A Pound a Glimpse
- A Smell of Burning: The Story of Epilepsy by Colin Grant
Cape, 242 pp, £16.99, August 2016, ISBN 978 0 224 10182 0
- The End of Epilepsy?: A History of the Modern Era of Epilepsy, 1860-2010 by Dieter Schmidt and Simon Shorvon
Oxford, 208 pp, £39.99, September 2016, ISBN 978 0 19 872590 9
In 1926, Graham Greene received a diagnosis of epilepsy. In all likelihood, he didn’t have the disorder. His only symptoms were three isolated episodes of lost consciousness: once in the school chapel at Berkhamsted; once in the London home of his psychoanalyst; and once in the offices of the Times, where he was working as an assistant editor. But the neurologist he consulted thought these episodes were sufficient grounds for the diagnosis, and the news nearly led Greene to throw himself in front of a speeding tube train. He was 22 years old and engaged to be married. He’d written two unpublished novels. The diagnosis threatened to upend all of his cherished plans for the future, not least his plans for a family: his analyst had told him (not quite accurately) that epilepsy could be inherited. But what unnerved Greene was something less rational. He seems to have felt that he had been prematurely and indelibly marked. Epilepsy wasn’t just a difficult chronic condition, like migraines or gout. Nor was it something dishonourable he could at least blame on his own behaviour, like syphilis. It was an affliction, an awesome, inexplicable curse. As he wrote decades later in A Sort of Life, the first volume of his autobiography, ‘epilepsy, cancer and leprosy – these are the three medical terms which rouse the greatest fear in the untutored and at 22 one is unprepared for so final a judgment.’
Epilepsy, cancer and leprosy. Who, in 2017, would think to group these conditions together, let alone declare them the most fearsome trio in the medical pantheon? Of the three, only leprosy retains any aura of external judgment, and that aura is both anachronistic, a hangover from the medieval period, and obsolete: in the West, leprosy has been all but eradicated. Cancer, of course, is still a scourge, but it is hardly monolithic and, given modern radiation, chemotherapy and surgery, not to mention advances in genetics, not necessarily ‘final’. As for epilepsy – who thinks of it much at all any more? Who now considers it to be a life-shattering sentence? What young person today, on receiving a diagnosis of epilepsy, would say to himself, as Greene did: ‘This is it. This is the end of the line for me’?
But in 1926, Greene’s despair made perfect sense. The medical historian Owsei Temkin has shown, in his monumental book The Falling Sickness (1945), that, socially speaking, there has never been a good time to have epilepsy. To the Greeks and Romans, Temkin writes, the epileptic was ‘an object of horror and disgust’. An epileptic fit was a terrifying omen, a sign of the gods’ displeasure, and a common response to witnessing one was to spit, to avoid contamination. In Europe, from the beginning of the Christian era up to the Renaissance, the condition was usually considered a sign of demonic possession, black magic or witchcraft. At best, it was evidence of a wicked character. (Kent to Oswald in King Lear: ‘A plague upon your epileptic visage!’)
But there has probably never been a worse time in history to have epilepsy than the century beginning around 1850. At that time, a noxious stew of pseudoscientific, Social Darwinist ideas linked the condition to a whole range of undesirable and ignominious traits: idiocy, madness, sexual deviancy, immorality, criminality. Two of the most common beliefs about epilepsy throughout the period – beliefs held by both doctors and non-doctors – were that observing an epileptic seizure could cause one, and that masturbation caused epilepsy by weakening the nervous system. By the start of the 20th century, most of the epileptics who had previously been confined in asylums had been removed from them and segregated – not to improve their treatment but to protect ordinary lunatics from becoming infected. Consequently, epileptics began to hide themselves from public view, or else, as in the case of Prince John, the epileptic fifth son of George V, they were hidden away by their families.
The rise of eugenics made things much worse. Eugenicists fixated on epilepsy as a hereditary threat to be tackled by both negative and positive countermeasures. By 1914, 13 US states had barred people with epilepsy from marrying. In 1927, a year after Greene had stood on an underground platform contemplating suicide, the US Supreme Court in Buck v. Bell ruled in favour of the forced sterilisation of ‘mental defectives’, including epileptics. ‘It is better for all the world,’ Justice Oliver Wendell Holmes Jr wrote, ‘if instead of waiting to execute degenerate offspring for crime or to let them starve for their imbecility, society can prevent those who are manifestly unfit from continuing their kind.’ Over the next thirty years, as many as 50,000 men and women with epilepsy were sterilised in the US. Under the Action T4 programme in Nazi Germany (where officials had noted Buck v. Bell with approval), thousands were simply murdered.
