Managing Respiratory Symptoms in Advanced MS – Practical

Monday 7th November 2016 Rachael Moses

Consultant Physiotherapist Complex Ventilation and Airway Clearance

@rachaelmoses rachael.moses@lthtr.nhs.uk

Ineffective cough as a result of weakness and ensuing restrictive lung

disease

Restrictive lung disease as a result of respiratory

muscle weakness and spinal deformity

Atelectasis as a result of secretion retention and restrictive lung disease

Chronic aspiration as a result of dysphagia and

exacerbated by an ineffective cough

Immobility as a result of muscle weakness or

disco-ordination

Identify what you need to treat

I cant take a deep breath

I cant cough

I cant talk for long

I’m sick of getting chest

infections

I get short of breath

Identify what you need to treat

Reduced FVC Reduced MIP

Reduced MEP

Reduced PCF Repeated

chest infections

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

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

• This is often repeated 4-6 x in same treatment cycle

• 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

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)

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.

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.

When to consider MI-E

• When combined MIC/MAC fail to produce a PCF > 160

• If MIC/MAC are ineffective in clearing secretions

• If a patient has inadequate carer support to provide regular MIC/MAC

• For patients who have regular hospital admissions with aspiration pneumonia

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

Need more help?

• http://www.irrd.ca/default.asp

• http://www.icid.salisbury.nhs.uk/ClinicalManagement/SpinalInjuries/Pages/AssistedCoughing.aspx

• http://www.acprc.org.uk/

• https://www.networks.nhs.uk/nhs-networks/silvah

• rachael.moses@lthtr.nhs.uk

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 rachael.moses@lthtr.nhs.uk

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