NEUROMUSCULAR DISEASE LISA F. WOLFE, MD A SSOCIATE P ROFESSOR IN M EDICINE -P ULMONARY AND N EUROLOGY N ORTHWESTERN U NIVERSITY F EINBERG S CHOOL OF M EDICINE C HICAGO , IL Dr. Lisa Wolfe is currently an Associate Professor in Medicine-Pulmonary and Neurology in the Northwestern University Feinberg School of Medicine. She is a member of the Neurology Faculty in both Sleep and Neuromuscular Medicine. She also works as the Director of Respiratory Care at the Rehabilitation Institute of Chicago, and is a pulmonary consultant to the Les Turner ALS foundation, the Sleep Medicine program of the Lurie Children’s Hospital, and the Muscular Dystrophy Association of Chicago. Dr. Wolfe obtained her Medical Degree from The Ohio State University, and trained in Internal Medicine at Northwestern University and then stayed on to complete fellowship training in Pulmonary, Critical Care, and Sleep Medicine. She has remained at Northwestern as faculty and developed a special interest in home based ventilation focusing particularly on the needs of those with neuromuscular disorders. Her current research focus on new trends in home ventilation including advanced modes such as servo-ventilation and volume assured pressure support as well as the use of alternative devices such as diaphragmatic pacing. OBJECTIVES: Participants should be better able to: 1. Consider the options available for advanced speech and communications for those patients with respiratory impairment in the setting of neuromuscular disease. 2. Establish treatment options utilizing appropriate new modes of non invasive ventilation for those patients with respiratory impairment in the setting of neuromuscular disease. 3. Develop broad range of inputs to best evaluate the need for mechanical ventilation with mask for those patients with respiratory impairment in the setting of neuromuscular disease. 4. Utilize non invasive ventilation download applications to encourage/ support self care for those patients with respiratory impairment in the setting of neuromuscular disease 5. Recognize the need for multiple modality airway clearance plans for patients with respiratory impairment in the setting of neuromuscular disease. SATURDAY, MARCH 5, 2016 8:30 AM
3/8/2016 NAMDRC 2016: The Role of Technology in Pulmonary, Critical Care and Sleep Medicine Neuromuscular Disease Lisa F. Wolfe MD Associate Professor of Medicine Northwestern University Feinberg School of Medicine Chicago , Illinois Dr. Wolfe has received research grants from ResMed, Synapse Biomedical, Hill Rom, and Respironics, but these do not create a conflict related to the following presentation. 1
3/8/2016 COI • No conflicts related to the topic of this talk • No of label uses will be discussed in this talk • Conflicts with research studies and consulting including: – ResMed – Philips Respironics – Synapse Biomedical – Hill- Rom Neuromuscular Disease: The Role of Technology The case for full mechanical ventilation Technology for the NMD patient with non-invasive interface • • Airway clearance technologies Battery – New cough assist technologies – Safety – New vibration based technologies – Portability • Communication technologies • Sip Ventilation – Leak speech – CO2 – Voice banking – Mortality – Gaze Device • Breath stacking • New/ Old NIV device technologies – LVR – Biphasic cuirass ventilation (BCV) – Cough • New NIV Devices – Swallow – ST devices – VAPS – Communication – ASV • 24 hour NIV • Device monitoring technologies • Cons 2
3/8/2016 Goals of Respiratory Care in Neuromuscular Disorders Weakness related respiratory Areas of weakness/ challenge complications • Glottic weakness • Secretion clearance • Swallow impairment – Nasal • Diaphragmatic – Chest – Weakness – Control of ventilation – Oral • Accessory muscle drop out • Atelectasis – Inspiratory muscles – Expiratory muscles – Lung volume recruitment • Core muscle weakness – Micro aspiration • Musculoskeletal • Communication failure – Scoliosis – Seating/ positioning • Hypoventilation – Chest wall Question 1 • For patients with neuromuscular disorders an airway clearance plan is helpful. All of the above are important outcomes of regular airway clearance but which is the most important: 1. Improve voice amplification 2. Reduction in work of breathing 3. Mitigate risk of pneumonia 4. Palliate fatigue with eating 3
