Pulmonary Complications of Neuromuscular Disease (Respiratory Medicine) (Sep 19, 2024)_(3031653343)_(Humana).pdf

This book provides clinicians with a comprehensive resource on the topic of respiratory complications of neuromuscular disease. It includes chapters by neurologists, pediatric pulmonologists, adult pulmonologists, anesthesiologists, and sleep physicians. The book begins with a review of the pathophysiology and epidemiology of the neuromuscular diseases that affect the respiratory system. It then includes chapters on pediatric concerns and the transition from pediatric providers to adult providers. There is additionally a focus on various therapeutic approaches and the monitoring of noninvasive ventilation. Finally, it includes a chapters on secretion clearance, palliative care and end of life decision making. The book appeals to a broad medical audience including adult pulmonologists, pediatric pulmonologists, respiratory therapists, nurses, sleep medicine providers, and neurologists. Contents Contributors Chapter 1: Overview of the Pathophysiology and Epidemiology of Neurologic Disorders Affecting the Respiratory System Brief Overview of the Neural Control of Respiration Sensory Input Signal Transmission Motor Output Feedback Loop Research and Caveats Epidemiology of Neurological Disorders Affecting the Respiratory System Respiratory Dysfunction Aspect of Neurological Disorders Disorders Affecting the Brainstem Neuromyelitis Optica Multiple Sclerosis Stroke Motor Neuron Diseases Amyotrophic Lateral Sclerosis Spinal Muscular Atrophy Other Motor Neuronopathies Disorders Affecting the Phrenic Nerve Phrenic Nerve Injury Brachial Plexopathies/Radiculopathies Guillain–Barre Syndrome Chronic Inflammatory Demyelinating Polyneuropathy Neuromuscular Junction-Related Disorders Myasthenia Gravis Lambert–Eaton Myasthenic Syndrome Botulism Myopathies Muscular Dystrophies and Other Inherited Myopathies Acquired Myopathies References Chapter 2: Assessing Respiratory Function in the Patient with Neuromuscular Disease Introduction Physiology of Respiratory Pump Failure Clinical Manifestations of Neuromuscular Respiratory Failure Dyspnea and Respiratory Muscle Weakness Sleep Disturbances Bulbar Signs and Symptoms Pulmonary Function Tests in Neuromuscular Respiratory Failure Basic Pulmonary Function Testing (PFTs): Spirometry and Lung Volumes Testing for Respiratory Muscle Strength: MIP, MEP, and SNIP PFTs in Patients with Tracheostomy Ancillary Tests Assessing Respiratory Muscle Weakness Fluoroscopic Sniff Test Diaphragmatic Electromyography Diaphragmatic Ultrasonography Assessing Alveolar Ventilation in Neuromuscular Disease Implications and Detection of Hypercapnia in Neuromuscular Disease Blood Gas Analysis Noninvasive PCO2 Measurement End-Tidal Pressure of Expired CO2 (PetCO2) Transcutaneous CO2 Serum Bicarbonate Conclusion References Chapter 3: Sleep-Disordered Breathing in Neuromuscular Disease Natural History of Hypoventilation in Neuromuscular Disease-Related Sleep Night-Time Symptoms Precede Daytime Symptoms REM Symptoms Precede Non-REM Symptoms Sleep-Disordered Breathing in Duchenne Muscular Dystrophy: REM-Related Obstructive Sleep Apnea Precedes Muscular Weakness How to Elicit Sleep-Related History in Patients with Neuromuscular Disease Use of Polysomnography in Neuromuscular Disease Necessary Accommodations in the Sleep Lab for Patients with Disabilities Requirements for Assessing Sleep-Disordered Breathing in Neuromuscular Disease Diagnostic Standards in Polysomnography Apneas and Hypopneas Evaluating Nocturnal Hypoventilation for Neuromuscular Diseases Different Definitions of Hypoventilation Scoring Polysomnography Pseudo-Obstructive and Pseudo-Central Events How Scoring Polysomnography Differs in Neuromuscular Disease Titration of Non-invasive Ventilation