It all started with a phone call, at the beginning of 1993. ‘It’s a Dr. Steele,’ Kate said. ‘John Steele, from Guam.’ I had had some contact with a John Steele, a neurologist in Toronto, many years before – could this possibly be the same one? And if so, I wondered, why should he be calling me now, calling from Guam? I picked up the phone, hesitantly. My caller introduced himself; he was indeed the John Steele I had known, and he told me that he now lived in Guam, had lived and worked there for a dozen years.
Guam had a special resonance for neurologists in the 1950s and ‘60s, for it was then that many descriptions were published of an extraordinary disease endemic on the island, a disease the people of Guam, the Chamorros, called lytico-bodig. The disease, seemingly, could present itself in different ways – sometimes as ‘lytico,’ a progressive paralysis which resembled amyotrophic lateral sclerosis (ALS, or motor neuron disease), sometimes as ‘bodig,’ a condition resembling parkinsonism, occasionally with dementia. Ambitious researchers converged on Guam from all over the world, eager to crack this mysterious disease. But, strangely, the disease defeated all comers, and, with repeated failures, the excitement died down. I had not heard anyone mention the lytico-bodig for twenty years, and presumed it had died out quietly, unexplained.
This was far from the case, John now told me. He still had hundreds of patients with lytico-bodig; the disease was still very active – and still unexplained. Researchers had come and gone, he said, few stayed too long. But what had especially struck him, after twelve years on the island, and seeing hundreds of these patients, was the lack of uniformity, the variability and richness, the strangeness of its presentations, which seemed to him more akin to the range of post-encephalitic syndromes seen in vast numbers after the encephalitis lethargica epidemic in the First World War.
The clinical picture of bodig, for example, was often one of a profound motionlessness, almost catatonia, with relatively little tremor or rigidity – a motionlessness which might suddenly dissolve or switch explosively into its opposite when these patients were given the smallest dose of L-DOPA – this, he thought, seemed extremely similar to what I had described with my post-encephalitic patients, in Awakenings.
These post-encephalitic disorders have all but disappeared now, and since I had worked with a large and unique population of (mostly elderly) post-encephalitic patients in New York during the 1960s and ‘70s, I was among the very few contemporary neurologists who had actually seen them.[45] So John was most eager that I come to see his patients in Guam, so that I could make direct comparisons and contrasts between them and my own.
The parkinsonism which affected my post-encephalitic patients had been caused by a virus; other forms of parkinsonism are hereditary, as in the Philippines; and yet others have been linked to poisons, as with the parkinsonian manganese miners in Chile or the ‘frozen addicts’ who destroyed their midbrains with the designer drug MPTP. In the 1960s, it had been suggested that the lytico-bodig was also caused by a poison, acquired through eating the seeds of the cycad trees which grew on the island. This exotic hypothesis was all the rage in the mid-sixties when I was a neurology resident – and I was especially taken by it because I had a passion for these primitive plants, a passion which went back to childhood. Indeed, I have three small cycads in my office – a Cycas, a Dioon, and a Zamia, all clustered around my desk (Kate has a Stangeria beside hers) – and I mentioned this to John.
‘Cycads – this is the place for them, Oliver!’ he boomed. ‘We have them all over the island; the Chamorros love to eat the flour made from their seeds – they call it fadang or federico…Whether this has anything to do with lytico-bodig is another matter. And on Rota, north of here, a short hop in a plane, you can see absolutely untouched cycad jungles, so thick, so wild, you’d think you were in the Jurassic.
‘You’ll love it, Oliver, whichever hat you wear. We’ll go around the island seeing cycads and patients. You can call yourself a neurological cycadologist, or a cycadological neurologist – either way, it will be a first for us on Guam!’
As the plane began its descent, circling the airport, I got my first glimpse of the island – it was far bigger than Pohnpei, and elongated, like a giant foot. As we skimmed over the southern end of the island, I could see the small villages of Umatac and Merizo nestled in their hilly terrain. One could see, from a height, how the entire northeastern part of the island had been turned into a military base; and the skyscrapers and superhighways of central Agana rapidly loomed as we descended.
