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Withdrawal of invasive ventilation in a patient with motor neurone disease and total locked-in syndrome
  1. Aoife Gleeson,
  2. Faye Johnson
  1. Department of Palliative Medicine, Aneurin Bevan University Health Board, Gwent, UK
  1. Correspondence to Dr Aoife Gleeson, Department of Palliative Medicine, Aneurin Bevan University Health Board, Ysbyty Ystrad Fawr, Ystrad Mynach, Gwent CF82 7EP, UK; aoife.gleeson{at}


Withdrawing invasive ventilation from a person with motor neurone disease who lacks the relevant mental capacity raises ethical issues such as the withdrawal of life-sustaining treatment and establishing best interests. There is little available information on providing optimal symptom management to these patients during the withdrawal process. We describe a man with motor neurone disease who also had total locked-in syndrome at the time of ventilation withdrawal, and we document the legal, ethical, emotional and symptom control issues encountered in supporting him.


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Respiratory muscle weakness is inevitable among patients with motor neurone disease (MND), and respiratory failure is their leading cause of death.1 Fewer than 1% of people with MND in the UK receive long-term tracheostomy ventilation, occuring usually at the onset of terminal respiratory insufficiency and often before the underlying neurological condition is diagnosed.2 3

For some patients with MND who are invasively ventilated (a selected population), tracheostomy ventilation permits an acceptable quality of life.4 Kaub-Wittemer et al 4 assessed quality of life in 21 invasively ventilated patients with MND, noting that 81% would choose tracheostomy ventilation again. Nevertheless, MND causes an increasing symptom burden, alongside progressive physical decline.4 At its most advanced stage, particularly when death due to respiratory failure is delayed with tracheostomy ventilation, MND can result in a complete absence of voluntary motor function and communication, known as total locked-in syndrome.5

Several case reports document requests by patients with MND to withdraw tracheostomy ventilation at that time or in the future via an advance decision, underlining the practical, ethical and legal challenges involved.2 6 7 The difficulty in establishing decision-making capacity due to impaired communication is intensified by secondary cognitive dysfunction developing in 20%–40% of cases.5 Palliating the sudden onset of symptoms due to hypoxia after withdrawing ventilation is also challenging.8 Case reports show a wide variation in practice and suggest that healthcare professionals consider ventilation the most difficult life-sustaining therapy to withdraw.2 6 7 A search of medical databases (eg, Embase, Ovid, Medline and the Cochrane Library) identifies a dearth of high-quality evidence to inform this situation.

We describe a patient with MND who developed total locked-in syndrome while on tracheostomy ventilation and whose ventilation was withdrawn in accordance with his wishes documented in an advance care plan. We illustrate the diversity and magnitude of the challenges encountered in carrying out his wishes.


The patient was diagnosed with MND when aged 42 years, after 6 months of progressive dysarthria and right-hand and leg weakness. Following diagnosis, he started riluzole. Two months later, after a holiday in Spain and a viral chest infection, he was admitted with a 10-day history of progressive limb weakness and dyspnoea. He was diagnosed with type II respiratory failure and right lower lobe collapse. He was admitted to the high-dependency unit and started on non-invasive ventilation. Over the next week, he was reviewed by clinical psychology and by specialist palliative care, who discussed the future place of care and role of non-invasive ventilation and symptom control medications. The next day, he was intubated, and over the subsequent 6 months in hospital, he lost strength in all four limbs, was treated for two episodes of ventilator-associated pneumonia, started percutaneous endoscopic gastrostomy feeding and had a suprapubic catheter inserted.

He was discharged home with 24-hour package of care, including one qualified and one unqualified nurse at all times. His support team also included the complex care team, his general practitioner, a consultant anaesthetist, the community palliative care team and a palliative medicine consultant. The anaesthetist reviewed him monthly, and the palliative care consultant fortnightly. His head and eye movements were initially preserved, allowing communication via an alphabet board. He could therefore discuss symptom control issues, psychosocial issues and his advance care wishes. He was clear that he wanted ventilation and treatment to be withdrawn when he could no longer communicate. Alongside his ongoing medical and nursing care, he managed to enjoy ‘normal’ activities, including going to football matches, going to the pub, going on holidays within the UK and spending time with his wife and son. His wife struggled emotionally with watching his deterioration and was supported by the clinical psychology team.

