Session 4: Living with and managing nocturnal hypoventilation in MND

Living with and managing nocturnal hypoventilation in MND Ventilation has to be supported… or less commonly The airway supported How do we do it?

It used be with one of these… Or one of these..

Then it was with one of these…

And now with one of these

What is NIV? Non-invasive ventilation (NIV) refers to the provision of ventilatory support through the upper airway using a mask or similar device. Initially and most frequently at night

Two types of NIV CPAP: Continuous positive airway pressure splints upper airway and recruits unused alveoli. (OSA, Acute type 1 respiratory failure) BiPAP: Bilevel Positive airway pressure (type 2 respiratory failure) (nippy, nippv, vpap) When used outside of hospital NIV is often referred to as Home Mechanical Ventilation (HMV)

How does BiPAP help ? generally NIV supports the patients own breathing increases tidal volume supports and rests tired respiratory muscles supports upper airway improves sleep efficiency and quality improves mortality and morbidity improves quality of life 8

When to introduce in MND Best time to introduce is when people have symptoms of sleep disordered breathing or nocturnal hypoventilation. Few people tolerate NIV without symptoms. Some patients, particularly those with MND, have what appears to be normal lung function and normal sleep study. Caveat: Not everyone will want ventilation or tolerate it. We are trying to improve quality of life not worsen it.

Bilevel ventilation Bilevel devices are designed to deliver inspiratory positive airway pressure (IPAP) when a patient takes breath in, pushing air into lungs This is followed by a lower expiratory positive airway pressure (EPAP) that allows patient to exhale Bilevel does not allow specific tidal volume Numerous devices

What is IPAP and EPAP? IPAP – pressure on inspiration to increase tidal volume size. This will ensure sufficient removal of carbon dioxide and will aid breathlessness EPAP – splints airway open during expiration to overcome obstruction or airway collapse. This aids gaseous exchange – aids oxygenation.

Pressure Support or Pressure Control? Senses breathing pattern of patient Delivers IPAP and EPAP Synchronises with patient Has timed back up breaths should respiratory rate drop PRESSURE CONTROL Provides pressure support with time being added Sometimes more tolerable in neuromuscular disease Added length of time of IPAP allows more time for carbon dioxide removal

NIPPY 3+

Interfaces Proper fit paramount Nasal/facial Full face preferable if mouth breather Total facemask also available

Interfaces

Sizing gauge

Does it work? In short…Yes

General outcomes mortality excellent outcome at 5 years for post polio syndrome 100% survival rate over 50% of those with DMD now survive to over 25 because of HMV excellent outcome in stable muscular skeletal and neuromuscular disease (73% of kyphoscoliosis pts 5 year survival (60% O2 alone) improving mortality in MND (200 days median improvement in length expectancy) poorer outcome in bronchiectasis, CF and COPD.

The evidence NB Probability of continuing NIV is equivalent to survival as the main reason for discontinuation was death. (Simonds and Elliott 1995)

The evidence DMD Ishikawa Y et al Neuromusc Disord 2011;21:47-51

The Evidence in MND Non-invasive ventilation Can improve QoL and length of life in non-bulbar MND Can Improve QoL but not length of life in severe bulbar MND

Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial Bourke et al 2006

Oxygen in MND Target saturation range is 88-92%. Only employ oxygen to keep patients in that range.

Hypoxaemic drive Control of ventilation is complex but includes a hypercapnic respiratory drive. A small percentage of patients who hypoventilate can have chronically high CO2. Such patients become insensitive to CO2 and use a hypoxaemic drive to their respiration. As paO2 increases, ventilation will fall raising paCO2 and decreasing pH.

VQ mismatch due to increased alveolar oxygen Poorly ventilated alveolar capillary units become hypoxic and therefore poorly perfused due to hypoxic pulmonary vasoconstriction. If widespread, then vasoconstriction across the pulmonary circulation.

VQ mismatch due to increased alveolar oxygen High flow oxygen improves alveolar hypoxia and therefore pulmonary vasoconstriction. The alveolar units remain poorly ventilated and CO2 pours into the blood stream, raising paCO2 and decreasing pH. The patient becomes narcosed and can die of acidaemia.

