Every Third Thought Page 6

  ‘Part of the trouble we have with Alzheimer’s is that it’s always a difficult diagnosis to make definitively,’ says Lees. Now, more than ever, younger people in their forties and fifties are getting panicky about dementia. He notes that he’s getting a lot of self-referring, middle-aged patients who demand to be seen by a neurologist. ‘It’s becoming quite an epidemic,’ he says. The problem with this surge in anxiety about Alzheimer’s is that, unlike Parkinson’s, there’s no bio-marker to confirm that an individual is unequivocally afflicted – or in the clear. A century since it was first discovered, Alzheimer’s continues to baffle and frustrate neurologists.

  When Dr Alois Alzheimer first identified this condition in the early 1900s, the post-mortem examination of Auguste Deter (who died aged fifty-six on 28 April 1906), revealed that her diseased brain had become small and shrivelled with deep fissures. The vandalism perpetrated by Alzheimer’s on the 100,000 million tiny nerve cells that make up our ‘grey matter’ is both horrifying and horribly destructive. This network of neurons, which has been the basis of memory, habit, language, and, ultimately, human consequence, cannot survive the assault. Deep in the cortex, the seahorse-shaped part of the brain known as the hippocampus creates humanity by processing a myriad external sensations, creating memory. Without memory, the human animal becomes, quite simply, a brute beast deprived of character and personality.

  Taking a global perspective, the World Health Organisation has declared that dementia should become a global public-health priority. This looks like a quixotic campaign. From 2000 to 2012, about 99 per cent of newly developed dementia drugs failed to pass their clinical trials. Not only is there no cure for Alzheimer’s, there’s none in prospect either.

  Lees, the off-duty magician, with a penchant for the psychedelic, cherishes the unpredictability of his calling. Who else, for instance, would take pleasure in reporting, as he did in a recent issue of the magazine Brain, that when he first came to study at University College Hospital, one of his mentors recommended him to read The Complete Stories of Sherlock Holmes? Lees described how William Gowers liked to teach his pupils that Arthur Conan Doyle had not only specialized in neuro-degenerative diseases as a doctoral student, but also that Holmes’ famous ‘method’ was one that the professional neurologist could profitably follow.

  In neurology, Lees notes, the diagnosis should begin the moment a patient walks into the room, based on the observation of his or her speech, aspect, and gait. Lees himself will say that even the most advanced technology can never replace the powers of simple observation. A hospital, he remarks, ‘is still a crime scene’, where the finest minds are needed to solve its mysteries. The National Hospital in Queen Square, Lees reports, is ‘my laboratory’. For twenty years, as an out-patient and stroke convalescent, I have been lucky enough to witness some of the work performed in this lab.

  Today, as we talk about his work, Lees describes how, as a younger man, he had developed the use of apomorphine through self-experimentation, inspired by Burroughs. ‘It was with some trepidation,’ he confesses, ‘that I injected myself with 1mg of apomorphine as the prelude to a fuller clinical investigation.’ As a result of his willingness to take such risks, apomorphine became licensed for routine treatment, with some remarkable results during the 1970s. Initially, this was the focus of Lees’ research. He would not move into the grimmer world of Alzheimer’s treatment until the 1980s and ’90s. Today, he is one of its foremost specialists in the world.

  *

  As a literary critic, I have occasionally speculated about ‘the narrative gene’ in our brains. How else to explain the human animal’s timeless and universal fascination with ‘story’, or the importance of good storytelling in the conduct of culture, politics, and society? Today, I find that this special opportunity to discuss ‘losing the plot’ with Professor Lees stimulates my imaginative response to the tale he begins to tell, the experience of witnessing a human mind becoming progressively untethered from its reason. In fact, I’m riveted.

