Evaluation and Management of Dyspnea in Hypermobile Ehlers-Danlos Syndrome and Generalized Hypermobility Spectrum Disorder: Protocol for a Pilot and Feasibility Randomized Controlled Trial.

BACKGROUND: Dyspnea is a prevalent symptom in individuals with hypermobile Ehlers-Danlos Syndrome (hEDS) and generalized hypermobility spectrum disorder (G-HSD), yet its contributors have not been identified. One known contributor to dyspnea is respiratory muscle weakness. The feasibility and effectiveness of inspiratory muscle training (IMT) in combination with standard-of-care rehabilitation (aerobic, resistance, neuromuscular stabilization, and balance and proprioception exercises) in improving respiratory muscle strength and patient-reported outcomes in patients with hEDS or G-HSD have not been evaluated. OBJECTIVE: This study aims to evaluate dyspnea, respiratory muscle strength, and patient-reported outcome measures (PROMs) in hEDS or G-HSD compared with healthy controls and to assess the feasibility of a randomized controlled trial of IMT and standard-of-care rehabilitation for improving respiratory muscle strength, exercise capacity, and PROMs compared with standard-of-care rehabilitation in hEDS and G-HSD. METHODS: The study will include 34 participants with hEDS or G-HSD and 17 healthy, age- and sex-matched controls to compare respiratory muscle structure and function and PROMs. After baseline assessments, participants with hEDS or G-HSD will be randomized into the intervention group and provided IMT combined with Ehlers-Danlos Syndrome standard-of-care rehabilitation or into the usual care group, and provided only standard-of-care rehabilitation for 8 weeks. The intervention group will be prescribed IMT in their home environment using the POWERbreathe K5 IMT device (POWERbreathe International Ltd). IMT will comprise 2 daily sessions of 30 breaths for 5 days per week, with IMT progressing from 20% to 60% of the baseline maximal inspiratory pressure (MIP) over an 8-week period. Feasibility will be assessed through rates of recruitment, attrition, adherence, adverse events, and participant satisfaction. The primary pilot outcome is MIP change over an 8-week period in hEDS or G-HSD. Secondary outcomes will include the evaluation of dyspnea using Medical Research Council Scale and 18-point qualitative dyspnea descriptors; diaphragmatic thickening fraction using ultrasound; respiratory muscle endurance; pulmonary function; prefrontal cortical activity using functional near-infrared spectroscopy; aerobic capacity during cardiopulmonary exercise testing; quality of life using Short Form-36; and scores from the Depression, Anxiety, and Stress scale-21. These measures will also be performed once in healthy controls to compare normative values. Multivariable regression will be used to assess the contributors to dyspnea. Paired 2-tailed t tests will be used to assess the changes in MIP and secondary measures after 8 weeks of IMT. RESULTS: Study recruitment began in August 2021 and, with several disruptions owing to COVID-19, is expected to be completed by December 2023. CONCLUSIONS: This study will provide a better understanding of the factors associated with dyspnea and the feasibility and effectiveness of IMT combined with standard-of-care rehabilitation. IMT may be a novel therapeutic strategy for improving respiratory muscle function and patient-reported outcomes in individuals with hEDS or G-HSD. TRIAL REGISTRATION: ClinicalTrials.gov NCT04972565; https://clinicaltrials.gov/ct2/show/NCT04972565. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/44832.

Early Change in Lower Limb Strength and Function in Lung Transplant Patients After Center-Based and Telerehabilitation.

PURPOSE: The aim of this study was to examine the change and relationship among quadriceps torque (QT) and physical function in adult lung transplant (LTx) patients undergoing rehabilitation. METHODS: A prospective study assessed 6-min walk test (6MWT) distance, QT, and Short Physical Performance Battery (SPPB) at the start of pre-habilitation and 10-12 wk post-LTx. Functional outcomes were examined for within-group differences for participants who completed center-based rehabilitation between September 2019 and March 2020 and participants who completed telerehabilitation ("telerehab") between March 2020 and June 2021 during COVID-19. Relationships between QT, SPPB, and 6MWT were examined pre- and post-LTx. RESULTS: A total of 49 LTx recipients were included (30 men, 61 [56-67] yr, 26 center-based rehab, and 23 telerehab). The 6MWT increased (median 75 m: 95% CI, 35 - 117, P < .0001), and the telerehab group showed an LTx decrease in QT (-9.6 Nm: 95% CI, -29 to -2.3, P = .02) and an increased gait speed (0.21 m/sec: 95% CI, 0.11 - 0.47, P < .0001). Pre-LTx QT showed a moderate correlation to pre-LTx SPPB (r = 0.41, P = .004) and weak correlations to gait speed and 6MWT (r ranging from 0.21 to 0.35, P < .05). Post-LTx QT showed moderate correlations to post-LTx SPPB (r = 0.43, P = .002), gait speed (r = 0.54, P < .001), five-time sit-to-stand (r =-0.57, P < .0001), and 6MWT (r = 0.62, P < .0001). CONCLUSIONS: Early post-LTx 6MWT and gait speed increased with no improvement in QT or other SPPB components. Correlations between QT and measures of exercise capacity and lower limb function were stronger post-LTx. Serial measurements may further inform functional trajectories and rehabilitation models.

