Session 6: Withdrawal of ventilation

Disease progression Respiratory muscle function will worse, and if untreated respiratory complications eventually cause death. MND is a progressive neurodegenerative disease that attacks the upper and lower motor neurones Non-invasive ventilation (RCT) and weak cough management (Ex Op) will increase length of life in non-bulbar MND

Disease progression and NIV dependence PwMND who tolerate NIV: self terminate treatment become dependent on NIV or die before they become dependent. Ventilator dependence can occur slowly or suddenly and can relate to a physiological and/or psychological cause. Simonds 2007 defines it as “an inability to discontinue ventilation for long periods.” For some, ventilator dependence can mean death without the ventilator within hours or minutes. Consent for image use, not for sharing on social media. Ann’s increasing NIV dependence

Managing NIV dependence Some require other the adjuncts such as mouth piece ventilation. Most can be treated with NIV Some, particularly those with bulbar involvement may request/require tracheostomy ventilation. Permission as before.

Ventilator dependence Patients who are dependent on ventilation can die quickly if the ventilation is stopped: The NIV or TV may have become a life support device. Most patients will die whilst using NIV or TV, this can be sudden death or otherwise. APM tentatively estimate 1 -5% of ventilator dependent patients request withdrawal, around 10% in a recent TV in MND audit Permission as before

Withdrawal of ventilation in MND Assisted ventilation (NIV or TV) is a medical treatment that can improve quality of life, symptoms and survival in selected patients. Patients have to consent to medical treatments and have a right to discontinue medical treatments. Discontinuation of ventilation appears to generate more concern than withdrawing other life-prolonging treatments.

Withdrawal of ventilation in MND If a patient has capacity and wants to stop ventilation and stopping that ventilation will cause them to die that is: acceptable legal ethically the correct thing to do in reality can be confusing, difficult and concerning.

The withdrawal experience Although the ethics and legality are, in theory, very clear, in practice many professionals felt and to a lesser extent still feel very uncertain about these aspects “Professionals have said that providing the care for a ventilator dependent patient who has asked for assisted ventilation to be withdrawn is practically and emotionally challenging.” Families recount how care during withdrawal has often fallen short of what they and the patient needed

Four ethical principles of medicine much criticised but widely used Autonomy Beneficence-doing good Non-maleficence-doing no net harm Justice (Beauchamp T, Childress J- Principles of Biomedical Ethics -1st ed OUP-1979)

Withdrawing ventilation David is a 27 year old man with MND, lives with his father and two younger siblings, his mother died of MND when he was 10. Diagnosed at 23 NIV at 25 TV at 26 After one year on ventilation he feels his quality of life is very poor and asks for ventilation to be stopped.

Autonomy As long as a person has capacity they have the right to make their own decisions relating to medical care Individuals must be given help and information to make a decision themselves Unwise, eccentric or odd decisions are appropriate and allowed ADRTs should be encouraged in case capacity is lost and follow the same guidance as for someone who hasn’t lost capacity. MCA 2005 ENGLAND AND WALES

David has capacity Ventilation is a treatment and can be declined. David has the right to say “I no longer want ventilation”, even if this rapidly causes death. But isn’t this suicide? If I turn off the ventilator am I euthanising David?

Suicide? Suicide: the action of killing oneself intentionally Ventilation has prevented death from MND and prolonged life Stopping ventilation is re-establishing the chain of causality, it isn’t an action designed to caused death David isn’t committing suicide. The MND is causing his death

Euthanasia? Euthanasia: The painless killing of a patient suffering from an incurable and painful disease or in an irreversible coma. Turning off the ventilator is re-establishing the chain of causality, it isn’t an action designed to caused death. Stopping ventilation is re-establishing the chain of causality, it isn’t an action designed to caused death. David isn’t being euthanised, MND is causing his death.

Artificial postponement of death - Chain of Causality

Patients don’t always die quickly when ventilation is withdrawn In an unpublished audit of 28 withdrawals of NIV in the UK between November 2015 and November 2017, 4 patients took over 2 hours to die, one with a high spinal injury took 24 hours to die.