Fifteen months after his discharge from hospital, a first professionals meeting was held to discuss and document the end-of-life care plan for him. The plan comprising his stated wishes was informed by a literature review, and this was discussed and ratified by the health board’s palliative care and intensive care consultant groups.

Communication became increasingly arduous, and although he tried several different aids, he had to continue to use the alphabet board, as his eye movements weakened. By 19 months postdischarge from the hospital, he could spell only one sentence per hour. Eye fatigue and recurrent conjunctivitis became problematic, while staffing his package of care also became difficult. After much discussion, he agreed to a respite admission to the local hospice, on condition that his usual staff continued to support his care.

Two years after discharge, his eye movements were very limited and were carefully monitored using a pro forma developed by the clinical team. An ethics review meeting led to an application to the Court of Protection for a declaration that tracheostomy ventilation could be withdrawn. Following the provision of statements and review of clinical case notes by the trust solicitor, it was decided that court proceedings were not required. A further professionals meeting was held, and the general practitioner, the palliative care consultant and the anaesthetic consultant undertook separate medical reviews. He had no observed eye movements during these three reviews; hence, we initiated the end-of-life care plan agreed by the patient, his family and the professional team.

A 24-hour continuous subcutaneous infusion with 100 mg midazolam, 100 mg diamorphine and 100 mg levomepromazine was set up. This was started 24 hours before ventilator withdrawal, because the absence of physiological signs of distress due to the total locked-in syndrome gave no clues to guide dosing. The next morning, the complex-care case manager, palliative care consultant and anaesthetist were present when the ventilation was withdrawn in his home, and they remained with him until his heart stopped 45 min later. There was no visible distress or tachycardia when ventilation was withdrawn. The patient had agreed months before his death which personnel would be present. His wife chose not to be in the room until his heart had stopped. He died 29 months after his discharge from hospital, having been on ventilation via tracheostomy for 35 months.


This report highlights the complexity of managing a patient transitioning from locked-in syndrome to total locked-in syndrome: a state of complete ophthalmoplegia, in which communication is no longer possible. In particular, we highlight the diagnostic difficulty and, hence, the ethical implications of confirming complete ophthalmoplegia. The case also illustrates the challenges associated with assessing and alleviating symptoms in a patient with MND who lacks motor function. The relative preservation of autonomic function meant that his heart rate stability was the only physiological clue to his comfort.9 There is also the financial cost of providing 24-hour care in the home: the baseline package of nursing care that enabled him to remain at home from discharge until death cost £484 744 per annum.

This case highlights some complex legal issues. According to the Mental Capacity Act 2005 (England and Wales), fundamental to diagnosing incapacity is the presence of both a disorder of mind or brain, and an inability to make decisions. Once he lost the ability to communicate, according to the Mental Capacity Act, he could no longer make decisions.10 Although cognitive dysfunction was an issue previously, establishing normal cognitive function became impossible due to total locked-in syndrome. We therefore managed him as someone lacking capacity.

According to Section 4 of the Mental Capacity Act, relating to making best-interest decisions, his advance statement of wishes were to be taken into account when making a treatment decision in his best interest. However, it lacked the legal credence of a valid and applicable Advance Decision to Refuse Treatment.10 Thus, we sought a Court of Protection Order to obtain legal permission to withdraw tracheostomy ventilation. This was later deemed unnecessary due to the unequivocal evidence of his prior autonomous wishes, which the clinical team had extensively documented. His previously expressed wishes were therefore determinative in the decision made in his best interests to withdraw tracheostomy ventilation once capacity was lost.

