An ideal ventilator for neuromuscular patients. F. Lofaso, Raymond Poincaré Hospital Inserm U 1179

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1 An ideal ventilator for neuromuscular patients F. Lofaso, Raymond Poincaré Hospital Inserm U 1179

2 Neuromuscular diseasescausing progressive respiratoryfailure Cerebral diseases: strokes, tumors. Brainstem:primaryhypoventilation, polio, multiple sclerosis Motor nerves: ALS, SMA Neuromuscular juction: myasthenia gravis.. Myopathies : musculardystrophy, myotonicdystrophy, Pompe disease.. Respiratory failure is a major cause of morbidity and mortality Benditt and Boitano, AJRCCM 2013

3 Pathogenisof respiratoryfailure in NMD Inspiratory Muscle Weakness Expiratory Muscle Weakness Upper airway Muscle Weakness Ventilatory Dysfunction Cough Dysfunction Swallowing Dysfunction Neuromuscular Respiratory Failure Benditt and Boitano, AJRCCM 2013

4 The ventilator management must be tailored to the needs and desires of the individual patient It should be effective on breathing, swallowing and coughing functions. To prolong survival and noninvasive techniques it should be tailored to the patient s desires.to improve quality of life : Benditt and Boitano, AJRCCM 2013

5 The French welfare system standard : Ventilator requirement depends on MV duration MV < 12h: Home-care ventilator support device (ISO ) MV> 12h: Home care ventilator for ventilator-dependant patient with alarm sound and internal battery (ISO ) MV>16h: an external battery pack and a back-up ventilator must be provided

6 Patients desires differ accorging to severity/ MV duration Regarding the ventilator technical characteristics Very important 5 4 Low noise 3 Small size Low weight 2 Battery autonomy Not important 1 0 noct NIV noct + d NIV Trach Adaptability to wheelchair Lofaso et al, Respiratory Care 2014

7 Nocturnal ventilation assistance Whichmode of MV?

8 Modes of mechanicalventilation Volume-targeted Constant flow Guarantees adequate MV VT will not change even if respiratory system mechanic changes Pressure-targeted Variable flow. Easy to use/well tolerated. Can compensate for minor leaks Highermeanairwaypressure If same VTis used in both modes, P-mode is associated with lower maximal Paw

9 Nocturnal NIV in NM Volume-vs Pressure-targetedmode ChochraneReview Reversal of daytime hypoventilation kpa Annane et al, Chochrane 2014

10 Nocturnal NIV in NM Volume-vs Pressure-targetedmode ChochraneReview Maximal pressures cmh 2 O Annane et al, Chochrane 2014

11 Nocturnal NIV in NM Volume-vs Pressure-targetedmode ChochraneReview Nocturnal SaO 2 minutes below 90% Sameresultsfor AHI : in favourof Volume TargetedMode Annane et al, Chochrane 2014

12 Nocturnal ventilation assistance conclusion Volumetric modes better improve respiratory function during sleep than the Pressure targeted modes Annane et al, Chochrane 2014

13 Daytimeventilation assistance Battery life effectiveness? Interface/Mode? How to reduce swallowing dysfunction? How to reduce cough dysfunction?

14 Batterylife withexternalbatterypack Differences between ventilators Leaks increase battery life during VC/ decrease battery life during PSV Battery life decreases when respiratory rate increases(but it is not proportionnal) Falaize et al, Respiratory Care 2014

15 Batterylife withexternalbatterypack 72:00:00 60:00:00 48:00:00 Eole 3 XLS 36:00:00 24:00:00 Elisée 150 Monnal T50 PB560 Vivo 50 Trilogy :00:00 0:00:00 VC - fr 10/min VC - fr 20/min VC 15/min + leaks PSV 15/min PSV 15/min + leak

16 Daytimeventilation :Interfaces/Modes 100 Interfaces Percent of patients Mouthpiece Other NIV interfaces 0 < 8 [8-12[ [12-20[ 20 Mechanical ventilation duration (h/24h) Percent of patients Modes of MV Barometric Volumetric Hybrid 0 Nocturnal NIMV N + Daytime NIMV N + DaytimeIMV Lofaso et al, Respiratory Care 2014

