Respiratory Disorders in Neuromuscular Disease: Management and Practice Principles

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Giuseppe Fiorentino, MD (Editor) – UOC Fisiopatologia and Respiratory Rehabilitation AO Dei Colli, Naples, Italy
Antonio Esquinas, MD (Editor) – Intensive Care Unit; Hospital Morales Meseguer, Murcia, Spain

Series: Advances in Respiratory and Critical Care Medicine
BISAC: HEA039120
DOI: https://doi.org/10.52305/BJYU4836

Over the years, the life expectancy of patients with neuromuscular disease has improved thanks to new knowledge and technological innovations. This new book is entirely dedicated to the knowledge of the complex and unique profile of patients suffering from neuromuscular pathology. Many key points are discussed by specialists dedicated to neuromuscular patient care.
Respiratory support is often indispensable to sustain the patient’s life. Non-invasive ventilation (NIV) has taken on an increasingly important role in the care of patients with chronic respiratory failure secondary to neuromuscular disease. The NIV approach and complementary techniques are described. The management of airways and clearance of secretions are important aspects which are dealt with in a dedicated session, and a new theme – laryngeal response pattern during NIV and cough assist techniques – is also addressed.

The timing of the tracheostomy and choice of cannula are decisive in the management of the neuromuscular patient, thus the use of invasive mechanical ventilation. Topics of interest also include perioperative respiratory management, weaning from mechanical ventilation and extubation. The use of imaging techniques, such as videofluoroscopy of swallowing and sonography can support the clinician in the diagnosis of complications. Today, even pulmonary rehabilitation is an integral and indispensable part of patient care.

The text – developed with the contribution of experts dedicated to the daily care of patients with neuromuscular disorders – is an ambitious project which aims to provide an extensive discussion of the topic and support for those who already work with this type of patient, as well as for those who are starting to engage in this type of patient care.

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Table of Contents

Preface

Introduction: Overview of Neuromuscular Disorders (NMDs) Affecting Respiratory Function
(Giuseppe Fiorentino, MD, Anna Annunziata, MD and Antonio Esquinas, MD-UOC Fisiopatologia and Respiratory Rehabilitation AO Dei Colli, Naples, Italy, et al.)

Section 1. Physiology Neuromuscular Disorders

Chapter 1. Respiratory Phenotypes of Pediatric Patients
(Adele Corcione, Melissa Borrelli and Francesca Santamaria-Departments of Translational Medical Sciences, Federico II University, Naples, Italy)

Chapter 2. Respiratory Muscle Strength
(Aditi S. Shah and Jeremy D. Road-Division of Respiratory Medicine, University of British Columbia, Vancouver, Canada and Department of Medicine, University of British Columbia, Vancouver General Hospital, Vancouver, Canada)

Chapter 3. Cough Function Abnormalities
(Mariano Mollica, MD, Martino Flora, MD, Elena Sciarrillo, PT, and Salvatore Musella, PT-UOC Fisiopatologia e Riabilitazione Respiratoria AO dei Colli, Naples, Italy)

Chapter 4. Diurnal Pulmonary Function Testing in Neuromuscular Disorders
(Francesca Simioli, MD, Maria Martino, MD and Sara Gioia, MD-Department of Respiratory Pathophysiology and Rehabilitation, Monaldi Hospital – A.O. Dei Colli, Naples, Italy)

Chapter 5. Sleep Disorders in Neuromuscular Diseases
(Michalis Agrafiotis, MD and Paschalis Steiropoulos, MD, PhD-Department of Pulmonary Medicine, “Georgios Papanikolaou” General Hospital of Thessaloniki, Exohi, Greece, et al.)

Section 2. Methodology/ Non Invasive Ventilation

Chapter 6. Initiation of Non-Invasive Ventilation in Children with Neuromuscular Disease
(Sherri Lynne Katz-Division of Respiratory Medicine, Department of Pediatrics, Children’s Hospital of Eastern Ontario/University of Ottawa, Ottawa, Ontario, Canada)

Chapter 7. Initiation of NIV in Adult
(Eugenio Sabato, MD and Emanuela Profilo, PhD-Department of Pneumology, “A. Perrino” Hospital, Brindisi, Italy)

Chapter 8. Modes of Ventilation
(Asier Bengoechea Calafell and Salvador Díaz Lobato-Emergency Medicine and Pulmonologist in Hospital de La Ribera, Valencia, Spain)

Chapter 9. Interface, Mouthpiece, Nasal Face/Alternative Interface
(Anna Annunziata, Maurizia Lanza, Antonio Esquinas and Giuseppe Fiorentino-Unit of Respiratory Physiopathology, Monaldi Hospital, Naples, Italy, et al.)

