Box 1: The map: locked-in syndrome and clinical uncertainty

Locked-in syndrome (also known as coma vigilante) poses problems for clinicians, who just do not understand that their patient is a silent and unresponsive witness to everything that is happening. It is more often relatives than medical staff who realise the patient’s predicament (usually by noticing intuitively that the patient is awake and registering what is going on). Nick’s mother and his girlfriend pleaded with the medical staff to realise that he was aware of what was happening, and when the clinicians appreciated that the diagnosis was locked-in syndrome, the climate of care changed. Locked-in Syndrome Essay. A patient in locked-in syndrome cannot interact with us because he or she has lost the ability to control his or her body (except, in most cases, the ability to move the eyes up and down) but, importantly, the subliminal cues that intuitively alert us to the presence of another person are all that is needed for the suspicion to form and then the diagnosis to be confirmed by imaging and bedside interaction. Locked-in syndrome is caused either by a lesion in the brainstem (usually vascular) or by extensive demyelination, denying the brain its peripheral connections.

What locked-in syndrome is not

Nick, aged 23 at the time, was plunged into locked-in syndrome by a dissection of the vertebral arteries during a rugby game. The syndrome is quite different from other forms of coma or the persistent vegetative state, although they may be confused at the bedside. The confusion affects ethics as well as clinical assessment, but the ethical implications of the two conditions are quite different.

Locked-in syndrome and persistent vegetative state

In a persistent vegetative state (as distinct from locked-in syndrome) the higher levels of the brain have been devastated, usually by a combination of shearing stress injury to neurones and global cerebral anoxia. These selectively damage the higher brain—the cortical system and its ramified connections, so that there are no longer enough “megabytes” to do the work required for conscious experience and cognition. Both comprise mental activity on data gathered from the environment (in the philosophical literature—intentional functions) and they make use of the extensive processing capacity of the neocortex. Locked-in Syndrome Essay. Consciousness therefore rests on the coordinated and holistic functioning of widespread and dynamically interconnected cerebral functions. Nick’s story makes it evident that he was from the beginning interacting with his environment in as many ways as his impairments allowed despite the fact that only a few people realised it. He recognised objects and people, conceptualised experiences, and experienced a range of feelings. These states do not survive in persistent vegetative state because there is insufficient (and insufficiently integrated) brain function to support them so that a patient in this state no longer has typical persistent vegetative state and is a consequence of the loss of the neural capacity required for that intelligent adaptation to the environment that forms the basis of human mental life. To put it simply, someone in a persistent vegetative state is not conscious because he or she cannot engage in the many acts of being conscious of the things of which Nick was conscious.

Box 2: A “locked-in” witness to ethical decisions

In Nick’s case, a clear diagnosis was made based on the anatomy of the injury and careful attention to the subtle signs of consciousness that he showed, but notice the tendency to marginalise or “absent” Nick as an intelligent participant despite the fact that he is an intelligent witness to these conversations about life and death. We can imagine the sheer awfulness of hearing others discuss turning off one’s life support.

Locked-in syndrome, mind, and consciousness

The lesion in locked-in syndrome causes a loss of control of all the bodily functions that make a person accessible to others; in fact, once the care team realised what was going on, they then developed and used a series of interactive systems (such as eye movements) to open up lines of communication.Locked-in Syndrome Essay.  However, despite this realisation, he heard things said about his prognosis and his life that paid little regard to what he might have been thinking.

It is worth dwelling for a moment on a philosophical claim by Wittgenstein: “Consciousness is as clear in his face and behaviour as it is in myself.” This remark is apparently disproved both by Nick’s testimony and our common intuition that our conscious life is private and internal. Wittgenstein argues that the meaning of any term is a function of its use in a sufficient range of typical or paradigmatic cases, so we might infer that where the normal conditions do not hold, our normal ability to recognise what we are seeing may be impaired. Therefore, if we normally judge that something or someone is conscious on the basis of the myriad subtle cues we pick up in everyday interaction with an intact human being, then certain kinds of neurological damage will make it hard to make that judgment. Locked-in syndrome is exactly such a situation, where the fact that the patient is conscious is unrecognisable to everybody except the person concerned (and perhaps those particularly “attuned” to him or her). Nevertheless he or she uses all the perceptual and cognitive skills that normally reveal what goes on around us.

