Ventilator Management in Neuromuscular Diseases 27/08/2018 Ventilator Management in Neuromuscular Diseases From Non-invasive to Invasive

Objectives Discuss respiratory disorders in neuromuscular diseases (NMD) Discuss impact of ventilatory assistance on NMD Review indications and contraindications of non-invasive ventilation (NIV) in NMD Discuss initiation of NIV in NMD Review indications of invasive ventilation in NMD

Pathophysiology of Ventilatory Failure Reduced capacity Weakness of respiratory muscles Capacity Drive Increase load Atelectasis UAW resistance due to bulbar weakness LOAD

Evolution of Respiratory Insufficiency in NMD Normal Breathing Chest Infections FVC < 40% pred REM related SDB NREM and REM related SDB FVC < 20% pred Daytime Hypercania AK Simmonds Chest 2006 Death

Signs and Symptoms Cardiac Signs Cardiopulmonary CNS Constitutional 27/08/2018 Vital Signs Constitutional Cardiopulmonary CNS Sleep Respiratory Signs Cardiac Signs Signs and Symptoms  Constitutional       Generalized fatigue       Weakness    Cardiopulmonary       Dyspnea Orthopnea       Lower extremity edema Secretion retention    CNS       Early morning headaches Mood disturbances       Daytime hypersomnolence Psychiatric problems    Sleep       Restless sleep Enuresis       Nightmares Frequent arousals Signs    Vital Signs       Tachypnea Tachycardia    Respiratory       Accessory chest and abdomen muscle use       Paradoxical breathing pattern       Diminished excursion    Cardiac       Distended neck veins Increased P2       Edema Cyanosis

Signs and Symptoms Cardiopulmonary CNS Constitutional Sleep 27/08/2018 Dyspnea Orthopnea Lower extremity edema Secretion retention Cardiopulmonary Dyspnea Orthopnea Lower extremity edema Secretion retention Early Morning headache mood disturbances daytime hypersomn Psychiatric problems Early Morning headache mood disturbances daytime hypersomn Psychiatric problems CNS Weakness Generalized Fatigue Weakness Generalized Fatigue Constitutional Signs and Symptoms Restless sleep Frequent arousals nightmares Sleep Restless sleep Frequent arousals nightmares Tachycardia Tachypnea Vital Signs Tachycardia Tachypnea  Constitutional       Generalized fatigue       Weakness    Cardiopulmonary       Dyspnea Orthopnea       Lower extremity edema Secretion retention    CNS       Early morning headaches Mood disturbances       Daytime hypersomnolence Psychiatric problems    Sleep       Restless sleep Enuresis       Nightmares Frequent arousals Signs    Vital Signs       Tachypnea Tachycardia    Respiratory       Accessory chest and abdomen muscle use       Paradoxical breathing pattern       Diminished excursion    Cardiac       Distended neck veins Increased P2       Edema Cyanosis Distended neck veins Edema Cyanosis Accessory muscle use Paradoxical breathing pattern Diminished excursion Respiratory Signs Distended neck veins Edema Cyanosis

Average age of DMD survivors from 19 to 25 27/08/2018 Average age of DMD survivors from 19 to 25 85% 73% Plateau effects over the first 5 – 6 years of ventilation tx Simmonds at al. 1998

Survival of ALS patients without Bulbar Involvement 27/08/2018 Survival of ALS patients without Bulbar Involvement Broken Line: Initiation of NIV therapy before protocol initiation Solid Line: Initiation of NIV therapy post protocol initiation Protocol=early respiratory evaluation Survival times with NIV therapy in patients without bulbar impairment according to the initiation of NIV therapy before protocol initiation (broken line) or after protocol initiation (solid line). Ferrero et al. 2005

Survival of hypercapnic ALS patients with bulbar impairment Broken Line: Intolerance of NIV Solid Line: Tolerance of NIV Ferrero et al. 2005

How can NIV possibly help NMD patients? Prevent respiratory decompensation Alter chest wall/lung growth characteristics – NIV hyperinflates, stretches lung Rest respiratory muscles Control nocturnal hypoventilation with or without symptoms Treat chronic hypercapnic respiratory failure Palliate symptoms Improved quality of sleep Enhanced sleep efficiency More stage 3 and stage 4 sleep

Indications of NIV in NMD patients Signs and Symptoms of respiratory insufficiency AND Daytime hypercapnia PCO2 > 45 Nocturnal hypoventilation (SpO2 < 88%, for > 5minutes) FVC<50% predicted MIP<60% predicted Repeated hospitalizations from chest infections Cor pulmonale Exceptions Significant daytime hypercapnia of PCO2 > 50, do not need symptoms as symptoms might not be present

ALS Practice Guidelines: Respiratory management

44 patients with FVC > 75% 12 patients with FVC > 75%, not on NIV

Patients with FVC < 75% on NIV 27/08/2018 Patients with FVC < 75% on NIV FVC Rate of decline in FVC slower in patients with NIV with significant differeces Patients with FVC < 75% without NIV Months

