• motor neuron disease

Making decisions about withdrawing life-sustaining treatment is something that a neurologist will most frequently be involved with in the intensive care unit setting, in people who have suffered a catastrophic brain injury. The ethical position is relatively clear when there is no prospect of meaningful recovery and, inevitably, the patient lacks awareness. Such decisions are made on the basis of futility and on a best-interest basis. The decision will be made in consultation with any appointed attorney for such decisions or following discussion with family members about any wishes the patient may have expressed and recorded, including any advance statements.

However, when patients themselves request the withdrawal (or refuse the continuation) of life-sustaining treatment, this raises significant challenges for doctors and others and is the subject of the General Medical Council (UK) and other guidance. In UK law, a patient with capacity for that decision has the right to refuse any medical intervention or treatment. This request by the patient may be made either directly or through an advance decision to refuse treatment, which becomes applicable when the patient loses capacity.

Withdrawal of life-sustaining treatment at a patient’s request through an advance decision to refuse treatment is especially challenging in the circumstances when a patient who is thought to be mentally aware loses the ability to communicate: the circumstances of the locked-in syndrome. In this issue, Gleeson and Johnson discuss the diversity and magnitude of the challenges in the withdrawal of assisted ventilation from a man with motor neurone disease who had consented to have tracheostomy ventilation to manage his respiratory failure.1

In the described case, the patient’s life was probably prolonged by 2–3 years. During that time, at least early on, he had enjoyed attending football matches and going on holiday with the family among other things. Perhaps things became more difficult later on; in the last year of life, he could spell only one sentence an hour with an eye board. The impact on his wife required support from the clinical psychology team. Overall, one gets the sense that the tracheal ventilation here was beneficial.

The case described by Gleeson and Johnson highlights the strengths of the regulatory framework for this situation and the anxiety that this provokes; despite correctly understanding the situation, the clinicians involved wanted and sought legal affirmation. The clear recording of the patient’s wishes, along with the regular monthly discussions to review these wishes, helped to assess the validity and applicability of the advance directive to refuse treatment when he lost ability to communicate. Reaching the decision within the legal and ethical framework is often complicated, but here, we see the ease with which this can be done following the the multidisciplinary team’s commendable approach.

The more challenging aspects of this case were how to decide if communication had been lost and how to ensure that the patient was not experiencing distress during withdrawal of tracheal ventilation, if they could not communicate. The case highlights the lack of evidence regarding how to determine if communication is lost, what physiological measurements are valid and useful for monitoring distress and what constitutes effective and proportionate use of medications for symptom management.

Guidance from the Association for Palliative Medicine of Great Britain and Ireland gives five standards for care, based on in-depth qualitative interviews with professionals and bereaved carers with experience of withdrawal of assisted ventilation in motor neurone disease, together with consensus expert opinion regarding symptom management.2 The guidance does not cover the approach to a locked-in patient; this case report provides useful additional insights for clinicians managing similar cases.

Invasive tracheostomy is rarely performed as a planned procedure for people with motor neurone disease in the UK, in contrast to the practice in several other countries. The reasons are unclear but may include cultural differences, preconceptions about the quality of life of motor neurone disease patients and/or doctors, availability of services able to provide the 24-hour specialist nursing care and funding. Clinicians occasionally use a tracheostomy to help to manage oral sections and aspiration in patients with bulbar disease, as described in a further case by the patient himself and his carers in this issue.3 There is currently little formal evidence regarding this approach and several unanswered issues; how does the symptomatic benefit balance against the longer term issues, such as withdrawal (as highlighted in the first case1)?

Recognising the need to develop the evidence base further, the Motor Neurone Disease Association and the Association for Palliative Medicine are supporting a registry of cases where non-invasive ventilation or tracheal ventilation is withdrawn. Readers with historical cases or those with cases in the future are encouraged to register the case details through submitting a core dataset. Further information can be found in the Association for Palliative Medicine guidance, on the website and by email (christinafaull@loros.co.uk or c.j.mcdermott@sheffield.ac.uk).2 4