Presentation by Rachael Moses, delivered at the MS Trust 2015 Annual Conference

1. Managing Respiratory Symptoms in
Advanced MS
Tuesday 10th November 2015
Rachael Moses
Consultant Physiotherapist
Complex Ventilation and Airway Clearance
@rachaelmoses rachael.moses@lthtr.nhs.uk

2. 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.

3. Pulmonary function and dysfunction in
multiple sclerosis.
Smeltzer et al 1988

5. Respiratory dysfunction in multiple sclerosis
Tzelepis and McCool 2015

6. Respiratory dysfunction in multiple sclerosis:
A prospective analysis of 60 patients
Buyse et al Eur Respir J, 1997; 10: 139–145
• Sixty patients
• Mean age 48
• EDSS mean score 6.5
• FSS:
o pyramidal 3.4
o brain stem 1.9
o mental 1.3
o cerebellar 2.2
o sphincter 1.8
o visual 1.4
o sensory 2.0

7. Pulmonary Function Tests
Patient Numbers Absolute Value % predicted
VC L 60 3.0 80
TLC L 35 5.6 100
RV L 35 2.1 115
RV/TLC % 35 38 113
FEV1 L 60 2.3 76
FEV1/VC % 60 75 93
PEF L 60 4.6 62
MEP cmH2O 58 48 30
MIP cmH2O 58 47 47

8. Disability score and pulmonary function
EDSS ≥7 EDSS <7
Mean n Mean n p-value
VC % pred 72 26 95 18 <0.001
FEV1 % pred 70 26 91 18 <0.002
PEF % pred 55 26 93 18 <0.001
Duration of disease, pulmonary function and neurological disability scores
Duration of disease
≥18 yrs <18 yrs
Mean n Mean n p-value
EDSS score 7.1 19 6.0 23 <0.05
FSS score
Mental
1.6 19 1.1 29 <0.001

9. Learning points
• For individuals with normal lung function expiratory muscle
strength may be reduced
• Impaired innervation of the upper airway may result in a
diminished awareness of coughing.
• Paralysis in advanced MS tends to ascend slowly from lower
extremities to upper extremities.
• As a result, the first respiratory muscles to be affected are
the abdominal muscles followed by the intercostal muscles.
• The diaphragm, which is innervated by the phrenic nerve
may be expected to be the last to be affected
• Bulbar dysfunction may predispose patients to aspiration
with desaturation, especially at night

10. Learning points
• Reports demonstrating abnormalities of respiratory control
in MS patients may only be observed during acute
exacerbations and therefore reversible
• No significant correlation was found between lung function
and duration of disease
• The prevalence of respiratory muscle dysfunction in MS
patients confirms the almost unpredictable course of the
disease
• This leads to variable respiratory and muscular
involvement.
• Despite marked respiratory muscle dysfunction some MS
patients never complain of pulmonary symptoms, such as
cough or SOB

11. What’s the point?
• MS patients are at risk
• Bulbar dysfunction
• Combination reduced lung volumes,
inspiratory and expiratory muscle weakness
and glottic dysfunction impairs cough
effectiveness
• PCF inversely related to degree of disability,
Chiara et al (2006), Aiello et al (2008)

12. Survival and cause of death in multiple sclerosis: a
prospective population-based study
Hirst et al 2008
• A population-based survey performed in South
Wales in 1985 identified 441 patients with MS
• The most common cause of death was
respiratory disease (47.5%).
• The standardised mortality ratio was 2.79 (95% CI
2.44 to 3.18) so that MS patients were almost
three times more likely to die prematurely
relative to the general population.

