ABSTRACT
Introduction: Myotonic dystrophy type 1 (DM1) is related to muscle weakness, impaired balance, and a high risk of falls resulting in decreased quality of life and social participation. Indeed, DM1 is considered as a model of premature aging. However, the Covid-19 pandemic has exacerbated these health problems by decreasing the access to rehabilitation services and significantly decrease volunteer total weekly physical activity (PA) level. A pragmatic tool: PACE (Promoting Autonomy through exer-CisE), which has been developed to allow safe and adapted physical activity (APA) practice in older adults at home, could be a solution to counter physical deficiencies and lockdown restrictions in DM1 population. Objectives: 1. To adapt the PACE tool (decisional tree related to 35 APA programs) for the DM1 population (Pace-DM1 tool);2. To evaluate if assessing functional and muscular capacity in remote mode is feasible and valid in the DM1 population compared to in person modality;3. To evaluate the acceptability, feasibility and usability of implementing the PACE-DM1 tool for the health professionals and the patients. Methods: O-1: Two patient-partners, 3 participants who will experience the PACE-DM1 APA program, 2 health professionals from the Neuromuscular Disease Clinic of Jonquière (NMC), 1 NMC manager and 3 researchers were recruited to adapt the PACE tools using a co-creation design. O-2: Participants are assessing functional and muscular capacity [Time Up and Go, 30sec. chair stand test, 5 reps of sit-to-stand, 4-meter gait speed, balance and functional reach test] per and post-intervention (12 weeks) in remote (zoom) and in-person methods. O-3: After co-creation meetings, health professional were asked to evaluate the acceptability (satisfaction assessed via Likert scales;semi-opened questions), usability (System Usability Scale questionnaire) and feasibility (adherence, recruitment rate, etc.) of the PACE-DM1 tool. Results: First, co-creation meetings allowed us to adapt PACE tool (e.g. decisional tree cut points) for DM1 participants. Health professionals found PACE-DM1 tool exercises safe and adapted to the physical deficiencies of the DM1 population. Our preliminary data (n=2) using the adapted decisional tree showed similar results between inperson and remote assessment. More precisely, both assessment modality identified the same physical deficiencies (lower limb cardio-muscular, trunk mobility and stability, and balance) and prescribed the same program difficulty (out of a possible 5 levels). In the pre-intervention phase, implementing the PACE-DM1 tool was considered acceptable, usable and feasible for health professionals. Conclusion: Preliminary results demonstrate that an APA pragmatic, easy-to-use and adapted tool is implementable to prevent care trajectory of people with DM1. Nevertheless, these promising results need to be confirmed and validated with a larger sample.