Chapter 10. Negative Mechanical Ventilation
(U. Vincenzi, MD)

Chapter 11. Intermittent Abdominal Mechanical Ventilation
(Paola Pierucci, MD, MSc, Valentina Di Lecce, MD and Onofrio Resta, MD-Cardio Thoracic Department, Respiratory ad Sleep Medicine Unit, Bari Policlinic. “Aldo Moro” University School of Medicine, Bari, Italy)

Chapter 12. MPV and Others Emergent Therapies
(Anna Annunziata, Ediva Myriam Borriello and Giuseppe Fiorentino-Department of Critic Area, Unit of Respiratory Disease, Monaldi Hospital, Naples, Italy)

Chapter 13. Noninvasive Ventilation
(Nanette C. Joyce, DO, MAS-Clinical Physical Medicine and Rehabilitation Department of Physical Medicine and Rehabilitation University of California, Davis School of Medicine, USA)

Chapter 14. Continuous Noninvasive Ventilator Support
(Fabrizio Rao, Marino Iatomasi and Lucia Greco-NeMO Clinical Centre, Milano, Italy and NeMO Clinical Centre, Arenzano, Genova, Italy, et al.)

Chapter 15. Risk of Unsuccessful Non-Invasive Ventilation in Neuromuscular Patients
(Paola Pierucci, MD, MSc, Andrea Portacci MD and Carla Santomasi MD-Cardio Thoracic Department, Respiratory ad Sleep medicine Unit, Bari Policlinic. “Aldo Moro” University School of Medicine, Bari, Italy)

Section 3. Monitoring

Chapter 16. Monitoring and Synchrony Ventilator Patient
(Mariano Alberto Pennisi and Paolo De Santis-UOSD Trauma e schock, Policlinico Gemelli Roma, Italy)

Section 4. Airways and Clearance

Chapter 17. Laryngeal Response Patterns during NIV and Mechanically Assisted Cough
(Tiina M. Andersen, PhD and Maria Vollsæter, PhD-Norwegian Advisory Unit on Home Mechanical Ventilation, Thoracic Department, Bergen, Norway; Department of Physiotherapy Haukeland University Hospital, Bergen, Norway and The Faculty of Health and Social Sciences, Western Norway University of Applied Sciences, Bergen, Norway, et al.)

Chapter 18. Tracheostomy Timing and Choice of Cannula
(Piero Ceriana and Cinzia Lastoria-Pneumologia Riabilitativa e terapia sub-intensiva respiratoria, ICS Maugeri IRCCS, Pavia, Italy)

Chapter 19. Cough Management
(Alexander Müller-Institute for Physical and Rehabilitation Medicine Cardiorespiratory Therapy Team, Clinic Floridsdorf, Vienna, Austria)

Section 5. Methodology/ Invasive Ventilation

Chapter 20. Invasive Ventilation
(David Orlikowski-Intensive care unit, Home Ventilation Department, Neuromuscular Disease Center Paris, North, East Raymond Poincaré Hospital, Paris Saclay University Hospital, Versailles Saint Quentin University)

Chapter 21. Weaning from Mechanical Ventilation and Extubation
(Piero Ceriana and Cinzia Lastoria-Pneumologia Riabilitativa e Terapia Sub-Intensiva Respiratoria,
ICS Maugeri IRCCS – Pavia, Pavia, Italy)

Chapter 22. Ventilatory Dependent Patient’s Unweanable Conditions
(A. Marotta, MD, R. Maffucci, MD and G. Cioffi, MD-Department of Respiratory Pathophysiology and Rehabilitation, Monaldi Hospital – A. O. Dei Colli, Naples, Italy)

Section 6. Perioperative Respiratory Management

Chapter 23. Perioperative Respiratory Management of Pediatric Patients with NMD
(Domenico Faticato, MD-Anesthesiology and Hyperbaric Medicine Unit, A.O.R.N. Santobono Pausilipon, Naples, Italy)

Chapter 24. Anesthetic Considerations for Neuromuscular Diseases
(A. Corcione, MD, L. Bucci, MD and C. Esposito, MD-Department of Critical Care Area, Monaldi Hospital, Ospedali dei Colli, Naples, Italy)

Chapter 25. Management of Acute Respiratory Failure in Pregnancy
(Antonietta Coppola, Giuseppe Fiorentino, MD and Antonio Esquinas, MD-UOC Fisiopatologia e Riabilitazione Respiratoria AO Ospedali dei Colli, Naples, Italy, et al.)

