Managing Respiratory Symptoms in Advanced MS by Rachael Moses
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Managing Respiratory Symptoms in Advanced MS Monday 22 nd February 2016 Rachael Moses Consultant Physiotherapist Complex Ventilation and Airway Clearance @rachaelmoses [email protected]
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Transcript of Managing Respiratory Symptoms in Advanced MS by Rachael Moses
Managing Respiratory Symptoms in Advanced MS
Monday 22nd February 2016 Rachael Moses
Consultant Physiotherapist Complex Ventilation and Airway Clearance
@rachaelmoses [email protected]
What’s the evidence?
Mutluay FK, Gurses HN, Saip S (2005) Effects of multiple sclerosis on respiratory functions.
Clinical Rehabilitation 19: 426–432.
Smeltzer et al. (1992) Respiratory function in multiple sclerosis. Utility of clinical assessment of respiratory muscle function. Chest 101: 479–484.
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.
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.
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
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
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
Reflection 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
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
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)
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.
Sound Familiar?
• EDSS > 7
• Inability to deep breathe
• No cough
• Repeated aspiration pneumonia
• Poor Voice
• SOBAR
• Inability to speak in sentences
No real evidence in how to manage the respiratory symptoms for
people with MS
However….
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
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
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
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
Stages of cough
• Inspiratory Phase
• Glottis closure
• Expiratory Phase
Vital Capacity
Assessing inspiratory stage of cough
Sniff Nasal inspiratory pressure (SNIP)
Maximal Inspiratory Pressure (MIC)
Stages of cough
• Inspiratory Phase
– Vital Capacity
– MIP
– SNIP
• Glottis closure
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”
Instrumental analysis
Stages of cough
• Inspiratory Phase
– Vital Capacity
– MIP
– SNIP
• Glottis closure
– Staccato expiration
– Instrumental analysis
• Expiratory Phase
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
Teach MAC and / or MIC
PCF < 270
PCF < 155
MI-E
+/- MAC
PCF < 245
MI-E
Combine MAC and / or MIC
Michelle Chatwin
Stages of cough
• Inspiratory Phase – Vital Capacity – MIP – SNIP
• Glottis closure – Staccato expiration – Instrumental analysis
• Expiratory Phase – MEP – PCF
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
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=player_detailpage&v=Dy1QDIM-rPI
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
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
• 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.
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.
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
Patient characteristics at the first visit
• 82% of patients had an
FVC < 80% predicted
• Mean FVC was only 56% predicted
• PCF was also significantly reduced, with 80% of patients having a PCF < 270
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.
• The MIC did not decline significantly with an average rate of decline of 50.6 mL/y.
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.
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.
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.
Combination of MIC and MAC
Lung volume recruitment
Manual assisted cough
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
Expiratory muscle augmentation
The use of expiratory aids to clear secretions significantly decreased hospitalization rates for respiratory complications of neurologic disease
Tzeng AC, Bach JR, Chest 2000
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
Mechanical In-Exsufflation (MI-E)
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.
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
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.
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.
Cough augmentation with mechanical insufflation/exsufflation in
patients with neuromuscular weakness Chatwin et al, 2003
Development of a system to allow independent home use of a mechanical in-exsufflator (M I-E device) for a patient with advanced
neuromuscular disease (MS) Moses, Armstrong, Bullock 2013
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.
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
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
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
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
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
Managing Respiratory Symptoms in Advanced MS
Thanks for listening.
Questions?
Email or tweet if you think of something later!
@rachaelmoses [email protected]
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
References
• Gosselink R, Kovacs L, Decramer M (1999) Respiratory muscle involvement in multiple sclerosis. European Respiratory Journal 13: 449–454.
• Aisen M, Arlt G, Foster S. Diaphragmatic paralysis without bulbar or limb paralysis in multiple sclerosis. Chest 1990; 98: 499–501.
• Balbierz JM, Ellenbergh M, Honet JC. Complete hemidiaphragmatic paralysis in a patient with multiple sclerosis. Am J Phys Med Rehab 1988; 67: 161–165.
• Cooper CB, Trend P St J, Wiles CM. Severe diaphragm weakness in multiple sclerosis. Thorax 1985; 40: 633–634.
