ISBN : 9780323957472
Author : Oscar Henry Mayer
Publisher : Academic Press
Year : 2023
Language : English
Type : Book
Description : Table of contents Cover image Title page Table of Contents Copyright Dedication Contributors Preface References Section 1: Introduction Chapter 1: Types of neuromuscular disease Abstract Introduction Genetic neuromuscular disease Acquired neuromuscular disease Conclusion References Chapter 2: Pulmonary pathophysiology of neuromuscular disease Abstract Introduction Components of respiratory system and mechanisms of respiratory progression Clinical features of respiratory involvement in neuromuscular disease References Chapter 3: The natural history of pulmonary disease Abstract References Section 2: Assessment Chapter 4: Assessment of pulmonary function Abstract Introduction Clinical evaluation: History and physical examination Volume Flow Pressure Gas exchange Conclusion References Further reading Chapter 5: Assessment of sleep-disordered breathing Abstract Introduction Pathophysiology Sleep-disordered breathings events associated with NMD Definitions of SDB and hypoventilation Evaluation of SDB Conclusion References Chapter 6: Assessment of need for airway clearance Abstract Introduction History and physical examination Pulmonary function testing Summary References Section 3: Clinical management Chapter 7: Disease-modifying pharmacotherapies Abstract Introduction Duchenne muscular dystrophy Spinal muscular atrophy Other rare neuromuscular diseases Pharmacoeconomics Conclusion References Chapter 8: Airway clearance Abstract The need for airway clearance Cough assessment Classification of airway clearance techniques (Table 8.1) Proximal airway clearance techniques Distal airway clearance techniques Medications Co-morbid conditions that can impact cough efficacy Considerations with tracheostomy/endotracheal tubes Clinical care guidelines Conclusion References Chapter 9: Aerodigestive problems and management in children with neuromuscular disorders Abstract Aerodigestive management in children with neuromuscular disorders Normal swallow Evaluation Management Noninvasive and invasive ventilation and aspiration References Chapter 10: Chronic mechanical ventilation Abstract Introduction Role and benefits of mechanical ventilation in NMD Initiation of mechanical ventilation in NMD Interfaces and modes Tracheostomy Monitoring: Clinical Monitoring: Electronic Ventilator data collection methods Ventilator manufacturer-specific platforms Summary Vignettes References Chapter 11: Thoraco-spinal disease Abstract Introduction Respiratory function in children with scoliosis Respiratory function before and after treatment in children with neuromuscular weakness and scoliosis References Chapter 12: Challenges in managing hypertonic neuromuscular disease Abstract Aspiration Spinal and chest wall deformities Airway clearance Upper airway obstruction and sleep-disordered breathing Conclusions References Chapter 13: Management of acute respiratory failure in neuromuscular disease Abstract Introduction Baseline risk factors for acute respiratory failure Causes of acute respiratory failure in NMD Acute management of respiratory illness in children with NMD Airway clearance Weaning Tracheostomy and invasive ventilation Conclusion References Chapter 14: Home management Abstract Preparation Home monitoring Community resources Routine health maintenance Contingency planning Quality of life References Section 4: Patient focused Chapter 15: Transition from pediatric to adult care in neuromuscular disease Abstract Introduction Transition stakeholders Rationale for transition planning in NMD Transition challenges in patients NMD and chronic respiratory failure Components of a successful transition program Health care transition timeline Transition lessons from other chronic disease models Special considerations for individuals with NMD during transition Future research Conclusion References Section 5: Conclusion Chapter 16: The evolving course of neuromuscular disease Abstract Defining natural history Spinal muscular atrophy Duchenne muscular dystrophy Conclusion References Index