13. Sound Familiar?
• EDSS > 7
• Inability to deep breathe
• No cough
• Repeated aspiration pneumonia
• Poor Voice
• SOBAR
• Inability to speak in sentences

14. No real evidence in how to manage
the respiratory symptoms for
people with MS
However….

15. Effect of Upper Respiratory Tract Infection in Patients with
Neuromuscular Disease
Poponick et al 1997
• Insert • Effects of acute URI on
subjects with
neuromuscular disease
• Vital capacity
• Maximal inspiratory
pressures
• Maximal expiratory
pressures
• Transcutaneous oxygen
saturation
• End-tidal PC02

16. Why cant NMD patients cough?
• Reduced lung volumes and weak abdominal
muscles result in an inadequate cough
• Normal peak cough flow (PCF) >360l/min
• The minimum required to remove secretions is
160-200L/min
• The primary cause of respiratory infection in
patients with NMD is the inability to effectively
clear tracheal secretions
• For a patient that has a normal PCF of around
270ml the likelihood is this will deteriorate

17. Deep inspiration
(95% TLC, > 1.5l VC, >2.5l
air inspired)
Epiglottis closes, vocal
cords shut tightly to
entrap air within lung
Abdominal muscles
contract forcefully ,
pushing against the
diaphragm followed by
intercostal contraction
With closed glottis the
intrathoracic pressure rises
> 100cmH20 causing
tracheal narrowing
Rapid opening of vocal
cords and epiglottis with a
large pressure difference
and a narrow trachea
results rapid flow rates
A Cough is stimulated and
air is expelled at around
75-100mph

19. Muscle groups essential for cough
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
Inspiratory
Muscles
Expiratory
Muscles
Bulbar
Function

20. Stages of cough
• Inspiratory Phase
• Glottis closure
• Expiratory Phase

21. Vital Capacity

22. Assessing inspiratory stage of cough
Sniff Nasal inspiratory pressure
(SNIP)
Maximal Inspiratory Pressure
(MIC)

23. Stages of cough
• Inspiratory Phase
– Vital Capacity
– MIP
– SNIP
• Glottis closure
• Expiratory Phase

24. 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”

25. Stages of cough
• Inspiratory Phase
– Vital Capacity
– MIP
– SNIP
• Glottis closure
– Staccato expiration
– Instrumental analysis
• Expiratory Phase

26. 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

27. Teach MAC and / or MIC
PCF < 270
PCF < 155
MI-E
+/- MAC
PCF < 245
MI-E
Combine MAC and / or MIC
Michelle Chatwin

28. Stages of cough
• Inspiratory Phase
– Vital Capacity
– MIP
– SNIP
• Glottis closure
– Staccato expiration
– Instrumental analysis
• Expiratory Phase
– MEP
– PCF

29. Maximum insufflation capacity (MIC)
• The maximum lung volume that can be held by
air stacking.
• It requires intact bulbar function
• The Maximum Insufflation Capacity (MIC)
measurement (litres) is the maximum volume of
air stacked within the patient’s lungs beyond
spontaneous vital capacity.
• It is measured after a patient takes a deep breath
until maximal capacity is reached and air is then
exhaled into a spirometer

30. Glossopharangeal Breathing
• This technique uses the
glottis to add an
inspiratory effort by
projecting blouses of air
into the lungs.
• The glottis closes with
each gulp.
• Individuals find it helps
them to have more
breath so they can talk
for longer/breathe for
longer and cough.
http://www.youtube.com/watch?feature=play
er_detailpage&v=Dy1QDIM-rPI

31. Lung Volume Recruitment Bag
• Patients with low lung
volume; either from
injury or medical
condition.
• Has a one way valve to
prevent loss of volume.
• Low cost, Versatile,
Light weight

32. Lung volume recruitment in DMD
McKim et al 2012
• 3-5 breaths were
delivered over 2-3
seconds to achieve
MIC for a total of 3-5
cycles
• Twice daily
• If secretions present a
MAC was also
performed

33. • At LVR initiation, FVC was
21.8 and CPF was 270L
• Annual decline of FVC was
4.7% predicted a year
before LVR and 0.5 percent-
predicted a year after LVR
initiation.
• The difference, 4.2 percent-
predicted a year represents
an 89% improvement in the
annual rate of FVC decline.
• Regular LVR dramatically
improved the rate of FVC
decline in DMD
McKim et al, 2012.