  Lees starts, quite gently, with the mental slowing and inflexibility naturally associated with ageing. Cerebral atrophy, he tells me, will make the brain of some ninety-year-olds look like a shrivelled walnut, a quite tangible decline. A healthy adult in the prime of life has a brain that’s like a balloon which has been crumpled up to be stuffed into the skull. The more evolved the mammal, the more convoluted the brain becomes. Very simple mammals have relatively smooth brains. Our brains are very convoluted. As we get older, the substance of the brain shrinks. Our increasing difficulties with decision-making in later life, the origin of a proverb like ‘You can’t teach an old dog new tricks’, hold a special fascination for Lees. He is all too aware of the way in which the changes attributable to ageing contribute to the negative stereotype of the elderly as obstinate, selfish, infantile, incompetent, and crotchety, and adds that ‘the saving grace of wisdom depends heavily on long-term memory’.

  Here, he makes this prediction: ‘As society’s attitudes to Alzheimer’s evolve and the threshold for diagnosis lowers, more and more people over the age of sixty will be asked a series of questions designed to probe their orientation in time and place, their short-term memory, and their language skills.’ In the near future, then, we are likely to blame the qualities we associate with age less on the individual and more on the afflictions of dementia.

  Once the difficult diagnosis of Alzheimer’s has been made, the narrative of this disease defines one of the greatest scourges of the twenty-first century. This is a neuro-degenerative affliction that strips the patient of all the qualities we recognize as human. If that sounds like hyperbole, consider a typical account of the physical and mental trajectory experienced by an Alzheimer’s sufferer. At first, invoking the language of the consulting-room or the lab, Lees describes the trajectory of Alzheimer’s as ‘distressing’. When pressed, he concedes that it’s ‘scary’.

  The poet W. H. Auden once compared Death to ‘the rumble of distant thunder at a picnic’. The first three stages of Alzheimer’s typically occur as darkening storm clouds forming, at first on a distant horizon, as it were, at the end of a perfect summer’s day. Every sixty-something knows the thin line in mundane consciousness between accepting a measure of mild forgetfulness (‘Now where did I put my car keys?’), and worrying about the darker significance of a more persistent memory loss. At a certain age, almost everyone will joke about ‘losing the plot’. Many Alzheimer’s patients, in their fifties and sixties, will be active and vigorous, successful citizens living full rich lives.

  The initial symptoms – occasional flashes of anger, inexplicable forgetfulness – are so slight that many families will miss them, attributing sudden bouts of irrationality to the frustrating mystery of old age. As we have already seen in the case of Terry Pratchett, more alert and self-aware patients may sense that something is wrong, something that, at this stage, many outsiders, and even some doctors, might overlook.

  The second phase in the progress of the illness becomes the unmistakable onset of memory loss: an inability to recognize acquaintances, or some repetitive questions in everyday conversation. Once again, the patient’s friends and family may be inclined to attribute this to ageing – until it becomes clear that something terrible is happening to the patient’s brain.

  Finally, in the third stage of Alzheimer’s, Lees says that it becomes impossible to ignore the disintegration of the patient’s mind. Mothers and fathers, uncles and grandparents, become palpably ‘mad’. Now a full medical examination and diagnosis becomes inevitable. For families and loved ones, in particular, there can be no more denial. In the end, says Lees, ‘you are no longer able to treat the patient. All you can do is do what you can to help their families.’

  Sometimes, the brain can be strangely merciful. At this dreadful turning point, it is likely that when the patient is informed of his or her fate, they will no longer be competent to retain, or respond to, this information. As Lees puts it, ‘Alzheimer’s ignites in the hippo
campus and the entorhinal cortex. Once these parts of the emotional brain have been burned down, there can be no more episodic memory.’

  This terrible moment spells the end of an individual’s character and personality. With episodic memory gone, past and present become for ever dissociated. A thousand daily functions – for example, knowing how to make a cup of tea, and remembering where to find, and how to use, milk and sugar – become impossible. Once episodic memory has gone, the Alzheimer’s patient is at the mercy of cerebral Furies, a process that falls into further patterns of horrifying dehumanization. ‘You have to ask yourself this question,’ says Lees. ‘Are we human without memory? Almost the only means of human communication at the end will be the comforts of touch, and perhaps some music.’

