1. Ventilator Management in Neuromuscular Diseases
27/08/2018
Ventilator Management in Neuromuscular Diseases
From Non-invasive to Invasive

2. Objectives
Discuss respiratory disorders in neuromuscular diseases (NMD)
Discuss impact of ventilatory assistance on NMD
Review indications and contraindications of non-invasive ventilation (NIV) in NMD
Discuss initiation of NIV in NMD
Review indications of invasive ventilation in NMD

3. Pathophysiology of Ventilatory Failure
Reduced capacity
Weakness of respiratory muscles
Capacity
Drive
Increase load
Atelectasis
UAW resistance due to bulbar weakness
LOAD

4. Evolution of Respiratory Insufficiency in NMD
Normal Breathing
Chest Infections
FVC < 40% pred
REM related SDB
NREM and REM related SDB
FVC < 20% pred
Daytime Hypercania
AK Simmonds Chest 2006
Death

5. Signs and Symptoms Cardiac Signs Cardiopulmonary CNS Constitutional
27/08/2018
Vital
Signs
Constitutional
Cardiopulmonary
CNS
Sleep
Respiratory Signs
Cardiac Signs
Signs
and Symptoms
 Constitutional       Generalized fatigue       Weakness    Cardiopulmonary       Dyspnea Orthopnea       Lower extremity edema Secretion retention    CNS       Early morning headaches Mood disturbances       Daytime hypersomnolence Psychiatric problems    Sleep       Restless sleep Enuresis       Nightmares Frequent arousals Signs    Vital Signs       Tachypnea Tachycardia    Respiratory       Accessory chest and abdomen muscle use       Paradoxical breathing pattern       Diminished excursion    Cardiac       Distended neck veins Increased P2       Edema Cyanosis

6. Signs and Symptoms Cardiopulmonary CNS Constitutional Sleep
27/08/2018
Dyspnea
Orthopnea
Lower extremity edema
Secretion retention
Cardiopulmonary
Dyspnea
Orthopnea
Lower extremity edema
Secretion retention
Early Morning headache mood disturbances daytime hypersomn
Psychiatric problems
Early Morning headache mood disturbances daytime hypersomn
Psychiatric problems
CNS
Weakness
Generalized Fatigue
Weakness
Generalized Fatigue
Constitutional
Signs
and Symptoms
Restless sleep Frequent arousals
nightmares
Sleep
Restless sleep Frequent arousals
nightmares
Tachycardia
Tachypnea
Vital
Signs
Tachycardia
Tachypnea
 Constitutional       Generalized fatigue       Weakness    Cardiopulmonary       Dyspnea Orthopnea       Lower extremity edema Secretion retention    CNS       Early morning headaches Mood disturbances       Daytime hypersomnolence Psychiatric problems    Sleep       Restless sleep Enuresis       Nightmares Frequent arousals Signs    Vital Signs       Tachypnea Tachycardia    Respiratory       Accessory chest and abdomen muscle use       Paradoxical breathing pattern       Diminished excursion    Cardiac       Distended neck veins Increased P2       Edema Cyanosis
Distended neck veins
Edema
Cyanosis
Accessory muscle use
Paradoxical breathing pattern
Diminished excursion
Respiratory Signs
Distended neck veins
Edema
Cyanosis

7. Average age of DMD survivors from 19 to 25
27/08/2018
Average age of DMD survivors from 19 to 25
85%
73%
Plateau effects over the first 5 – 6 years of ventilation tx
Simmonds at al. 1998

8. Survival of ALS patients without Bulbar Involvement
27/08/2018
Survival of ALS patients without Bulbar Involvement
Broken Line: Initiation of NIV therapy before protocol initiation
Solid Line: Initiation of NIV therapy post protocol initiation
Protocol=early respiratory evaluation
Survival times with NIV therapy in patients without
bulbar impairment according to the initiation of NIV therapy
before protocol initiation (broken line) or after protocol initiation
(solid line).
Ferrero et al. 2005

9. Survival of hypercapnic ALS patients with bulbar impairment
Broken Line: Intolerance of NIV
Solid Line: Tolerance of NIV
Ferrero et al. 2005

10. How can NIV possibly help NMD patients?
Prevent respiratory decompensation
Alter chest wall/lung growth characteristics –
NIV hyperinflates, stretches lung
Rest respiratory muscles
Control nocturnal hypoventilation with or without symptoms
Treat chronic hypercapnic respiratory failure
Palliate symptoms
Improved quality of sleep
Enhanced sleep efficiency
More stage 3 and stage 4 sleep

11. Indications of NIV in NMD patients
Signs and Symptoms of respiratory insufficiency
AND
Daytime hypercapnia PCO2 > 45
Nocturnal hypoventilation (SpO2 < 88%, for > 5minutes)
FVC<50% predicted
MIP<60% predicted
Repeated hospitalizations from chest infections
Cor pulmonale
Exceptions
Significant daytime hypercapnia of PCO2 > 50, do not need symptoms as symptoms might not be present

12. ALS Practice Guidelines: Respiratory management

13. 44 patients with FVC > 75%
12 patients with FVC > 75%, not on NIV

14. Patients with FVC < 75% on NIV
27/08/2018
Patients with FVC < 75% on NIV
FVC
Rate of decline in FVC slower in patients with NIV with significant differeces
Patients with FVC < 75% without NIV
Months