with Polysomnography Location of Non-invasive Ventilation Titration Is Diagnostic Testing Needed in a Sleep Lab? Alternative Measurements of Respiratory Muscle Strength Titration Protocols for Technologists: Nuts and Bolts Bilevel Spontaneous/Timed (S/T) Volume-Assured Pressure Support (VAPS) References Chapter 4: Ventilatory Support for the Neuromuscular Patient: Noninvasive and Invasive Ventilation Introduction Indications and Considerations for Eligibility Contraindications Interface Type Nasal Masks Oral and Full-Face Masks Mouthpiece Ventilation Modes of Ventilation Spontaneous, Spontaneous/Timed, and Pressure-Control Modes VAPS Mouthpiece Ventilation Monitoring Non-Invasive Ventilation Indications for Tracheostomy with Mechanical Ventilation Invasive Versus Noninvasive Ventilation for Acute Respiratory Failure Desirable Versus Undesirable Effects of Tracheostomy with Mechanical Ventilation Survival Quality of Life and Caregiver burden Tracheostomy Impact on Speech and Swallowing Cost Tracheostomy Selection with NMD Modes of Invasive Mechanical Ventilation and Monitoring Decannulation of “Unweanable” Patients Transition to Hospice Care Ethical Considerations in Noninvasive Ventilation Summary References Chapter 5: Non-invasive Approaches to Secretion Clearance in Neuromuscular Disease Introduction Respiratory Parameters in Patients with Neuromuscular Diseases Insufflation capacity Maximum Insufflation Capacity (MIC) and Maximum Insufflation Capacity–Vital Capacity Difference (MIC–VC Difference) Peak Cough Flow (PCF) Cough Augmentation Lung Volume Recruitment (Breath Stacking) Manually Assisted Cough Mechanical Insufflation/Exsufflation Expiratory Muscle Training (EMT) Assisted Insufflation Non-invasive Ventilation Intermittent Positive Pressure Ventilation (i.e. Mouthpiece Ventilation) Glossopharyngeal Breathing Manual Airway Clearance Techniques Percussion Vibration Secretion Clearance Devices Intrapulmonary Percussive Ventilation High-Frequency Chest Wall Oscillations or Compression Inhaled Therapies Conclusion References Chapter 6: Monitoring Patients on Long-Term Noninvasive Ventilation Introduction Ventilation Devices Ventilator Data Acquisition Ventilator Manufacturer-Specific Platforms ResMed Philips Respironics Ventec Life Systems Breas Data Components, Reliability, and Accuracy Leak Tidal Volume Airway Pressures Spontaneous Triggers and Cycles Patient-Ventilator Asynchrony Respiratory Monitoring beyond the Ventilator Spirometry Pulse Oximetry Noninvasive Measurement of Carbon Dioxide Evidence for Telemonitoring of Assisted Ventilation Clinical Applications of Noninvasive Ventilation Monitoring Initial Setup Refinement of Initial Settings Troubleshooting and Long-Term Monitoring Troubleshooting Scenarios Low Adherence High Leak Inadequate Tidal Volume Daily Adherence Leak Mode Inspiratory Time and Cycling Control Inspiratory Airway Pressure Obstructive Events Lack of Telemonitoring Data Daytime Hypercapnia Legal and Ethical Issues with Telemonitoring Reimbursement Future Considerations for Telemonitoring References Chapter 7: Approach to the Pediatric Patient with Respiratory Complications of Neuromuscular Disease Introduction Epidemiology Pathophysiology Pediatric Airway Pediatric Chest Wall Airway Clearance Impairment Bulbar Dysfunction, Dysphagia, and Sialorrhea Parenchymal Lung Disease and Hypoxemia Hypercarbic Respiratory Failure Chest Wall Restriction Hypoventilation Sleep-Disordered Breathing Challenges of Respiratory Assessments in Children Indications for Testing Laboratory Testing Gas Exchange Tests of Respiratory Muscle Strength and Endurance Lung Volumes Treatment Lung Volume Recruitment Airway Clearance Cough Augmentation: Manually Assisted Cough Cough Augmentation: Mechanical Insufflation-Exsufflation Mucus Mobilization Chest Physiotherapy