Moderator: Michael Richardson, MD, FACP Presenters: Toni Chiara, PhD, MHS, MSPT Charles J. Gutierrez, PhD, RRT, FAARC Jim Hunziker, MSN, ARNP

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1 Moderator: Michael Richardson, MD, FACP Presenters: Toni Chiara, PhD, MHS, MSPT Charles J. Gutierrez, PhD, RRT, FAARC Jim Hunziker, MSN, ARNP 1

2 Disclosures This continuing education activity is managed and accredited by Professional Education Services Group. The information presented in this activity represents the opinion of the authors or faculty. Neither PESG, nor any accrediting organization endorses any commercial products displayed or mentioned in conjunction with this activity. Commercial Support was not received for this activity. 2

3 Toni Chiara, PhD., MHS., MSPT Has no financial interest or relationships to disclose Charles J. Gutierrez, PhD, RRT, FAARC Has no financial interest or relationships to disclose Jim Hunziker, MSN, ARNP Has no financial interest or relationships to disclose CME Staff Disclosures Professional Education Services Group staff have no financial interest or relationships to disclose. 3

4 At the conclusion of this activity, the participant will be able to: Describe changes that occur to the respiratory system due to SCI/D List diagnostic tests that can be used to diagnose pulmonary dysfunction Employ pulmonary exercise strategies that can be used to minimize the effects of pulmonary dysfunction due to SCI/D Utilize respiratory assistive devices with Patients with pulmonary dysfunction in SCI/D Summarize a novel pulmonary rehabilitation strategy to minimize mechanical ventilation in Veterans with SCI/D 4

5 Jim Hunziker, MSN, ARNP MS Center of Excellence - West 5

6 Consists of Breathing in (inspiration) Breathing out (expiration) Types of breathing Quiet diaphragm does 80% of work Active breathing accessory, internal intercostal, scalene, sternocleidomastoid, abdominal, shoulder and back muscles all assist diaphragm during deep or forced breathing 6

7 Issues occur when anything interferes with this process Decrease in elasticity of rib cage Mechanical blockage of bronchi/bronchioles Constriction when breathing in (Asthma) Floppy tubes that flatten on exhalation (COPD) Lack of signal to muscles of breathing SCI Neuromuscular disease (ALS, MS, Guillain-Barre Syndrome, etc.) 7

8 Neural Medulla: regulates respiratory depth and rate Pons: is the pacemaker of respiration Apneustic center: initiates respiration Pneumotaxic Center: inhibits respiration by blocking Apneustic Center signals Mechanical Stretch receptors Irritant receptors Juxtacapillary receptors Chest wall receptors Chemical receptors Central chemoreceptors Peripheral chemoreceptors 8

9 Diaphragm Flattens which sucks air into lungs Abdominal muscles Elevate sternum/ribs, increase front/back dia. (A-P) Accessory muscles Expand the ribcage Intercostals between the ribs Externals elevate ribs, internal pull together ribs Increase A-P diameter by 20% Stenocleidomastoid muscles go up into the neck, elevate rib cage 9

10 Accessory Muscles External Intercostals Diaphragm 10

11 Diaphragm and chest wall muscle weakness Reduction in vital capacity and inspiratory force Hypoventilation with increase in PaCO 2 Usually seen first at night AM headache, nocturnal awakening, night sweats, lethargy, depression Most notable with FVC < 50% 11

12 Abdominal and internal intercostal weakness Inability to close glottis Reduced peak cough airway flow Inability to clear secretions Atelectasis and infection 12

13 Internal Intercostals Abdominal Muscles 13

14 Low lung volumes Atelectasis Reduced lung compliance Stiffer chest wall Associated kyphoscoliosis Multiple lung infections 14

15 In MS, respiratory decline occurs more frequently than in the general population Respiratory involvement early in the course of the disease is mostly due to reversible neuromuscular failure Later in the course of the disease, bulbar weakness can lead to aspiration, pneumonia, and death 15

16 Common even in those with normal spirometry w/no complaints (31% of pts had abnormalities w/no Sx) People w/edss 5.5 have decreased ability to cough 3 In MS, most common cause of death (47.5%), and three times more likely than the general population to die of Pulm Disease 2,4 People w/ms more likely to have COPD 1 Night desaturation common (70%) in one study 47% of this group had normal spirometry Unknown cause for rest Correlation between MS and higher rate of Narcolepsy Higher sedative 5 and Baclofen use in this group 16

17 SCI Spinal cord is cut or injured, preventing signal for muscular contraction/relaxation to reach muscles Neuromuscular ALS Motor neurons degenerate in motor cortex and lateral spinal tract Progresses to LMN with destruction of anterior horn cells in spine MS Demyelination of nerve preventing or slowing down electrical signal to muscles 17

18 Intimately related to cough function Bulbar muscle involvement Portends poorer prognosis Difficulty in managing secretions Choking episodes and aspiration Pneumonia and respiratory failure 18