17 Daytimeventilation withmouthpiece Boitano and Benditt, Respiratory Care 2005 Benditt and Boitano, AJRCCM 2013

18 A specificmouthpieceventilation mode for thisdevice(mpv) The «Kiss» trigger : patient connection is detected by its effects on a constant small flow. Then insufflation is delivered An interlocking support system combines with the breathing circuit to make access easy for patients An alarm which notifies absence of spontaneous breath within a selected period, thus no back-up rate is necessary

19 Benefitsof mouthpieceventilation ranked by priority Followed by : 5) better vision, 6) reduction in skin injury, 7) facilitation of swallowing, 8) decrease of aerophagia Khirani et al, Respiratory Care 2014

20 Breathing/Swallowing interaction Swallowing characteristics in healthy subjects SUBMENTAL - EMG LARYNX MOTION THORAX MOTION Pharyngeal activity(millisecond) Healthy Subject Gross et al JAP 2003

21 Breathing/Swallowing interaction Ventilatorydysfunction in NM disorders and in COPD may be associated with swallowing disorders and dysfunctional breathing-swallowing interactions In NM disorders Shaker et al, American Journal of Physiol 1992 Terziet al, AJRCCM 2007 Gross et al, AJRCCM 2009 Terzi et al, Neuromuscul Disord 2010 SUBMENTAL - EMG LARYNX MOTION THORAX MOTION Normal Subject Neuromscularpatient Terziet al AJRCCM 2007

22 How to improveeatingin patients with littleventilator-free breathing? Terziet al, CCM 2014 Modifiedhome ventilator (Elysée150, ResMed, San Diego, USA) Switch activation witholds ventilation Garguilo et al, 2015

23 Results All patients found the device useful No episode of aspiration No episode of auto-triggering with device Swallowing comfort stable under NIV Dyspnea sensation during swallowing inductive plethysmography BORG dyspnea scale(/10) P = ml 10 ml 0 SB NIV Garguilo et al, 2015

24 Results 5 ml-bolus 10 ml-bolus Yogurt ANOVA (p) SB NIV SB NIV SB NIV Size effect Texture effect NIV effect Number of swallows (per bolus) 2.0± ± ± ± ± ± Duration of swallowing (sec) Swallowing fragmentation (respiratory events per bolus) 5.4± ± ± ± ± ± ± ± ± ± ± ± % of swallows followed by an inspiration 43±23 10±8 46±24 18±19 46±21 21 ± < Garguilo et al, 2015

25 Results 5 ml-bolus 10 ml-bolus Yogurt ANOVA (p) SB NIV SB NIV SB NIV Size effect Texture effect NIV effect Number of swallows (per bolus) 2.0± ± ± ± ± ± Duration of swallowing (sec) Swallowing fragmentation (respiratory events per bolus) 5.4± ± ± ± ± ± ± ± ± ± ± ± % of swallows followed by an inspiration 43±23 10±8 46±24 18±19 46±21 21 ± < Garguilo et al, 2015

26 Results 5 ml-bolus 10 ml-bolus Yogurt ANOVA (p) SB NIV SB NIV SB NIV Size effect Texture effect NIV effect Number of swallows (per bolus) 2.0± ± ± ± ± ± Duration of swallowing (sec) Swallowing fragmentation (respiratory events per bolus) 5.4± ± ± ± ± ± ± ± ± ± ± ± % of swallows followed by an inspiration 43±23 10±8 46±24 18±19 46±21 21 ± < Garguilo et al, 2015

27 Coughassistance Very important Patients vs Prescribers desires differ Not important Synchronization Efficacy>Comfort Integrated Cough assistance Lofaso et al, Respiratory Care 2014

28 Cough assistance Mechanical in-exsufflation vs Breath stacking IPPB With or without Manually Assisted Cough (MAC)

29 Coughassistance Peak Cough Flow (L/sec) P<0.01 P<0.01 Baseline MI-E IPPB + MAC MI-E + MAC Lacombe et al, Respiration 2014

30 Coughassistance Flow (L/sec) Beginning of inspiration insufflation flow from upper airway volume reduction exsufflation glottic opening peak cough flow Mask Pressure (cmh 2 O) positive pressure sec Lacombe et al, Respiration 2014

31 Coughassistance positive pressure during cough with MI-E + MAC Peak Cough Flow (L/sec) Baseline MI-E MI-E + MAC IPPB + MAC 270 l/min 180 l/min Lacombe et al, Respiration 2014

32 Conclusion Ventilators should include : A Volume Targeted Mode Efficient Batteries Specific modes for: mouthpiece ventilation eating/swallowing coughing These adaptations should be evaluated