Box 3: Misjudging patients with locked-in syndrome

In the locked-in case, the mind of the patient is fully functioning but the integrated functioning of the psychosomatic whole is disturbed: connecting lines are cut, even though the internal hard drive is more or less intact. Therefore, as in other cases, such as profound cerebral palsy, the state of the person may be completely misjudged because, although it is subtly manifest, it may not be readily manifest to us. This is the implication of the fact that people who are attuned to the patient or on the alert for the condition can discern what is happening.Locked-in Syndrome Essay.  But the thinking that affects our acute assessments also, and quite evidently, blinds us to the ongoing work of “self remaking” that someone like Nick is doing.

When we understand the problem, we can see exactly how it comes about that the person is still interacting with the world around him. But it is all one way traffic in that, however things strike him, he cannot show it. His showing and our ability to recognise his “presence” to us depends, in locked-in syndrome, on a myriad of subtle manifestations evident in eye and face and bodily habitus, even though voluntary activity and obvious responsiveness is what we normally rely on (especially in clinical life). Therefore in locked-in syndrome we can, and do, make mistakes about what is happening to the patient. We must make special efforts to reach past the locked-in syndrome barrier and allow the patient to connect with us. Locked-in syndrome is the kind of state that defeats our capacities to know how it is with another person, and when we are given an insight into his or her situation we can readily identify with Nick’s frustration at being unable to convey how it is with him.

Notice that a similar strategy defeats any attempt to argue for the abiding worry that can affect our attitudes to patients in a persistent vegetative state, where we worry about an enduring presence incommunicable to “us outside.” But in a patient in such a state, the capacity to be conscious has been destroyed so that it is almost the exact reverse of locked-in syndrome. Locked-in Syndrome Essay. The cognitive or experiential core of human life is gone in persistent vegetative state whereas in locked-in syndrome this core is intact, as Nick so eloquently tells us.

Box 4: Ethical implications

We have touched on one set of ethical issues to do with the life of somebody who has survived locked-in syndrome and there are many others that take us far beyond the discussion so far. One might concur with Nick when he says that his life in the state to which he has been reduced is not worth living and that at times he just wants to be left to die but also be overwhelmed by the courage and determination he shows by going on living. In fact his determination to go on living is congruent with the majority response of patients in this condition.Locked-in Syndrome Essay. A person such as Nick has been tragically struck down and needs all the help he or she can get to rediscover something worth living for. After that has been done, it is his or her decision what then happens.

Locked-in syndrome and persistent vegetative state

It seems that we can, if we listen to a story such as Nick’s, find a real life situation against which to assess our ethical thinking about persistent vegetative state and other brain injured states. In fact, the ethical implications of locked-in syndrome and persistent vegetative state are completely different. In a persistent vegetative state, the experience of a person as a person (“a conscious living thing that hath reason and reflection,” to quote English philosopher John Locke) is ended and can never be restored because of a massive loss of neural circuitry. Thus the story of the person’s life, as a story experienced “from the inside,” has ended.

A patient in a persistent vegetative state cannot take part in life or in any decisions that are made about him or her. We are therefore left only with an advanced declaration (if one exists) or an objective standard of substantial benefit in making an end of life decision such a case. But Nick presents us with a different challenge. He requires only that we care enough to rebuild the tools of communication (through interactive technology and massive personal commitment) so that he can begin to live again among us, albeit with severe disabilities.

ORDER A PLAGIARISM-FREE PAPER NOW

Additional educational resources for patients and professionals

Locke J (ed. P. Nidditch). An essay concerning human understanding. Oxford: Clarendon, 1689, 1975.

Smith E Delargy M. Locked in syndrome. BMJ 2005;330: 406-9. [PMC free article][PubMed] [Google Scholar]

Auckland Area Health Board v Attorney-General [ 1993] NZLR 235. [PubMed] Locked-in Syndrome Essay.

Gillett G. Bioethics in the clinic. Baltimore: Johns Hopkins University Press 2004. (Especially chapter 10.)

Multi-society task force on PVS medical aspects of the persistent vegetative state. New Engl J Med 1994;1499: 1503. [PubMed] [Google Scholar]

Gillett G McMillan J. Consciousness and intentionality. Amsterdam: John Benjamins. 2001.

Gillett G. Wittgenstein’s startling claim: consciousness and the persistent vegetative state. In: Elliot C, ed. Slow cures and bad philosophers. Durham: Duke University Press, 2001: 70-88.

Wittgenstein L. Zettel. Oxford: Blackwell, 1967: 221.