These are not just disturbing facts. They are disturbing facts that lie in uncomfortable proximity to our own time. Nowhere else in medicine or psychiatry do we find a stigma as ancient, persistent and ultimately florid as that which has dogged epilepsy. Any worthwhile account of epilepsy must therefore ask not only what caused the condition to be reviled but what has become of that revulsion. Are we really so enlightened now? Has the stigma that reigned only decades ago disappeared, or has it merely become less visible, less garish?
A Smell of Burning, Colin Grant’s fourth book (he has also written about reggae and about Marcus Garvey), is preoccupied by questions of stigma – haunted by them, even. The book is a hybrid of memoir, medical history and social history of a kind that has become familiar in the past twenty years. Like many contributions to the genre, it was born out of anguish. When Grant was in his twenties, his teenage brother, Christopher, the youngest, most loved member of the family, began to have seizures. At first, Christopher’s seizures took the form of fainting spells. One moment he would be upright, the next on the floor, unconscious. Grant and his family called these episodes ‘collapses’. As time went on, the seizures grew more varied. Grant describes episodes of full-blown convulsions – commonly known as grand mal seizures – in which Christopher would writhe and thrash on the ground, his teeth clenched, his neck twisted. He describes ‘complex partial’ seizures during which Christopher would lose awareness but continue to move through the world: more than once police found him walking in the road without his shoes. And he describes moments of absence – short-lived, blank-eyed lapses of awareness. He also describes the grand mal seizure that killed Christopher at the age of 39. The final seizure was no different in kind from its predecessors. The coroner listed the cause of death as Sudden Unexplained Death in Epilepsy, SUDEP. The name concedes the reality of medical knowledge about the phenomenon. Half of all epilepsy-related deaths are from SUDEP. No one knows what causes it. The heart just stops.
For years, Grant has worked as a radio producer for the BBC, but he first studied to be a doctor. This helps to account for the book’s tone, which is one of apology, a kind of nagging guilt. It’s hard enough to watch as your brother’s life is proscribed and finally curtailed by disease – as he struggles to find steady work, makes his way in and out of hospitals, loses the right to drive (you must have been free of seizures for two years to hold a licence), and becomes fixated on his own funeral (Christopher insisted on bagpipes). When you have been trained to alleviate suffering but are helpless to alleviate, let alone cure, your brother’s it’s harder still. It’s cold comfort that, as Grant acknowledges, the clinical picture of epilepsy is extraordinarily fraught. As he writes late in the book, ‘many forms of epilepsy are idiopathic: science has yet to discover what causes it in the majority of cases, and why some individuals are predisposed to it.’
Even to speak of epilepsy in the singular, as a unified disorder, is in fact misleading. Just as ‘cancer’ refers to the tendency of a person’s cells, of whatever type, to proliferate abnormally, ‘epilepsy’ refers to the tendency of a person’s brain cells, of whatever location or network of locations, to fire abnormally. The name of the condition merely points to the presence of a symptom – seizures. Since brain cells control all human functions, the potential consequences of their misfiring are enormous. Seizures can cause everything from graphomania to aphasia, déjà vu to jamais vu, obsession to apathy, tingling to hallucinations, tics to convulsions. Grant tells the story, first reported in the Lancet in 1954, of a patient at the Maudsley Hospital in London whose petit mal seizures were somehow connected to his ability to be sexually gratified by the sight of a safety pin. He also speculates that the novelist Philip K. Dick’s 8000-page late work, Exegesis, was inaugurated by a temporal lobe seizure that led Dick to believe with certainty that he had become a first-century Christian fleeing Roman persecution.
