Objectives Mechanical ventilation (MV) has been shown to improve survival and quality of life in motor neuron disease (MND). However, during the progression of MND, there may come a point when MV is no longer felt appropriate. Association of Palliative Medicine Guidelines have been recently published to help clinicians withdraw MV at the request of patients with MND in a safe and compassionate manner to ensure that symptoms of distress and dyspnoea are minimised.
Methods In this report, we discuss the palliative and ventilatory management of six ventilator-dependent patients with MND who had requested the withdrawal of MV as part of their end-of-life care.
Results We have withdrawn MV from six patients with MND at their request and our practice has been influenced by the Association of Palliative Medicine Guidelines.
Conclusion Withdrawal of MV in MND at a patient’s request is challenging but is also a fundamental responsibility of healthcare teams. We discuss the lessons we have learnt which will influence our practice and help other teams in the future.
- end of life care
- neurological conditions
- respiratory conditions
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Mechanical ventilation (MV) is commonly used to treat respiratory failure in a diverse range of conditions. Non-invasive ventilation (NIV) has been shown to improve quality of life and survival in patients with motor neuron disease (MND).1 A minority of patients with MND undergo tracheostomy ventilation (TV).
In MND, a time may be reached when MV ceases to be appropriate. At this time, it is the right of the patient to request the withdrawal of MV and the duty of healthcare teams to withdraw MV.2 The presence of the ventilator during the dying process makes this process unnatural with equipment at the bed side but also does not allow hypoventilation and build-up of carbon dioxide which accompany normal dying.
Patients who are nocturnally ventilated can simply refuse the treatment. However, this is more challenging in patients receiving TV and in ventilator-dependent patients (patients using MV for more than 12 hours in a 24-hour period). These patients are unable to breathe without MV without experiencing dyspnoea.
Withdrawal of MV at the request of ventilator-dependent patients with MND challenges healthcare professionals posing ethical and moral considerations and concerns about legal repercussions. We have demonstrated the benefits of education to healthcare professionals caring for patients with MND improving confidence with the ethics, legalities and practicalities of withdrawal of MV.3
Standards have been produced as part of guidance from the Association of Palliative Medicine (APM) pertaining to the withdrawal of MV at the request of patients with MND.2 The standards highlight the importance of autonomy and the right to discontinue MV as with any therapy. They sanction pre-emptive medications to prevent dyspnoea during withdrawal of MV from MV-dependent patients. The guidance also gives a detailed review of the relevant law including Advance Decision to Refuse Treatment (ADRTs), Lasting Powers of Attorney and questions of best interests. The standards refer specifically to the legal and ethical position within the UK.
Reported experience of MV withdrawal is rare. A Danish cohort study with large numbers of patients with MND receiving MV reported only 12 cases over a 9-year period.4 To our knowledge, this is the first UK case series and the first since publication of specific APM guidance.
We present six patients with MND who requested withdrawal of MV. We discuss the symptom and ventilatory management and conclude with lessons we have learnt.
Patient A—a 55-year-old man with disease onset in January 2013 commenced NIV in January 2014. His dependency increased over 8 months to complete NIV dependency. Elective tracheostomy was performed in October 2014. His ADRT stated that when prolonged unconsciousness due to MND occurred, TV should be discontinued. TV was withdrawn in March 2015 at home.
Patient B—a 56-year-old man with disease onset in April 2012 commenced NIV in January 2015. He rapidly became NIV dependent. His ADRT stated that he wanted MV withdrawn if his bulbar function or mobility deteriorated. NIV was withdrawn in August 2015 at home.
Patient C—a 63-year-old woman with disease onset in January 2011 commenced NIV in July 2014. She became NIV dependent in November 2015. She was admitted to a hospice for palliative management. At that stage, options were explored and the withdrawal of MV was requested as part of those discussions. NIV was withdrawn in December 2015 in the hospice.
Patient D—a 31-year-old man with disease onset in September 2014 commenced NIV in May 2016. He became NIV dependent following pneumonia in July 2016. He stabilised to allow home discharge and requested withdrawal of NIV. NIV was withdrawn in September 2016 at home.