  *

  Alzheimer’s patients exhibit many responses to this ‘silent plague’. First, there is incomprehension. This can sometimes be a comparatively benign state in which the patient becomes merely childlike. The novelist Iris Murdoch, who was finally diagnosed with Alzheimer’s in 1997 (she died in 1999), would spend her days watching children’s TV. Her husband, John Bayley, reported in his memoir, Iris, that his wife ‘remains her old self in many ways’. He goes on:

  The power of concentration has gone, along with the ability to form coherent sentences, and to remember where she is, or has been. She does not know she has written twenty-seven remarkable novels . . . If an admirer asks her to sign a copy of one of her novels . . . it takes her some time, but the letters are still formed with care, and resemble, in a surreal way, her old handwriting. The old gentleness remains.

  Once forgetfulness advances more completely, language itself becomes redundant and – without words – how can life have any meaning? At this point – this is the next stage – the patient might become disturbed and violent. Subsequently, as dementia takes hold, there will be a need for hospitalization, where possible. In some cases, there will have to be a referral to a nursing home for twenty-four-hour care.

  Lees describes to me how, at the peak of his career, he would treat as many as a hundred dementia cases. Now he confines himself to just a handful of Alzheimer’s patients. He observes, with sadness, how the disease takes its toll on everyone: spouses, families, children, and partners. There will be no respite. Typically, all involved will long for the death of the afflicted, but this may not occur for five, seven, even ten years. In this acute stage, the fate of the Alzheimer’s sufferer is terrifying. The complete destruction of what Lees calls ‘the emotional brain’ may result in explosive rage, wild cravings for food, and a kind of manic fearlessness. Mixed in with an intolerable loss of social skills, there will be the almost total inability to recognize old friends and family, or recall past events. The annihilation of the self deprives the patient of all autonomy, reducing them to a sub-human husk.

  For the care-givers, the devastation wrought by Alzheimer’s is like a life sentence, in which every nuance of everyday life becomes a source of anxiety and pain. In Iris, John Bayley recalls ‘a brisk exchange’ with a fellow carer, a woman whose husband also has Alzheimer’s. ‘Like being chained to a corpse, isn’t it?’ says the woman cheerfully. ‘A much-loved corpse naturally,’ she amends, with an implicit suggestion that Bayley might be willing to abandon what he calls ‘the usual proprieties that went with our situation’. Meanwhile, alone at home, there are precious few ‘proprieties’. Bayley has to struggle to stay in touch with his ‘lost’ wife:

  Our mode of communication seems like underwater sonar, each bouncing pulsations off the other, and listening for an echo. The baffling moments at which I cannot understand what Iris is saying, or about whom or what . . . can sometimes be dispelled by a jokey parody of helplessness, and trying to make it mutual. Both of us at a loss for words.

  As well as the loss of language, there is the growing loss of affect, and facial expression. The Alzheimer face is often described as ‘the lion face’ – sombre, expressionless, and frozen in leonine impassivity, occasionally broken by a mad smile. Bayley, however, manages to find humanity and even fun in mundane tasks, like getting up in the morning. ‘Dressing most days’, he writes, ‘is a reasonably happy and comic business. I am myself still far from sure which way her underpants are supposed to go: we usually decide between us that it doesn’t matter . . . I ought to give her a bath, but I tend to postpone it from day to day.’

  Bayley does his best, in his stoic English way, to put a brave face on things, but an inevitable bleakness creeps in: ‘Most days are in fact for [Iris] a sort of despair, although despair suggests a conscious and positive state and this is a vacancy which frightens her by its lack of dimension.’ Bayley tries to deal with the anxiety by suggesting a distraction like a shopping trip in the car. ‘Something urgent, practical, giving the illusion of sense and routine.’

