Reducing The Cost Of Pulmonary Impairment In Children With Neuromuscular Disease

1. Reducing The Cost Of Pulmonary Impairment In Children With Neuromuscular Disease Chris Landon, MD, FAAP, FCCP Ventura County Medical Center Ventura, California Audrius Plioplys, MD, CMD,FRCPC, FAAP Mercy Hospital Chicago, Illinois

2. Objectives • 1. Provide an overview of neuromuscular diseases and complications that predispose patients to frequent respiratory exacerbations. • 2. Identify treatment strategies/medically appropriate care to meet needs of this difficult patient population to promote improvements in quality of life, and positive clinical outcomes. • 3. Discuss evidence supporting the effect of airway clearance with The Vest system on reducing costs associated with pulmonary complications/exacerbations.

3. Neuromuscular Diseases: Overview • Children who experience varying degrees of neurological/neuromuscular dysfunction • Diagnoses include: cerebral palsy, muscular dystrophy, spinal muscular atrophy, brain injury, consequences of infectious disease, inherited metabolic disorders, etc. • One child in 1000 is institutionalized as a result of profound disability

4. Neuromuscular Diseases: Overview • Neuromuscular • Gastroesophageal • Immune system • Respiratory • Psychosocial Multi-system assessment necessary to determine risk of pulmonary involvement:

5. Neuromuscular Diseases Assessment of complications that predispose to pulmonary involvement • Neuro assessment • Skeletal muscle control • Respiratory and ventilatory muscle abnormalities • Weak/absent cough • Weak/absent gag reflex • Upper airway control and coordination • Seizure activity • Spasticity

6. Neuromuscular Diseases Assessment of complications that predispose to pulmonary involvement • Neuro assessment – Dysphagia • Oral motor dyskinesia/pseudobulbar palsy • True bulbar palsy • Dysfunctional arousal • Oral motor weakness • Increased secretions

7. Neuro assessment – Oral motor weakness Muscular dystrophies Myopathies Neuromuscular junction disorders Anterior horn cell disorders Typical symptoms Too weak to swallow Too weak to cough Easily fatigued Head position dependent Neuromuscular Diseases Assessment of complications that predispose to pulmonary involvement

8. Neuro assessment – Increased secretions Autonomic dysfunction Medication effects Frequent seizures Typical symptoms Constant drooling Worse with stress or infection Drowning in drool Neuromuscular Diseases Assessment of complications that predispose to pulmonary involvement

9. Poor Swallow Control (Dysphagia) • Oral motor dyskinesia / pseudobulbar palsy • True bulbar palsy • Dysfunctional arousal • Oral motor weakness • Increased secretions

10. Oral Motor Dyskinesia / Pseudobulbar Palsy • Diffuse or bilateral cortical damage • Basal ganglia damage • Cerebellar – brainstem damage

11. Oral Motor Dyskinesia / Pseudobulbar Palsy • Typical symptoms: • Increased gag, choking, vomiting • Nasal regurgitation • Tonguing – pushing • Poor tolerance of liquids and chunks

12. True Bulbar Palsy • Cranial nerves 9, 10, 12 • Pontine-medullary damage • Arnold-Chiari malformation • Bulbar syrinx • Moebius syndrome

13. True Bulbar Palsy • Typical symptoms • Decreased gag, poor palate movement • Pocketing of food • Rumination • Drooling • “O” sign

14. Dysfunctional Arousal • Disorder of excessive somnolence • Toxic encephalopathy • Drug induced stupor • Autistic disorder

15. Dysfunctional Arousal • Typical symptoms • Must be reminded to swallow • Falls asleep while eating • Poor cough reflex • “Q” sign, string bean sign

16. Oral Motor Weakness • Myopathies • Muscular dystrophies • Neuromuscular junction disorders • Anterior horn cell disorders

17. Oral Motor Weakness • Typical symptoms • Too weak to swallow • Too weak to cough • Easily fatigued • Head position dependent

18. Increased Secretions • Autonomic dysfunction • Medication effects • Frequent seizures

19. Increased Secretions • Typical symptoms • Constant drooling • Worse with stress or infection • Drowning in drool

20. Poor Breathing Control • Central neurogenic hypoventilation • Periodic breathing patterns • Ondine’s curse • Stupor and coma • Thoracic weakness

21. Central Neurogenic Hypoventilation • Diffuse cortical damage • Poor hypoxic response • Poor hypercarbic response • Worse with stress or infection

