Silent scream heard around the world

Published on Saturday 28 November 2009 19:12

FOR 23 years, Rom Houben – a one-time engineering student who could speak four languages – was imprisoned in his own body.

Paralysed in a car crash in 1983, doctors diagnosed him as being in a permanent vegetative state: that is they believed that, though his eyes were open, he was unaware of anything going on around him. In fact, according to neurologists in his native Belgium, Houben was always conscious: for more than two decades, he lay outwardly impassive, but "screaming" with frustration inside, as a succession of experts wrote him off.

Unable even to communicate with his eyes – as many paralysed people can – there was no way for him to alert medical staff to his plight. When he heard doctors say he was as good as dead, he couldn't tell them they had got it wrong; when his mother and sister told him his father had died, he was unable to express his sorrow.

With all form of interaction denied him, he learned to live entirely within his own mind. "I travelled with my thoughts into the past, or into another existence altogether," he has said. "Sometimes, I was only my consciousness and nothing else."

Today, at the age of 46, Houben is finally able to share his experience of "locked-in syndrome". In the three years since hi-tech brain scans carried out by pioneering doctor Steven Laureys at the Coma Science Centre at the University of Liege showed Houben's brain was functioning almost normally, he has undergone intensive physiotherapy and is now able to communicate through a computer keyboard, with the aid of a therapist.

And last week, Houben told his amazing story to a public aghast that such a thing could be possible. "I screamed, but there was nothing to hear," he wrote. "I dreamed myself away."

His story echoes that of Jean-Dominique Bauby, the former editor of Elle magazine who became "locked-in" after suffering a massive stroke at the age of 42. Bauby wrote a poignant account of his ordeal in the book The Diving Bell And The Butterfly, later made into a film. But for all Bauby's suffering, his "locked-in" condition was recognised quite quickly. He was able to dictate his memoirs by blinking his one good eye when the letter of the alphabet he wanted to use was spoken, and they were published shortly before his death. Houben's years of misdiagnosis is, on the other hand, the stuff of existential nightmare. More terrifying still is the thought that his is not an isolated case. According to two surveys carried out by the Royal Hospital for Neuro Disability in London in 2003, the proportion of misdiagnosis could be as high as 43 per cent.

That means that of the estimated 2,000 PVS patients in the UK, more than 800 could still have some degree of awareness.

The Royal College of Physicians' guidelines say only when a patient has been in a persistent vegetative state for a year can he or she be diagnosed as being in a permanent vegetative state. Once diagnosis is considered definite, moves can be made to have feeding tubes withdrawn, although – even now – each case has to be individually sanctioned by a court.

Houben's case has reawakened argument over euthanasia and living wills. Ever since the families of Hillsborough victim Anthony Bland in England and Janet Johnston, who had taken a drugs overdose in Scotland, won the right to have their feeding tubes removed, emotions have run high over such cases.

But campaigners are divided on what Houben's case does to the debate. For pro-lifers, the fact PVS patients could be conscious is further reason enough not to give up on them.

Those who back euthanasia, however, take a different message from the story. Some see Houben as a symbol of why those who have suffered horrific brain injuries should be allowed to die with dignity: if a patient is truly PVS, then at least they're not suffering unduly, their argument goes. But if they are in pain and unable to communicate, their lives must be intolerable and should be brought to an end.

Even more fevered than the ethical debate, is the discussion over whether the Houben case is a hoax. This allegation, gathering momentum in the US, is centred on the way Houben is said to communicate. In a TV clip released last week, his hand appears to be guided by the therapist, with Houben reportedly using "gentle pressure" to tell her which keys to type.

But according to the US sceptics – including renowned "demystifier of pseudoscientific bunk" James Randi and biologist PZ Myers – her hand is moving too fast for him to be controlling it, while his eyes are almost shut. They are calling for "an end to this farce".

"That's called 'facilitated communication'," Prof Arthur Caplan, a bioethics professor at the University of Pennsylvania, added. "That is Ouija board stuff. It's been discredited time and time again. It's usually the person doing the pointing who's doing the messages, not the person they claim they are helping."

Houben's story may well stretch the limits of our imaginations, but this is far from the first time this has happened. In 1996, for example, Mark Newton fell into a coma while diving. He had been unconscious for six months, and doctors had recommended turning off life support.

