Acouple of years ago a patient I’ll call Joan came to my GP clinic to tell me that her right leg had stopped working. ‘Sometimes it’ll hold me upright,’ she said, ‘and sometimes it won’t.’ I took a look at her legs. Her thigh muscles on the right seemed to me a bit weaker than on the left, and her reflexes were unusually brisk; when I tapped her knee with a little rubber hammer I had to dodge a vigorous kick. The rest of her examination was normal. ‘Hmm,’ I said, did some blood tests (also normal) and referred her to a neurologist. In the interval before the date of her appointment Joan called back to say that the vision in her left eye had become patchy. ‘Hmm,’ I said. ‘Would you mind getting an appointment with an optician – soon?’
Neurons in the brain and spinal cord have inputs (‘dendrites’) and an output (the ‘axon’). Broadly speaking the axon acts to influence the dendrites of other neurons in a network, or stimulates a muscle to make it move. Axons conduct electrical signals by means of sodium ions, not electrons, but like electrical cables they have insulation – a fatty sheath called myelin which ensures the reliable transmission of the signals. Sometimes the body’s immune system begins to attack myelin; as a result, neuronal signals can be slowed, jumbled, reversed or even blocked. I wondered if the neurons running through Joan’s spinal cord towards her right leg had become affected by this ‘demyelination’, as well as those connecting her left eye and her brain.
In Italo Svevo’s novel Zeno’s Conscience, the protagonist is told that taking a single step requires the co-ordination of no fewer than 54 muscles. ‘Walking became a difficult labour, and also painful,’ Svevo wrote. ‘Even today, while I write, if someone watches me in motion, the 54 movements become too much, and I’m at risk of falling over.’ A decade earlier, the naturalist B.F. Cummings, who published his journals under the pseudonym W.N.P. Barbellion, recorded his experience of demyelination: ‘In a horrible panic – the last few days – I believe I am developing locomotor ataxy. One leg, one arm and my speech are affected, i.e. the right side and my speech centre … I stutter a little in my speech when excited, I cannot write properly (look at this handwriting) and my right leg is rocky at the knee. My head swims.’
Joan’s MRI scan confirmed the diagnosis: several patches or ‘plaques’ of demyelination were visible in her brain and spinal cord. These plaques tend to become scarred over time, a process called ‘sclerosis’, visible to pathologists at postmortem as grey-pink blobs in the brain. The first neurologist to describe it was Jean-Martin Charcot of the Salpêtrière in Paris, who in 1868 called it ‘sclérose en plaques disseminées’. In German the disease became known as ‘Multiple Sklerose’ and in English ‘disseminated sclerosis’. By the 1950s the anglophone medical community had settled on ‘multiple sclerosis’.
Towards the end of a long walk on a summer’s day in 2017, Robert Douglas-Fairhurst noticed that his legs felt heavy and poorly co-ordinated. ‘By the time I reached my front door I was shuffling along like an old man in carpet slippers.’ He was in his late forties and wondered if these were the first flickering signs of old age, or if perhaps he had an iron deficiency. He went to see his GP, who tested his muscle strength and tapped his knees. ‘Hmm,’ the GP said, and referred him to a neurologist, who made him walk around the clinic until the carpet-slipper effect took hold. ‘Hmm,’ the neurologist said, and arranged an MRI scan.
MRI scans render the brain as a series of transverse slices, like a stack of plates, from the top of the head to the base of the skull. At his follow-up appointment, Douglas-Fairhurst was shown the scan of his own brain and it was pointed out to him that the white matter was speckled with little plaques. Neurologists are accustomed to breaking bad news. Douglas-Fairhurst’s doctor was brisk and businesslike: ‘I’m going to come right out with it,’ she said. ‘I think you have multiple sclerosis.’ He thanked her for her honesty, but left feeling he’d just been handed something ‘halfway between a life sentence and a death sentence’.
