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Respiratory Care in Neuromuscular Disease

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Respiratory Care in Neuromuscular Disease Cori Daines, MD Pediatric Pulmonary Medicine University of Arizona Neuromuscular Disease Duchenne s muscular dystrophy ... – PowerPoint PPT presentation

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Title: Respiratory Care in Neuromuscular Disease


1
Respiratory Care in Neuromuscular Disease
  • Cori Daines, MD
  • Pediatric Pulmonary MedicineUniversity of Arizona

2
Neuromuscular Disease
  • Duchennes muscular dystrophy
  • Beckers muscular dystrophy
  • Limb-Girdle muscular dystrophy
  • Spinal muscular atrophy
  • Myotonic dystrophy

3
Neuromuscular Disease
  • Genetically inherited
  • Muscle weakness
  • Extremities
  • Trunk/spine
  • Respiratory
  • Swallowing
  • Cardiac

4
  • Dr. John Bach

5
Neuromuscular disease
  • Controller failure
  • Chest wall compromise
  • Muscle weakness
  • Hypotonia
  • Hypertonia

6
Respiratory Control
  • Maintain homeostasis
  • Oxygen
  • Carbon dioxide
  • Hydrogen ion concentration (pH)
  • Optimize mechanical efficiency
  • Complex functions
  • Vocalization
  • Cough
  • Exercise
  • Adaptation to disease

7
Respiratory Muscles
  • Diaphragm
  • Intercostals
  • Accessory muscles (shoulder girdle)
  • Pharynx
  • Larynx
  • Abdominal wall
  • Perineum

8
Physiologic impact
  • Ventilatory impairment
  • Oxygenation impairment (A-a)DO2 ?
  • Sleep disordered breathing
  • Maintenance of lung volume
  • Growth of the lung in children
  • Lung clearance impairment
  • Lung inflammation from aspiration
  • Nocturnal vs diurnal dysfunction varies

9
Prevention
  • Assuming that there is no primary lung disease
    most NMD patients can have normal lungs
  • An ounce of prevention is worth a pound of cure
  • 4 Es Expansion, Evacuation, Evasion and
    Evaluation
  • i.e. expand the lungs, clear the airways, and
    avoid aspiration and infection
  • Evaluate how the patient progresses acutely and
    over the long term

10
Minimize work of breathing
  • Normally 15 of energy WOB
  • In NMD this can be exceeded
  • Decreased use of energy in movement
  • Increased work of breathing due to inefficient
    system and/or stiff/obstructed lungs
  • Increased WOB will lead to chronic hypercapnea
    and compensatory alkalosis

11
Expansion
  • Weakness leads to
  • Poor inspiration
  • Atelectasis and decreased compliance due to fluid
    accumulation and microatelectasis
  • Chest wall/Shoulder girdle contracture
  • Kyphoscoliosis (except DMD)
  • Loss of MIP correlates with loss of lung volume
    and MIP lt 30 are predictive of increases in CO2

12
Duchenne MD FVC vs Age
Bach et al. Arch Phys Med Rehabil 1981 62328
13
Expansion
  • Reduced TLC, VC, FRC, ERV
  • Balance between chest wall and diaphragm
  • Affects optimal position (Upright better with
    weak diaphragm)
  • Rate of loss of function affects degree of
    breathing intolerance Rapid is worse

14
Evacuation
  • Optimal humidity and warmth to maintain ciliary
    function
  • Cough
  • Sense
  • Close glottis
  • Pressurize pulmonary gas by tensing abdomen and
    perineum (200 cm H2O)
  • Explosively open airway
  • Continue cough to lower lung volume
  • Cough peak flow transients are 6 to 12 liters per
    second i.e. 360 to 720 L/min

15
Pulmonary clearance failure
  • Disrupted cilia due to drying and inflammation
  • Low tidal volume (lt 20 ml/kg)
  • Poor glottic closure
  • Poor abdominal compression
  • CPF lt 2.7 L/sec 160L/min predict failure of
    extubation in adults (i.e. lt 2 L/min/kg)
  • Poor coordination
  • Inablility to continue to low lung volume

16
Evacuation
  • Chest physiotherapy
  • Stacking with voluntary cough
  • Stacking with augmented cough
  • Mechanical insufflation-exsufflationTracheostomy

17
EVASION Avoid pulmonary damage
  • Growth failure
  • Poor expansion
  • Poor nutrition
  • Aspiration
  • Foreign body (tracheostomy)
  • Poor clearance with inflammatory processes

18
Evasion Aspiration
  • A tension exists between natural, pleasure giving
    aspects of feeding and danger of aspiration and
    inadequate nutrition
  • Often this leads to an illogical approach i.e.
    pt has to fail oral feeds to go to alternative as
    opposed to succeed with oral feedings to move off
    of supportive modalities (NG,GT etc)
  • Progresses early in SMA/Brainstem dysfunction and
    later in DMD
  • Oral hygiene important even if NPO