Shortly after, however, he regained his capacity to communicate and told doctors he had been aware of what was going on around him throughout his time in hospital. "I used to talk to people and just assume they could hear me," he said. "People used to look at me sitting with my Walkman on and say: 'Ah, he's listening to his tape.' I'd be shouting: 'No, I'm not; it's finished. Someone please turn it over'."

Dr Ashraff Ali is a consultant of neurological rehabilitation at the Royal Hospital of Neuro Disability – the most pioneering centre of its kind in the UK – and the place where Newton was treated. He says the video of Houben communicating through the keyboard is too short to draw any conclusions about the Belgian's cognitive capabilities. But every year, the hospital discovers signs of consciousness in a handful of supposedly PVS patients, usually within a few weeks of them being referred.

Many of those who have been wrongly diagnosed are visually impaired, making it more difficult for doctors to test their responses to external stimuli.

But there may also be a problem with lack of time and experience, with some diagnoses made on the basis of inadequate assessment. If the patient applies pressure with his hand later on, medics will tend to dismiss this as an involuntary reflex.

"Our assessments take 12 weeks and are carried out by a multi-disciplinary team of doctors, nurses, occupational therapists, music therapists and are based on a patient's response to certain stimuli: visual, auditory, olfactory and touch," Ali said.

"Before we do that, however, we have to make sure the patients are physically stable: that they are free of infection; that they are not taking medication that would make them drowsy and that they are comfortable and well-supported."

They have had many success stories. In one case, they discovered a man who had been diagnosed PVS and in a nursing home for eight years, was able to move one of his shoulders. "Over time he learned to use this movement to communicate and the first thing he did was to tell his wife 'I love you'," Ali said.

The Royal Hospital has lots of hi-tech equipment which helps patients use what little movement they have to communicate. For example, there is a device which allows the patient to scan a screen full of letters and trigger the one he wants to type with his eye. Sometimes, when the doctors are almost certain the original PVS diagnosis is correct, they will send them to Addenbrooke's Hospital for a functional MRI scan just to make sure.

But patients in the UK do not undergo the battery of tests, including PET scans and electroencephalography (EEG), which revealed Houben's high degree of consciousness. Last week, he described his reaction when his true condition was first discovered. "I especially felt relief," he wrote. Finally able to show that I was indeed there." He went on to explain how difficult it was to learn to communicate again after so many years in silence. "Just like with a baby, it happens with a lot of stumbling," he wrote.

In response to the growing number of doubters, Laureys, who has been described as "a very rigorous scientist and physician", said he had verified the facilitated communication was genuine by showing Houben objects when the therapist was out of the room and later asking Houben to recall them.

His mother Fina Nicolaes also insists her son has been communicating with her for three years. "At first he had to push with his foot on a sort of computer mouse which only had a yes-no side," she said.

She always believed her son was aware; she continued to take him on days out and arranged for him to go to the US five times for tests, before finally finding Laureys. Now she says she appreciates the jokes and black humour that lace her son's sentences.

The doubters remain unimpressed. But then perhaps they have their own agenda. American bioethicist Wesley J Smith believes many of those claiming the case is a hoax are "using scepticism as an ideological tool to keep society from drawing ethical conclusions that the sceptic might oppose". He believes some scientists have an agenda to use PVS patients for organ harvesting or experimentation.

As the moral debate rumbles on, Houben, who plans to write his autobiography, just wants to get on with his life. He has a lot of catching up to do.

Levels of consciousness

COMA: A coma is a profound state of unconsciousness in which a person cannot be awakened by pain or by vigorous stimulation. It can be caused by head injuries, strokes, metabolic imbalances, loss of oxygen to the brain or overdoses. If lower brain centres are damaged, a respirator may be required for the person to breathe. The damage may be reversible or irreversible.

PERMANENT VEGETATIVE STATE (PVS): Patients in a vegetative state are those who have been in a coma and progressed to a state of wakefulness, but without any detectable awareness. After four weeks in a vegetative state, they are said to be in a persistent vegetative state. After a year, they are said to be in a permanent vegetative state. Unlike a person in a coma, a person in a PVS has sleep-wake cycles and may cough, sneeze, scratch and even cry or smile at times.

LOCKED-IN SYNDROME: Locked-in syndrome is an exceptionally frustrating state of consciousness for a victim. Due to paralysis of the whole body, a person cannot communicate or move, but is aware and awake.

BRAIN DEATH: A brain dead state reflects the absence of brain function. Before life support equipment was invented, the body would die as soon as the brain died. Brain death is irreversible.