Chapter 26. Cardiac Manifestations of Neuromuscular Disease
(Gerardo Nigro, MD, PhD, V. Russo, MD, A. Rago, MD and A. A. Papa, MD-Department of Cardiothoracic and Respiratory Sciences, University of Campania “L. Vanvitelli,” Monaldi Hospital, Naples, Italy)

Chapter 27. Therapy of Scoliosis in Neuromuscular Pathology
(Valerio Pipola, Konstantinos Martikos, Emanuela Asunins and Alessandro Gasbarrini-Department of Spine Surgery, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy)

Section 7. Diagnosis Approach in Neuromuscular Disorders

Chapter 28. Imaging Techniques in NMD
(Tullio Valente, MD and Roberta Lieto, MD-Radiology, Azienda Ospedali dei Colli, Monaldi Hospital, Naples, Italy)

Chapter 29. Sonography in the Evaluation of the Disease
(Salvatore Guarino, MD, Christina Anne Jelly, MD and Tullio Valente, MD-Department of Radiology, Monaldi Hospital, Naples, Italy, et al.)
Section 8. Complementary Evaluations in Neuromuscular Disorders

Chapter 30. Vocal Cord Dysphonia and Sialorrhea
(Raffaele Dubbioso, PhD, MD and Rafael Soler, MD-Department of Neurosciences, Reproductive Sciences and Odontostomatology University of Naples Federico II, Napoli, Italy)

Chapter 31. Screening and Evaluation Tools of Dysphagia in Neuromuscular Disorders
(Maria Rosaria Valentino, MD Gerardo Langella, MD and Valentina Di Spirito, MD-Department of Respiratory Pathophysiology and Rehabilitation, Monaldi Hospital – A.O. Dei Colli, Naples, Italy)

Chapter 32. Aspiration Treatment and Decisional Algorithm
(Maurizia Lanza, MD-Unit of Respiratory Physiopathology, Monaldi Hospital, Naples, Italy)

Chapter 33. Infections in Neuromuscolar Disease Patients: A Point in the Right Direction
(Novella Carannante, MD, Eugenio Piscitelli, MD and Giuseppe Fiorentino, MD-Emergency Infectious Disease, Cotugno Hospital, AO Ospedali dei Colli, Naples, Italy, et al.)

Chapter 34. Nutritional Support in Neuromuscular Diseases
(D.Tammaro, M.Rispoli and A.Corcione-Critical Care Department, AO “Ospedali Dei Colli” – Monaldi Hospital, Naples)

Section 9. Pulmonary Rehabilitation

Chapter 35. Respiratory Muscle Training in Children and Adults with Neuromuscular Diseases
(Paolo Buonpensiero, PT and Emilia Privitera, PT, MSC-University of Neaples Federico II Departement of Pediatrics, Italy, et al.)

Chapter 36. Prevention of Pulmonary Morbidity
(Barbara Garabelli and Emilia Privitera-Pulmonary Unit, Centro Clinico Nemo c/o ASST Grande Ospedale Metropolitano, Niguarda, Milan, Italy, et al.)

Section 10. Home Mechanical Ventilation

Chapter 37. Discharge Planning from Hospital to Home Mechanical Ventilation
(Salvador Díaz Lobato, MD, PhD, Asier Bengoechea Calafell, MD and Johanny Vargas, MD-Nippon Gases Healthcare, Spain, et al.)

Chapter 38. Home Mechanical Ventilation
(Giorgio Emanuele Polistina, Pasquale Imitazione and Vittoria Graziani-Department of Pathophysiology and Respiratory Rehabilitation and Subintensive Respiratory Unit, Monaldi Hospital, Naples, Italy)

Section 11. Outcome

Chapter 39. Cause and Outcome of Acute Neuromuscular Respiratory Failure
(Saint-Clair Bernardes Neto and Antonio Sarmento-Gerontology – Catholic University of Brasília, Brasília/DF, Brazil, Department of Physical Therapy of Estácio Fatern, Federal University of Rio Grande do Norte (UFRN), SOMA Capacitações, Natal/RN, Brazil, et al.)

Section 12. Health Care and Ethical Aspects

Chapter 40. The Caregiver Formation
(Fabrizio Rao and Alice Pirola-NeMO Clinical Centre, Milano, Italy and NeMO Clinical Centre, Arenzano (Genova), Italy, et al.)

Chapter 41. The Role of Non Invasive Ventilation in Quality of Life in Neuromuscular Diseases
(E. Volpato, MS, PhD, F. Pagnini, PsyD, PhD and P. Banfi, MD-IRCCS Fondazione Don Carlo Gnocchi, Milan, Italy, et al.)

Chapter 42. Telemonitoring
(Giancarlo Garuti, Jacopo Garuti and Alessia Zanoli-Pulmonology Unit, Santa Maria Bianca Hospital, Mirandola MO, Italy, et al.)

Chapter 43. Palliative Care
(Salvador Díaz Lobato, MD, PhD, Sagrario Mayoralas, MD, PhD and Johanny Vargas, MD-Nippon Gases Healthcare, Spain, et al.)

Chapter 44. End-of-Life Care
(Francesca Trojsi, Giulia D’Alvano and Gioacchino Tedeschi-Department of Advanced Medical and Surgical Sciences, Università degli Studi della Campania “Luigi Vanvitelli,” Naples, Italy)

Chapter 45. Ethical and Medico-Legal Aspects
(Nicoletta Carmine, MD, Spinelli Sara, MD and Cauteruccio Rosa, MD-UOC Fisiopatologia and Respiratory Rehabilitation, AO Dei Colli, Naples, Italy)

Index

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