22. Periodic Breathing Patterns • Cheyne-Stokes respiration • Biot’s respiration • Rett’s syndrome • Apneustic breathing

23. Ondine’s Curse • Waking versus sleeping centers • Defect in shift to automatic breathing • “… if I should die before I wake…”

24. Stupor and Coma • Brainstem dysfunction from pressure • Brainstem dysfunction from ischemia • Brainstem suppression from drugs • Brainstem degeneration

25. Thoracic Weakness • Myopathies • Muscular dystrophies • Neuromuscular junction disorders • Anterior horn cell disorders

26. Recognition of Neurogenic Pulmonary Clearance Problems • Recognize the possible brain region involved • Perform a careful history • Perform a careful examination • Sleep study / Life Shirt • MRI – attention to brainstem • ? muscle studies ?

27. The Upper Airway-Swallowing and Aspiration Aspiration Associated Pneumonias Lower Esophageal Aspiration, Gastric Distention and Airway Remodeling Gastroesophageal Reflux Disease (GERD) Fundoplication Versus Medication and Airway Clearance Nutrition and the Immune System Gastroesophageal Function and Complications

28. The Faces of Dysfunction Arching Regurgitation Failure To Thrive Irritability Refusing Feedings Gagging and Choking

29. Sandifer’s Syndrome • Characterized by arching and turning the head to the side (opisthotonos) may give the appearance of a seizure. Functions to clear the esophagus of acid reflux by increasing intrathoracic pressure.

30. Cardiorespiratory Reflexes

31. Cardiorespiratory Reflexes

32. Refluxate

33. Refluxate Protection

34. Genetic Abnormalities Nutritional Compromise of the Immune System Stress and Immune Response Recurrent Infection and Frequent Use of Antibiotics: The Impacts Allergies Reactive Airway Disease (RAD) Airway Clearance Therapy The Immune System

35. Respiratory Medical History • Number of Pulmonary Infections Annually • Number of Hospital Admissions Annually • Number of ER Admissions Annually • Number of Courses of Antibiotics for Respiratory Infections Annually • Immunization History • History of Recurrent Infections with Respiratory Syncytial Virus (RSV)

36. Quality Airway Clearance Therapy Should • Clear secretions effectively and consistently • Preserve lung function • Reduce infectious exacerbations • Reduce dependence on antibiotic therapy and other medications • Reduce need for hospitalization and auxiliary medical services • Delay disease progression • Reduce the burden of care • Enhance the quality of life

37. Neuromuscular Disease - Perioperative Care

38. High Risk For Post-Operative Complications • Atelectasis • Pneumonia • Respiratory Failure • Need for prolonged ventilation • Tracheostomy • Death

39. Problems • Weak cough • Dyscoordinated swallow • Aspiration • Difficulty clearing secretions • Increased lower respiratory tract infections

40. Respiratory Weakness • May not be apparent on physical exam • Respiratory failure when work of breathing is increased

41. Chronic Respiratory Muscle Weakness • Reduced lung volumes • Microatelectasis • V/Q mismatch • Scoliosis • Decreased compliance of the chest wall • Decreased pulmonary compliance • Hypoxemia only during sleep • Hypoventilation due to muscle weakness • Hypoventilation due to central hypoventilation

42. Preoperative Assessment • Thorough history • Physical examination • Laboratory studies

43. Thorough History • Frequency and severity of respiratory tract infections • Pulmonary complications of previous surgeries • History suggestive of reactive airways disease • Even mildly increased airway obstruction may lead to respiratory failure in the postoperative period in a patient with severe respiratory muscle weakness

44. Physical Examination • Gag reflex • Cough • Adequacy of aeration • Presence of adventitial lung sounds

45. Ability To Cooperate With Post-Operative Pulmonary Therapy • General muscle strength • Physical and intellectual capacity

46. Laboratory Examinations • Chest x-ray • Arterial blood gases or mixed venous gas measurements and oximetry • Complete blood count

47. Pulmonary Function Tests • All children who are capable of performing them • Lung volumes • Pre and post bronchodilator • Maximal inspiratory and expiratory mouth pressures • frequently decreased more than lung volumes and flows • do not correlate with general muscle strength

48. Alternative/Competitive Airway Clearance Modalities

49. Chest Physiotherapy • Mechanical techniques for the noninvasive clearance of excessive secretions and aspirated materials from the airway

50. Prevent, Treat, or At Least Delay The Effects of Mechanical,Infectious, and Biochemical Sequelae • prevent resistance to airflow • work of breathing • hyperinflation • atelectasis • maldistribution of ventilation • ventilation-perfusion mismatch