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Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
Neuromuscular Disease
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Neuromuscular Disease

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Presentation of Dr. Dean Hess at 10th Pulmonary Medicine Update Course, Cairo, Egypt. Pulmonary Medicine Update Course is organized by Scribe : www.scribeofegypt.com …

Presentation of Dr. Dean Hess at 10th Pulmonary Medicine Update Course, Cairo, Egypt. Pulmonary Medicine Update Course is organized by Scribe : www.scribeofegypt.com

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  • 1. Mechanical Ventilation of the Patient with Neuromuscular Disease Dean Hess PhD RRT Associate Professor of Anesthesia, Harvard Medical School Assistant Director of Respiratory Care, Massachusetts General Hospital Editor in Chief, Respiratory Care
  • 2. Neuromuscular Diseases Causing Respiratory Failure <ul><li>Cerebral cortex : stroke, tumor </li></ul><ul><li>Brainstem : drugs, hemorrhage, anoxia, polio, multiple sclerosis, primary hypoventilation </li></ul><ul><li>Spinal cord : trauma, tumor, tetanus </li></ul><ul><li>Motor nerves : motor neuron disease (ALS, SMA), Guillain-Barré, critical illness neuropathy </li></ul><ul><li>Neuromuscular junction : drugs, myasthenia gravis, toxins (botulism, snake bite) </li></ul><ul><li>Myopathies : muscular dystrophy, myotonic dystrophy, Pompe disease </li></ul>
  • 3. <ul><li>Guillain-Barre (reversible) and myasthenia gravis (treatable) are most common </li></ul><ul><li>Inspiratory/expiratory muscle weakness, upper-airway dysfunction </li></ul><ul><li>Bedside PFTs can predict need for mechanical ventilation: </li></ul><ul><ul><li>Vital capacity <15 mL/kg or < 1 L </li></ul></ul><ul><ul><li>Maximal inspiratory pressure > -30 cm H 2 O </li></ul></ul><ul><ul><li>Maximal expiratory pressure < 40 cm H 2 O </li></ul></ul><ul><li>Insufficient evidence to recommend NIV in patients with Guillain-Barré syndrome or myasthenia gravis </li></ul>Primary NMD Causing Acute Respiratory Failure Mehta, Respir Care 2006; 51:1016
  • 4. <ul><li>Polyneuropathy and myopathy (coexist, myopathy most common); occurs in 25% of ventilated patients </li></ul><ul><li>Risk factors: sepsis, corticosteroids, hyperglycemia, neuromuscular blockade, severity of illness </li></ul><ul><li>Associated with adverse outcomes: mortality, longer time on ventilator, increased length of stay </li></ul><ul><li>Intensive insulin therapy reduces the incidence of ICU-acquired weakness </li></ul><ul><li>Ventilator-induced diaphragmatic dysfunction (VIDD) (Levine, N Engl J Med 2008;358:1327 ; Jubran, Respir Care 2006) </li></ul>ICU-Acquired Muscle Weakness
  • 5. Mobility in the ICU Needham, JAMA 2008 Bailey, Crit Care Med 2007; 35:139 Morris, Crit Care Med 2008; 36:2238 Schweikert, Lancet 2009; 373: 1874 Burtin Crit Care Med 2009; 37:2499
  • 6. Neuromuscular Respiratory Failure Benditt, Respir Care 2006; 51:829
  • 7. Respiratory Muscle Training <ul><li>Training can increase respiratory muscle strength and endurance Leith and Bradley, J Appl Physiol 1976; 41:508; Lotters, Eur Respir J 2002;20:570 </li></ul><ul><li>Training occurs slowly – weeks? (unlikely the result of a few SBTs) </li></ul>
  • 8. Cough Assist Techniques <ul><li>Evaluation of Cough </li></ul><ul><ul><li>Cough flow < 160 L/min: initiate cough assist </li></ul></ul><ul><ul><li>Cough flow < 270 L/min: risk for secretion retention </li></ul></ul><ul><ul><li>Low cough flow associated with extubation failure (Salam et al, Intensive Care Med 2004) </li></ul></ul><ul><li>Cough Assist Techniques </li></ul><ul><ul><li>Hyperinflation </li></ul></ul><ul><ul><li>Manually assisted cough </li></ul></ul><ul><ul><li>Mechanical In-Exsufflator (Cough Assist) </li></ul></ul>
  • 9. Noninvasive Ventilation
  • 10. NIV and ALS patients with normal or moderately impaired bulbar function patients with severe bulbar impairment Bourke, Lancet Neurol 2006;5:140
  • 11. NIV and ALS: Quality of Life patients with normal or moderately impaired bulbar function patients with severe bulbar impairment Bourke, Lancet Neurol 2006;5:140
  • 12. Indications for Chronic NIV <ul><li>Symptoms (fatigue, dyspnea, morning headache, orthopnea) and one of the following: </li></ul><ul><ul><li>PaCO 2 ≥ 45 mm Hg </li></ul></ul><ul><ul><li>Nocturnal desaturation ≤ 88% for 5 consecutive min </li></ul></ul><ul><ul><li>For progressive neuromuscular disease, maximal inspiratory pressures > -60 cm H 2 O or FVC < 50% predicted </li></ul></ul>Chest 1999; 116: 521–534
  • 13.  