The positive impact of a telemedicine education program on healthcare workers during the COVID-19 pandemic in Ontario, Canada.

Context The impact of the COVID-19 pandemic on health care service and delivery has been profound. In addition to shifting and expanding clinical responsibilities, rapidly evolving information and guidelines during the COVID-19 pandemic has made it difficult for healthcare workers (HCWs) to synthesize and translate COVID-19 information into practice. Objective The objectives of this study are 1) to examine the impact of a telemedicine education program on HCW's self-efficacy and satisfaction and 2) to explore HCWs' experience in the program and context of practice during the COVID-19 pandemic. Study Design We conducted a prospective mixed methods parallel-design study among ECHO COVID participants using pre-post questionnaires and focus group discussions (FGDs). We examined questionnaire results for changes in HCWs' self-efficacy and satisfaction. We analyzed FGD data to explore HCWs' experience in ECHO and the context of their practice during the COVID-19 pandemic. Setting Project Extension for Community Healthcare Outcomes (ECHO) model is a virtual, telemedicine education model that provides longitudinal support and addresses the emerging needs of HCWs. "ECHO Ontario: Managing COVID-19 Patients in the Community" (ECHO) was launched in July 2020. The goal of the program was to disseminate best practices regarding COVID-19 as they emerged and to increase HCW confidence and knowledge of COVID-19. Population Studied The study population were practicing HCWs in the province of Ontario, Canada. 1) HCW self-efficacy 2) HCW satisfaction Results Of 299 HCWs registered in ECHO COVID, 114 (38.1%) participated in this analysis. Median self-efficacy scores increased from 5 (IQR 4 - 6) to 6 (IQR 6 - 6) (p < 0.0001), independent of profession, years in practice, age group, or type of practice environment. Participants were highly satisfied with the ECHO sessions with a median score of 4 (IQR 4 - 5). FGD data indicated that HCWs gained knowledge through ECHO, established the context of their practice, and revealed facilitators for ECHO program success, including the transition to virtual care, the practicability of knowledge provided, and a "perspective from the trenches." Conclusions This study demonstrated that a telemedicine education program aimed to support HCWs in managing patients with COVID-19 had a positive impact on self-efficacy and satisfaction, and that this impact was specifically mediated through the ECHO program.

Eccentric Training in Pulmonary Rehabilitation of Post-COVID-19 Patients: An Alternative for Improving the Functional Capacity, Inflammation, and Oxidative Stress.

The purpose of this narrative review is to highlight the oxidative stress induced in COVID-19 patients (SARS-CoV-2 infection), describe longstanding functional impairments, and provide the pathophysiologic rationale that supports aerobic eccentric (ECC) exercise as a novel alternative to conventional concentric (CONC) exercise for post-COVID-19 patients. Patients who recovered from moderate-to-severe COVID-19 respiratory distress demonstrate long-term functional impairment. During the acute phase, SARS-CoV-2 induces the generation of reactive oxygen species that can be amplified to a "cytokine storm". The resultant inflammatory and oxidative stress process causes organ damage, particularly in the respiratory system, with the lungs as the tissues most susceptible to injury. The acute illness often requires a long-term hospital stay and consequent sarcopenia. Upon discharge, muscle weakness compounded by limited lung and cardiac function is often accompanied by dyspnea, myalgia, anxiety, depression, and sleep disturbance. Consequently, these patients could benefit from pulmonary rehabilitation (PR), with exercise as a critical intervention (including sessions of strength and endurance or aerobic exercises). Unfortunately, conventional CONC exercises induce significant cardiopulmonary stress and increase inflammatory and oxidative stress (OS) when performed at moderate/high intensity, which can exacerbate debilitating dyspnoea and muscle fatigue post-COVID-19. Eccentric training (ECC) is a well-tolerated alternative that improves muscle mass while mitigating cardiopulmonary stress in patients with COPD and other chronic diseases. Similar benefits could be realized in post-COVID-19 patients. Consequently, these patients could benefit from PR with exercise as a critical intervention.

Feasibility of a Home-Based Exercise Program for Managing Posttransplant Metabolic Syndrome in Lung and Liver Transplant Recipients: Protocol for a Pilot Randomized Controlled Trial.

