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.
Plain language summary
The use of standing devices for boys with Duchenne muscular dystrophy
Review question
What is the effect of using standing devices in boys with Duchenne muscular dystrophy (DMD)?
Background
DMD is a condition that affects only boys. The progressive muscle wasting that occurs with the condition results in decreased walking ability and eventually, use of a wheelchair for mobility. Specialist equipment is used routinely to assist boys with DMD to stand or bear weight. Such equipment includes standing devices (e.g. frames or standing wheelchairs) and orthoses (e.g. braces or splints). We have evidence from other groups of people that standing reduces physical problems, including pain, development of contractures (fixed shortening of muscles) and curvature of the spine. Standing also strengthens bones. However, we have no evidence about the effects of the use of standing frames or orthoses by boys and men with DMD.
We searched the medical literature for studies that met our inclusion criteria. We required studies to have assessed standing devices for use in DMD compared to no standing device, a different model of standing device, usual care, or an alternative aid to enable weight bearing. Studies had to randomly allocate participants to treatment groups (these studies usually provide the highest quality evidence).
Key results and quality of evidence
We found no studies that met our inclusion criteria.
Conclusion
We found no relevant trials eligible for this review to determine the effectiveness of standing devices for boys with DMD. We need further studies to determine whether standing devices have an impact on specific health problems, participation in activities, and quality of life. Future research studies should also focus on the ‘dose’ of standing (i.e. the duration, frequency and amount of weight bearing) and the use of different orthoses.
The evidence is current to September 2019.
