Non-invasive ventilation in the management of motor neurone disease

1. Non-invasive ventilation in the management of motor neurone disease Implementing NICE guidance July 2010 NICE clinical guideline 105

2. What this presentation covers • Background • Scope • Recommendations • Costs and savings • Discussion • Find out more

3. Definitions • ALS • Bulbar symptoms • Interface • MIP • Non-invasive ventilation (NIV) • Orthopnoea • PaCO2 • SpO2 • SNIP • Vital capacity (VC)

4. Background:1 • Motor neurone disease (MND) is an incurable and progressive neurodegenerative condition • Characterised by progressive muscular paralysis and wastage • This results clinically in weakness of the bulbar, limb, thoracic and respiratory muscles • Incidence is approximately 29 per million population, affecting slightly more men Image reproduced by kind permission of Motor Neurone Disease Association

5. Background:2 • Respiratory problems are the main cause of death in patients with MND • No single test of respiratory function or of respiratory muscle weakness can be used to reliably predict the onset of respiratory failure or to identify the most appropriate timing for starting non-invasive ventilation • Non-invasive ventilation is usually used initially for intermittent support to relieve symptoms of hypoventilation at night

6. Scope • The guideline covers, in adults (aged 18 and over) with a diagnosis of MND: • The identification and assessment of respiratory impairment, to determine who should be offered non-invasive ventilation and when • Ongoing management of the use of non-invasive ventilation, and decisions on its continuation or withdrawal, including during end-of-life care • Specific information and support needs of patients and carers

7. Multidisciplinary team A multidisciplinary team should coordinate and provide ongoing management and treatment for a patient with MND, including regular respiratory assessment and provision of non-invasive ventilation

8. Information and support: 1 • Offer to discuss the use of non-invasive ventilation in a timely and sensitive manner • Include information, appropriate to the stage of illness, about: • possible symptoms and signs of respiratory impairment • natural progression of MND • respiratory function tests and results • benefits and limitations of interventions • how NIV can improve symptoms and prolong life • how NIV can be withdrawn • palliative strategies • Inform relevant clinicians of key decisions agreed

9. Information and support: 2 • Provide support and assistance to manage non-invasive ventilation, including: • training • assistance with secretion management • information on palliative strategies • offer of ongoing emotional and psychological support • Ensure that families and carers have: • an initial assessment, including ability and willingness to assist and training needs • the opportunity to discuss concerns with the multidisciplinary team

10. Monitor for respiratory impairment

11. Respiratory function tests: 1 • Assess baseline respiratory function at, or soon after, diagnosis • Measure: • SpO2 (measured by pulse oximetry) • then one or both of: • forced vital capacity or vital capacity • MIP and/or SNIP • If the patient has severe bulbar impairment or severe cognitive problems, measure SpO2 but omit other tests if interfaces are unsuitable

12. Respiratory function tests: 2 • Perform tests every 3 months, but this can vary depending on symptoms and signs, patient preference and rate of progression of MND • Perform arterial or capillary blood gas analysis if SpO2 is low • Refer urgently to a specialist respiratory service if PaCO2 is greater than 6 kPa Image reproduced by kind permission of Motor Neurone Disease Association

13. Respiratory function tests: 3 If any of the results listed below are obtained, discuss impact, referral and treatment options

14. Non-invasive ventilation– offering a trial • Offer a trial of non-invasive ventilation if symptoms and signs and test results indicate that the patient is likely to benefit • Discuss benefits and limitations • In patients with severe bulbar impairment or severe cognitive problems, only consider a trial if specific benefits are possible Image reproduced by kind permission of Motor Neurone Disease Association

15. Non-invasive ventilation – risk assessment and care plan Before starting non-invasive ventilation, the multidisciplinary team should, after discussion with the patient and their family and carers, : • carry out and coordinate a patient-centred risk assessment • prepare a comprehensive care plan Offer the patient and their family and carers a copy of the care plan

16. Non-invasive ventilation – starting and reviews • When starting non-invasive ventilation: • perform initial acclimatisation during the day • usually start regular treatment at night • gradually build up hours of use as • necessary • Continue non-invasive ventilation if clinical reviews show appropriate improvement • Discuss all decisions to continue or withdraw treatment with the patient, and family and carers if patient agrees Image reproduced by kind permission of Motor Neurone Disease Association

17. Patients with dementia • Base decisions on respiratory function tests on considerations specific to the patient’s needs and circumstances • Before a decision is made on the use of NIV, the neurologist from the multidisciplinary team should carry out an assessment that includes: • the patient’s capacity to make decisions and give consent • the severity of dementia and cognitive problems • whether the patient is likely to accept treatment • whether the patient is likely to achieve improvements in sleep-related symptoms and/or behavioural improvements • a discussion with the patient’s family and/or carers

18. Planning end-of-life care • Offer to discuss end-of-life care with the patient and (if the patient agrees) their family and carers, at an appropriate time and in a sensitive manner • Discuss: • planning • advance decisions to refuse treatment • what to do if non-invasive ventilation fails • strategies to withdraw non-invasive ventilation if the patient wishes • involvement of family and carers

19. Costs per 100,000 population

20. Discussion • Who needs to join our multidisciplinary team? • How do we liaise with primary care? • How do we support families and carers at each stage when considering or using non-invasive ventilation? • Can non-invasive ventilation be accessed by all eligible patients? • How do we manage end-of-life care?

21. Find out more • Visit www.nice.org.uk/CG105 for: • the guideline • the quick reference guide • ‘Understanding NICE guidance’ • costing report and template • audit support