A search of Medline and Embase revealed a dearth of evidence relating to withdrawal of tracheostomy ventilation in MND. Gannon describes the issues faced by a UK hospice on receiving a referral for ventilator withdrawal. Unfamiliarity with the procedure and professional unease among hospice staff almost delayed the admission.11 Previous case reports indicate widely variations in doses and routes of medication and the length of time to achieve comfort/sedation. In one report, achieving adequate sedation took more than 48 hours.2 The dose of midazolam administered before ventilator withdrawal ranged from 5 mg intravenously to 100 mg via continuous subcutaneous infusion.2 12 As our patient had total locked-in syndrome, medication was not titrated and was started 24 hours before withdrawal. The Association for Palliative Medicine recently published guidance on withdrawing assisted ventilation in MND, informed by a large questionnaire and interview study.8 13 14 It outlines five standards that provide a structure for withdrawing both non-invasive and invasive ventilation in MND.8 However, the advice relating to symptom control suggests that medication be given according to the level of sedation, which would not be applicable to patients with total locked-in syndrome, as the physiological effects of sedation cannot be assessed.

The growing international evidence relating to the withdrawal of tracheostomy ventilation in MND suggests significant variation in the frequency with which tracheostomy ventilation is used in MND worldwide. Kettemann et al report increasing use of tracheostomy ventilation among German patients with MND, with a current rate suggested of 8%.15 In their cohort study, which analysed the management of patients with MND undergoing ventilator withdrawal, patients using tracheostomy ventilation (as opposed to non-invasive ventilation) tended to be ventilator dependent and usually required deep sedation for symptom control. Deep sedation was achieved with central venous access and the administration of propofol and morphine.15 Kettemann et al highlight that ophthalmoplegia is a rare development in the natural course of MND, but note that immediately before ventilator withdrawal, 41% of the patients in their study had ophthalmoplegia (partial or complete). They emphasise an association between ventilation in MND and locked-in syndrome.15

The frequency of tracheostomy ventilation in MND patients in Japan is high and rising.16 In a study between 1990 and 2010, data for 160 patients were analysed. The group was divided into two, with 80 among those analysed between 1990 and 1998 and 80 between 1999 and 2010. The rate of tracheostomy ventilation use increased from 28% in the first group to 38% in the second group.16 The authors suggest that reasons for the rising uptake of tracheostomy ventilation in Japan include religious and cultural beliefs and values, the fact that the Japanese government funds all cost of treatment and improved understanding of MND among healthcare professionals.16 The situation is similar in Italy, where around 30% of patients with MND undergo tracheostomy ventilation when bulbar symptoms become severe.17

Conversely, only 3% of patients with MND in a Dutch study by Maessen et al 18 received tracheostomy ventilation, but the typical mode of death for Dutch patients with MND differs from most other countries because of their Euthanasia Act 2002. These authors found that 16% of Dutch patients with MND underwent euthanasia or physician-assisted suicide and 15% of patients died during deep sedation, despite that only 2.9% of patients in the study were reported to be using tracheostomy ventilation.18

This present case study highlights the complexity of providing care for tracheostomy ventilated patients with MND. Although UK clinical guidance does not currently recommend invasive ventilation for managing respiratory failure in MND, there remains a proportion of patients who develop respiratory failure before their neurological diagnosis.3 19 Thus, the issues faced by the patient in this case are likely to affect a small proportion of UK patients with MND in the future.

While the report adds valuable information to the evidence base, it describes only a single case of total locked-in syndrome, so our findings may not be generalisable. Further studies may include assessment of services available to support tracheostomy ventilation in patients with MND in the community and specialist palliative care settings and an economic evaluation of provision of care for this patient group.

Key points

  • Tracheostomy ventilation in motor neurone disease (MND) is rare in the UK but more common internationally.

  • Tracheostomy ventilation alters the natural course of MND by postponing death from respiratory failure but leads to a high incidence of ophthalmoplegia and locked-in syndrome.

  • For patients lacking the relevant mental capacity who have not appointed a Lasting Power of Attorney and have not created an Advance Decision to Refuse Treatment, best interest decisions should be established in accordance with Section 4 of the Mental Capacity Act 2005.

  • Current evidence relating to the withdrawal of invasive ventilation in MND is limited in the UK.

  • Tracheostomy ventilation is started more frequently in countries such as Japan and Italy, and management during the last days of life also varies internationally.



  • Contributors AG: conception or design of the work, data collection, critical revision of the article. AG and FJ: data analysis or interpretation, final approval of the version to be published. FJ: drafting the article.

  • Competing interests None declared.

  • Patient consent Consent was obtained from the wife of the deceased patient.

  • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Chris McDermott, Sheffield, UK.

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