19
Evaluation
  • History and physical including QOL and sleep
    questionnaire
  • Chest film
  • Lung volumes
  • MIP/MEP
  • Sniff MIP
  • Inspiratory flow reserve
  • Maximum insufflation capacity
  • Cough peak flows

20
Patient status changes with viral respiratory
infections
  • Increased secretions
  • Decreased muscle strength
  • Surfactant dysfunction in LRI
  • Transient increase in need for support
  • We commonly evaluate patients when they have
    recovered from an illness or are stable

21
Dr. Bach Outpatient Protocol
  • Patients at risk
  • During chest colds w/ assisted PCF below 270 LPM
  • Patients prescribed
  • Oximeter and MIE device
  • Patients trained in
  • Air stacking insufflated volumes via mouth and
    nasal interfaces
  • Manually assisted coughing
  • Mechanical in-exsufflation at 35 to 50 to
    -35 to -50 cm H2O

22
Outpatient Protocol
  • Patients given 1-hour access to
  • Portable volume ventilator
  • Cough Assist MIE (J. H. Emerson Co., Cambridge,
    MA)
  • Various mouthpieces and nasal interfaces
  • Patients and care providers are instructed
  • SaO2 lt95 indicates hypoventilation or airway
    mucus accumulation that must be cleared to
    prevent atelectasis and pneumonia
  • Use SaO2 monitoring whenever fatigued, short of
    breath, or ill
  • Use noninvasive IPPV and manually and
    mechanically assisted coughing as needed to
    maintain normal SaO2 at all times

23
Outpatient Protocol
  • Patients with elevated EtCO2 or daytime SaO2 lt95
  • Undergo nocturnal SaO2 monitoring
  • When symptomatic or nocturnal SaO2 mean lt94
  • A trial of nocturnal nasal IPPV is provided
  • People continue to use nocturnal nasal IPPV when
    they felt less fatigue and nocturnal mean SaO2
    increases.
  • Most young patients use noninvasive IPPV for the
    first time to assist lung ventilation during
    chest infections.

24
Respiratory muscle aids Indications
  • Failure to maintain a healthy lung with growth
    and optimal ventilatory function
  • i.e. failing the 3 Es
  • Prevention is key
  • Optimize support in relation to the needs of the
    patient

25
Using what the patient has
  • Daytime spontaneous respiration with nocturnal
    support for control, airway obstruction,
    recruitment of lung volume
  • Glossopharyngeal breathing during the daytime
    with nocturnal ventilation
  • Optimizing cough and lung volume with stacking
    maneuvers

26
Glossopharyngeal breathing
27
Maximal insufflation capacity
  • Breath stacking
  • Measured unassisted with spontaneous breathes or
    GPB breaths
  • Commonly 1.5x the VC
  • Can be augmented with interface and manual
    resuscitator bag
  • Maintain lung volume and compliance and chest
    wall compliance

28
Inspiratory muscle aids
  • Rocking bed and abdominal belt
  • Disadvantage is no expansion of lung i.e. frc to
    less than frc
  • Negative pressure ventilators
  • Disadvantages are OSAS and aspiration
  • Non-invasive IPPV
  • Tracheostomy and IPPV

29
Nocturnal support
  • Used prior to need for 24/7 support
  • Improves daytime PaO2, PaCO2
  • Reduces respiratory muscle work at night and
    rests the muscles
  • Reverses cor pulmonale perhaps in addition to O2
    by improving lung volume

30
Nocturnal support
  • Increases MIP and lung volume
  • Improves compliance and FRC during the daytime
  • Can be used even in patients with severe
    breathing intolerance
  • CCHS or Quadraparesis with daytime diaphragmatic
    pacing
  • GPB during daytime
  • Can be transitioned to 24/7 with illnesses

31
NIPPV Interfaces
  • Full face mask
  • Nasal mask
  • Custom mask
  • Mouthpiece / Lipseal
  • Leakage and dental issues
  • Sipper mouthpiece

32
NIPPV Nasal mask / Prongs
  • 2-3 x preferred compared to mouthpiece
  • Problems
  • Leak, esp mouth
  • Nasal bridge pressure with mask
  • Gum erosion or compression with mask
  • Nasal erosion with prongs
  • Chin strap may be needed

33
NIPPV Full face mask
  • Decreased leak
  • Decreased
  • Cough
  • Talking
  • Eating
  • Nocturnal use with daytime nasal mask

34
NIPPV Sipper / Mouthpiece
  • Daytime use
  • Allows facial freedom
  • Flexed mouthpiece /- custom orthodontics
  • Intermittently used to augment breathing
  • Continuously used