High-Frequency Chest Wall Compressions Intrapulmonary Percussive Ventilation Ventilatory Assistance Noninvasive Ventilation Diurnal Ventilatory Support Tracheostomy Ancillary Treatments in Neuromuscular Disorders Sialorrhea Management Dysphagia Management Management of Chest Wall Deformities Novel Therapeutics Transitioning to Adult Care Summary References Chapter 8: The Pediatric to Adult Transition of Patients with Neuromuscular Disease Key Concepts in Transition Differences Between Pediatric and Adult Models of Care Potential Negative Health Consequences Related to Gaps in Transition Patient and Family Perspectives Barriers and Facilitators to Transition Patient and Family Factors Healthcare Systems Health Insurance Criteria for Successful Transition Transition Policy Co-Development Tracking and Monitoring: Aged 14–18 Transition Readiness: Aged 14–18 Transition Planning: Aged 14–18 Transfer of Care: Aged 18–21 Transition Completion: Aged 18–23 Future Considerations References Chapter 9: Respiratory Care of the Individual with Muscular Dystrophy Disease Presentation and Progression in Muscular Dystrophy Presentation Progression Morbidity and Mortality Pathophysiology of Respiratory Problems in MD Weak Cough/Atelectasis/Pneumonia Sleep Disordered Breathing/Diurnal Respiratory Failure Multi-disciplinary Aspects of Care That Impact the Respiratory System Cardiac Musculoskeletal Care Swallowing and Nutrition Transition to Adult Care Clinical Respiratory Assessment of the Individual with MD History Symptoms of Weak Cough Symptoms of Sleep Disordered Breathing Physical Examination Pulmonary Function Testing Nocturnal Gas Exchange Assessment Frequency of Clinical Evaluation Respiratory Cardiac Respiratory Management of MD General Vaccinations/Anticipatory Guidance Corticosteroids Secretion Management Proximal Airway Clearance Lung Volume Recruitment Manually Assisted Cough Mechanical In-exsufflation Respiratory Muscle Training Distal Airway Clearance Therapies Medications and Other Adjuncts Management of Sleep Disordered Breathing and Respiratory Failure Sleep Disordered Breathing Diurnal Respiratory Failure Non-invasive Ventilation Mouthpiece Ventilation Tracheostomy Emerging Therapies References Chapter 10: Respiratory Care of the ALS Patient Introduction Epidemiology Risk Factors Genes Sex, Aging, and Environmental Exposures Pathophysiology (See Fig. 10.1) Clinical Features Diagnosis El Escorial Criteria Gold Coast Criteria Staging and Prognosis Treatment Complications of ALS and Respiratory Manifestations Respiratory Dysfunction Bulbar Symptoms Cognitive Impairment Sleep Disorders Evaluation of Pulmonary Status in ALS Symptom Assessment and Physical Exam Pulmonary Function Testing Nocturnal Oximetry, Polysomnography, and Arterial Blood Gas (ABG) Management of Hypoventilation Ineffective Cough and Secretion Management Airway Clearance Techniques Oral Secretion Management Dysphagia, Aspiration, and Nutrition Management Palliative Care and End of Life Care in ALS Conclusion References Chapter 11: Respiratory Care in Spinal Cord Injury Introduction Epidemiology Anatomy and Pathophysiology Muscles of Inspiration Diaphragm External Intercostal Muscles and the Accessory Muscles of Inspiration Muscles of Expiration Internal Intercostal Muscles Rectus Abdominis and External and Internal Obliques Pectoralis Major Bulbar-Innervated Muscles Injury Patterns and Timing Tetraplegia C1–C4 Injury C5–C8 Injury Paraplegia T1–T12 Temporal Patterns in Spinal Cord Injury Assessment of Respiratory Function: Pulmonary Function Tests Spirometry Airway Hyperreactivity and Bronchodilator Responsiveness in Spinal Cord Injury Maximal Inspiratory Pressure/Maximal Expiratory Pressure Lung Volumes Peak Cough Flow Symptoms of Respiratory Dysfunction in Spinal Cord Injury Dyspnea Cough Bronchial Mucus