When to consider NIV is not suitable? Relative contraindications Severe swallowing impairment Need for full time ventilatory assistance, unless patient prefers NIV Absolute contraindications Upper airway obstruction Uncontrolled secretion retention Inability to cooperate Inability to cough effectively even with assistance Inability to fit mask

NIV Equipment Interface Nasal mask Full face mask Nasal Pillows Anti re-breathing valves Interface Nasal mask Full face mask Nasal Pillows Mouth piece Quick release: minimize risk of asphyxiation and aspiration when vomitting Note:Smart start/stop is disabled on FFM

Home NIV vs Home IV users Trach FFMV nasal

Mask Fitting Use mask template to optimize fitting The Mask-Fitting Feature allows the patient to check the quality of their mask fit and adjust if necessary in order to minimize leak Air pressure is delivered for a period of 3 minutes In CPAP mode: Pressure = set treatment pressure or 10 cm H2O, whichever is greater In S, ST, & T modes: Pressure = set EPAP pressure or 10 cm H2O, whichever is greater

Mouthpiece Ventilation Beneficial for patients with excessive mouth leak with nasal masks 24/7 ventilatory assistance with little or no vital capacity

Ventilators Bilevel devices Light Portable Simple to operate Quiet Low cost Ideal for non dependent patients/nocturnal use Variable tidal volume Simple to use Leak compensation

Multi mode ventilators Wide range of Alarms Internal battery Invasive and noninvasive options available Volume targeted modes allow patient to breathe stack ideal for patients with very poor cough Ideal for ventilator dependent users

Pressure limited Volume limited

Eurovent Study 2002

Initiating Home NIV: Step 1 Set goals with patients Individualize home care plans based on goals Settings of initiation: Hospital Sleep lab for night titration Physician’s Office/Clinic Home

Possible Goals of Home NIV Alleviate symptoms Stabilize and/or improve gas exchange Prevent nocturnal hypoventilation Improve sleep quality Enhance quality of life Reduce complications of respiratory failure (polycythemia, cor pulmonale) Provide comfort and convenience Simplify care with optimal portability Avoid invasive ventilation/tracheostomy Minimize cost of long-term care and burden on caregivers Prolong survival

Home Ventilation Initiation ADAPTATION PERIOD Weekly visit for few weeks Monitoring and Follow ups Monthly visits STABLE 4 to 6 monthly follow ups

NIV Titration: for NMD with no Respiratory Distress Begin with low settings IPAP 8 EPAP 4 or TV 6 ml/kg, RR 12 – 16 bpm Adaptation period: weekly visit for first few weeks Desensitization Gradually increase settings to achieve 8 ml/kg if tolerable Good Chest expansion No accessory muscle use Over days to weeks of titration Settings increased as tolerated: Improve ABG Improve symptoms such as daytime sleepiness, morning headache, fatigue Priority: Comfort

Monitoring Consider overnight titration monitoring SpO2 and ETCO2 for FVC < 30% predicted Monitor daytime ABG after more than 4 hours of use per day Consider PSG under the following circumstances: Pressure titration when initiating NPPV To evaluate for obstructive sleep apnea     Obese, snorer, or hypersomnolence To seek sleep hypoventilation in     Symptomatic patient without daytime hypercapnia or severe pulmonary function defect     Asymptomatic patient with daytime hypercapnia or severe pulmonary function defect*

<270L/min = insufficient to remove secretions

Use of Supplemental Oxygen - Caution “ The use of supplemental O2 (oxygen) should never be prescribed to the DMD patient to treat low SaO2 levels without testing to determine if the patient suffers from hypoventilation. End-tidal CO2 (carbon dioxide) levels or blood gas CO2 should be checked to determine if the patient is retaining CO2. Improper use of supplemental oxygen can mask hypoventilation, decrease breathing rate, and cause breathing to stop!” “Monitoring the SaO2 level while having the DMD patient take several deep breaths can often act as a quick check for hypoventilation. If the SaO2 level rises above 95%, hypoventilation due to weak chest muscles is a likely cause. (Bach JR, Quest 1995; 2:1)”

Selection of Settings for NPPV Backup rate 12 to 16 bpm, ventilatory assistance for central apneas and ineffective triggers Over months of titration and adaptation – increase IPAP to achieve TV 10 – 15 ml/kg, IPAP may be 12 – 18 cmH2O Daytime PCO2 improvement: 40s to 50s Improve morning headaches Fatigue Daytime sleepiness Adjust EPAP 3 – 5 cmH2O, priority: tolerable Higher EPAP is necessary in OSA and COPD

What if PCO2 is high or PO2 is low during a follow up session? Increase Backup rate Increase IPAP Increase use of NIV Increase naps Assess for presence of air leaks in the night Assess for presence of apneas in the night, may need to increase EPAP

When to Consider Tracheostomy? Substantial Glottic Dysfunction Increased risk of aspiration Inability to clear secretions despite cough augmentation Inability to generate adequate cough flow despite aggressive cough assistance Recurrent pneumonia on full time NIVV with adequate cough assist Substantial elevate PaCO2, despite optimal full time NIV Patient preference Benditt . Respiratory Care 2006

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