3/8/2016 QUESTION 1 For patients with neuromuscular disorders an airway clearance plan is helpful. All of the above are important outcomes of regular airway clearance but which is the most important: 55% 1. Improve voice amplification 42% 2. Reduction in work of breathing 3% 0% 3. Mitigate risk of pneumonia 1. 2. 3. 4. 4. Palliate fatigue with eating Airway Clearance Device Goals Device Options • Vibration • Vibration • Secretion Consistency: – Airway Vibration – at the lips – Chest wall Vibration – Drying vs Waterlike • Drying vs Wetting • Lung Volume Recruitment – Nebulization solution • Expiratory Support – Humidity – Saliva control • Lung Volume Recruitment – Ambu bag breath stacking – Mechanical “cough” assist – Breath stacking on vent 4
3/8/2016 Airway Clearance: Vibration Airway Vibration Chest Wall Vibrations • Pulm Rehab programs have • High Frequency Chestwall used devices where the Oscillation ( HFCWO) has most commonly been used in the setting vibrations require significant of bronchiectasis patient effort – Concerns in the setting of NMD • Hypersecreation sputum is not an – Concerns in the setting of NMD issue? – It can be an issue with: • Insufficient flow » Siallorhea • Weak “embouchure” » Swallow issues with micro aspiration – Devices – Advantages for those with: • PEP: • Cognitive impairment • Bulbar dysfunction – Flutter ,™ Acapella ™ – Devices AerobikA ™ and Cornet™ • Therapy Vest™ • PEP + Acoustic: • AffloVest – Vibralung ™ Airway Clearance: Vibration Airway Vibration • Pulm Rehab programs have Electro-Mechanical-Acoustical Airway Clearance: used devices where the vibrations require significant Promotes muckinesis by vibrating the column of gas in the airways patient effort with sounds at different – Concerns in the setting of NMD frequencies. It is unclear if this • Insufficient flow technology will have a role in NMD • Weak “embouchure” but there is a promise which other devices in the category don’t have. – Devices Patient generated flow is not • PEP: needed. – Flutter ,™ Acapella ™ AerobikA ™ and Cornet™ • PEP + Acoustic: McPeck M. Respir Ther 2014; 9(Oct-Nov): 45-47. – Vibralung ™ 5
3/8/2016 Airway Clearance: Vibration Chest Wall Vibrations Medical costs per member per month (PMPM) $10,000.00 - Total cost↓ by $1,111 (12.4%) (p=0.035) * $9,000.00 - Inpatient admission costs ↓by $1,812 (p=0.003) $8,000.00 - Pneumonia costs ↓ by $326 (p=0.031) $7,000.00 $6,000.00 * $5,000.00 $4,000.00 * $3,000.00 Pre-HFCWO * $2,000.00 Post-HFCWO * $1,000.00 $0.00 Total Medical Total Rx Allowed Total Medicial & Rx Inpatient Inpatient Allowed Pulmonary Pneumonia PMPM Allowed PMPM PMPM Allowed PMPM admissions/1000 PMPM Diagnosis PMPM • Data (N=426; 2007-11) obtained from two large databases of commercial insurance claims. Patient with NMD codes and started on HFCWO • Outcomes included total medical costs per member per month, pharmacy costs per member per month, inpatient hospitalizations, emergency room visits, and claims for pulmonary diagnoses or pneumonia. Lechtzin NL; Annals of the American Thoracic Society InPress; 2016 Airway Clearance: Secretion Consistency Thick Waterlike • Causes • Causes – Dehydration – Medications Dry palate – Over drying – • Mestinon and simultaneous medications – Immobile tongue drooling due • Anticholinergics to a weak – Mouth breathing tongue • Treatments • Treatments – Saline nebs WET – Humidifiers DRY – Papaya/ Pineapple/ Red grape juice 6
3/8/2016 Airway Clearance: Secretion Consistency Thick Waterlike New therapies to reduce saliva • Botox • Ligation • Suction • Ablation • Radiation • Future drug: Tropicamide Films Airway Clearance: Secretion Consistency Thick Waterlike New therapies to reduce saliva • • Suction • Botox Ligation • • The most effective way Complications: • No Bite V of treating sialorhea – Airway obstruction – Respiratory insufficiency – Sialoadenitis – Persistent fistula – floor-of-mouth cyst – Ranulas • Deep suctioning options Squires, N. Dysphagia (2014) 29:500 – 508 • Good for Lakraj, AA Toxins 2013, 5, 1010- Oral Care 1031 Greensmith, A. PLASTIC AND RECONSTRUCTIVE SURGERY Vol. 116, No. 5 Oct. 2005 7
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