The mystery of what causes epilepsy in the first place remains unsolved. In some cases, where there has been a cerebral tumour, drug withdrawal, or head trauma (the rate of epilepsy in veterans of the Iraq and Afghan wars is alarmingly high), the cause is clear. But in most it remains obscure, hidden by the almost cosmic complexity of the brain and all that influences it – genetic, physiological and environmental. Twenty-four hundred years ago a physician in the Hippocratic school argued, in the first surviving monograph on the subject, that epilepsy was a disorder of the brain. We’ve advanced leagues beyond that fact. The development of electroencephalography, or EEG, in the 1940s has allowed clinicians to distinguish between types of seizure and to discern where in the brain seizures begin and how they spread. More recently, neuroscientists have worked out the mechanisms of seizures down to the cellular and even the molecular level. We now know a great deal about the ‘how’ of epilepsy. We know what happens when epilepsy strikes. What remains hazy is why it strikes in the first place.
This doesn’t make epilepsy unique. We don’t know the ultimate cause of any number of disorders, including multiple sclerosis, autism, motor neurone disease and most cancers. In many diseases there is no single cause. Nature is not so neatly mechanistic. But a central point of Grant’s book is that epilepsy is different from other disorders because of the unusual, dramatic effects it has on people. Multiple sclerosis can’t make a man feel as if heaven had descended and engulfed him, placing him in direct contact with the divine, as epilepsy did for Dostoevsky. No one has ever described motor neurone disease as a ‘very psychedelic experience’, as Neil Young (whose childhood nickname was ‘Shakey’) has described epilepsy. In a word, epilepsy is strange. It affects behaviour, sensation, belief and even personality. It changes people. It is also diagnostically indelible. Once someone has shown a predisposition to seizures, that person is epileptic for life. The label and the identity – ‘being epileptic’ rather than ‘having epilepsy’ – stick. Not knowing what causes the condition can make it that much more difficult for us to shake the sense that the epileptic is someone apart from us, and that his sudden extravagant fits represent something more meaningful than a mere electrical glitch. There is a relationship between our ignorance about the condition and our response to it. Epilepsy, Grant writes, is still ‘an enigma and something of a taboo subject. We may have conquered our fear of carcinomas but conditions like epilepsy remain truly disturbing.’
This presses the case too far. But the virtue of Grant’s memoiristic approach is that it gives a nuanced and particular account of how the stigma associated with epilepsy persists, and how it can deform a life. Christopher bore his condition with stoicism, courage and a sense of irony. But one gets the impression that this was a defensive posture – a way to shield himself from, and when possible to outwit, the fears, apprehensions and preconceptions of the non-epileptic world. This world naturally included almost everyone he encountered: employers from whom he hid his epilepsy in order to get a job, only to lose it once his seizures proved disruptive; A&E doctors who mistook his post-seizure confusion for psychosis and moved to have him institutionalised; his own neurologist, who could talk to him about his condition only in terms of pharmaceutical dosages and medication compliance. It also included his family. Both of Christopher’s parents – Jamaican immigrants to England – responded to his epilepsy with a jumble of affection, concern and jittery superstition. His father referred to it only as ‘head trouble’, as if trying to establish a protective distance. His mother, an evangelical Christian, thought it might be the work of the devil. (Once, when Christopher had a fit in church, the pastor tried to exorcise the spirits.) His grandmother, who called it an ‘affliction’, considered it an opportunity for Christ to offer his mercy. ‘You see! Can’t you see?’ she said after Christopher received his diagnosis. ‘You see the light above his head? You see the light? Jesus is here.’
Throughout A Smell of Burning, Grant implicates himself in the problems of epilepsy and its meaning. His first chapter centres on an occasion as a medical student when he recoiled in confusion and horror from a patient’s epileptic seizure. The ‘shock at the spectacle’ exploded his clinical reserve, but along with his repulsion he detects a ‘prurient fascination in witnessing the otherworldliness of someone in the grip of a grand mal seizure’. This fascination, he recognises, can be just as disfiguring as disgust, and it never leaves him. It emerges in the book, and in his dealings with his brother, as a ‘tendency … to bestow a kind of nobility on people with epilepsy’. He parades a long line of geniuses, trailblazers and heroes – some of whom, like St Paul, Joan of Arc and Van Gogh, may or may not have had epilepsy, and some of whom, like Julius Caesar, Dostoevsky and Harriet Tubman, almost certainly did. He also keeps vigil over his brother, determined ‘to discover what it was that marked him with epilepsy’ – how he had been changed by the condition and what it was that made him different from the rest of us. Christopher only wants to be left alone. Feeling his brother’s eyes on him, he responds: ‘I’m going to start charging, you know. A pound a glimpse. A fiver a long hard stare.’
