Emergencies cards for neuromuscular disorders 1st Consensus Meeting from UILDM – Italian Muscular Dystrophy Association Workshop report

Fabrizio Racca 1, Valeria A. Sansone 2, Federica Ricci 3, Massimiliano Filosto 4, Stefania Pedroni 5, Elena Mazzone 6, Yaroslava Longhitano 1, Christian Zanza 1, Anna Ardissone 7, Rachele Adorisio 8, Angela Berardinelli 9, Claudia Bondone 10, Chiara Briani 11, Francesca Cairello 12, Elena Carraro 5, Giacomo P. Comi 13, Grazia Crescimanno 14, Adele D’Amico 15, Fabio Deiaco 16, Alessia Fabiano 17, Francesco Franceschi 18, Michelangelo Mancuso 19, Alessandro Massè 20, Sonia Messina 21, Tiziana Mongini 22, Isabella Moroni 7, Andrea Moscatelli 23, Olimpia Musumeci 21, Paolo Navalesi 24, Gerardo Nigro 25, Carlo Origo 26, Chiara Panicucci 27, Marika Pane 6, Martino Pavone 28, Marina Pedemonte 29, Elena Pegoraro 11, Marco Piastra 30, Antonella Pini 31, Luisa Politano 32, Stefano Previtali 33, Fabrizio Rao 34, Giulia Ricci 19, Antonio Toscano 21, Andrea Wolfler 23, Khristian Zoccola 26, Cristina Sancricca 35, Vincenzo Nigro 36, Antonio Trabacca 37, Andrea Vianello 38, Claudio Bruno 27,39

1 Department of Anesthesiology and Critical Care Medicine, Azienda Ospedaliera SS. Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; 2 Neurorehabilitation Unit, the NeMO Clinical Center in Milan, University of Milan, Milan, Italy; 3 Division of Child and Adolescent Neuropsychiatry, Department of Public Health and Pediatric Sciences, University of Turin, Turin, Italy; 4 Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy; NeMO-Brescia Clinical Center for Neuromuscular Diseases, Brescia, Italy; 5 Neuromuscular Omnicentre (NeMO), Fondazione Serena Onlus, Milan, Italy; 6 Paediatric Neurology and NeMO Center, Catholic University and Policlinico Gemelli, Rome, Italy; 7 Child Neurology Unit, Department of Pediatric Neuroscience Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy; 8 Bambino Gesù Hospital and Research Institute- ERN GUARD Heart, Rome, Italy; 9 IRCCS Mondino Foundation, Pavia, Italy; 10 Pediatric Emergency Department, University Hospital “Città della Salute e della Scienza di Torino”, Turin, Italy; 11 Department of Neurosciences, University of Padua, Padua, Italy; 12 Pediatric and Pediatric Emergency Unit, Pediatric Cardiology Service AO SS Antonio e Biagio e C. Arrigo, Alessandria, Italy; 13 Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Neurology Unit, Milan, Italy; Dino Ferrari Center, University of Milan, Milan, Italy; 14 Italian National Research Council, Institute for Biomedical Research and Innovation, Palermo, Italy; Regional Center for prevention and treatment of respiratory complications of rare genetic neuromuscular diseases, Villa Sofia-Cervello Hospital Palermo, Italy; 15 Neuromuscular and Neurodegenerative Unit, Department of Neurosciences. Bambino Gesù Paediatric Hospital, Rome, Italy; 16 Paediatric Emergency Department, University Hospital Città della Salute e della Scienza di Torino, Turin, Italy; 17 Pneumology Unit Azienda USL Romagna Infermi Hospital, Rimini, Italy; 18 Emergency Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy; 19 Department of Clinical and Experimental Medicine Neurological Institute, University of Pisa, Pisa, Italy; 20 Department of Orthopaedics and Traumatology, University of Turin, Turin, Italy; 21 Department of Clinical and Experimental Medicine, University Hospital of Messina, Messina, Italy; 22 Neuromuscular Unit, Department of Neurosciences RLM, University of Turin, Turin, Italy; 23 Neonatal and Pediatric Intensive Care Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy; 24 Department of Medicine - DIMED, University of Padua, Padua, Italy; 25 Department of Medical Translational Sciences, Division of Cardiology, Monaldi Hospital, University of Campania “Luigi Vanvitelli”, Naples, Italy; 26 Pediatric Orthopedic and Traumatology Unit Azienda Ospedaliera SS. Antonio e Biagio e Cesare Arrigo, Alessandria, Italy; 27 Center of Translational and Experimental Myology, IRCCS Istituto Giannina Gaslini, Genoa, Italy; 28 Pediatric Pulmonology & Respiratory Intermediate Care Unit, Sleep and Long Term Ventilation Unit, Pediatric Hospital Bambino Gesù Research Institute, Rome, Italy; 29 Pediatric Neurology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy; 30 Pediatric Anesthesiology and Intensive Care Unit, Università Cattolica, Rome, Italy; 31Pediatric Neuromuscular Unit, IRCCS Institute of Neurological Sciences, Bologna, Italy; 32 Cardiomiology and Medical Genetics, Department of Experimental Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy and Gaetano Torre for Muscular Dystrophy Association, Naples, Italy; 33 IRCCS San Raffaele Scientific Institute, Milan, Italy; 34 Neuromuscular Omnicentre (NeMO), Fondazione Serena Onlus La Colletta Hospital, Arenzano (GE), Italy; 35 Neuro-Rehabilitation Center, Fondazione UILDM Lazio Onlus, Rome, Italy; 36 Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy; 37 Scientific Institute IRCCS “E. Medea”, Unit for Severe disabilities in developmental age and young adults. (Developmental Neurology and Neurorehabilitation), Brindisi, Italy; 38 Respiratory Pathophysiology Division, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy; 39 Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health - DINOGMI, University of Genoa, Genoa, Italy

DOI 10.36185/2532-1900-081

Acute hospitalisation may be required to support patients with Neuromuscular disorders (NMDs) mainly experiencing respiratory complications, swallowing difficulties, heart failure, urgent surgical procedures. As NMDs may need specific treatments, they should be ideally managed in specialized hospitals. Nevertheless, if urgent treatment is required, patients with NMD should be managed at the closest hospital site, which may not be a specialized centre where local emergency physicians have the adequate experience to manage these patients. Although NMDs are a group of conditions that can differ in terms of disease onset, progression, severity and involvement of other systems, many recommendations are transversal and apply to the most frequent NMDs. Emergency Cards (EC), which report the most common recommendations on respiratory and cardiac issues and provide indications for drugs/treatments to be used with caution, are actively used in some countries by patients with NMDs. In Italy, there is no consensus on the use of any EC, and a minority of patients adopt it regularly in case of emergency. In April 2022, 50 participants from different centres in Italy met in Milan, Italy, to agree on a minimum set of recommendations for urgent care management which can be extended to the vast majority of NMDs. The aim of the workshop was to agree on the most relevant information and recommendations regarding the main topics related to emergency care of patients with NMD in order to produce specific ECs for the 13 most frequent NMDs.

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