Rachael Moses - Managing respiratory function in advanced Multiple Sclerosis

1. HELEN LEY LECTURE
MANAGING RESPIRATORY FUNCTION IN
ADVANCED MULTIPLE SCLEROSIS
Rachael Moses
Consultant Respiratory Physiotherapist
Complex Ventilation and Airway Clearance
Lancashire and South Cumbria Long Term Ventilation Service
MS Trust Annual Conference 5th November 2018 Leicester
@rachaelmosesrachael.moses@lthtr.nhs.uk

2. AIMS
• Describe the potential impact of MS on respiratory function
• Identify ways in which they can improve their practice to
better manage/prevent respiratory symptoms in MS
• Gain a greater understanding of the resources available to
help them support pwMS experiencing respiratory
symptoms

3. RESPIRATORY INVOLVEMENT – THE
INEVITABLE CONSEQUENCE OF MS?
Smeltzer et al. (1992) Respiratory function in multiple
sclerosis. Utility of clinical assessment of respiratory muscle
function. Chest 101: 479–484.

4. PULMONARY FUNCTION AND DYSFUNCTION
IN MULTIPLE SCLEROSIS.
SMELTZER ET AL 1988
• Studied pulmonary function in 25 MS patients with a range of motor
impairment.
• Ambulatory patients had normal spirometry
• Wheelchair-bound patients with upper extremity involvement had
reduced spirometry
• Bedridden patients had significantly lower spirometry results
• Spirometry decline correlated with higher EDS scores and expiratory
muscle weakness occurred most frequently.

5. PULMONARY FUNCTION AND DYSFUNCTION
IN MULTIPLE SCLEROSIS.
SMELTZER ET AL 1988
• Studied pulmonary function in 25 MS patients with a range of motor
impairment.
• Ambulatory patients had normal spirometry
• Wheelchair-bound patients with upper extremity involvement had
reduced spirometry
• Bedridden patients had significantly lower spirometry results
• Spirometry decline correlated with higher EDS scores and expiratory
muscle weakness occurred most frequently.

6. RESPIRATORY DYSFUNCTION IN MULTIPLE
SCLEROSIS
TZELEPIS AND MCCOOL, 2015
• Respiratory dysfunction frequently occurs in patients with advanced
MS and may manifest as acute or chronic respiratory failure:
• disordered control of breathing
• respiratory muscle weakness
• sleep disordered breathing
• or neurogenic pulmonary oedema
• The underlying pathophysiology is related to demyelinating plaques
involving the brain stem or spinal cord

7. RESPIRATORY DYSFUNCTION IN MULTIPLE
SCLEROSIS
TZELEPIS AND MCCOOL, 2015
• Respiratory complications such as aspiration, lung infections and
respiratory failure are typically seen
• Acute respiratory failure is uncommon and due to newly appearing
demyelinating plaques in the brain stem or spinal cord
• Early recognition of MS patients at risk for respiratory complications
allows for the timely implementation of care and measures to decrease
disease associated morbidity and mortality

8. RESPIRATORY DYSFUNCTION IN MULTIPLE
SCLEROSIS
TZELEPIS AND MCCOOL 2015

9. MUSCLE GROUPS ESSENTIAL FOR COUGH
Inspiratory
Muscles
Expiratory
Muscles
Bulbar
Function
Effective cough is a protective mechanism against respiratory tract infections, which are
the commonest cause of hospital admission in patients with respiratory muscle weakness
due to neuromuscular disease.
Chatwin et al 2003

10. STAGES OF COUGH
• Inspiratory Phase
• Vital Capacity
• MIP
• SNIP
• Glottis closure
• Staccato expiration
• Instrumental analysis
• Expiratory Phase

11. ASSESSING INSPIRATORY STAGE OF COUGH
Sniff Nasal Inspiratory Pressure
(SNIP)
Maximal Inspiratory Pressure
(MIP)

12. Lu I et al. J Appl Physiol 2006;101:1104-1112
©2006 by American Physiological Society
GLOTTIS CLOSURE
Staccato expiration
“eee, eee, eee”
“ahh, ahh, ahh”

13. INSTRUMENTAL ANALYSIS

14. ASSESSING EXPIRATORY STAGE
OF COUGH
• MEP (maximal expiratory pressure
or PeMax)
• Assessing PCF is a quick and easy
way of measuring expiratory
muscle function
• > 360 l min = Normal Cough
Function
• < 270 l min = Introduce strategies
for assisted airway clearance
• < 160 l min = Additional assisted
airway clearance strategies

15. Teach lung volume recruitment
PCF < 270
PCF < 155
Cough Assist plus manual
techniques
PCF < 245
Cough Assist Machine
Combine techniques
Michelle Chatwin

