Problem-solving Respiratory Issues in Children With Neuromuscular Disease. December 13, 2018 Eliezer Be eri, M.D.
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1 Problem-solving Respiratory Issues in Children With Neuromuscular Disease December 13, 2018 Eliezer Be eri, M.D.
2 About Our Presenter Eliezer Be eri, M.D. Alyn Rehabilitation Hospital Jerusalem, Israel
3 Objectives - Appreciate the major psychosocial issues that can undermine successful rehabilitation of a child on mechanical ventilation, if not identified and addressed at the outset of the rehab program, - Identify the pathophysiology of neuromuscular respiratory failure and how that influences strategies for ongoing mechanical ventilation and for mechanical ventilation weaning, - Discuss the indications, contraindications, benefits, and drawbacks of noninvasive ventilation versus tracheostomy ventilation, - Learn strategies to optimize speech in the ventilated patient, and - Understand strategies to diagnose and manage respiratory problems in the child with Cerebral Palsy.
4 n Pediatric Rehabilitation Hospita Jerusalem, Israel In-patient rehabilitation departments (50 beds), including a 19-bed respiratory rehab ward Out-patient rehabilitation clinics (40,000 visits/yr) School for children with chronic medical problems
5 Psychosocial Aspects of Respiratory Rehabilitation
6 Psychosocial aspects of Respiratory Rehabilitation The biggest barrier to discharge home and participation in society of ventilated children: Parental Psychological Paralysis
7 Psychosocial aspects of Respiratory Rehabilitation Enabling Participation A picnic at the zoo Hydrotherapy
8 Psychosocial aspects of Respiratory Rehabilitation Enabling Participation Explain that the brain is more important than the lungs
9 Pathophysiology of Neuromuscular Respiratory Failure
10 Pathophysiology of Neuromuscular Respiratory Failure INSPIRATORY and EXPIRATORY muscles work differently Diaphragm: Inspiration only Innervation: C 3,4 and 5 (keeps the diaphragm alive) Efficiency is dependent on: 1) stable rib cage (strong intercostals) otherwise get paradoxical breathing
11 Pathophysiology of Neuromuscular Respiratory Failure INSPIRATORY and EXPIRATORY muscles work differently Diaphragm: Inspiration only Innervation: C 3,4 and 5 (keeps the diaphragm alive) Efficiency is dependent on: 1) stable rib cage (strong intercostals) otherwise get paradoxical breathing 2) Optimal starting position (determined by abdominal pressure)
12 Pathophysiology of Neuromuscular Respiratory Failure INSPIRATORY and EXPIRATORY muscles work differently C 5 L 1 T 6 Abdominal Muscles: Expiration (coughing) Maintains normal intra-abdominal pressure keeps diaphragm in optimal starting position Innervation: T6 L1
13 Pathophysiology of Neuromuscular Respiratory Failure Pathophysiology of NM Respiratory Failure ABDOMINAL PARALYSIS DIAPHRAGM PARALYSIS INTERCOSTAL PARALYSIS ABDOMINAL PARALYSIS Inability to cough Inefficient diaphragm function Inadequate tidal volume Secretion retention Atelectasis Pneumonia Respiratory failure
14 Clinical Diagnosis and Treatment of Neuromuscular Respiratory Failure
15 Treatment of Neuromuscular Respiratory Failure The clinical presentations of INSPIRATORY muscle failure and of EXPIRATORY muscle failure are different Inspiratory Muscle Failure: Pathology: inadequate tidal volume Symptoms: Gradually worsening hypercapnea morning headache decreased intellectual functioning Drowsy ( in oxygen saturation occurs late) Gradual deterioration Expiratory Muscle Failure: Pathology: Ineffective cough Symptoms: Acute pneumonia or atelectasis after trivial respiratory infection (sudden in oxygen saturation) Sudden death
16 Treatment of Neuromuscular Respiratory Failure Treat the neuromuscular cause, not the hypoxia symptom Respiratory muscle weakness secretion clearance Weak cough Retained secretions Atelectasis Shallow breaths ventilation pneumonia supplem give oxygen po 2 pco 2 (<95%)
17 Treatment of Neuromuscular Respiratory Failure Strategies for Improving Ventilation
18 Treatment of Neuromuscular Respiratory Failure The ventilation strategy for treating neuromuscular respiratory failure is different from the strategy for treating parenchymal respiratory failure Parenchymal ventilation Neuromuscular ventilation Small, rapid breaths (to avoid barotrauma) SIMV mode Deep, slower breaths (to prevent atelectasis) Assist Control mode
19 Treatment of Neuromuscular Respiratory Failure Strategy for Weaning Neuromuscular Patients from Ventilation Short periods of intense effort (disconnection from the ventilator), so as to strengthen respiratory muscles
20 Treatment of Neuromuscular Respiratory Failure The Effect of Tidal Volumes on the Time to Wean Persons With High Tetraplegia From Ventilators. Peterson WP et al. Spinal Cord Retrospective review of 42 ventilated high SCI patients (C3/C4) 23 patients treated with low Vt ventilation (<20ml/Kg) Atelectasis: 32% on admission, 52% after 2 weeks Time to wean: 58 days 19 patients treated with high Vt ventilation (>20ml/Kg) Atelectasis: 84% on admission, 16% after 2 weeks Time to wean: 37 days High Vt ventilation resulted in less atelectasis (p=0.01) and quicker weaning (p=0.02)