What about those who lack capacity? MND frequently affects the bulbar and facial muscles causing difficulty with communication NIV and TV affect the ability to communicate Up to 15% have associated frontotemporal dementia Inability to communicate desires, concerns and consent to continue treatment (TV patients can become locked in) and/or Loss of capacity

Withdrawal in those lacking capacity Hopefully patient who is or becomes ventilator dependent will have an ADRT in place. Enactment of a valid ADRT in a patient who has lost capacity is appropriate and legal In the absence of an ADRT but where there is LPA (health & well being) requesting withdrawal, the MDT must agree withdrawal is in the best interests of the patient Withdrawal in a patient who has lost capacity is then appropriate and legal. In the absence of an ADRT and LPA, withdrawal decisions must be based on a best interest determination as set out in the MCA 2005. Withdrawal in a patient who has lost capacity is then appropriate and legal.

How to do it? Withdrawal of Assisted Ventilation at the Request of a Patient with Motor Neurone Disease Link to guidance

How to do it? Ensure its legal: Patient with capacity must be making an informed choice, at least 2 conversations with 2 different professionals, one should be a senior doctor. Patient who has lost capacity must have a valid ADRT or LPA for Health and Wellbeing and best interest meeting. Patient who has lost capacity and does not have a valid ADRT or LPA for Health and Wellbeing needs a best interests determination. If there are divergent views between the patient and their family or the healthcare team, it is useful to obtain professional and medico legal support and guidance.

How to do it? Communicate and involve patient (from the outset of NIV) family team.

How to do it? Plan and co-ordinate The 4 W’s: when, where, who and how Team Approach with a nominated lead (usually the palliative care physician or GP) Who will do what: administer meds manage and switch off ventilator including the alarms who will remove the mask or disconnect from the tracheostomy tube support the family

How to do it? Withdraw Adequate anticipatory sedation for the patient must be administered. This can includes high doses of opiates and benzodiazpines to prevent any distress, particularly to highly dependent ventilator patients. Levomepromazine may be a useful second-line sedative. The intravenous route would be the best method, although difficult to achieve at home.

How to do it? Withdraw Check adequacy of sedation, highly dependent patients will need to be unresponsive to prevent unnecessary dyspnoea, anxiety and distress. Reducing ventilator settings or stopping ventilation for a short period. If adequately sedated or symptom control remove ventilation. Don’t remove the oxygen: it may help with symptoms and may prevent seizure 2nd to hypoxaemia.

How to do it? Completion After death support the family and the team. Complete the medical certificate of cause of death in a timely fashion. Such a certificate may read for example: 1a ventilatory failure (due to) 1b advanced motor neurone disease. Complete an audit form Reflect, they are all different.

Beneficence/non-maleficence withdrawal of ventilation very likely to cause: dyspnoea anxiety distress appropriate sedative medication is essential to promote beneficence and prevent maleficence even if such medications reduce respiratory drive as with all palliative care, the intent must be solely to avoid or ameliorate symptoms of discomfort or distress

UK Audit of withdrawal of ventilation in NIV dependent patients (Nov 2015 - Jan 2016) 16 withdrawals (14 MND, 3 TV), from across the UK. Audit data collected using the audit tool 6 at home, 8 at hospice all were using NIV for >22 hours per day 13 patients had capacity, nobody had an ARDT which informed the process lead in all but two cases were palliative care doctors Data collected will inform the next edition of the APM Withdrawal Guidance (Unpublished data collected by Withdrawal of ventilation in MND Working Party 2017) More information and a copy of the audit tool are included in guidance produced by the Association for Palliative Medicine – appendix 6.

UK Audit of withdrawal of ventilation in NIV dependent patients (Nov 2015 - Jan 2016) Overall: withdrawal was a positive experience for the family only in one case did a family member become very distressed withdrawal was a positive experience for the team In 12 cases it was thought the process was positive for the patient being involved improves confidence.

UK Audit of withdrawal of ventilation in NIV dependent patients (Nov 2015 - Jan 2016) Doses used: Mean Midazolam 26mg (Range 10mg- 120mg) Mean Morphine 15mg (Range 10-52.5mg) 7 patients required levomepromazine, mean dose 102mg (Range 25- 200mg) Time until death after removal: immediately up to 24hours Time until death from administration of first drug: 30 mins-3 days

Summary NIV and TV prolongs life in MND a significant number of patients will become ventilator dependent a small number of patients will ask for ventilatory support to be removed removal of ventilation is legal and in keeping with good and ethical medical care withdrawal needs to be appropriately managed to minimise distress for everyone.