19 Increased Dyspnea Decreased Endurance Decreased Activity 19

20 Neuromuscular Disease Upper Airway Muscle Weakness Expiratory Muscle Weakness Inspiratory Muscle Weakness Aspiration Cough Impairment Respiratory Failure and Pneumonia 20

21 Initially, by Patient report Borg scale (10 point shortness of breath scale) 6 Minute Walk PFT s 21

22 Peak Cough Flow Normal > 500 lpm Worrisome < 200 lpm Maximal Expiratory Pressure Measured in the literature Questionable predictive value for clinical care 22

23 Improve and stabilize gas exchange Ameliorate symptoms Improve sleep quality Improve quality of life Extend survival 23

24 24

25 Encourage smoking cessation Rehabilitation: Traditional pulmonary rehabilitation Pulmonary device only rehabilitation Ventilatory support (Rehabilitation and vent support will be discussed over the rest of the program) 25

26 Maintain aerobic conditioning as long and as much as possible Arm ergometry Hand cycling Ambulation if safe Use of pedometer (give goals, change over time) Consider Inspiratory and expiratory muscle training Standard Pulmonary Rehabilitation if patient can participate 26

27 Consider in-clinic measurement of NIF, FVC, Peak Flow or Peak Cough Flow annually or more often if indicated Consider at home monitoring of Peak Flow or Peak Cough Flow Refer for sleep study as appropriate Teach Quad Coughing to caregivers Provide MIE (can also be used as non-invasive ventilatory support) 27

28 Invasive Ventilation -tracheotomy Non-Invasive Ventilation -negative pressure ventilation -diaphragmatic pacing -non-invasive positive pressure ventilation (NPVV) 28

29 Practice at VA Puget Sound: Any negative response on the following assessment should trigger a referral to speech pathology After assessment by speech pathology there may also be Barium swallowing study Direct endoscopic visualization 29

30 Swallowing evaluation Any negative response on this assessment should trigger a referral to speech pathology POSITIVE NEGATIVE THE PATIENT DOES NOT DOES complain about difficulty swallowing. THE PATIENT HAS HAS NOT been taking sufficient fluids and calories THE PATIENT NOT HAD HAD a recent history of aspiration pneumonia. THE PATIENT HAS NOT HAS had a recent feeding tube. THE PATIENT DOES NOT DOES have a tracheostomy. THE PATIENT DOES NOT DOES have signs of aspiration, i.e. chest congestion. THE PATIENT IS IS NOT adequately alert. THE PATIENT DOES DOES NOT speak clearly, without slurring. THE PATIENT S VOICE IS IS NOT clear, without wetness. THE PATIENT DOES NOT DOES pocket food in the mouth. THE PATIENT DOES NOT DOES have signs of neglect. THE PATIENT IS IS NOT swallowing saliva without need for suctioning. THE PATIENT IS NOT IS drooling. THE PATIENT CAN CANNOT produce a strong cough. THE PATIENT DOES DOES NOT chew and swallow readily with 1/2 tsp. ice chips. THE PATIENT DOES NOT DOES cough when given 10cc of water. THE LARYNX DOES DOES NOT elevate on swallowing. THE PT S VOICE DOES DOES NOT remains clear after each swallow. 30

31 1. Egesten A, Brandt L, Olsson T, Granath F, Inghammar M, Löfdahl CG, Ekbom A. Increased prevalence of multiple sclerosis among COPD patients and their first-degree relatives: a population-based study. Lung May-Jun;186(3): Epub 2008 Mar Tripodoro VA, De Vito EL. Management of dyspnea in advanced motor neuron diseases. Curr Opin Support Palliat Care Sep;2(3): Aiello M, Rampello A, Granella F, Maestrelli M, Tzani P, Immovilli P, Franceschini M, Olivieri D, Chetta A. Cough efficacy is related to the disability status in patients with multiple sclerosis. Respiration. 2008;76(3): Epub 2008 Mar Hirst C, Swingler R, Compston DA, Ben-Shlomo Y, Robertson NP. Survival and cause of death in multiple sclerosis: a prospective population-based study. J Neurol Neurosurg Psychiatry Sep;79(9): Buyse B, Demedts M, Meekers J, Vandaer L, Rochette F, Kerkhofs L. Respiratory dysfunction in Multiple Sclerosis: a prospective analysis of 60 patients. Eur Respir J, 1997,10:

32 RM Strength and Endurance verse Pulmonary Function in Veterans diagnosed with ALS, MS, and SCI Toni Chiara, PhD, MHS, MSPT

33 1. MIP < -60 cm H2O - 2/2 Diaphragm Muscle Weakness 2. Complain of Orthopnea a. Sensation of dyspnea when laying flat b. Nocturnal hypoventilation i. Poor sleep ii. Fatigue iii. Excessive daytime sleepiness iv.morning headdaches c. Exertional dyspnea d. Dyspnea at rest advanced weakness