1. Respiratory Management of MND patients in Scotland
Dr Scott Davidson
WoS Long Term Ventilation Unit
Queen Elizabeth University Hospital
Glasgow

2. Introduction
Majority of patients will die in respiratory failure

3. Introduction Respiratory muscle weakness Alveolar hypoventilation
< 20% aware of symptoms at presentation
Develops in all as disease progresses
Alveolar hypoventilation
Respiratory failure
Late
Acute

4. Respiratory presentations
Breathlessness
Orthopnoea
Sleep disturbance
Morning headaches
Daytime hypersomnolence
Ineffective cough
Excessive secretions
Aspiration
Panic attacks
Important to emphasise that sleep related symptoms are often unrecognised unless specifically sought

5. Respiratory presentations
Sleep abnormalities
Sleep disordered breathing ( %)
Common SDB pattern is
REM sleep related hypopnoea and oxygen desaturation
With overall hypoventilation with more severe weakness
More severe patients
Particularly susceptible during REM when ventilation almost entirely dependant on diaphragm
Sleep disordered breathing and disturbed sleep architecture are both common in MND. Sleep disruption can result from SDB and orthopnoea as well as muscle cramps/difficulty dealing with secretions for example
Discrete events relatively infrequent and consequently the AHI is an insensitive index of the severity of SDB in MND
Bulbar weakness appears to have little effect on the severity or type of SDB

6. Respiratory presentations
But
Sleep disruption may be from
SDB
Orthopnoea
Muscle cramps
Difficulty managing secretions

7. Respiratory monitoring

8. Respiratory monitoring
Postal survey Neurologists 2009
612 referrals for NIV
444 successfully initiated (75%)
38% respondents monitored baseline function
20% monitored routinely
32% symptoms alone for referral
43% combination symptoms and physiology
26% use O2 without considering NIV 1st
J Neurol Neurosurg Psychiatry 2009

9. NICE Guidelines 2010

10. NICE 2010 3 monthly assessment SpO2 FVC/VC SNIP/MIP
ABG< 92% lung disease or < 94% if not
pCO2 > 6 immediate referral
pCO2 < 6 but symptoms/signs refer
FVC/VC
SNIP/MIP
Particularly orthopnoea
Bear in mind that an elevated paCO2 usually implies severe weakness and a poor prognosis
Nocturnal transcutaneous CO2 is elevated before daytime paCO2 is raised

11. Respiratory monitoringVC
> 70%
breathlessness unusual
< 70%
Decompensation common within 12/12
< 30%
Respiratory failure virtually inevitable
Link here is want to pick up problems in anticipatory fashion

12. If any of these discuss respiratory impairment / treatment options and consider referral specialist respiratory service for assessment
Remember that pressure measurements are more sensitive in early stages of disease and FVC will fall only with moderate or severe weakness
Overall SNIP is the best predictor of daytime paCO2

13. NIV in MND

14. NIV in MND Bourke et al, Lancet Neurol 2006;5:140-7 n = 92 enrolled
n = 41 randomised to either NIV or standard care when 1 or more of:
Orthopnoea
MIP < 60%
Symptomatic daytime hypercapnia
n = 19 standard care
n = 22 NIV
19 did not meet inclusion criteria
10 met but declined
92 enrolled and 41 met criteria for randomisation during surveillance period

15. Survival

16. Survival
Better bulbar function group showed survival advantage 205 days

17. Quality of Life - SF36

18. Quality of Life - SF36

19. Quality of Life – SAQLI

20. Quality of Life – SAQLI

21. Study key points Normal or moderate bulbar Improved survival
NIV 216 days versus 11 best supportive care (p = )
Beyond that of riluzole
Maintenance of and improvement in QoL
Maintained above 75% of baseline in NIV group
Severe bulbar
Improvement in sleep related symptoms
Unlikely to confer large survival benefit
BUT we know paper by Ferrero 2005 in chest >50% bulbar patients initiated successfully and attribute to less nihilisticc attitude, inpatient initiation and overnight adaptation of settings 21

22. NIV - when to start?
Optimum time is unclear

23. NIV - when to start – suggested criteria?
Any one of
Orthopnoea with evidence respiratory muscle weakness
Daytime hypercapnia
? Nocturnal desaturation
( SaO2 < 90% for > 5% sleep or 5 consecutive minutes )
? Nocturnal hypercapnia
( tCO2 > 6.5kPa )
Have left off resp muscle tests as we tend not to use as an indication ? Add in better of MIP or SNIP < 40

24. NICE Guidelines 2010
Offer to discuss the use of NIV in a timely and sensitive manner
Include information, appropriate to the stage of illness, about:
possible symptoms and signs of respiratory impairment
natural progression of MND
respiratory function tests and results
benefits and limitations of interventions
how NIV can improve symptoms and prolong life
how NIV can be withdrawn
palliative strategies
Inform relevant clinicians of key decisions agreed

26. When to involve Respiratory/LTVU?
At diagnosis
This allows us to discuss appropriately as per nice guidance

27. Withdrawal of NIV

28. Withdrawal of NIV Can be discontinued as a matter of choice
Death may be minutes to days
Decision may be obvious concern to
Family
Healthcare professionals
(managers)
Bereavement services
Staff support mechanisms
A competent patient has the right to stop any treatment they are receiving. Complying with patient wishes is good medical practice and not euthanasia
Further evidence of the importance of Anticipatory care planning

29. Withdrawal of NIV Anticipatory Planning Current / future focus of care
Review previous and formulate new plans
Resuscitation
Preferred place of death
Home
Hospice
hospital

30. Withdrawal of NIV
Practicalities

31. Withdrawal of NIV Who will be present? Who will remove the mask?
Opportunity to say goodbye essential
Explanation of process
Explanation of potential symptoms
Explanation of unknown timeframe
Who will remove the mask?
Who will turn off the ventilator?
Who will coordinate the process?
Here comment on potential for colour chnge/gasping etc
Comment here also on who will not be present and explore reasons etc

32. Withdrawal of NIV Anticipatory prescribing Syringe driver plus bolus
Morphine
Midazolam
Haloperidol
Glycopyrronium
Oxygen
Glycopyrronium if excessive secretions an issue and start with lowest doses that control symptoms

33. Withdrawal of NIV Bereavement and counselling
Withdrawal of NIV a complex emotional process

34. Tracheostomy ventilation

35. Tracheostomy ventilation
Traditionally emergency setting
Cuffed protects airway but
Swallow and speech problems
Immobility
Bronchospasm
Tracheomalacia
Haemorrhage and fistula formation
Few long term residential facilities
Carer burden progression to locked in

36. Summary LTV involvement early with patients essential
Respiratory monitoring
Symptoms
Physiology
Sleep (tosca)monitoring
NIV complex
Starting
Optimising
Withdrawal
Cough
To discuss in afternoon sessions

37. Discussion

38. Patients with dementia
Assessment pre NIV decision:
the patient’s capacity to make decisions and give consent
the severity of dementia and cognitive problems
whether the patient is likely to accept treatment
whether the patient is likely to achieve improvements in sleep-related symptoms and/or behavioural improvements
a discussion with the patient’s family and/or carers
About 5% of patients develop frontotemporal dementia (associated whilst milder degrees of cognitive function are more common