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. Does it work?
In short…Yes

11. 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.

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

13. The evidence DMD
NIV significantly improves length of life in DMD, more so than tracheostomy ventilation.

14. 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.

15. 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

16. 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.

17. Supporting the Mucociliary escalator
mobilise the phlegm
hydration
mucolytics
breathing techniques.
mucociliary escalator video

18. 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.

19. Supporting the inspiratory muscles
Breath stacking.
Lung Volume Recruitment (LVR) bag.

20. Supporting the inspiratory muscles
Breath Stacking
Lung Volume Recruitment bag
Glossopharyngeal breathing video

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

22. 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)

23. 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

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

25. 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.

26. 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.

27. 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.

28. 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.