The terminal was teeming with people of a dozen nations, scurrying in all directions – not only Chamorros, Hawaiians, Palauans, Pohnpeians, Marshallese, Chuukese, and Yapese, but Filipinos, Koreans, and, in vast numbers, Japanese. John was waiting at the barrier, an easy figure to pick out among the bustling crowds, for he was tall and fair, with very pale hair and a ruddy complexion. He was the only person in the entire airport, as far as I could see, wearing a suit and tie (most were dressed in brightly colored T-shirts and shorts). ‘Oliver!’ he boomed, ‘Welcome to Guam! So good to see you! You survived the Island Hopper, eh?’
We walked through the steaming airport and out through the parking lot to John’s car, a battered white convertible. We skirted Agana, and started toward the southern part of the island, to the village of Umatac, where John lives. I had been somewhat taken aback by the airport, but now as we drove south, the hotels, the supermarkets, the Western bustle, died away, and we were soon in gentle, undulating country. The air grew cooler as the road climbed higher and wound along the slopes of Mount Lamlam, the highest point on the island. We stopped at a lookout point, got out, and stretched. There were grassy slopes all around us, but higher, on the mountain, a thick cloak of trees. ‘You see those bright green dots, standing out against the darker foliage?’ John asked me. ‘Those are the cycads, with their new foliage. You’re probably used to Cycas revoluta, the bristly, low Japanese cycad, which one sees everywhere,’ he added. ‘But what we have here is a much larger, indigenous species, circinalis – they look almost like palms from a distance.’ Pulling out my binoculars, I scanned them with delight, glad I had made the long journey to this island of cycads.
We got back into the car, drove a few minutes more, and then John stopped again at a final ridge. There, spread out below us, glinting in the sun, was the Bay of Umatac, the bay where Magellan had anchored his ships in the spring of 1521. The village clustered around a white church by the water, with its spire rising above the surrounding buildings; the hillside sloping down to the bay was dotted with houses. ‘I’ve seen this a thousand times,’ said John, ‘but I never get tired of it. It is always as beautiful as the first time I saw it.’ John had been rather formal, in manner as well as dress, when we met at the airport, but now, as he looked down at Umatac, a different aspect of him appeared. ‘I have always loved islands,’ he said, ‘and when I read Arthur Grimble’s book, A Pattern of Islands – do you know it? – anyhow, when I read it, I knew I would never be happy unless I lived on a Pacific island.’
We got back into the car and started the winding descent to the bay. At one point, John stopped the car again, and pointed to a graveyard on a hilly slope. ‘Umatac has the highest incidence of lytico on the entire island,’ he said. ‘That’s how it ends.’
There was a large cantilevered bridge – ornate, gaudy, startling – spanning a gulch as the main road entered town. I had no idea of its history or function; it was as absurd, in its way, as the transplanted London Bridge in Arizona – but it looked festive, fun, as it leapt into the air, a pure effervescence of high spirits. As we entered the village, and drove slowly through, people waved or called out greetings to John as we passed, and with this, it seemed to me, the remaining reserve fell away – suddenly he looked completely at ease, at home.
John has a low, comfortable house, a little to one side of the main village, sheltered by palms, banana trees, and cycads. He can retreat to his study and immure himself among his books – or, in a minute, be with his friends and patients. He has a new passion, beekeeping; hives, in wooden hutches, stood by the side of the house, and I could hear the murmur of bees as we pulled up.
While John went to make tea, I waited in his study and glanced at his books. I had seen a Gauguin reproduction in the living room above the sofa, and now my eye was instantly caught by seeing Gauguin’s Intimate Journal, wedged between copies of the Annals of Neurology. The juxtaposition was striking: Did John see himself as a neurological Gauguin? There were hundreds of books and leaflets and old prints of Guam, especially relating to the original Spanish occupation – all mixed, higgledy-piggledy, with his neurology books and papers. John returned as I was looking at these, with a large pot of tea and a strange, phosphorescent purple confection.