  For the Alzheimer’s nurse, there remains the consolation that their patients are usually oblivious to their condition. In the clinical atmosphere of the National, I am finding it hard to imagine such a state of mind, and Lees is trying to elucidate his experience for my benefit. Reflecting on this stage of the disease, he reports that ‘some sufferers do remain conscious of their state, paradoxical as this seems. The torment of knowing that you cannot speak or think what you want must be intolerable, and I have met patients in whom such torment is clearly visible.’ Discussing this unprecedented torture of the mind, Lees projects a kind of priest-like serenity that must, I speculate, have been a great comfort to his patients and, most especially, their carers.

  Iris Murdoch, contrariwise, seems to have retained some sense of reality. When Bayley surrendered to his care-giver’s compulsion to demonstrate how bad things were, his wife would surprise him. ‘I made a savage comment today,’ he writes, ‘about the grimness of our outlook. Iris looks relieved and intelligent. She says: “But I love you.”’ Her husband fights with his rage (‘I get Iris into a seat and give her a violent surreptitious punch on the arm’), and also with the torment that this brings:

  The exasperation of being followed about the house now by Iris is as strong and genuine as is my absolute need for it. Were she to avoid me, I would pursue her as anxiously, if not quite so obsessively, as she now pursues me.

  Heartbreakingly, every now and then Iris would articulate her feeling that she was ‘sailing into darkness’.

  If he were a neurologist, Bayley concludes, he would find it hard to believe in such flashes of lucidity. ‘The words which Iris used [as a novelist] with such naturalness and brilliance cannot be stacked there, silently, sending out an occasional signal. Or can they?’ He notes that his wife’s ‘eerie felicities’ are like the things a young child will come out with to delight and amuse parents and friends.

  Finally, there is always Bayley’s own terror of imminent mortality, a strange, sad coda to an infinitely sad and affecting memoir:

  What will [Iris] do if I die? If I’m ill and have to go to hospital; if I have to stay in bed – what will she do then? I make these demands with increasing hostility and violence. I am furious to see my words are getting nowhere, and yet relieved too by this, so that I can continue my fury.

  Andrew Lees, musing now on a lifetime’s experience of neuro-degenerative diseases, tells me that, as a doctor, he can never predict his patients’ response to their plight. ‘It’s hard,’ he concludes, ‘not to present their predicament as anything but terribly bleak,’ and yet there are occasional moments of humanity.

  When former US president Ronald Reagan, who also suffered from Alzheimer’s, first became conscious of his failing memory, he would cover up his forgetfulness with feeble jokes. Lees says such strategies are quite common, as a defence mechanism. ‘Especially at the beginning, there’s a lot of anxiety. But occasionally you’ll find a quite different reaction.’ He recalls a recent patient, a man in his sixties whom he described to me as ‘a happy man’. At one consultation, to test his condition, Lees had asked the patient if he knew what day of the week it was.

  ‘Why do you ask
me that?’ said the man, in a tone of puzzlement. ‘At my age, the day of the week really does not matter. I’m retired. I walk the dog. I watch television. In the afternoon, I have a little sleep. Days of the week don’t matter to me.’

  To every question that Lees put to him, this man had ‘a smart answer’ of Zen-like equanimity. A bit later in the consultation Lees asked, ‘Do you know where you are?’ To which his patient cheerfully replied, ‘I’ve no idea, but it doesn’t matter. My wife brings me. She’s here; she’ll take me home. That’s enough.’ Lees can only laugh. ‘His wife was sitting behind him, and she was almost beside herself. She’d had it up to here.’ He shakes his head. ‘In the end, it’s the families that stop visiting. There’s no point. It becomes too painful.’

  With some regret, even dismay, Lees will concede how painfully slow the progress of neurological research has been throughout his career, noting that there are no good animal models available for research into neuro-degeneration. ‘We still don’t know if old primates can develop Alzheimer’s,’ he observes. ‘We have to look at human tissue to try to understand what’s going on in the brain, and we are making some progress with “brain-mapping”, though that can be over-interpreted.’