Sander Oorschot

The quality of evidence for improving physical fitness in people with neuromuscular diseases (NMDs) through aerobic exercise is low. The aim of this study was to evaluate the efficacy of combined personalized home-based aerobic exercise and coaching on physical fitness in people with NMD, compared with usual care. In a multicenter, assessor-blinded, randomized controlled trial, participants with different types of NMD were randomized (1:1 ratio) to a 6-month intervention (personalized home-based aerobic exercise and coaching) or usual care. Assessments were performed at baseline, directly after intervention, and at 6 and 12 months after intervention. The primary outcome was physical fitness, measured as peak oxygen uptake (VO_2peak) directly after intervention. Secondary outcomes included daily physical activity, quality of life, physical functioning, metabolic syndrome markers, and creatine kinase level. We conducted intention-to-treat linear mixed-model analyses for all outcomes, with the baseline value of the particular outcome as covariate. Ninety-one participants (median age = 64.0, 60% female) were randomized to the intervention (n = 44) or usual care (n = 47) group. The mean group difference in VO_2peak directly after intervention was 2.2 mL/min/kg (95% CI 0.2–4.1, p = 0.028) and 1.7 mL/min/kg (95% CI 0.1–3.4, p = 0.039) on average over time, in favor of the intervention group. There were no significant between-group differences in the secondary outcomes. Twenty-five and 22 adverse events were reported in the intervention and usual care groups, respectively. Creatine kinase levels remained unchanged. This study provides evidence that combined personalized home-based aerobic exercise and coaching is safe and improves physical fitness in people with NMD, but without evidence of improved physical functioning, daily physical activity, quality of life, or metabolic syndrome markers. This home-based approach has good potential for wider implementation. Future research should explore the association between changes in VO_2peak and functional outcomes and strategies to counteract the slightly diminishing long-term intervention effect. The study was registered in the Netherlands Trial Register (ID: NL7344) on November 5, 2018. The first participant enrolled on September 19, 2018. However, the Ethics Review Committee of the Amsterdam Medical Center approved the study protocol on November 7, 2017. No adjustments were made to the approved study protocol, and the register corresponds one on one with the approved study protocol. This study provides Class II evidence that a 6-month personalized aerobic exercise program combined with coaching improves maximal aerobic capacity in patients with NMDs without effective cures.

Objective: To determine the physical strain of walking and assess its relationship with daily steps and intensity of daily activity in people with neuromuscular diseases. Design: Cross-sectional study. Subjects/patients: Sixty-one adults with neuromuscular diseases. Methods: Physical strain of walking, defined as oxygen consumption during comfortable walking relative to peak oxygen uptake. Daily step count and daily time spent in moderate and vigorous physical activity were assessed using accelerometry and heart rate measurements, respectively. Regression analyses assessed the relationships between log daily step count and log daily time spent in moderate and vigorous physical activity, and physical strain of walking. Results: The mean (standard deviation) physical strain of walking was 73 (20)% Log daily step count and physical strain were negatively associated (β = –0.47). No association was found with log daily time spent in moderate and vigorous physical activity. Conclusions: The highly increased physical strain of comfortable walking indicates that walking is very demanding for people with neuromuscular diseases and is associated with a reduction in daily step activity. The absence of a relationship between intensity of activities and physical strain indicates that, despite a reduction in daily step activity, strenuous daily activities may still be performed.

Background: Home-based aerobic exercise in people with neuromuscular diseases (NMDs) has benefits compared to exercise in the hospital or a rehabilitation center because traveling is often cumbersome due to mobility limitations, and societal costs are lower. Barriers to home-based aerobic exercise include reduced possibilities for monitoring and lack of motivation. To overcome these and other barriers, we developed a mobile health app: Keep on training with ReVi (hereafter referred to as ReVi). Objective: We aimed to determine the usability of the ReVi app. Methods: Patients followed a 4-month, polarized, home-based aerobic exercise program on a cycle or rowing ergometer, with 2 low-intensity sessions and 1 high-intensity session per week supported by the ReVi app. The app collected training data, including heart rate and ratings of perceived exertion, provided real-time feedback on reaching target intensity zones, and enabled monitoring via an online dashboard. Physiotherapists instructed patients on how to use the ReVi app and supervised them during their training program. Patients and physiotherapists separately evaluated usability with self-developed questionnaires, including 9 questions on a 5-point Likert scale, covering the usability elements efficiency, effectiveness, and satisfaction. Results: Twenty-nine ambulatory adult patients (n=19 women; mean age 50.4, SD 14.2 years) with 11 different slowly progressive NMDs participated. Both patients and physiotherapists (n=10) reported that the app, in terms of its efficiency, was easy to use and had a rapid learning curve. Sixteen patients (55%) experienced 1 or more technical issue(s) during the course of the exercise program. In the context of effectiveness, 23 patients (81%) indicated that the app motivated them to complete the program and that it helped them to exercise within the target intensity zones. Most patients (n=19, 70%) and physiotherapists (n=6, 60%) were satisfied with the use of the app. The median attendance rate was 88% (IQR 63%-98%), with 76% (IQR 69%-82%) of time spent within the target intensity zones. Four adverse events were reported, 3 of which were resolved without discontinuation of the exercise program. Conclusions: The usability of the ReVi app was high, despite the technical issues that occurred. Further development of the app to resolve these issues is warranted before broader implementation into clinical practice.