21 Treatment of Neuromuscular Respiratory Failure Strategies For Improving Secretion Clearance
22 Treatment of Neuromuscular Respiratory Failure Coughing is better than Catheter Suction Catheter suction Cough
23 Treatment of Neuromuscular Respiratory Failure Limitations of Catheter Suction Limited to trachea and mainstem bronchi Catheter suction misses the left lung 92% of the time 70% of pneumonia in SCI occurs in left lung, only 30% in right lung (Fishburn, 1990)
24 Treatment of Neuromuscular Respiratory Failure Mechanical Inexsufflation Simulates a natural cough by alternating positive pressure insufflation with negative pressure exsufflation Can generate effective cough flows (>160 l/min) in paralyzed patients Generates airflow from 5 th generation bronchi, bilaterally May be attached to facemask or ET tube Completely noninvasive
25 Troubleshooting Tracheostomy Problems
26 Troubleshooting Tracheostomy problems Types of Tracheostomy Tubes Cuffed Uncuffed Protection from aspiration Best ventilation Precludes speech Fewer traumatic complications (scarring) Have to manage air-leak Facilitates speech
27 Troubleshooting Tracheostomy problems Speech Valves
28 Speech in the Non-Ventilated Patient With a Tracheostomy
29 Troubleshooting Tracheostomy problems Phonation during natural breathing occurs only during EXPIRATION and with cuff deflated cuff inflated cuff deflated
30 Troubleshooting Tracheostomy problems Speech valve prevents exhalation through the cannula, forcing more leak around cannula no speech valve with speech valve
31 Troubleshooting Tracheostomy problems Speech Valve Benefits Voice production May partially enable valsalva (increases trunk stability and limb strength) More natural swallowing? Contraindications No air leak around cannula (small children) Inflated cuff Upper airway obstruction
32 Troubleshooting Tracheostomy problems To Improve Natural Speech: Use non-cuffed cannula instead of cuffed Try smaller cannula Use speech valve if possible Laryngeal vibrator Try fenestrated cannula
33 Speech in the Ventilated Patient
34 Troubleshooting Tracheostomy problems Phonation during mechanical ventilation occurs loudest Inspiration during INSPIRATION, weakly Expiration during expiration, and only with cuff deflated Inspiratory speech Expiratory speech
35 To improve ventilation speech Hoit et al Am J Speech-Lang Pathol 1997 Hoit el al Chest 2003 Shea et al J Speech Hear Res 1994 Inspiratory speech Expiratory speech
36 Noninvasive Ventilation
37 Noninvasive Ventilation Noninvasive Ventilation Advantages Better speech No risk of sudden death No increased secretions Psychological: gradual, acceptable Cosmetic
38 Noninvasive Ventilation Noninvasive Ventilation Disadvantages Labor intensive Doctors Parents Fussy (lots of possibility for error) Pressure sores Conjunctivitis Dry mouth Abdominal distension Full face mask may prevent speech Deformation of the maxilla The biggest hurdle: lack of a philosophy of noninvasion in acute care facilities.
39 Noninvasive Ventilation Duchenne Muscular Dystrophy 24-hour vent dependent (3 years) Daytime mouthpiece ventilation Nighttime facemask ventilation
40 Noninvasive Ventilation Siblings with Nemaline Myopathy Both fully vent dependent since age 2 Both facemask and tracheostomy are options
41 Noninvasive Ventilation Contraindications to Noninvasive Ventilation Uncooperative or hyperactive child Incompetent or unmotivated parents Airway obstruction: Vocal cord paralysis Poor secretion control
42 Understanding Respiratory Pathology in Cerebral Palsy
43 Pneumonia and its Prevention in CP GE Reflux Cough Reflex Food Aspiration (Gastrostomy) Poor Swallowing Poor Secretion Clearance (Inexsufflation) (Fundoplication) PNEUMONIA (Body Mobilization) Dependent Secretions (Scopoderm) (Atropine Drops) (Salivary Gland Botox) Saliva Aspiration Body Movement Muscle Paralysis Severe Scoliosis Muscle Tone
44 Conclusions Neuromuscular disease has a unique impact on respiratory function, and should be managed with appropriate strategies Effective respiratory rehabilitation requires an integrated, holistic and multi-disciplinary approach
45 Continuing Education Credits IPRC thanks our partners at WeeFIM for sponsoring today s CEUs. To obtain CEUs for today s event, each Occupational Therapist must have attended the full presentation and complete an online evaluation to receive a certificate. An evaluation link and instructions will be ed to all registrants. Please share with all attendees.
46 Surveys & Certificates of Attendance IPRC values your opinion. Following the webinar, a survey link will be ed to all registrants. Please complete and share with all attendees. A certificate of attendance for today s presentation may be requested via the survey. surveymonkey.com/r/iprcwebinarsurvey
47 Thank you! Questions? I welcome any comments and questions: Eliezer Be eri, MD ebeeri@alyn.org
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