16. LUNG VOLUME RECRUITMENT BAG

17. LUNG VOLUME RECRUITMENT IN MULTIPLE
SCLEROSIS
SROUR ET AL 2013
• 10 year study
• LVR was attempted in patients with FVC 80% predicted.
• Regular twice daily LVR was prescribed
• A baseline FVC 80% predicted was present in 82% of patients and
80% of patients had a PCF insufficient for airway clearance
• There was a significant decline in FVC and PCF over a median follow-
up time of 13.4 months (Mean FVC was only 56% pred + PCF < 270)

18. COMPARATIVE TIME COURSE OF AVERAGE
FVC, MIC, PCF AND PCF LVR
• FVC, PCF and PCF LVR
declined significantly at
an average rate of
• 89.9 mL/y,
• 154 mL/s/y
• 89.1 mL/s/y respectively

19. COMPARATIVE TIME COURSE OF AVERAGE
FVC, MIC, PCF AND PCF LVR
• FVC, PCF and PCF LVR
declined significantly at
an average rate of
• 89.9 mL/y,
• 154 mL/s/y
• 89.1 mL/s/y respectively
Pulmonary function and cough declines in MS patients
over time
LVR is associated with a slower rate of decline in lung
function and peak cough flow.

20. RANDOMIZED CONTROL TRIAL OF EFFECTS OF A 10-WEEK
INSPIRATORY MUSCLE TRAINING PROGRAM ON MEASURES OF
PULMONARY FUNCTION IN PERSONS WITH MULTIPLE SCLEROSIS
FRY ET AL 2007
• 10-week home exercise inspiratory training program or a nontreatment
control group
• 46 ambulatory individuals with clinically diagnosed MS EDSS (2.0-6.5)
• The intervention group demonstrated significantly greater improvement than
the control group in maximal inspiratory pressure (P < 0.001)
• Baseline and post exercise training comparison of secondary pulmonary
expiratory outcomes were significant in the intervention group for
• FEV1 (P = 0.014)
• FVC (P = 0.041)
• FEF 25-75% (P = 0.011)
• No significant changes were noted for the control group

21. COUGH ASSIST DEVICES
NIPPY Clearway Philips E-70Emersen (Respironics)

22. IS THERE ANY EVIDENCE?
• Significantly increases peak cough flow
Bach 1993, Sivasthy 2001, Chatwin 2003, Mustafa 2001
• Decreased treatment time Chatwin 2009
• Improved oxygen saturation and decreased dyspnoea
Winck 2004
• Potential to decrease mechanical ventilation time
Vianello 2005
• Is well tolerated and safe
Miske 2004,Fauroux 2008
• Prevent hospitalisation during exacerbations
Lacombe 2014

23. RESPIRATORY FAILURE
• Some MS Patients will have other respiratory conditions:
• Asthma
• COPD
• Sleep Apnoea
• Most MS Patients do not develop nocturnal hypoventilation, but some do:
• Waking through the night
• Vivid dreams/nightmares
• Inability to lie flat
• Morning headaches
• Daytime sleepiness/lethargy
• Reduced appetite/weight loss

24. SOUND FAMILIAR?
• EDSS > 7
• Inability to deep breathe
• No cough
• Repeated ‘aspiration pneumonia’
• Poor Voice
• Short of Breath
• Inability to speak in sentences

25. THE USE OF MI-E AS A COST EFFECTIVE ADMISSION
AVOIDANCE STRATEGY FOR PATIENTS WITH ADVANCED
MULTIPLE SCLEROSIS (MS)
MOSES 2015
Diagnosis PCF
Reading
(ml)
Number of
admissions
in 12
months
Average
bed days
Readmissions in 12
months (following
provision of NIPPY
clearway)
Potential
Bed
days
saved
Potential
cost
saving (£)
MS 110 4 14 1 42
MS <50 6 9 0 54
MS 100 7 18 0 126
7,600
11,200
32,800
(cost saving is based on a hospital bed day costing £300, minus cost of equipment and consumables) Department of health
https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/213060/2011-12-reference-costs-
publication.pdf
The provision of a MI-E device for patients with MS can prevent future hospital admissions and is
therefore a cost effective admission avoidance strategy for patients with MS
Patients should be offered MI-E devices as part of a planned discharge package.

26. HELEN LEY LECTURE
MANAGING RESPIRATORY FUNCTION IN
ADVANCED MULTIPLE SCLEROSIS
Rachael Moses
Consultant Respiratory Physiotherapist
Complex Ventilation and Airway Clearance
Lancashire and South Cumbria Long Term Ventilation Service
MS Trust Annual Conference 5th November 2018 Leicester
@rachaelmosesrachael.moses@lthtr.nhs.uk

27. REFERENCES
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31. REFERENCES
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