BACKGROUND: Posttransplant metabolic syndrome (PTMS) is a common contributor to morbidity and mortality among solid organ transplant recipients in the late posttransplant period (≥1 year). Patients diagnosed with PTMS are at a higher risk of cardiovascular disease and frequently experience decreased physical function and health-related quality of life (HRQL). Studies in the early posttransplant period (<1 year) have shown the benefits of facility-based exercise training on physical function and HRQL, but have not evaluated the effects on metabolic risk factors. It remains unclear whether home-based exercise programs are feasible and can be delivered at a sufficient exercise dose to have effects on PTMS. This protocol outlines the methodology of a randomized controlled trial of a partly supervised home-based exercise program in lung transplant (LTx) and orthotopic liver transplant (OLT) recipients. OBJECTIVE: This study aims to evaluate the feasibility (ie, recruitment rate, program adherence, attrition, safety, and participant satisfaction) of a 12-week individualized, home-based aerobic and resistance training program in LTx and OLT recipients initiated 12 to 18 months after transplantation, and to assess estimates of intervention efficacy on metabolic risk factors, exercise self-efficacy, and HRQL. METHODS: In total, 20 LTx and 20 OLT recipients with ≥2 cardiometabolic risk factors at 12 to 18 months after transplantation will be randomized to an intervention (home-based exercise training) or control group. The intervention group will receive an individualized exercise prescription comprising aerobic and resistance training, 3 to 5 times a week for 12 weeks. Participants will meet on a weekly basis (via videoconference) with a qualified exercise professional who will supervise exercise progression, provide support, and support exercise self-efficacy. Participants in both study groups will receive a counseling session on healthy eating with a dietitian at the beginning of the intervention. For the primary aim, feasibility will be assessed through recruitment rate, program adherence, satisfaction, attrition, and safety parameters. Secondary outcomes will be measured at baseline and 12 weeks, including assessments of metabolic risk factors (ie, insulin resistance, abdominal obesity, blood pressure, and cholesterol), HRQL, and exercise self-efficacy. Descriptive statistics will be used to summarize program feasibility and effect estimates (means and 95% CIs) for sample size calculations in future trials. RESULTS: Enrollment started in July 2021. It is estimated that the study period will be 18 months, with data collection to be completed by December 2022. CONCLUSIONS: A partly supervised home-based, individually tailored exercise program that promotes aerobic and resistance training and exercise self-efficacy may be an important intervention for improving the metabolic profile of LTx and OLT recipients with cardiometabolic risk factors. Thus, characterizing the feasibility and effect estimates of home-based exercise constitutes the first step in developing future clinical trials designed to reduce the high morbidity associated with PTMS. TRIAL REGISTRATION: ClinicalTrials.gov NCT04965142; https://clinicaltrials.gov/ct2/show/NCT04965142. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/35700.

Positive impact of a telemedicine education program on practicing health care workers during the COVID-19 pandemic in Ontario, Canada: A mixed methods study of an Extension for Community Healthcare Outcomes (ECHO) program.

INTRODUCTION: In addition to shifting and expanding clinical responsibilities, rapidly evolving information and guidelines during the COVID-19 pandemic has made it difficult for health care workers (HCW) to synthesise and translate COVID-19 information into practice. This study evaluated whether a COVID-19-specific telemedicine education program (ECHO COVID) would impact health care workers' self-efficacy and satisfaction in the management of patients with COVID-19. METHODS: A prospective mixed methods parallel-design study was conducted among ECHO COVID participants using pre-post questionnaires and a focus group discussion. Questionnaire results were examined for changes in health care workers' self-efficacy and satisfaction. Focus group discussion data were analysed to explore health care workers' experience in ECHO COVID and the context of their practice during the COVID-19 pandemic. RESULTS: 239 health care workers registered in ECHO COVID and 114 (47.7%) completed questionnaires and attended at least one ECHO COVID session. Median self-efficacy scores increased from 5 (IQR 4-6) to 6 (IQR 6-6) (p < 0.0001), independent of profession, years in practice, age group, or practice environment. Participants were highly satisfied with ECHO COVID sessions with a median score of 4 (IQR 4-5). Focus group discussion data indicated that health care workers gained knowledge through ECHO COVID and revealed facilitators for ECHO COVID program success, including the transition to virtual care, the practicability of knowledge provided, and a 'perspective from the trenches.' DISCUSSION: This study demonstrated that a telemedicine education program aimed to support health care workers in managing patients with COVID-19 had a positive impact on health care workers' self-efficacy and satisfaction. This impact was specifically mediated by the ECHO COVID program.

Telerehabilitation for Lung Transplant Candidates and Recipients During the COVID-19 Pandemic: Program Evaluation.