‘It’s called Ube,’ he said. ‘Very popular here. Made from the local purple yams.’ I had never had an ice cream so mealy, so mashed potato-like, nor one of so extraordinary a color; but it was cool and sweet, and grew on me as I ate it. Now that we were in his library, relaxing over tea and Ube, John started to tell me more of himself. He had spent his formative years in Toronto (indeed we had exchanged letters when he was there, more than twenty years earlier, on the subject of children’s migraines and the visual hallucinations which sometimes accompany them). When John was a resident, in his twenties, he and his colleagues had discovered an important neurological condition (progressive supranuclear palsy, now called Steele-Richardson-Olszewski syndrome). He did further postgraduate work in England and France and a brilliant academic career seemed to be opening for him. But he also felt obscurely conscious of wanting something quite different and had a strong desire to care for patients as a general physician, as his father and grandfather had before him. He taught and practiced in Toronto for another few years, and then in 1972 he moved to the Pacific.
Arthur Grimble, whose book had so excited John, had been a district officer in the Gilbert and Ellice Islands before the First World War, and the picture he gave of life in these islands determined John to go to Micronesia. Had he been able to, he would have gone to the Gilberts, like Grimble – for though these islands had changed their name (to Kiribati), they remained otherwise unchanged, hardly contaminated by commerce or modernization. But there were no medical postings available there, so John went instead to the Marshall Islands, to Majuro. In 1978, he moved to Pohnpei, his first experience of a high volcanic island (and it was here that he learned of the maskun, the hereditary colorblindness among the Pingelapese, several of whom he saw in his practice at this time). Finally, in 1983, having sampled the Marshalls and the Carolines, he went to the Marianas and to Guam. He hoped he might settle here and live the quiet life of a country doctor, an island practitioner, surrounded by community and relationship – though also, at the back of his mind, there was always the riddle of the Guam disease, and the thought that he, perhaps, might be the one to solve it.
He had lived first in noisy, Westernized Agana, but soon felt an overwhelming need to move to Umatac. If he was to work with the Chamorros and their disease, he wished to be among them, surrounded by Chamorro food, Chamorro customs, Chamorro lives. And Umatac was the epicenter of the disease, the place where it had always been most prevalent: the Chamorros sometimes referred to the lytico-bodig as ‘chetnut Humatag,’ the disease of Umatac. Here in this village, within the span of a few hundred acres, the secret of lytico-bodig must lie. And with it, perhaps, the secret of Alzheimer’s disease, Parkinson’s disease, ALS, whose varied characteristics it seemed to bring together. Here in Umatac is the answer, John said, if we can find it: Umatac is the Rosetta Stone of neurodegenerative disease, Umatac is the key to them all.
John had sunk into a sort of reverie as he recounted the story of his wandering, lifelong passion for islands, and his finally coming to Guam, but now he suddenly jumped to his feet, exclaiming, ‘Time to go! Estella and her family are expecting us!’ He seized his black bag, donned a floppy hat, and made for the car. I too had sunk into a sort of trance, but was precipitated out of this by the urgent tone of his voice.
Soon we were whizzing down the road to Agat – a drive which made me slightly nervous, for John was now launching into another reminiscence, a very personal history of his own encounter with the Guam disease, the vicissitudes of his thought, his work, and his life on Guam. He spoke with passion, and with vehement, darting gestures, and I feared his attention was not fully on the road.