Patient E—a 50-year-old man with disease onset in January 2012 commenced NIV in May 2013. He became NIV dependent in October 2014 and underwent elective tracheostomy. A hospital admission was followed by discharge to a long-term care facility. He was discharged home in August 2015. His ADRT stated that when prolonged unconsciousness due to MND occurred, TV should be discontinued. TV was withdrawn in January 2017 at home.
Patient F—a 78-year-old man with disease onset in in August 2014 commenced NIV in November 2016. He became NIV dependent in February 2017. He requested withdrawal of NIV at home which took place in September 2017 at home.
The medication and ventilatory management are summarised in tables 1 and 2, respectively.
We present six patients with MND who requested withdrawal of MV whose requests were facilitated in accordance with best medical practice and the APM MND practice guideline.2
There are lessons which we feel are important to take into future practice and are pertinent to all involved in this area of palliative care.
All healthcare professionals should read the APM guidance for withdrawal of MV for reassurance that the course of action requested by the patient is ethical and legal.
All healthcare professionals should meet with the family and with each other prior to the date of withdrawal of MV to allow adequate planning. We felt it important that one member of the healthcare team co-ordinated the planning of MV withdrawal.
The patient’s wishes on the timing of MV withdrawal should be respected where possible. An evening withdrawal is often easier to deliver in terms of the workload of healthcare staff involved but may represent a challenge for community staff and drug availability.
It is not practical for the home ventilation team to have any role in the delivery of palliative medication. Palliative medications can be administered by district nurses, but we found the input of specialist palliative services invaluable. This has the advantage of involving other senior clinicians and sharing the responsibility for medication dosing and the assessment of response to medications.
It was useful to have a room where drugs could be prepared away from the patient and family both for safety and privacy.
If patients are on long-term opiates, then substituting oral medications with a subcutaneous infusion has the advantage of predictable drug delivery and flexibility in dosing. Doing this in advance of the withdrawal of MV has the advantage of reaching a steady state of drug concentration before any dose titration.
The doses of opiates and benzodiazepines were variable, but in general the higher the level of consciousness of patients pre-withdrawal, the higher the dose of drugs required to manage and control symptoms.
The pre-insertion of two or more subcutaneous lines is helpful. Repeated subcutaneous injection of large volumes of drugs can result in localised drug collections and may cause discomfort and unpredictable drug absorption. Rotating administration sites addressed this issue.
We reduced the respiratory rate and only begun to reduce the ventilatory pressures and tidal volumes when the respiratory rate had been halved and the level of consciousness had reduced. Our experience of stably ventilated patients with MND is that they are symptomatically dependent on adequate lung expansion.
During our first planned domiciliary withdrawal of MV, we recognised the importance of ensuring that all ventilator and humidifier alarms were disabled. This prevented unnecessary distress to either the patient or family.
One patient continued to breathe for 32 hours following the withdrawal of MV. This experience enabled us to counsel relatives and patients and explain that the role of the home ventilation team is to ensure that MV is withdrawn without causing any distress to the patient and that spontaneous ventilation may continue.
Following the withdrawal of MV, patients often had an increased respiratory rate. Some of the team were concerned that this represented distress. However, lack of consciousness and eyelash reflex reassured all present that this represented a physiological response to hypercapnia rather than patient distress.
Two patients opened their eyes at the moment of death despite being deeply unconscious prior to withdrawal of MV. Given the level of consciousness immediately prior to withdrawal of MV and minimal respiratory support due to the very gradual withdrawal of MV, we also felt this represented a physiological reflex to hypoxaemia rather than patient distress.
We have successfully withdrawn MV from six patients with MND at their request. We discuss the challenges posed by this practice and our learning from them. This experience should prove useful to other home ventilation, MND and palliative care teams and informs future practice. No two patients were the same in terms of levels of consciousness, ventilatory support or acute illness and therefore our management was, and will remain, necessarily bespoke.
Preparation and communication between team members and with the patient and their families is critical to building trust at this most challenging of times.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.