1. Session 4: Living with and managing nocturnal hypoventilation in MND

2. Living with and managing nocturnal hypoventilation in MND
Ventilation has to be supported…
or less commonly
The airway supported
How do we do it?

3. It used be with one of these…
Or one of these..

4. Then it was with one of these…

5. And now with one of these

6. What is NIV?
Non-invasive ventilation (NIV) refers to the provision of ventilatory support through the upper airway using a mask or similar device. Initially and most frequently at night

7. Two types of NIV
CPAP: Continuous positive airway pressure splints upper airway and recruits unused alveoli. (OSA, Acute type 1 respiratory failure)
BiPAP: Bilevel Positive airway pressure (type 2 respiratory failure) (nippy, nippv, vpap)
When used outside of hospital NIV is often referred to as Home Mechanical Ventilation (HMV)

8. How does BiPAP help ?
generally NIV supports the patients own breathing
increases tidal volume
supports and rests tired respiratory muscles
supports upper airway
improves sleep efficiency and quality
improves mortality and morbidity
improves quality of life 8

9. When to introduce in MND
Best time to introduce is when people have symptoms of sleep disordered breathing or nocturnal hypoventilation.
Few people tolerate NIV without symptoms.
Some patients, particularly those with MND, have what appears to be normal lung function and normal sleep study.
Caveat:
Not everyone will want ventilation or tolerate it.
We are trying to improve quality of life not worsen it.

10. Bilevel ventilation
Bilevel devices are designed to deliver inspiratory positive airway pressure (IPAP) when a patient takes breath in, pushing air into lungs
This is followed by a lower expiratory positive airway pressure (EPAP) that allows patient to exhale
Bilevel does not allow specific tidal volume
Numerous devices

11. What is IPAP and EPAP?
IPAP – pressure on inspiration to increase tidal volume size. This will ensure sufficient removal of carbon dioxide and will aid breathlessness
EPAP – splints airway open during expiration to overcome obstruction or airway collapse. This aids gaseous exchange – aids oxygenation.

12. Pressure Support or Pressure Control?
Senses breathing pattern of patient
Delivers IPAP and EPAP
Synchronises with patient
Has timed back up breaths should respiratory rate drop
PRESSURE CONTROL
Provides pressure support with time being added
Sometimes more tolerable in neuromuscular disease
Added length of time of IPAP allows more time for carbon dioxide removal

13. NIPPY 3+

14. Interfaces Proper fit paramount Nasal/facial
Full face preferable if mouth breather
Total facemask also available

15. Interfaces

16. Sizing gauge

17. Does it work?
In short…Yes

18. General outcomes mortality
excellent outcome at 5 years for post polio syndrome 100% survival rate
over 50% of those with DMD now survive to over 25 because of HMV
excellent outcome in stable muscular skeletal and neuromuscular disease (73% of kyphoscoliosis pts 5 year survival (60% O2 alone)
improving mortality in MND (200 days median improvement in length expectancy)
poorer outcome in bronchiectasis, CF and COPD.

19. The evidence
NB Probability of continuing NIV is equivalent to survival as the main reason for discontinuation was death. (Simonds and Elliott 1995)

20. The evidence DMD
Ishikawa Y et al Neuromusc Disord 2011;21:47-51

21. The Evidence in MND Non-invasive ventilation
Can improve QoL and length of life in non-bulbar MND
Can Improve QoL but not length of life in severe bulbar MND

22. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial Bourke et al 2006

23. Oxygen in MND Target saturation range is 88-92%.
Only employ oxygen to keep patients in that range.

24. Hypoxaemic drive
Control of ventilation is complex but includes a hypercapnic respiratory drive.
A small percentage of patients who hypoventilate can have chronically high CO2.
Such patients become insensitive to CO2 and use a hypoxaemic drive to their respiration.
As paO2 increases, ventilation will fall raising paCO2 and decreasing pH.

25. VQ mismatch due to increased alveolar oxygen
Poorly ventilated alveolar capillary units become hypoxic and therefore poorly perfused due to hypoxic pulmonary vasoconstriction.
If widespread, then vasoconstriction across the pulmonary circulation.

26. VQ mismatch due to increased alveolar oxygen
High flow oxygen improves alveolar hypoxia and therefore pulmonary vasoconstriction.
The alveolar units remain poorly ventilated and CO2 pours into the blood stream, raising paCO2 and decreasing pH.
The patient becomes narcosed and can die of acidaemia.

