flexor length observed in a previous case series of PWSD users with DMD, 11 although these findings were not significant and may have reflected increased anterior pelvic tilt rather than true hip extension. This contrasts with increased hip contracture observed in one series of 34 adolescents during their first year of wheelchair use 8 .…”

“…Ongoing therapy programmes include active and passive stretching regimens with use of ankle‐foot orthoses 10 but these programmes do not always prevent contracture 7,9 . The PWSD provides capacity for more frequent change in body position which could mitigate against increasing deformity, as observed in four individuals with DMD who used a PWSD over a 6–12‐month period 11 . Poorer emotional wellbeing has been observed, particularly when walking becomes limited 12,13 with accompanied life‐style restrictions, reduced independence and social isolation.…”

Brazilian consensus on Duchenne muscular dystrophy. Part 2: rehabilitation and systemic care

“…Stretching continues. Long orthosis, for joint positioning while standing allows for longer periods of stretching 47 (Level of evidence: 3B, Class of recommendation: B) 48 (Level of evidence: 4C, Class of recommendation: C). Attention to the degree of muscle weakness and fatigue.…”

Rehabilitation Management of the Patient With Duchenne Muscular Dystrophy

“…The importance of initiating the preventive use of supported standing before the development of contractures should be emphasized. 112 Many advocate for the continued use of supported standing devices and a powered stand-and-drive motorized wheelchair into late-nonambulatory stages and throughout adulthood if contractures do not limit positioning and if devices are tolerated. Motorized stand-anddrive wheelchairs obviate the need for transfers to use supported standing, decreasing the risk of falls and increasing the number of hours per day of functional, comfortable, supported standing (Supplemental Fig 5).…”