Douglas-Fairhurst teaches English at Oxford. In his closing lecture to first years he likes to quote Proust: ‘The last page was read, the book was finished … Then, what?’ He wants his students to think about the ways in which studying literature will help them to navigate their lives. His own response to the news he’d been given was to head to the library. The result is Metamorphosis, a richly textured and syncopated book, alternately erudite and irreverent; Douglas-Fairhurst tries not to take himself too seriously, despite his subject. In its mingling of absurdity and earnest inquiry the book echoes Barbellion’s account of his (untreated) MS. Fairy tales figure prominently – they are good training for unhappy endings – though Douglas-Fairhurst notes that fairy-tale characters with misshapen bodies usually turn out to have misshapen souls too. The day of his diagnosis a trapdoor opened beneath his feet and, like Alice, he has been tumbling ever since. Alice ‘reminds us that one person has the potential to be many different things … but already the unpredictability of living with MS meant that I had begun asking myself the same question. “Who are you?”’ His next stop is Kafka’s Metamorphosis. Like Gregor Samsa, Douglas-Fairhurst has had to learn a new way of being and moving; he also needs the help of others. He worries that his story and Samsa’s will turn out to have other similarities. ‘It appeared that all I had to look forward to was a time in which I would be first cared for, then neglected, and finally abandoned.’ In a letter to his friends and colleagues, he asks them to do precisely nothing: ‘Don’t stop inviting me to things. Don’t put on a concerned look if I seem a bit wobbly. In fact, don’t even ask me how I am if you can help it … If anything, I’d prefer people to make jokes about it. (By and large we only make jokes about the things that really matter.)’
We still don’t really understand the mechanism by which multiple sclerosis attacks the myelin around the axons in the brain and spinal cord, or how to stop it. Vitamin D is involved somehow, and there’s some evidence that supplements can help. The disease tends to manifest itself in one of two modes: ‘relapsing-remitting’ MS, in which the nervous system partially recovers between episodes, and ‘primary progressive’ MS, which tends to increase in severity from the outset (Douglas-Fairhurst has the latter). The categories are not distinct, and relapsing-remitting MS can shift in pattern towards secondary progressive over time. MRI scans are useful in diagnosis, but the severity of the picture on the scan doesn’t match up perfectly with the patient’s degree of impairment – you can be mildly affected but have plaques all over the place, or have very few plaques yet still be severely affected.
MS isn’t uncommon in the UK. It affects around two people in every thousand (the average GP will have three or four patients with MS) and its incidence increases the further north you go – perhaps through a combination of genetic susceptibility and cloudier skies. Twenty years ago, when I worked in Edinburgh’s department of clinical neurosciences, there were few treatments: we used to give infusions of immunoglobulin, and sometimes interferon (a protein that affects signalling between white blood cells). The advice at that time was to withhold treatment until late in the progress of an episode. Though the disease remains incurable there are now various treatments that can slow its progress and diminish its severity. These days my neurology colleagues treat early and aggressively, and there are several immunomodulator drugs available that tweak the actions of the white blood cells involved in generating the plaques.
Joan, my patient, was diagnosed with relapsing-remitting MS and was started on infusions of a drug called Ocrelizumab, which works by hampering the immune system. (It thereby exposes you to greater risks from infection.) Her symptoms settled down, and now she is waiting to see when – or if – her condition will flare up again. Once a year she goes to see a specialist at a clinic in Edinburgh. I visited the clinic recently and saw a succession of patients who were in remission thanks to the new drugs.
In the last few years another treatment for MS has evolved, as neurologists have taken inspiration from a procedure developed by haematologists to treat leukaemia. It involves ‘harvesting’ stem cells from the patient’s blood, using strong chemotherapy to quell the part of the bone marrow that generates white blood cells, then putting the stem cells back in again. It is a transplant of sorts, but an ‘autologous’ one, in which donor and recipient are the same person. The procedure has the unlovely name ‘autologous haematopoietic stem cell transplantation’, or aHSCT for short. Douglas- Fairhurst describes it as ‘the medical equivalent of rewriting a song by setting new words to the same tune’; elsewhere he hears it described as a ‘reboot’. But it’s a risky one: some trials have reported a 6 per cent mortality rate from the procedure, which, as Douglas-Fairhurst points out, suggests not so much a reboot as something like smashing up a computer and attempting to put it back together again. Just fifteen months after his diagnosis, having considered then rejected self-referral to Dignitas (‘a package of medical costs, funeral expenses and official fees would cost 10,500 Swiss francs – approximately £7700 – plus airfare’), he signed up for aHSCT.
