Assisted standing for Duchenne muscular dystrophy

Abstract

Background: Duchenne muscular dystrophy (DMD) is the most common X-linked neuromuscular disorder. When boys with DMD reach the second decade of life, they lose their ability to walk and become wheelchair dependent. Standing devices and orthoses are considered to be an essential component in the therapy management of DMD. Clinical opinion and research from other neurological conditions highlight the proposed benefits of standing device use, however, its effect within this population is currently unknown. A review of the evidence for the use of standing devices and orthoses is necessary to inform all stakeholders, including people with DMD, clinicians, decision makers and funders, and to guide future research.

Objectives: To assess the effects of standing devices and orthoses on musculoskeletal impairments (such as pain, contracture, scoliosis development and bone density) in boys and men with DMD, and secondarily to determine their effect on quality of life, participation in activities, and patient experience (satisfaction). We also considered any adverse events associated with their use.

Search methods: We searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, AMED, PsycINFO, CINAHL Plus, PEDro, and ProQuest Dissertations & Theses Global up to 5 September 2019. We checked references in identified trials, handsearched journal abstracts, and searched trials registries.

Selection criteria: We planned to include randomised controlled trials (RCTs) and quasi-RCTs of any model of standing device for use in DMD. The control interventions would have been any other comparison group, including no standing device, a different model of standing device, usual care, or an alternative form of assistive weight bearing.

Data collection and analysis: We used standard Cochrane methodological procedures.

Main results: Although we identified 13 potentially relevant studies, none met the inclusion criteria for this review.

Authors' conclusions: Since there were no RCTs or quasi-RCTs available to evaluate the effectiveness of standing devices in people with DMD, studies are needed to investigate the effectiveness of standing devices in this population.

Conflict of interest statement

IB: none known

CK: received no support from any organisation for the submitted work; she has received a research grant in partnership with Leckey.

SL: none known.

SMcD: none known

KP: was funded by Health and Social Care (HSC) Research and Development Northern Ireland for the completion of this Cochrane Review.

Figures

1

Study flow diagram.