35
NIPPV Sipper / Mouthpiece
  • Large VT set on ventilator or High insp flow if
    pressure controlled
  • Allows stacking maneuvers
  • Head/neck control for intermittent use
  • Use of flexed mouthpiece with a back pressure of
    2-3 cmH2O can reduce low pressure/disconnect
    alarms

36
Complications of NIPPV
  • Facial and orthodontic changes
  • Aerophagia (PIP gt 25 cmH2O)
  • Nasal drying/congestion humidify
  • Volutrauma - air leak

37
Tracheostomy
  • Controversial
  • Current view in rehab circles is that with proper
    care a tracheostomy is never needed
  • Our experience is that tracheostomy may have a
    role
  • Patient preference
  • Upper airway dysfunction
  • Severe central airway obstruction by secretions

38
Ventilators
39
Ventilators
  • Pressure cycled vs Volume cycled
  • Pressure cycled are often triggered by flow
    sensing reducing work of breathing
  • Flow sensing is also important in pts with high
    respiratory rates infants/toddlers

40
Ventilators
  • Leak can vary with sleep, position, and effort
    which is problematic with volume cycled
    ventilators
  • Variable airway resistance and/or pulmonary or
    chest wall compliance better with volume settings
  • Pressure cycling limits ability to stack

41
Ventilator triggering and rate
  • Small/weak or brainstem/CNS pts may not trigger
    well
  • Spontaneous-timed modes are useful with a backup
    rate higher than spontaneous when initiating
    ventilation in infants/young children
  • Back-up rates lower than spontaneous once
    comfortable

42
Minute ventilation strategy
  • High Vt/Low RR vs Low Vt/High RR
  • High Vt High IPAP-EPAP span
  • Maintains lung volume and compliance especially
    when asleep or sick
  • Maintains Vd/Vt ratio if patient status changes

43
Ventilation goals
  • Healthy lungs with good volumes and no
    atelectasis
  • Rate on the low side and Vt or PIP on the high
    side
  • PaCO2 35 5 mmHg
  • Room air
  • Patient comfort
  • Ability to trigger vent
  • Ability to deliver needed volume/flow in time
  • No auto-PEEP
  • No auto-cycling / Ventilator-Patient dysynchrony
  • Primary lung disease may change this approach

44
BiPAP settings
  • S/T mode / High span IPAP/EPAP
  • If OSAS is main issue low span is appropriate
  • IPAP range 15-18
  • May need higher with high UA resistance,
    non-compliant lungs, obesity/non-compliant chest
    wall
  • May need to be lower with high spont rates
  • EPAP range 2-4
  • Depending upon circuit may need 4 cmH2O to avoid
    rebreathing
  • High EPAP is rarely needed

45
Other issues
  • Inspiratory time
  • IE of 12
  • Ti of 0.5 min (infant) and 1.0 (gtinfant)
  • Insp flow rate necessary to achieve pressure
    comfortably
  • Trigger sensitivity set to reduce WOB, but not
    autocycle
  • Pressure support may improve comfort with
    spontaneous breaths
  • Ultimately creates an S/T mode depending upon
    settings

46
LTV system - Pulmonetics
47
LTV system - Pulmonetics
48
LTV system
49
LTV Features
50
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51
Control mode ventilation
Limited respiratory control / Inability to
trigger breaths
52
Assist Control Mode
Can trigger breaths, but needs support with each
breath
53
SIMV Mode
Most patients, improved comfort, stable CO2s
54
Bilevel Mode
Mimic BiPAP / No Backup Rate
55
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56
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57
Rise Time
  • Pressure control
  • Pressure support
  • Flow in volume control is set by Ti and Vt

58
Rise Time
  • Slow rise time
  • Small ET
  • Bronchospasm / AOD
  • Pressure overshoot on PIP
  • Fast rise time
  • Short Ti / High respiratory rate
  • Vary with age i.e. larger VT faster rise time

59
Home ventilation reality
  • Every patient is unique
  • These are more guidelines rather than rules
  • Vary settings, interfaces, strategies to achieve
    goals of good health and optimized quality of
    life

60
Discharge home Medical Issues
  • Presence of a stable airway
  • FiO2 less than 40
  • PCO2 safely maintained
  • Nutritional intake optimal
  • Other medical conditions well controlled
  • Above may vary if palliative care

Jardine E, Wallis C. Thorax 1998 53762
61
Discharge Home Support
  • Goals and plans clarified with family and
    caregivers
  • Family and respite caregivers trained in the 4
    Es and all equipment
  • Nursing support arranged for nighttime
  • Equipment lists developed and implemented with
    re-supply and funding addressed
  • Funding and insurance issues addressed
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