Oxygen in MND Target saturation range is 88-92% Only employ oxygen to keep patients in that range Oxygen alerts and venture packs may help acute teams

Living with and managing a weak cough To support a weak cough you need to: support the mucocillary escalator support the inspiratory muscles support the vocal cords support the expiratory muscles or all of these.

European Neuromuscular Workshop on Airway Clearance Techniques 3rd – 5th March 2017 Lack of comprehensive and comparable research Very few recommendations that can be used by all people with NMD Clinicians, researchers and consumer representation from 12 countries to develop a consensus approach for airway clearance in NMD.

Suggested strategy for initiation of cough augmentation techniques

Supporting the mucocillary escalator mobilise the phlegm hydration mucolytics breathing techniques. the mucocillary escalator video

Supporting the inspiratory muscles Breath stacking It allows patients to achieve an inspiratory lung volume close to total lung capacity. FRC = functional residual capacity; TLC = total lung capacity. Modified by permission from McKim D. Canadian Respiratory Journal 2008:15.

Supporting the inspiratory muscles Breath stacking Lung Volume Recruitment (LVR) bag Series of stacked breaths (with no breath out between Inspirations) Single breath (with expiration/ cough)

Contraindications Haemotypsis Pneumothorax Bullae Care in COPD Raised ICP Impaired consciousness/inability to communicate Inflated tracheostomy cuff/ET tube Tracheoesophageal fistula Severe bulbar insufficiency Care if treating after meals/Stop feed

Supporting the inspiratory muscles Breath Stacking Lung Volume recruitment bag. Glossopharyngeal Breathing

Supporting the expiratory muscles Manual Assisted Cough Own and patient size? Eight of bed? Strength, pain relief? Suction?

MAC Precautions Indwelling abdominal or pelvic catheters Patients with severe scoliosis PEG’s/RIG’s Contraindications Rib fractures Other chest injuries Severe osteoporosis Abdominal surgery/trauma Untreated haemoptysis Clotting disorders Unstable spine Paralytic ilieus Pregnancy

Doing all three: Mechanical insufflator/exsufflator Emerson Cough assistor Nippy Clearway E 70 Cough assistor ‘The MI/E uses positive pressure to promote maximal lung inflation followed by an abrupt switch to negative pressure to the upper airway – the rapid change stimulates the flow changes that occurs during a cough’ (Chatwin et al 2003)

MI-E Utilisation Introduce when PCF < 160l/min Can be used via FM, mouthpiece, ETT and trache Manual and automatic modes Acute and community use +/- 0-60cmH20 in/exsufflation pressures Need insufflation pressure sufficient to reach MIC Need expiratory flow bias to move secretions (10-20cm H20 difference)

Contraindications Bullous emphysema Undrained pneumothorax or pneumo-mediastinum CV instability Tracheoesphageal fistula Recent or existing barotrauma Spinal instability Acute pulmonary oedema Acute lung injury Facial trauma

Considerations In MND with bulbar dysfunction Manual techniques more effective than MI-E in early stages of disease Recognised that MI-E becomes more effective with disease progression Both inspiratory and active expiratory phase of MI-E can lead to dynamic upper airway narrowing and/or closure resulting in a reduction rather than the desired improvement in cough flow efficacy (Anderson et al 2017)

Suggested strategy for MI-E in bulbar MND (Anderson et al 2017)

Discharge planning Family/Carers to be trained and competency framework completed Instruction leaflet with written document of settings given to pt District Nursing to provide portable suction machine Provide handover to community/palliative care physiotherapist Email prescription to Respicare and request follow up visit re maintenance of machine/consumables

Increasing muscle weakness Expiratory techniques Schematic representation of the management of cough augmentation (Chatwin & Simonds 2007) Increasing muscle weakness Combination of all techniques Cough assist Combination of inspiratory and expiratory techniques Inspiratory techniques: Pressure breathing (IPPB) Breath stacking Non invasive ventilation (NIV) Intermittent positive pressure breathing (IPPB) Expiratory techniques Cough assist techniques Worsening peak cough flow