Rescuing those misdiagnosed as in persistent vegetative state: we’ve been here before

Two weeks ago, the UK press ran a sensational story about a patient, diagnosed by medical specialists as having been for years in persistent vegetative state (PVS) with no consciousness or ability to communicate, discovered through a state-of-the-art scanning system to have been fully conscious throughout, and now enabled to communicate through a specially designed computer keyboard.  The Guardian‘s account, published on 24 November 2009, began:

For 23 years Rom Houben was ­imprisoned in his own body. He saw his doctors and nurses as they visited him during their daily rounds; he listened to the conversations of his carers; he heard his mother deliver the news to him that his father had died. But he could do nothing. He was unable to communicate with his doctors or family. He could not move his head or weep, he could only listen.  Doctors presumed he was in a vegetative state following a near-fatal car crash in 1983. They believed he could feel nothing and hear nothing. For 23 years.

Then a neurologist, Steven Laureys, who decided to take a radical look at the state of diagnosed coma patients, released him from his torture. Using a state-of-the-art scanning system, Laureys found to his amazement that his brain was functioning almost normally. “I had dreamed myself away,” said Houben, now 46, whose real “state” was discovered three years ago, according to a report in the German magazine Der Spiegel this week.  Laureys, a neurologist at the ­University of Liege in Belgium, published a study in BMC Neurology earlier this year saying Houben could be one of many cases of falsely diagnosed comas around the world. He discovered that although Houben was completely paralysed, he was also completely conscious — it was just that he was unable to communicate the fact.

Houben now communicates with one finger and a special touchscreen on his wheelchair – he has developed some movement with the help of intense physiotherapy over the last three years.

[Guardian, 24 November 2009]

In September 2006 I wrote a post for this blog (at https://barder.com/568):

“Persistent Vegetative State”: some background to last week’s story

September 9th, 2006

Last Friday (8 Sept 06) the Guardian ran a (perhaps deservedly) sensational front page story announcing that “For first time, doctors communicate with patient in persistent vegetative state: Brain scans showed woman was able to imagine playing tennis and walking round her flat”….  [http://www.guardian.co.uk/frontpage/story/0,,1867567,00.html]

I went on to express surprise — in 2006! — at the failure of the Guardian‘s September 2006 story —

…to connect the latest developments with the extensive work already done on Persistent Vegetative State issues over many years, and its implications for patients who have been diagnosed as being in PVS.  A world expert and author of break-through research on the subject is Professor Dr Keith Andrews, Director of the Institute of Complex Neurological Disability at the Royal Hospital for Neuro-Disability in Putney, London (on whose governing body I was privileged to sit for some years).  An early and still centrally important paper on PVS by Dr Andrews and his collaborators was “Misdiagnosis of the vegetative state: retrospective study in a rehabilitation unit” (Andrews K, Murphy L, Munday R, Littlewood C., British Medical Journal, 1996; 313: 13-16).  Based on his studies of a sizeable sample of patients referred to the Royal Hospital after having been diagnosed by specialists elsewhere as being in PVS, Dr Andrews concluded (in the words of a later article) that –

Out of 40 patients diagnosed as being in a persistent vegetative state, 17 (43%) were later found to be alert, aware, and often able to express a simple wish. The study is one of the largest, most sustained analyses of severely disabled people presumed to be incapable of conscious thinking, communication, or awareness of their surroundings. The author, London neurologist Dr. Keith Andrews, said, “It is disturbing to think that some patients who were aware had for several years been treated as being vegetative.”

So in 2006 I was citing a paper on PVS published in 1996 about the disturbing frequency with which patients were being misdiagnosed as being in persistent vegetative state when in fact they were fully or partially conscious, and could often be enabled to communicate using recently developed techniques.

Once upon a time newspapers kept a clippings library to enable their writers to refer back to earlier material about an apparently new story that they were about to write. Now Google News — and their own hard disks — could easily do that job of elementary research for them. Yet they still regularly announce the invention of the wheel with a fanfare of trumpets. The hacks really ought to have a notice stuck to their computer monitors bearing the legend: “There is nothing new under the sun”. It’s as if the collective consciousness of the media is itself in persistent vegetative state.

But the really worrying moral of this curious tale is that at least 13 years after the publication of a major article about the common misdiagnosis of patients wrongly thought to be in PVS, and techniques for re-establishing communication with a high percentage of them, patients are still, in 2009, being wrongly diagnosed. Each time a patient is rescued from this nightmarish imprisonment in his or her own body, conscious but unable to communicate, the medical triumph is trumpeted as if such a thing had never happened before. The question we should be asking is how many other misdiagnosed patients are languishing in their mental prisons, listening to their doctors and relatives discussing whether to turn off their life support machines? How many life support machines have been switched off while the patient lies there, unable to communicate his awareness of the fate about to destroy him? And lest anyone should suppose that a person trapped in such a nightmare would surely welcome death as a merciful release, it’s worth remembering that not one of those rescued by Dr Andrews from their misdiagnosed condition, and able at last to describe the experience of the captive years, ever said that he or she would rather have died.