‘It’s an extraordinary story, Oliver,’ he started, ‘whatever way you look at it – in terms of the disease itself, and its impact on the people here on the island, the tantalizing, round-and-round search for its cause.’ Harry Zimmerman, he said, had first seen it in 1945, as a young navy doctor arriving after the war; he had been the first to observe the extraordinary incidence of ALS here, and when two patients died he was able to confirm the diagnosis at autopsy.[46] Other physicians stationed on Guam provided further, richer documentation of this puzzling disease. But it required perhaps a different sort of mind, the mind of an epidemiologist, to see the greater significance of all this. For epidemiologists are fascinated by geographic pathology, so to speak – the special vicissitudes of constitution or culture or environment which predispose a population to a specific disease. Leonard Kurland, a young epidemiologist at the National Institutes of Health in Washington, realized at once when he read these initial reports that Guam was that rare phenomenon, an epidemiologist’s dream: a geographic isolate.
‘These isolates,’ Kurland was later to write, ‘are sought constantly, because they stimulate our curiosity and because the study of disease in such an isolate may demonstrate genetic or ecological associations that otherwise might not be appreciated.’ The study of geographic isolates – islands of disease – plays a crucial role in medicine, often leading to the identification of a specific agent of disease, or genetic mutation, or environmental factor that is linked to the disease. Just as Darwin and Wallace found islands to be unique laboratories, hothouses of nature which might show evolutionary processes in an intensified and dramatic form, so isolates of disease excite the epidemiological mind with the promise of understandings to be obtained in no other way. Kurland felt that Guam was such a place. He shared his excitement with his colleague Donald Mulder, at the Mayo Clinic, and they decided to go to Guam right away, to launch a major investigation there, with all the resources of the NIH and the Mayo.
This was not, John suspected, just an intellectual moment for Kurland, but an event which changed his life. His initial visit, in 1953, opened intoxicating horizons for him – a love affair, a mission, which was never to stop. ‘He is still writing and thinking about it, and coming here,’ John added, ‘forty years later – once it gets to you, it never lets you go.’
When Kurland and Mulder arrived they found more than forty cases of lytico on the island, and these, they felt, were only the most severely affected, milder cases probably having escaped medical attention. A tenth of all the adult Chamorro deaths on Guam were due to the disease, and its prevalence was at least a hundred times greater than on the mainland (in some villages, like Umatac, it was over four hundred times greater). Kurland and Mulder were so struck by this concentration of the disease in Umatac that they wondered whether it might have originated here and then spread to the rest of the island. Umatac, John pointed out, had always been the most isolated, least modernized village on Guam. There was no access by road in the nineteenth century, and even in 1953, the road was often impassable. Sanitary and health conditions were poorer than anywhere else on the island at that time, and traditional customs remained very strong.
Kurland was also struck by the way in which certain families seemed predisposed to get lytico: he mentioned one patient who had two brothers, a paternal uncle and aunt, four paternal cousins, and a nephew with the disease (and he observed that health records back to 1904 showed this family to have been singled out even then). Many of the family, John said, were now his patients. And there were other families, like the one we were on our way to see, who seemed particularly vulnerable to the disease.
‘But you know,’ said John, gesturing violently, and causing the car to lurch to one side, ‘there was something else very interesting which Len described then, but which he first regarded as unconnected. He found not only forty-odd people with lytico, but no less than twenty-two with parkinsonism – far more than one would expect to see in a community of this size. And it was parkinsonism of an unusual sort: it would often begin with a change in sleeping habits, with somnolence, and go on to profound mental and physical slowing, profound immobility. Some had tremor and rigidity, many had excessive sweating and salivation. He thought at first that it might be a form of postencephalitic parkinsonism – there had been an outbreak of Japanese B encephalitis a few years earlier – but he could find no direct evidence for this.’
Kurland started to wonder about these patients, the more so as he found another twenty-one cases of parkinsonism (some with dementia as well) in the following three years. By 1960 it seemed clear that these could not be post-encephalitic in origin, but were cases of what the Chamorros called bodig, a disease, like lytico, endemic for at least a century in Guam. Now, when the patients were examined more closely, many of them seemed to have signs of both bodig and lytico; and Kurland wondered if the two might in some way be allied.
