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Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease
1st Edition - April 13, 2023
Editor: Oscar Henry Mayer
Language: English
Paperback ISBN:9780323957472
9 7 8 - 0 - 3 2 3 - 9 5 7 4 7 - 2
eBook ISBN:9780323957489
9 7 8 - 0 - 3 2 3 - 9 5 7 4 8 - 9
Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease covers the broad medical problems and specific pulmonary conditions in patients with pediat…Read more
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Pulmonary Assessment and Management of Patients with Pediatric Neuromuscular Disease covers the broad medical problems and specific pulmonary conditions in patients with pediatric neuromuscular disease, ranging from the different types of neuromuscular disease, their pathophysiology, and assessment and management, including both novel disease modifying pharmacotherapies and state-of-the-art clinical management. This book provides evidence-based guidance for pediatric patients with neuromuscular disease, and is a valuable resource to pediatric pulmonologists, critical care physicians, and respiratory therapists who want an update and understanding on the cutting-edge therapies and standards of care for managing this population.
Provides a single comprehensive source of information to properly guide pulmonary assessments of pediatric neuromuscular disease
Discusses recent advancements in both medications and clinical respiratory management for pediatric patients
Covers different types of neuromuscular disease, including spinal muscular atrophy, Duchenne muscular dystrophy, congenital muscular dystrophy, and more
Pediatric pulmonologists, critical care physicians, respiratory therapists, neurologists and general pediatricians
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
No. of pages: 254
Language: English
Edition: 1
Published: April 13, 2023
Imprint: Academic Press
Paperback ISBN: 9780323957472
eBook ISBN: 9780323957489
OM
Oscar Henry Mayer
Dr Mayer is Attending Physician, Division of Pulmonology at the Children’s Hospital of Philadelphia and Professor of Clinical Pediatrics, at the Perelman School of Medicine, University of Pennsylvania. He has been managing patients with pediatric neuromuscular disease over the last 20 years and has become a recognized international expert in the field, has served on committees to determine standard of care practices and has lectured extensively on a variety of different topics in this field.
Affiliations and expertise
Professor of Clinical Pediatrics, The Perelman School of Medicine, University of Pennsylvania, USA
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