BACKGROUND: The COVID-19 pandemic resulted in a rapid shift from center-based rehabilitation to telerehabilitation for chronic respiratory disease and lung transplantation due to infection control precautions. Clinical experience with this delivery model on a large scale has not been described. OBJECTIVE: The aim of this study is to describe usage and satisfaction of providers and lung transplant (LTx) candidates and recipients and functional outcomes following the broad implementation of telerehabilitation with remote patient monitoring during the first wave of the COVID-19 pandemic. METHODS: This study was a program evaluation of providers, LTx candidates, and early LTx recipients who used a web-based, remote monitoring app for at least four weeks between March 16 and September 1, 2020, to participate in telerehabilitation. Within-subjects analysis was performed for physical activity, Self-efficacy For Exercise (SEE) scale score, aerobic and resistance exercise volumes, 6-minute walk test results, and Short Physical Performance Battery (SPPB) results. RESULTS: In total, 78 LTx candidates and 33 recipients were included (57 [51%] males, mean age 58 [SD 12] years, 58 [52%] with interstitial lung disease, 34 [31%] with chronic obstructive pulmonary disease). A total of 50 (64%) LTx candidates and 17 (51%) LTx recipients entered ≥10 prescribed exercise sessions into the app during the study time frame. In addition, 35/42 (83%) candidates agreed the app helped prepare them for surgery and 18/21 (85%) recipients found the app helpful in their self-recovery. The strongest barrier perceived by physiotherapists delivering the telerehabilitation was patient access to home exercise and monitoring equipment. Between the time of app registration and ≥4 weeks on the waiting list, 26 LTx candidates used a treadmill, with sessions increasing in mean duration (from 16 to 22 minutes, P=.002) but not speed (from 1.7 to 1.75 mph, P=.31). Quadriceps weight (pounds) for leg extension did not change (median 3.5, IQR 2.4-5 versus median 4.3, IQR 3-5; P=.08; n=37). On the Rapid Assessment of Physical Activity questionnaire (RAPA), 57% of LTx candidates scored as active, which improved to 87% (P=.02; n=23). There was a decrease in pretransplant 6-minute walk distance (6MWD) from 346 (SD 84) meters to 307 (SD 85) meters (P=.002; n=45) and no change in the SPPB result (12 [IQR 9.5-12] versus 12 [IQR 10-12]; P=.90; n=42). A total of 9 LTx recipients used a treadmill that increased in speed (from 1.9 to 2.7 mph; P=.003) between hospital discharge and three months posttransplant. Quadriceps weight increased (3 [IQR 0-3] pounds versus 5 [IQR 3.8-6.5] pounds; P<.001; n=15). At three months posttransplant, 76% of LTx recipients scored as active (n=17), with a high total SEE score of 74 (SD 11; n=12). In addition, three months posttransplant, 6MWD was 62% (SD 18%) predicted (n=8). CONCLUSIONS: We were able to provide telerehabilitation despite challenges around exercise equipment. This early experience will inform the development of a robust and equitable telerehabilitation model beyond the COVID-19 pandemic.

Subject validation of reusable N95 stop-gap filtering facepiece respirators in COVID-19 pandemic.

INTRODUCTION: The COVID-19 pandemic has unveiled widespread shortages of personal protective equipment including N95 respirators. Several centers are developing reusable stop-gap respirators as alternatives to disposable N95 respirators during public health emergencies, using techniques such as 3D-printing, silicone moulding and plastic extrusion. Effective sealing of the mask, combined with respiratory filters should achieve 95% or greater filtration of particles less than 1um. Quantitative fit-testing (QNFT) data from these stop-gap devices has not been published to date. Our team developed one such device, the "SSM", and evaluated it using QNFT. METHODS: Device prototypes were iteratively evaluated for comfort, breathability and communication, by team members wearing them for 15-30min. The fit and seal were assessed by positive and negative pressure user seal checks. The final design was then formally tested by QNFT, according to CSA standard Z94.4-18 in 40 volunteer healthcare providers. An overall fit-factor >100 is the passing threshold. Volunteers were also tested by QNFT on disposable N95 masks which had passed qualitative fit testing (QLFT) by institutional Occupational Health and Safety Department. RESULTS: The SSM scored 3.5/5 and 4/5 for comfort and breathability. The median overall harmonic mean fit-factors of disposable N95 and SSM were 137.9 and 6316.7 respectively. SSM scored significantly higher than disposable respirators in fit-test runs and overall fit-factors (p <0.0001). Overall passing rates in disposable and SSM respirators on QNFT were 65% and 100%. During dynamic runs, passing rates in disposable and SSM respirators were 68.1% and 99.4%; harmonic means were 73.7 and 1643. CONCLUSIONS: We present the design and validation of a reusable N95 stop-gap filtering facepiece respirator that can match existent commercial respirators. This sets a precedence for adoption of novel stop-gap N95 respirators in emergency situations.