34. Maintaining pulmonary compliance
• LVR will help to prevent atelectasis and
improving chest wall compliance.
• A daily regimen of 8 to 10 hyperinflation
manoeuvres has been suggested as a
maintenance therapy for pulmonary and chest
wall compliance
• In UK, recommend 2-4 x a day of the
prescribed regime.

35. 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

36. Conclusions
• The FVC rate of decline was significantly lower in
those who had an improvement in PCF with LVR
at the first visit than in those without
improvement (p<0.0001)
• As was the PCF rate of decline (p = 0.042)
*in an analysis where the baseline FVC or PCF
respectively were included as covariates to account
for differing baseline values between the groups.

37. Conclusions
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.

38. Combination of MIC and MAC
Lung volume
recruitment
Manual assisted
cough

39. Combination of MIC and MAC
Lung volume
recruitment
Manual assisted
cough
Combining manual hyperinflation with the
abdominal thrust manoeuvre has been shown to
produce a higher peak cough flow than by using
either therapy alone
Trebbia et al 2005

40. Limits of Effective Cough-Augmentation Techniques
in Patients With Neuromuscular Disease
Toussaint et al 2012
• Patients with VC >
340 mL and MEP < 34
cm H2O would
optimally benefit
from the combination
of breath-stacking
plus manually assisted
cough to improve PCF
to > 180 L/min

41. Mechanical In-Exsufflation
(MI-E)

42. What is MI-E
• MI-E consists of insufflation of the lungs with
positive pressure
• Followed by a rapid change into negative-
pressure to give an active exsufflation
• That creates a peak and sustained flow high
enough to provide adequate shear and
velocity
• Loosen and mobilises secretions toward the
mouth for suctioning or expectoration.

43. Mechanical Insufflation–Exsufflation Improves Outcomes for
Neuromuscular Disease Patients with Respiratory Tract Infections
Vianello et al, 2005
Treatment failure (need for minitracheostomy or intubation)
2/11 (p 0.05) 10/16
Treatment
MI-E plus Chest Physio Chest Physio
URTI
11 NMD 16 matched controls

44. Cough augmentation with mechanical insufflation/exsufflation in
patients with neuromuscular weakness
Chatwin et al, 2003
• Adults and children with NMD exhibit weak
cough and are susceptible to recurrent chest
infections, a major cause of morbidity and
mortality.
• MI-E may improve cough efficacy by
increasing PCF
• It was hypothesised that MI-E would produce
a greater increase in peak cough flow than
other modes of cough augmentation.

45. Cough augmentation with mechanical insufflation/exsufflation in
patients with neuromuscular weakness
Chatwin et al, 2003
• 22 patients (median 21 yrs) with NMD and 19
age-matched controls were studied.
• Spirometry was performed and respiratory
muscle strength measured
• Peak cough flow was recorded during maximal
unassisted coughs, followed in random order by
coughs assisted by physiotherapy, NIV,
insufflation and exsufflation, and exsufflation
alone
• Subjects rated strength of cough, distress and
comfort on a visual analogue scale.

46. Cough augmentation with mechanical insufflation/exsufflation in
patients with neuromuscular weakness
Chatwin et al, 2003

47. 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.