27. Oxygen in MND Target saturation range is 88-92%
Only employ oxygen to keep patients in that range
Oxygen alerts and venture packs may help acute teams

28. Living with and managing a weak cough
To support a weak cough you need to:
support the mucocillary escalator
support the inspiratory muscles
support the vocal cords
support the expiratory muscles
or all of these.

29. European Neuromuscular Workshop on Airway Clearance Techniques
3rd – 5th March 2017
Lack of comprehensive and comparable research
Very few recommendations that can be used by all people with NMD
Clinicians, researchers and consumer representation from 12 countries to develop a consensus approach for airway clearance in NMD.

30. Suggested strategy for initiation of cough augmentation techniques

31. Supporting the mucocillary escalator
mobilise the phlegm
hydration
mucolytics
breathing techniques.
the mucocillary escalator video

32. Supporting the inspiratory muscles
Breath stacking
It allows patients to achieve an inspiratory lung volume close to total lung capacity. FRC = functional residual capacity; TLC = total lung capacity.
Modified by permission from McKim D. Canadian Respiratory Journal 2008:15.

33. Supporting the inspiratory muscles
Breath stacking
Lung Volume Recruitment (LVR) bag
Series of stacked breaths (with
no breath out between
Inspirations)
Single breath (with expiration/
cough)

34. Contraindications Haemotypsis Pneumothorax Bullae Care in COPD
Raised ICP
Impaired consciousness/inability to communicate
Inflated tracheostomy cuff/ET tube
Tracheoesophageal fistula
Severe bulbar insufficiency
Care if treating after meals/Stop feed

35. Supporting the inspiratory muscles
Breath Stacking
Lung Volume recruitment bag.
Glossopharyngeal Breathing

36. Supporting the expiratory muscles
Manual Assisted Cough
Own and patient size? Eight of bed? Strength, pain relief? Suction?

37. MAC Precautions Indwelling abdominal or pelvic catheters
Patients with severe scoliosis
PEG’s/RIG’s
Contraindications
Rib fractures
Other chest injuries
Severe osteoporosis
Abdominal surgery/trauma
Untreated haemoptysis
Clotting disorders
Unstable spine
Paralytic ilieus
Pregnancy

38. Doing all three: Mechanical insufflator/exsufflator
Emerson Cough assistor
Nippy Clearway
E 70 Cough assistor
‘The MI/E uses positive pressure to promote maximal lung inflation followed by an abrupt switch to negative pressure to the upper airway – the rapid change stimulates the flow changes that occurs during a cough’ (Chatwin et al 2003)

39. MI-E Utilisation Introduce when PCF < 160l/min
Can be used via FM, mouthpiece, ETT and trache
Manual and automatic modes
Acute and community use
+/- 0-60cmH20 in/exsufflation pressures
Need insufflation pressure sufficient to reach MIC
Need expiratory flow bias to move secretions
(10-20cm H20 difference)

40. Contraindications Bullous emphysema
Undrained pneumothorax or pneumo-mediastinum
CV instability
Tracheoesphageal fistula
Recent or existing barotrauma
Spinal instability
Acute pulmonary oedema
Acute lung injury
Facial trauma

41. Considerations In MND with bulbar dysfunction
Manual techniques more effective than MI-E in early stages of disease
Recognised that MI-E becomes more effective with disease progression
Both inspiratory and active expiratory phase of MI-E can lead to dynamic upper airway narrowing and/or closure resulting in a reduction rather than the desired improvement in cough flow efficacy (Anderson et al 2017)

42. Suggested strategy for MI-E in bulbar MND (Anderson et al 2017)

43. Discharge planning
Family/Carers to be trained and competency framework completed
Instruction leaflet with written document of settings given to pt
District Nursing to provide portable suction machine
Provide handover to community/palliative care physiotherapist
prescription to Respicare and request follow up visit re maintenance of machine/consumables

44. Increasing muscle weakness Expiratory techniques
Schematic representation of the management of cough augmentation (Chatwin & Simonds 2007)
Increasing muscle weakness
Combination of all techniques
Cough assist
Combination of inspiratory and expiratory techniques
Inspiratory techniques:
Pressure breathing (IPPB)
Breath stacking
Non invasive ventilation (NIV)
Intermittent positive pressure breathing (IPPB)
Expiratory techniques
Cough assist techniques
Worsening peak cough flow