Managing Respiratory Symptoms in Advanced MS

Sunday 6th November 2016 Rachael Moses

Consultant Physiotherapist Complex Ventilation and Airway Clearance

@rachaelmoses rachael.moses@lthtr.nhs.uk

Midbrain

Pons

Medulla Oblongata

Brainstem

Normal Breathing

• FVC = forced vital capacity = volume exhaled after maximal inspiration through to maximal expiration

• FEV1 = forced expiratory volume in 1 sec

• FEV1/FVC = ratio

• MIP/MEP/SNIP

• PCF = peak cough flow

Measurement Approximate value

Male Female

Forced vital capacity (FVC)

4.8 L 3.7 L

Tidal volume (Vt) 500 mL 390 mL

Total lung capacity (TLC)

6.0 L 4.7 L

Measuring lung function

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.

Respiratory dysfunction in multiple sclerosis Tzelepis and McCool 2015

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

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

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

Optimal medical management

• Vaccinations • Exercise and prevention • Smoking cessation • SLT and nutritional assessment • Oral secretion management • Mucolytics • Reflux management • Antibiotics • Early detection of chest infections and use of

rescue packs

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

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

• Expiratory Phase

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

Surveillance Monitoring

Lung Volume Recruitment Bag

Mechanical Aids

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

Ethical Dilemma’s

• Resus status

• Intubation status

• Tracheostomy

• Tracheostomy and ventilation

• Non-Invasive Ventilation

• Access to Airway Clearance

• Advanced Care Planning

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

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

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