The title of Grant’s book refers to the warning signs, or ‘auras’, that often precede an epileptic attack. Sights, sounds, smells and physical sensations can signal that a fit is imminent and give an epileptic time to prepare and safeguard himself from injury. (Neil Young has said there are ‘about 45 seconds’ between his experience of an aura and the onset of a seizure.) The title of Dieter Schmidt and Simon Shorvon’s book echoes the title of the final chapter of Temkin’s seminal history, ‘The end of falling sickness?’ Falling sickness was the popular name for epilepsy from the second century on. Temkin’s question mark denotes his caution about assuming we’ve left old ideas behind. His history extends from ancient Greece to the second half of the 19th century, when the modern discipline of neurology and more sophisticated medical investigations of epilepsy emerged in tandem. It was Temkin’s hope that, with further scientific progress, epilepsy might cease to be ‘a paradigm of the suffering of both body and soul’.
Schmidt and Shorvon are both eminent neurologists – at the Freie Universität in Berlin and University College London respectively – and their book is a kind of modest, reflective sequel to Temkin’s. The End of Epilepsy? picks up where Temkin left off, with the work of the 19th-century neurologist John Hughlings Jackson, whose insight that seizures are caused by sudden, excessive ‘discharges’ of brain tissue revolutionised our understanding of epilepsy and still forms the core of our definition today. Schmidt and Shorvon adopt Temkin’s approach and consider epilepsy as ‘a fundamentally social as well as medical phenomenon’. Seizures, they write, are occasional and fleeting, ‘but what has been described as “being epileptic” is a permanent internal (existential) state of being, a psychic position.’ In the last fifty years, they report, around 200,000 articles have appeared on epilepsy or epileptic seizures. Governments, universities and foundations have spent billions of dollars on medical research and development. Schmidt and Shorvon look at how this has improved clinical outcomes for epileptics, and consider whether it has enabled them to live their lives in a freer, fuller way. Anyone who wants a cogent overview of the science and modern history of epilepsy would do well to read this book. But they reach conclusions which, as they admit, many of their colleagues may find idiosyncratic and over-critical. For example, they acknowledge that in clinical terms epileptics are pretty well served. Blockbuster anti-epileptic drugs such as carbamazepine and valproate provide relief from seizures in more than 80 per cent of patients. This makes epilepsy ‘one of the most treatable of all neurological conditions, with a success rate that is much higher than in stroke, Parkinson’s disease, dementia or brain tumour’. Two out of three patients will experience complete remission of their seizures within twenty years. Still, Schmidt and Shorvon are disappointed. The drugs are helpful, yes, but they are only palliative. They suppress seizures; they don’t stop them. (No one knows exactly why seizures stop when they do.) They also cause side effects that range from mild fatigue to depression to heart failure. Worldwide, 65 million people have epilepsy. More than 20 million of them will at one time or other become ‘drug-resistant’, and a typical clinical course means a time-consuming layering of different drugs to find the right balance. ‘A physician,’ they write, ‘might ask a patient to start taking a common anti-epileptic drug for a few months and see if it works. If it doesn’t, they start over with a different drug, which is often added to the medication. The fraught process can mean weeks of suffering and false hope for patients.’ To make matters worse, once you start taking an anti-epileptic drug, it’s very hard to get off it. You risk withdrawal symptoms, psychological effects and, of course, the recurrence of seizures. As a result, many epileptics choose to live permanently with medication and its side effects.
This state of affairs is common to many psychiatric disorders: the frustrating trial and error, the agitated waiting, the sense that even in success you are a slave to a blister pack. The situation is unarguably better than it was a hundred and fifty years ago, when the only anti-epileptic drug available was potassium bromide, which could cause a host of horrifying side effects, including hallucinations, diarrhoea and weeping sores that sometimes required surgery. The pre-modern treatment of epilepsy could be grotesque. Over the centuries epileptics have been fed hippo testicles, crocodile faeces, clumps of camel hair, diseased mule skin, and a drink made of scorched human bones (in the last case, Galen reports, the patients were usually kept in the dark, ‘lest they should be nauseated’). In ancient Rome, epileptics waited in the wings of the Colosseum to suck the blood of slaughtered gladiators straight from their wounds.