Hypersecretion Respiratory Complications of Spinal Cord Injury Atelectasis Pneumonia Sleep-Disordered Breathing Respiratory Failure Interventions Chest Physiotherapy/Postural Drainage/Manually Assisted Cough Glossopharyngeal Breathing Lung Volume Recruitment Respiratory Muscle Strength Training Abdominal Binders Mechanical Insufflation-Exsufflation, Suction Catheters, and Bronchoscopy Bronchodilators β2-Adrenergic Agonists Anticholinergics Non-invasive Ventilation Daytime Ventilation and Mouthpiece Ventilation Nocturnal Non-invasive Ventilation Invasive Mechanical Ventilation Tidal Volumes Positive End-Expiratory Pressure Weaning Mechanical Ventilation Tracheostomy and Decannulation Respiratory Muscle Electrostimulation: Diaphragmatic Pacing Conclusion References Chapter 12: Approach to the Patient with Neuromuscular Diseases Causing Acute Respiratory Failure Introduction Diagnostic Evaluation and Clinical Approach History Timeline Provoking Illness and Provoking Factors Specific Symptoms to Focus on in the History Physical Examination: Neurologic Mental Status Cranial Nerve Examination Special Cranial Nerve Exam Maneuvers Cogen’s Lid Twitch Fatigable Upgaze Tongue and Facial Weakness Extraocular Movement Abnormalities Motor Examination Fatigable Weakness Neck Flexion and Extension Single Breath Count Objective Testing of Respiratory Strength and Reserve Negative Inspiratory Force (NIF) Testing Additional Considerations of the Neurologic Examination General Examination History and Examination Summary General Overview and Early Clinical Assessment ICU Assessment and Monitoring Review of Common Neuromuscular Pathology in the ICU Myasthenia Gravis Myasthenic Crisis Clinical Presentation Diagnosis Management Immediate Management of Myasthenic Crisis Acetylcholinesterase Inhibitor Management Immediate Management of Myasthenic Crisis: Initiation of Immunotherapy Weaning from Mechanical Ventilation Guillain-Barré Syndrome (GBS) Clinical Presentation Diagnosis Differential Diagnosis Important Complications of GBS in the ICU Dysautonomia Syndrome of Inappropriate Antidiuretic Hormone Secretion Management Considerations for ICU-Level Care Treatment: Immunosuppression Dysautonomia Monitoring and Management ICU Management: Additional Early Complications ICU Management: Early Tracheostomy Intubation and Mechanical Ventilation Considerations for Ventilator Weaning, Mechanically Assisted Cough, and Sedation The Deconditioned Diaphragm Extubation Failure Consideration for Tracheostomy Conclusions References Chapter 13: The Global Perspective on Respiratory Care for Neuromuscular Disease Trends in Prevalence and Global Impact of Neuromuscular Disorders Relevance of Population Studies in ALS and Their Limitations Origins of Geographical Gradient and Ethnic Diversity in ALS Healthcare Impact of NMD Duchenne Muscular Dystrophy Health Burden Spinal Muscular Atrophy (SMA) Disease Burden (Including the Impact of Nusinersen and Gene Therapy) Screening for SMA Basic Research in NMD Patient Support Groups Holistic Medical Care Pathways in NMD Standards of Care Delivery of Care International Comparisons in Home Mechanical Ventilation in NMD Relationship Between the Prevalence of HMV and National Wealth New Modes of Care Delivery: Remote Care and Telemonitoring International Impact of COVID-19 in NMD References Chapter 14: Palliative Care and End-of-Life Decision-Making for People with Neuromuscular Disease Introduction Overview of Palliative Care Overview of Neuromuscular Diseases Specific Areas to Be Addressed and Interventions Respiratory Management Nutrition and Hydration Pain Fatigue Depression and Anxiety Communication Advance Care Planning Surrogate Decision-Making Ethical Considerations Psychological and Spiritual Support End of Life and Hospice End-of-Life Decision-Making References Index