BACKGROUND Home-based aerobic exercise in people with neuromuscular diseases (NMDs) has benefits compared to exercise in the hospital or a rehabilitation center because traveling is often cumbersome due to mobility limitations, and societal costs are lower. Barriers to home-based aerobic exercise include reduced possibilities for monitoring and lack of motivation. To overcome these and other barriers, we developed a mobile health app: Keep on training with ReVi (hereafter referred to as ReVi). OBJECTIVE We aimed to determine the usability of the ReVi app. METHODS Patients followed a 4-month, polarized, home-based aerobic exercise program on a cycle or rowing ergometer, with 2 low-intensity sessions and 1 high-intensity session per week supported by the ReVi app. The app collected training data, including heart rate and ratings of perceived exertion, provided real-time feedback on reaching target intensity zones, and enabled monitoring via an online dashboard. Physiotherapists instructed patients on how to use the ReVi app and supervised them during their training program. Patients and physiotherapists separately evaluated usability with self-developed questionnaires, including 9 questions on a 5-point Likert scale, covering the usability elements efficiency, effectiveness, and satisfaction. RESULTS Twenty-nine ambulatory adult patients (n=19 women; mean age 50.4, SD 14.2 years) with 11 different slowly progressive NMDs participated. Both patients and physiotherapists (n=10) reported that the app, in terms of its efficiency, was easy to use and had a rapid learning curve. Sixteen patients (55%) experienced 1 or more technical issue(s) during the course of the exercise program. In the context of effectiveness, 23 patients (81%) indicated that the app motivated them to complete the program and that it helped them to exercise within the target intensity zones. Most patients (n=19, 70%) and physiotherapists (n=6, 60%) were satisfied with the use of the app. The median attendance rate was 88% (IQR 63%-98%), with 76% (IQR 69%-82%) of time spent within the target intensity zones. Four adverse events were reported, 3 of which were resolved without discontinuation of the exercise program. CONCLUSIONS The usability of the ReVi app was high, despite the technical issues that occurred. Further development of the app to resolve these issues is warranted before broader implementation into clinical practice.

Background: In individuals with neuromuscular diseases (NMD), symptoms of muscle weakness, fatigue and pain may limit physical activity. Inactivity leads to reduced physical fitness, which further complicates daily life functioning. Due to inconclusive evidence regarding exercise in NMD, the optimal training approach and strategies to preserve an active lifestyle remain to be determined. The physical activity programme I’M FINE, consisting of individualized aerobic exercise to improve physical fitness and coaching to preserve an active lifestyle, was therefore developed. The primary objective of this study will be to evaluate the efficacy of the I’M FINE programme in terms of improved physical fitness in individuals with slowly progressive NMD, compared to usual care.Methods: A multicentre, assessor-blinded, two armed, randomized controlled trial will be conducted in a sample of 90 individuals with slowly progressive NMD. Participants motivated to improve their reduced physical fitness will be randomized (ratio 1:1) to the I’M FINE intervention or usual care. The I’M FINE intervention consists of a six-month physical activity programme, including individualized home-based aerobic exercise to improve physical fitness (i.e. peak oxygen uptake), and motivational interviewing coaching (e.g. goal setting, self-management) to adopt and preserve an active lifestyle. Measurements will be performed at baseline, post-intervention, and at 12- and 18-months follow-up. The primary outcome is peak oxygen uptake (VO₂peak) directly post intervention. Main secondary outcomes are physical capacity, muscle strength, self-efficacy, daily activity, quality of life and markers of metabolic syndrome. The primary analysis compares change in VO₂peak post-intervention between the intervention and usual care group, with analysis of covariance.Discussion: The I’M FINE study will provide evidence regarding the efficacy of a physical activity intervention on the physical fitness and active lifestyle over the short- and long-term in individuals with slowly progressive NMD. These outcomes could potentially improve the (inter)national guidelines for efficacy of aerobic exercise programmes and provide insight in achieving a more active lifestyle in NMD.Trial registration: (5/11/2018): Netherlands Trial Register NTR7609 (retrospectively registered),https://www.trialregister.nl/trial/7344. However, the Ethics Review Committee of the Amsterdam Medical Center (AMC) approved the study protocol on 7/11/2017. No adjustments were made to the approved study protocol before the first participant enrolment and registration. Registration was done after the second participant enrolment and the information in the register corresponds one on one with the approved study protocol.