  • 14. Respir Care 2006; 51:1005
  • 15. How to Choose Settings for NIV <ul><li>Empiric </li></ul><ul><ul><li>Short-term symptoms: comfort, accessory muscle use </li></ul></ul><ul><ul><li>Long-term symptoms: less morning headache, fatigue, and daytime sleepiness </li></ul></ul><ul><li>Physiologic: tidal volume, gas exchange, esophageal/gastric pressure measurements </li></ul><ul><li>Polysomnography </li></ul><ul><ul><li>Long wait time </li></ul></ul><ul><ul><li>Sleep labs less familiar with NMD than OSA </li></ul></ul><ul><li>Overnight oximetry </li></ul><ul><ul><li>Does not assess sleep quality </li></ul></ul>
  • 16. NIV Settings for NMD <ul><li>Back-up rate (periodic breathing) </li></ul><ul><li>Trigger, cycle, rise time per patient comfort </li></ul><ul><li>EPAP: 3 – 4 cm H 2 O (low as possible unless OSA) </li></ul><ul><li>IPAP: 8 – 15 cm H 2 O as tolerated; may need higher settings with acute illness </li></ul><ul><li>Ramp off </li></ul><ul><li>Unclear role for newer modes like AVAPS </li></ul>
  • 17. NIV Settings for NMD <ul><li>FIO 2 : room air unless acute illness </li></ul><ul><li>Humidity: routine </li></ul><ul><li>Inhaled bronchodilators and steroids not necessary </li></ul><ul><li>Nasal symptoms: humidity, OTC remedies, nasal steroids and anticholinergics </li></ul>
  • 18. Approaches to Intolerance: Chronic Use <ul><li>Start with low settings </li></ul><ul><li>Practice wearing mask without pressure </li></ul><ul><li>Short periods with distraction (watching TV) </li></ul><ul><li>Use during naps </li></ul><ul><li>Short times at night, gradually increasing </li></ul><ul><li>Personal motivation, family support </li></ul><ul><li>Knowledge of the evidence (using NIV is life prolonging) </li></ul>
  • 19. Indications for Tracheostomy <ul><li>Patient preference </li></ul><ul><li>Inability to tolerate NIV or failing NIV </li></ul><ul><li>Bulbar involvement: aspiration, pneumonia </li></ul><ul><li>Inadequate cough despite cough assist </li></ul><ul><li>Consider need for resources to manage trach and ventilator </li></ul>
  • 20. Ventilator Settings for Trach Patient <ul><li>Volume control ventilation (avoid pressure support and pressure control) </li></ul><ul><li>Rate and tidal volume per comfort, gas exchange, and safety </li></ul><ul><li>PEEP 5 cm H 2 O; room air </li></ul><ul><li>Cuff deflation for leak speech; adjust PEEP, tidal volume, inspiratory time to improve speech </li></ul>
  • 21. Ventilator Settings for Trach Patient <ul><li>Choose ventilator for size (fits on wheelchair) and battery life </li></ul><ul><li>Transition to homecare ventilator in acute care setting </li></ul><ul><li>Do not wean patient with progressive disease (e.g., ALS) </li></ul><ul><li>Use lung protective ventilation strategies if patient develops acute lung injury (Crit Care Med 2007; 35:1815) </li></ul>
  • 22. Effect of PaCO 2 on CBF 20 40 60 P aCO 2 (mm Hg)
  • 23. Effect of PO 2 on CBF P aO 2 (mm Hg) 0 60 120 100 50
  • 24. Effect of PCO 2 and PCO 2 on CBF
  • 25. Hyperventilation in Acute Brain Injury <ul><li> PaCO 2   CBF   ICP </li></ul><ul><li>Transiently effective (minutes to hours) </li></ul><ul><li>Prophylactic hyperventilation harmful (J Neurosurg 1991) </li></ul><ul><li>Brain Trauma Foundation Guidelines (J Neurotrauma, 2007) </li></ul><ul><ul><li>Prophylactic hyperventilation is not recommended </li></ul></ul><ul><ul><li>Use as a temporizing measure for increased ICP </li></ul></ul><ul><ul><li>Should not be used in the first 24 hrs after head injury </li></ul></ul><ul><ul><li>If used, cerebral oxygenation should be monitored </li></ul></ul>