• Kuwahira I, Kondo T, Ohta Y, Yamabayashi H. Acute respiratory failure in multiple sclerosis. Chest 1990; 97:246–248.
• Noda S, Umezaki H. Dysarthria due to loss of voluntary respiration (Letter). Arch Neurol 1982; 39: 132.
References
• Mutluay FK, Gurses HN, Saip S (2005) Effects of multiple sclerosis on respiratory functions. Clinical Rehabilitation 19: 426–432.
• Smeltzer SC, Skurnick JH, Troiano R, Cook SD, Duran W, et al. (1992) Respiratory function in multiple sclerosis. Utility of clinical assessment of respiratory muscle function. Chest 101: 479–484.
• Smeltzer SC, Utell MJ, Rudick RA, Herndon RM (1988) Pulmonary function and dysfunction in multiple sclerosis. Archives of Neurology 45: 1245–1249.
• Altintas A, Demir T, Ikitimur HD, Yildirim N (2007) Pulmonary function in multiple sclerosis without any respiratory complaints. Clinical Neurology & Neurosurgery 109: 242–246.
• Foglio K, Clini E, Facchetti D, Vitacca M, Marangoni S, et al. (1994) Respiratory muscle function and exercise capacity in multiple sclerosis. European Respiratory Journal 7: 23–28.
• Tzelepis , McCool (2015) Respiratory dysfunction in multiple sclerosis. Resp Care.
References
• Yamamoto T, Imai T, Yamasaki M. Acute ventilatory failure in multiple sclerosis. J Neurol Sci 1989; 89: 313 324.
• Carter JL, Noseworhty JH. Ventilatory dysfunction in multiple sclerosis. Clin Chest Med 1994; 15: 693–703.
• Chiara T, Martin AD, Davenport PW, Bolser DC (2006) Expiratory muscle strength training in persons with multiple sclerosis having mild to moderate disability: effect on maximal expiratory pressure, pulmonary function, and maximal voluntary cough. Arch Phys Med Rehabil 87: 468–473.
• Aiello M, Rampello A, Granella F, Maestrelli M, Tzani P, et al. (2008) Cough efficacy is related to the disability status in patients with multiple sclerosis. Respiration 76: 311–316.
• Trebbia G, Lacombe M, Fermanian C, et al. Cough determinants in patients with neuromuscular disease. Respir Physiol Neurobiol. 2005;146(2–3):291–300
References
• McKim DA, Katz SL, Barrowman N, Ni A, Leblanc C (2012) Lung Volume Recruitment Slows Pulmonary Function Decline in Duchenne Muscular Dystrophy. Arch Phys Med Rehabil.
• Bach JR, Bianchi C, Vidigal-Lopes M, Turi S, Felisari G (2007) Lung inflation by glossopharyngeal breathing and ‘‘air stacking’’ in Duchenne muscular dystrophy. Am J Phys Med Rehabil 86: 295–300.
• Kang SW, Bach JR (2000) Maximum insufflation capacity. Chest 118: 61–65. • Vitacca M, Paneroni M, Trainini D, Bianchi L, Assoni G, Saleri M, Gile` S,
Winck JC, Gonc¸alves MR: At Home and on Demand Mechanical Cough Assistance Program for Patients With Amyotrophic Lateral Sclerosis. Am J Phys Med Rehabil 2010;89:401–406
• Winck JC, Gonc¸alves MR, Lourenc¸o C, Viana P, Almeida J, Bach JR. Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumberance. Chest 2004;126(3):774–780.
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 Phys Med Rehabil 2005;84:83–88.
• Chatwin M, Ross E, Hart N, Nickol AH, Polkey MI, Simonds AK. Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J 2003; 21: 502–508.
• Lung Volume Recruitment in Multiple Sclerosis. Nadim Srour, Carole LeBlanc, Judy King, Douglas A. McKim. 2013. PLOS ONE | www.plosone.org
• Hirst, Swingler, Compston, Ben-Shlomo, Robertson. Survival and cause of death in multiple sclerosis: a prospective population-based study. J Neurol Neurosurg Psychiatry 2008;79:1016-1021
