Date Presented 03/27/20
This study is a longitudinal, randomized clinical trial exploring the benefits of two wheelchair-mounted dynamic arm supports (KINOVA O540 and JAECO WREX) in nonambulatory individuals with DMD experiencing upper-extremity weakness. Data collected will provide information about upper-extremity movement patterns and performance, as well as participant-reported outcomes regarding function, goal attainment, independence, quality of life, and user satisfaction.
Primary Author and Speaker: Danielle Hall
Contributing Authors: Natalie Silverman, Michelle Beck, Margaret Feltman, Amy Hartman, Roxanna Bendixen
PURPOSE: We explore the benefits of wheelchair-mounted dynamic arm supports in non-ambulatory males with Duchenne muscular dystrophy (DMD) who are experiencing upper extremity (UE) weakness. As a progressive neuromuscular disorder, males with DMD lose function in their UE, resulting in decreased independence. Dynamic arm supports (DAS), which use mechanical gravity compensation to support the UE, may increase participation and independence in DMD. Prior research on DAS has shown positive influence on psychological factors, social participation, and independence. There is, however, a significant need for standardization of evaluation and training methods and determination of time and cost savings. In comparison to passive arm supports, actively actuated DAS are new to the rehabilitation field with limited information available to determine efficacy and suitability in populations with UE deficits.
DESIGN: This longitudinal randomized clinical trial evaluates the use of two DAS in the participants’ natural home environment: 1) KINOVA O540 (powered/actively actuated); 2) JAECO WREX (non-powered/passive). Individuals with DMD recruited through the Muscular Dystrophy Association and the Duchenne Registry are invited to participate if they are over 14 years old and experiencing UE weakness. The study includes initial testing and installation of the randomized device, a 4-week home device trial, and final testing and removal of the device. The participant may elect to enroll in a second device trial with the non-randomized device.
METHOD: The Actigraph GT9x, a wrist-worn activity monitor, is worn during a 2-week baseline period, the 4-week device trial, and a 2-week post-device period to capture UE movement patterns. Functional testing is completed initially without the device and then re-administered with the device to evaluate impact of the device on function. Testing includes the Performance of Upper Limb Scale (PUL), the Goal Attainment Scale (GAS), and Neuro-Quality of Life (QoL) survey. A repeated measures ANOVA will be used to analyze UE function, the GAS, and QoL across our two cohorts at 2 time-points. Descriptive statistics will be used to explore technology usage and satisfaction through self-report assessments including the Upper Limb ADL self-report and caregiver logs. For the cross-over trial, a paired samples t-test will explore differences within subjects for all outcomes.
RESULTS: Currently 14 participants (mean age 18.64 yrs (SD=4.07)) are enrolled. There have been 12 trials with the KINOVA O540, and 5 with the JAECO WREX. Following the 4-week in home trial, on average all participants scored +1.00 (SD=1.06) on the GAS. Across all participants, PUL scores increased by +3.08 (SD=5.30). Thus far, these results indicate that participants are having positive increases in UE performance and attainment of meaningful goals using the dynamic arm support as compared to baseline. Actigraphy data are currently being analyzed. Additional data collection and analyses are still in progress.
CONCLUSION: The overall use of both DAS appears to benefit males with DMD at variable rates both within and between our cohorts. The information gleaned from this study guides understanding of the feasibility and usefulness of DAS in DMD. IMPACT: The results of this study will provide important objective knowledge regarding use of DAS, which will be beneficial to all stakeholders including manufacturers improving the technology design; clinicians recommending, fitting, and training individuals in use; payers and funding sources; and males with DMD and their caregivers who may directly benefit from use of the device.
References
Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens P, Cripe L, Kaul A, Kinnett K, McDonald C. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. The Lancet Neurology. 2010;9(1):77-93.
Van der Heide, L. A., van Ninhuijs, B., Bergsma, A., Gelderblom, G. J., van der Pijl, D. J., & de Witte, L. P. (2014). An overview and categorization of dynamic arm supports for people with decreased arm function. Prosthetics and Orthotics International, 38(4), 287-302.