Schmidt and Shorvon want epilepsy eradicated. ‘If you think about it,’ they write, ‘it is quite surprising that we have not been able to remove epilepsy from the long list of serious and intractable brain disorders, given the billions of dollars and the best medical efforts to develop more effective epilepsy drugs and to improve the outcome of epilepsy surgery.’ They survey their field and what they see – or what, out of a constructive impulse, they choose to emphasise – is a ‘mire of confusion’ in which chemical compounds are thrown like darts at the problem and experts can’t even agree on how to define epilepsy, let alone how to distinguish and classify its myriad effects. We’ve come a long way, but we haven’t come nearly far enough.
On the other hand, Schmidt and Shorvon find much to applaud in society and culture. Here, too, they recognise that ‘not all is rosy.’ But the progress that has been made, they judge, has been nothing less than revolutionary – an unprecedented transformation in the relationship between epileptics and the world they live in. They believe that ‘the modification of social attitudes around epilepsy in the last forty years’ has been the greatest improvement for patients with epilepsy in the modern era.
It isn’t just ‘social attitudes’ that concern Schmidt and Shorvon, but social attitudes as reflected and influenced by institutions. Clinicians and researchers have been looking at epilepsy through an enlightened materialist lens – as ‘epilepsy’ rather than ‘the falling sickness’ – for a century and a half. But as Greene knew well, medical sophistication doesn’t necessarily obviate prejudice. At one point, Schmidt and Shorvon quote from the Harvard neurologist William Lennox’s Epilepsy and Related Disorders (1960). Lennox, who served as both editor of the prestigious journal Epilepsia and president of the International League against Epilepsy, described the average epileptic as an ‘unreasonable, stubborn, fault-finding, shallow-minded, tiresomely loquacious and reiterative individual … [who] will not be welcomed in any social group’. Until 1970, the UK Matrimonial Causes Act held that a marriage could be declared void if it was discovered that one of the spouses had been epileptic at the time of the union. In the mid-1970s, Schmidt and Shorvon write, ‘it was still legal in the United States to deny people with seizures entry to restaurants, theatres, recreational centres and other public places.’
In a short lifetime, then, the formal slander and legal proscription of people with epilepsy has become as taboo as the condition itself once was. Schmidt and Shorvon don’t go into much detail about how or why this transformation occurred. Revelations about the Nazi eugenics programme may gradually have made anti-epilepsy sentiment less acceptable. More generally, they point to a postwar ‘consensus on human rights’ that in time yielded powerful anti-discrimination laws. They point to a growing trend in the West towards diversity and inclusion – a trend that encompasses not just epilepsy but also autism, homosexuality, physical disabilities, mental illness and a score of other conditions, identities and ways of being. Epilepsy is now not about moral weakness, or even necessarily just about sickness. It is also about difference, and the right to maintain it. If the beginning of the 20th century was the worst time in history to have epilepsy, the beginning of the 21st is surely the best.
Stigma unarticulated and prohibited, however, is not stigma erased, and certainly not unfelt. According to the charity Young Epilepsy, almost nine out of ten people with the condition feel they have been victims of discrimination. More than half do not reveal their epilepsy to new acquaintances for fear of a negative reaction. Meanwhile, as the experience of Grant’s family suggests, the ‘West’ is not so stable or concrete a designation. Shame can cross borders – and in many places it remains potent. In Nigeria, almost half of schoolteachers believe children with epilepsy are insane, and almost a third believe the condition is contagious. In Taiwan and China, 72 per cent of parents would prefer their children not to have playmates with epilepsy.
Schmidt and Shorvon make careful note of these figures. But the very fact that researchers set out in good faith to record evidence of prejudice gives the authors confidence. As they were putting together The End of Epilepsy?, newspaper articles were appearing for International Epilepsy Day that bemoaned the continued stigmatising of the disorder. ‘Nevertheless,’ they write, ‘the fact that the articles exist and the fact that there are “epilepsy days” show how public perception has changed over the period.’ One might add that the fact that these two books exist shows it too.