  • 26. Monitoring Brain Oxygenation <ul><li>Jugular venous oximetry (SjO 2 ): measured in jugular bulb </li></ul><ul><ul><li>Reflects global oxygen extraction by brain </li></ul></ul><ul><ul><li>Normal 60-70% </li></ul></ul><ul><ul><li>< 55% = ischemia </li></ul></ul><ul><li>Brain tissue PO 2 (PbrO 2 ): measured by probe placed directly into brain </li></ul><ul><ul><li>Local PO 2 measured in an area extending 1.4 cm from probe tip </li></ul></ul><ul><ul><li>Normal: 25 - 30 mm Hg </li></ul></ul><ul><ul><li>< 10 - 15 mm Hg associated with poor outcome </li></ul></ul>
  • 27.  FIO 2 to  PbrO 2 ? <ul><li>What is PbrO 2 measuring? </li></ul><ul><li>Does increasing PbrO 2 matter? </li></ul><ul><li>Does the risk of hyperoxia outweigh the benefit? </li></ul><ul><ul><li>Oxygen toxicity - acute lung injury </li></ul></ul><ul><li>Use should be limited until further studies demonstrate outcome benefit </li></ul>
  • 28. ALI/ARDS in Acute Brain Injury <ul><li>Lung protective ventilation may be associated with  PCO 2 </li></ul><ul><ul><li>Effect on ICP small, transient, and safe (Acta Neurochir (Suppl) 81, 2002) </li></ul></ul><ul><ul><li>Benefit outweighs risk of  ICP </li></ul></ul><ul><li>Effect of PEEP on ICP </li></ul><ul><ul><li>If compliance low, PEEP has no effect (Neurosurgery 1981) </li></ul></ul><ul><ul><li>With recruitment, PEEP has no effect on ICP; with hyperinflation, PEEP increases ICP (Intensive Care Med 2005) </li></ul></ul>
  • 29. Crit Care Med 2007; 35:1815
  • 30. Does Level of consciousness Predict Extubation Success? <ul><li>GCS ≥ 8: 75% successful </li></ul><ul><li>GCS < 8: 33% successful* </li></ul><ul><ul><li>* patients included that ultimately had support withdrawn when extubation failed </li></ul></ul><ul><ul><li>Reintubation rate 16% </li></ul></ul><ul><ul><li>29% of patients underwent tracheotomy </li></ul></ul>Namen et al, ARCCM 2001
  • 31. Does Level of Consciousness Predict Extubation Success? <ul><li>136 patients with acute brain injury </li></ul><ul><ul><li>Patients without reasonable hope of survival after extubation excluded </li></ul></ul><ul><ul><li>Overall extubation success: 82% </li></ul></ul><ul><ul><ul><li>Success not affected by GCS, gag, extubation delay </li></ul></ul></ul><ul><ul><ul><li>Spontaneous cough,  suctioning frequency associated with success </li></ul></ul></ul><ul><ul><li>Extubation delay associated with  LOS, pneumonia </li></ul></ul><ul><ul><li>Only 4% of patients underwent tracheotomy </li></ul></ul>Coplin et al, AJRCCM 2000
  • 32. Crit Care Med 2008; 36:2986
  • 33. Summary <ul><li>A number of acute and chronic neuromuscular diseases have respiratory muscle involvement </li></ul><ul><li>Treatments such a noninvasive ventilation and airway clearance can be life prolonging </li></ul><ul><li>Lung protective ventilation should be used in the patient with neuromuscular disease </li></ul><ul><li>Level of consciousness should not preclude extubation </li></ul><ul><li>Secretions and cough are important factors in determining extubation success </li></ul>

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