48. Tracheostomy and Mechanical In-Exsufflation for patients
with advanced Multiple Sclerosis. Should it be a standard
treatment intervention when indicated?
Moses 2015
• Tracheostomies and long term ventilation are now a
recognised treatment for many patients with
neuromuscular disease.
• There is little evidence to support tracheostomy and
mechanical in-exsufflation (MI-E) for patients with
advanced progressive multiple sclerosis (MS) and obvious
ethical implications of both.
• This case study describes the journey of a 50 year old man
with advanced MS who was readmitted with left sided lung
collapse and in severe hypoxic respiratory failure
• His decision to have a tracheostomy saved his life, one he
does not regret

49. Hyperbaric Oxygen Therapy
Anecdotally some people with MS have
reported relief from some symptoms after
HBO therapy, but scientific studies have
repeatedly failed to reproduce these claims

50. Optimal medical management
• Vaccinations
• SALT and nutritional assessment
• Oral secretion management
• Mucolytics
• Reflux management
• Antibiotics
• Early detection of chest infections and use of
rescue packs
• Exercise and prevention

51. The future
• Regular spirometry testing
• PCF measurements
• LVR offered as a standard intervention
• MI-E devices being trialled and offered
• RCTs and prospective studies
• Inclusion into NICE Guidance
• Personal care budgets
• Commissioning

52. Closing thoughts
• People with MS that become immobile will develop
respiratory insufficiency with varying degrees
• There is lots of evidence for lung volume recruitment,
secretion clearance and optimisation of respiratory
function for people with NMD
• The evidence is transferable and may make the lives of
people with MS more manageable with a reduction in
respiratory side effects, hospital admissions and
therefore secondary complications

53. Managing Respiratory Symptoms in
Advanced MS
Thanks for listening.
Questions?
Email or tweet if you think of something later!
@rachaelmoses rachael.moses@lthtr.nhs.uk

54. LVR Procedure
• Position patient – preferably in upright sitting and explain procedure
• Establish with your patient the signal he/she will use to notify you that
MIC is reached.
• With nose clips in place, ask the patient to take a deep breath and hold.
• Ask the patient to place lips tightly around the mouthpiece to prevent air
from escaping.
• As you gently squeeze the resuscitation bag, coordinate with the patient’s
inspiration. Squeeze the bag 2-5 times until you feel the lungs are full or
when the patient sends you a signal that MIC is reached.
• Once the patient’s lungs are full, take the mouthpiece out of the mouth,
ask the patient to hold the maximum insufflation for 3 to 5 seconds, and
then allow the patient to exhale gently.
• Repeat steps 3 to 5 times.
http://www.irrd.ca/education/policy/LVR-policy.pdf

55. References
• Gosselink R, Kovacs L, Decramer M (1999) Respiratory muscle
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• Balbierz JM, Ellenbergh M, Honet JC. Complete hemidiaphragmatic
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• Cooper CB, Trend P St J, Wiles CM. Severe diaphragm weakness in
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• Kuwahira I, Kondo T, Ohta Y, Yamabayashi H. Acute respiratory
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• Noda S, Umezaki H. Dysarthria due to loss of voluntary respiration
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56. References
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• Smeltzer SC, Skurnick JH, Troiano R, Cook SD, Duran W, et al. (1992)
Respiratory function in multiple sclerosis. Utility of clinical assessment of
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Resp Care.

57. References
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• Carter JL, Noseworhty JH. Ventilatory dysfunction in multiple
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• Trebbia G, Lacombe M, Fermanian C, et al. Cough determinants in
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58. References
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59. References
• Chatwin M and Simonds A. The addition of mechanical
insufflation/exsufflation shortens airway-clearance sessions in
neuromuscular patients with chest infection. Respir Care
2009;54(11):1473– 1479.
• Vianello A, Corrado A, Arcaro G, Gallan F, Ori C, Minuzzo M, Bevilacqua M.
Mechanical insufflation– exsufflation improves outcomes for
neuromuscular disease patients with respiratory tract infections. Am J
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• Chatwin M, Ross E, Hart N, Nickol AH, Polkey MI, Simonds AK. Cough
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• Lung Volume Recruitment in Multiple Sclerosis. Nadim Srour, Carole
LeBlanc, Judy King, Douglas A. McKim. 2013. PLOS ONE | www.plosone.org
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