15. When to consider NIV is not suitable?
Relative contraindications
Severe swallowing impairment
Need for full time ventilatory assistance, unless patient prefers NIV
Absolute contraindications
Upper airway obstruction
Uncontrolled secretion retention
Inability to cooperate
Inability to cough effectively even with assistance
Inability to fit mask

16. NIV Equipment Interface Nasal mask Full face mask Nasal Pillows
Anti re-breathing valves
Interface
Nasal mask
Full face mask
Nasal Pillows
Mouth piece
Quick release: minimize risk of asphyxiation and aspiration when vomitting
Note:Smart start/stop is disabled on FFM

17. Home NIV vs Home IV users
Trach
FFMV
nasal

18. Mask Fitting Use mask template to optimize fitting
The Mask-Fitting Feature allows the patient to check the quality of their mask fit and adjust if necessary in order to minimize leak
Air pressure is delivered for a period of 3 minutes
In CPAP mode:
Pressure = set treatment pressure or 10 cm H2O, whichever is greater
In S, ST, & T modes:
Pressure = set EPAP pressure or 10 cm H2O, whichever is greater

19. Mouthpiece Ventilation
Beneficial for patients with excessive mouth leak with nasal masks
24/7 ventilatory assistance with little or no vital capacity

20. Ventilators Bilevel devices Light Portable Simple to operate Quiet
Low cost
Ideal for non dependent patients/nocturnal use
Variable tidal volume
Simple to use
Leak compensation

21. Multi mode ventilators
Wide range of Alarms
Internal battery
Invasive and noninvasive options available
Volume targeted modes allow patient to breathe stack
ideal for patients with very poor cough
Ideal for ventilator dependent users

22. Pressure limited
Volume limited

23. Eurovent Study 2002

24. Initiating Home NIV: Step 1
Set goals with patients
Individualize home care plans based on goals
Settings of initiation:
Hospital
Sleep lab for night titration
Physician’s Office/Clinic
Home

25. Possible Goals of Home NIV
Alleviate symptoms
Stabilize and/or improve gas exchange
Prevent nocturnal hypoventilation
Improve sleep quality
Enhance quality of life
Reduce complications of respiratory failure (polycythemia, cor pulmonale)
Provide comfort and convenience
Simplify care with optimal portability
Avoid invasive ventilation/tracheostomy
Minimize cost of long-term care and burden on caregivers
Prolong survival

27. Home Ventilation Initiation
ADAPTATION PERIOD
Weekly visit for few weeks
Monitoring and Follow ups
Monthly visits
STABLE
4 to 6 monthly follow ups

28. NIV Titration: for NMD with no Respiratory Distress
Begin with low settings IPAP 8 EPAP 4 or TV 6 ml/kg, RR 12 – 16 bpm
Adaptation period: weekly visit for first few weeks
Desensitization
Gradually increase settings to achieve 8 ml/kg if tolerable
Good Chest expansion
No accessory muscle use
Over days to weeks of titration
Settings increased as tolerated:
Improve ABG
Improve symptoms such as daytime sleepiness, morning headache, fatigue
Priority: Comfort

29. Monitoring
Consider overnight titration monitoring SpO2 and ETCO2 for FVC < 30% predicted
Monitor daytime ABG after more than 4 hours of use per day
Consider PSG under the following circumstances:
Pressure titration when initiating NPPV
To evaluate for obstructive sleep apnea    
Obese, snorer, or hypersomnolence
To seek sleep hypoventilation in   
 Symptomatic patient without daytime hypercapnia or severe pulmonary function defect    
Asymptomatic patient with daytime hypercapnia or severe pulmonary function defect*

30. <270L/min = insufficient to remove secretions

31. Use of Supplemental Oxygen - Caution
“ The use of supplemental O2 (oxygen) should never be prescribed to the DMD patient to treat low SaO2 levels without testing to determine if the patient suffers from hypoventilation. End-tidal CO2 (carbon dioxide) levels or blood gas CO2 should be checked to determine if the patient is retaining CO2. Improper use of supplemental oxygen can mask hypoventilation, decrease breathing rate, and cause breathing to stop!”
“Monitoring the SaO2 level while having the DMD patient take several deep breaths can often act as a quick check for hypoventilation. If the SaO2 level rises above 95%, hypoventilation due to weak chest muscles is a likely cause. (Bach JR, Quest 1995; 2:1)”

32. Selection of Settings for NPPV
Backup rate 12 to 16 bpm, ventilatory assistance for central apneas and ineffective triggers
Over months of titration and adaptation – increase IPAP to achieve
TV 10 – 15 ml/kg, IPAP may be 12 – 18 cmH2O
Daytime PCO2 improvement: 40s to 50s
Improve morning headaches
Fatigue
Daytime sleepiness
Adjust EPAP 3 – 5 cmH2O, priority: tolerable
Higher EPAP is necessary in OSA and COPD

33. What if PCO2 is high or PO2 is low during a follow up session?
Increase Backup rate
Increase IPAP
Increase use of NIV
Increase naps
Assess for presence of air leaks in the night
Assess for presence of apneas in the night, may need to increase EPAP

34. When to Consider Tracheostomy?
Substantial Glottic Dysfunction
Increased risk of aspiration
Inability to clear secretions despite cough augmentation
Inability to generate adequate cough flow despite aggressive cough assistance
Recurrent pneumonia on full time NIVV with adequate cough assist
Substantial elevate PaCO2, despite optimal full time NIV
Patient preference
Benditt . Respiratory Care 2006

35. Thank You for your Attention!