Update [pm 5 Dec 09]: Comments by Tony Hatfield and Peter Harvey (below) discuss serious doubts about the genuineness of the technique, known as Facilitated Communication or FC, used to enable Rom Houben, the subject of the current reports, allegedly to communicate.  The links helpfully supplied by Tony in his comment point to a mass of evidence that this is either a hoax or a sad case of self-delusion, and that the texts supposedly being typed out by the patient are actually being composed by the facilitator holding Houben’s finger to peck at the letters on the flat keyboard, supposedly in response to faint twitches that she claims to detect in his finger.  The video clip of this process seems to me to demonstrate beyond reasonable doubt that the unfortunate Mr Houben is not in any sense doing the typing or even controlling it.  It seems unlikely therefore that this patient can in fact now communicate, and doubtful whether any particular level of consciousness has really been detected in his case, although the jury is still out on that.

However, as to the restoration of communication, the almost certain lack of credibility of this case, and apparently of FC generally, can’t be taken as discrediting well documented cases of patients wrongly diagnosed as in PVS and ultimately found not only to have been conscious at some level throughout, but also to be able to communicate, however laboriously, by blinking, or exerting slight pressure of a heel, elbow, ball of a thumb, or other part of the anatomy, on a button that operates a buzzer — one buzz for ‘yes’, two buzzes for ‘no’, or whatever.  These cases are, I believe, well documented and not seriously challenged.  In the case of the researches conducted by Professor Dr Keith Andrews at the Royal Hospital for Neuro-Disability, referred to in my 2006 blog post and quoted above, Keith Andrews and his assistants several years ago developed a ‘SMART’ (Sensory Modality Assessment and Rehabilitation Technique) kit that used stimuli of all five senses, even including taste, in order to try to identify some means by which a supposedly vegetative patient might be able to do enough to answer a question ‘yes’ or ‘no’: a twitch, a slight pressure, a blink, a frown.  Once that’s established, the rest follows:  the patient can communicate words by indicating ‘yes’ when the correct letter of the alphabet is read out, and so on.  Computers using predictive text can then speed up this process.  I don’t think that ‘locked in syndrome’ can seriously be dismissed as illusory, or that it can never be unlocked by techniques such as those discussed.  Below is a selection of the comments on one of the websites referred to by Tony Hatfield which appear to bear this out.  But in any case the purpose of this post is not to suggest a verdict on the genuineness or otherwise of the particular Houben case which has revived media interest in the whole subject in recent weeks, nor of FC generally, but rather to point out that there’s a long history of research and clinical practice in this field, including some virtually indisputable successes, going back for well over a decade.  Even if the Houben case were, improbably, to turn out to be genuine, it certainly wouldn’t be the first of its kind, as much of the mainstream media has been suggesting.

Here are some of the relevant comments, with their URLs for those wishing to check:

Locked-in syndrome is a condition, usually caused by a brainstem stroke but also sometimes caused by traumatic brain injury, in which the patient is fully aware and awake but unable to communicate because nearly all voluntary muscles are paralyzed. Usually, locked-in patients can only blink their eyes. From my perspective it is a fate worse than death. The most famous case of being locked-in is Jean-Dominique Bauby, a French journalist who developed locked-in syndrome after suffering a stroke in 1995. Bauby could communicate through blinking his left eye, which was the only part of his body over which he had any control left. Amazingly, he was able to dictate slowly and painfully his experiences by blinking when the correct letter of the alphabet was reached by the person transcribing his memoirs, which were published as The Diving Bell and the Butterfly and later made into a movie.
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php

I work in brain injury advocacy, and have been active in the young people in nursing homes campaign here. I know of several people discharged from hospital with PVS diagnoses who are now clearly, independently communicating (in a couple of cases, become peer support leaders/advocates around YPINH [Young People In Nursing Homes] issues themselves)
Posted by: Maria Strong | November 25, 2009 6:08 AM
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php#comment-2100686

I don’t think adaptive technology is advanced enough, but one twitch for yes, two for no, seems doable. If human fingers could detect the twitch, technology would be able to.
Posted by: LW | November 25, 2009 10:23 AM
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php#comment-2101041

“I don’t think adaptive technology is advanced enough”
Oh yes it is. For years, Dr Stephen Hawking has been able to ‘talk’ just by twitching a single muscle. And he’s not alone, there are dozens like him who can use the same basic technology to communicate.
Posted by: sophia8 | November 25, 2009 11:10 AM
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php#comment-2101163

Indeed, the technology IS advanced enough. There are AAC devices that respond to tiny finger twitches, eye blinks, even gaze-tracking (i.e. all you have to do is scan the board and hold your gaze for a moment on the picture/word/letter you want). Cost is an obstacle to getting access to the more advanced systems, but they do exist. This man’s speech therapist ought to know that, same as she ought to know that FC is complete bunk.
Posted by: borealys | November 25, 2009 12:11 PM
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php#comment-2101348

Notably, though, [Professor Stephen Hawkings has] been declining lately. He can’t work the clicker as easily as he used to. He’s starting to have to use a form of communication that might be described as facilitated, though it’s not what’s shown in the video above. He works closely with an undergrad (who may, by now, be postdoc; I don’t know), who has gotten to know him well enough to be able to predict what he’s saying before he’s finished selecting the particular word. To avoid tiring Hawking, the student sometimes lists a series of words, stopping when Hawking indicates that he’s found the correct one. Notably, Hawking still has facial control, so people can read his emotions to some degree. Even so, it still takes considerable patience on the student’s part. It is very tedious compared to the facilitated communication triumphs that are sometimes claimed.
Posted by: Calli Arcale | November 25, 2009 12:48 PM
http://scienceblogs.com/insolence/2009/11/another_contender_for_the_worst_reportin.php#comment-2101442

Brian