Metamorphosis has an unusual structure.It begins with Chapter 42 (‘Falling’), descends towards ‘Chapter Zero’ about nine- tenths of the way through, then climbs back up to end on a second Chapter 7. At first it seems as if, like Barbellion’s journal, the narrative is moving towards a truly definitive conclusion (the first published volume of Barbellion’s journal ends ‘Barbellion died on 31 December’ though in fact he lived another six months), but instead chapter zero describes what Douglas-Fairhurst calls his ‘rebirthday’ – the day his bone marrow was repopulated with new stem cells. Barbellion, when he was dying, wrote of the beauty of watching Ctenophores in a glass of seawater, caught in refracted light. As Douglas-Fairhurst lies in his sterile room watching specks of dust dancing in sunlight he reflects that his room is so clean these must be his own skin cells. The chapter is called ‘Handful of Dust’.
Metamorphosis is as much an account of the rigours of aHSCT as it is an account of living with MS, though it ends before we find out how effective the treatment has been. Primary progressive MS doesn’t usually respond well to aHSCT and isn’t available on the NHS; Douglas-Fairhurst paid for it privately, parting with a sum you’d need to buy ‘a terraced house in some parts of the country’. I have heard of clinics in Mexico that profit from the desperation of MS patients by offering aHSCT of dubious quality, but Douglas-Fairhurst’s hospital is in London, the ‘brightest and shiniest’ he has yet visited.
He doesn’t seem to have been fortunate in his physicians: ‘When the doctors and nurses look at you it quickly becomes apparent that it is not really you that they see. Your identity has been whittled down to a set of symptoms and the medical data contained in your scans and charts. In effect, you have become just another puzzle for them to solve.’ This is the kind of attitude that drove me away from hospital medicine into general practice, and the kind that A.J. Lees, a professor of neurology in London, sets out to dismantle in his short memoir Brainspotting. Like Barbellion, Lees was an enthusiastic naturalist as a child, and believes that his hobby trained him in the sort of discriminating pattern recognition you need to practise as a professional diagnostician. He doesn’t trust private hospitals, having looked beyond the new carpets, shiny elevators and the smell of coffee and seen the disasters their brochures don’t mention: ‘Private hospitals are there to generate income and all the rhetoric of quality, safety and patient satisfaction is in truth no more than a public relations exercise,’ he writes; private neurologists are ‘factory workers in what has become a service industry’.
In Charcot’s day, neurology and psychiatry were united in one discipline, but by 1914 the Harvard pathologist Elmer Southard had divided them into two camps: ‘brain spot men’ gazing down the viewfinders of their microscopes and ‘mind twist men’, whose focus was on their patients’ memories, dreams and experiences. Even today the line between neurology and psychiatry is blurred. For example, psychiatrists tend to look after people with dementia, an organic brain disease, while neurologists tend to look after patients with Tourette’s, which involves flights of mood and activity and can be tempered with small doses of the kinds of drug used to treat psychosis. And about a third of patients referred to neurology clinics have a problem that has come about through their beliefs and expectations rather than through a biological process affecting their brains or nerves. Lees hopes, as he approaches retirement, to resurrect a clinical attitude that ‘embraces anecdote, cordial laughter and tacit knowledge but never lapses into sentimentality’. A lifetime of practising neurology has helped him accept the ‘arbitrary and often brutal nature’ of diseases of the brain, and MS in particular, which he singles out for the deceptive way in which it can attack, recede, then return with renewed vigour, its symptoms changing with each new episode. At the end of Metamorphosis, Douglas-Fairhurst records the checklist with which he now begins each day. It helps him gauge how his MS is going to affect him, and how much, therefore, he can expect of himself: ‘eyes, brain, bladder, legs, mood’. I used the same checklist myself the last time I spoke to Joan.
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