Finally, when Asao Hirano, a young neuropathologist (and student of Zimmerman’s), came to Guam in 1960 to do a postmortem study of the brains of those who had died from lytico and from bodig, he was able to show that both diseases involved essentially the same changes in the nervous system, though with varying distributions and severity. So pathologically it seemed that lytico and bodig might not be separate diseases, but a single disease which could present in very different ways.[47]
This again was reminiscent of the encephalitis lethargica: when this first broke out in Europe, there seemed to be half a dozen separate diseases rampaging – so-called epidemic polio, epidemic parkinsonism, epidemic schizophrenia, etc. – and it was not realized until pathological studies were done that all of these were in fact manifestations of the same disease.
‘There is no standard form of lytico-bodig,’ John said, as we pulled up in front of a house in the little village of Agat. ‘I could show you a dozen, two dozen patients, and no two would be the same. It is a disease which is polymorphous in the extreme, which can take three, or six, or twenty different forms – you’ll see with Estella and her family.’
We were welcomed by a young woman, who shyly motioned us to come in. ‘Hello, Claudia,’ said John. ‘It’s nice to see you. How is your mother today?’ He introduced me to the family: Jose and Estella, Claudia and her two brothers, in their twenties, and Jose’s sister, Antonia. I was struck by Estella as soon as we entered the house, because she looked so much like one of my post-encephalitic patients as she stood, statuelike, with one arm outstretched, her head tilted back, and an entranced look on her face. One could put her arms in any position, and they would be maintained like this, apparently effortlessly, for hours. Left alone, she would stand motionless, as if spellbound, staring blankly into space, drooling. But the moment I spoke to her, she answered – appropriately, with wit; she was perfectly capable of lucid thought and speech, provided somebody started her going. Similarly, she could, if she was with someone, go shopping, or to church, always pleasant and alert, but with a sort of detached, preoccupied, sleepwalking air, a strange immurement in herself. I wondered how she might react to L-DOPA – it had not yet been tried with her – for such catatonic patients, in my experience, could show the most dramatic reactions to the drug, bursting out of their catatonia with projectile-like force, and sometimes, with the continuation of the drug, developing multiple tics. Perhaps the family had some inkling of this, I am not sure; when I asked them, they said only that she did not seem to be suffering, that she never complained of her catatonia, that she seemed to be perfectly serene inside.
I found myself in two minds at this. Part of me wanted to say: But she is ill, catatonic, she can’t fully respond – don’t you want to bring her back? She has a right to be medicated, we have a duty to medicate her. But I hesitated to say anything, feeling an outsider. Later, when I asked John about this, he said, ‘Yes – that would have been my reaction, when I came here in ‘83. But the attitude to illness is different here.’ In particular, he said, the Chamorros seem to have a certain stoicism or fatalism – he hardly knew which word to use – about illness, and the lytico-bodig in particular.
With Estella, specifically, there was the sense of calm, of her being in her own world, the sense of an achieved equilibrium both within her, and in relation to her family and community – and the fear that medication might ‘stir her up’ and imperil this.
But it was very different for Jose, her husband: physiologically different, as a start – for he had the most intense jamming, clenching, locking parkinsonism, where muscle groups, rigid, fought against each other and jammed each movement at its inception. If he wished to straighten his arm, activation of the triceps was at once opposed by an activation of its antagonist, the biceps (which normally relaxes to allow the arm’s extension), and vice versa – so that the arm got locked, perpetually, in strange positions, and he could neither bend nor straighten it. Similar jams, similar binds, affected all his muscle groups – the whole innervation of the body was perverse. He would go red in the face with the intensity of his effort to get through the block, and sometimes it would give way suddenly, and then the force of his effort would make him jerk violently or fall.
In this sort of parkinsonism, the ‘explosive-obstructive’ sort, the whole body, so to speak, is set against itself, locked in irre-soluble inner conflict. It is a state full of tension, effort, and frustration, a tormenting condition which one of my patients once called ‘the goad and halter.’ Jose’s state was wholly different from the strange muscular compliance, the waxy flexibility, which went with Estella’s catatonia. One could see, in this one couple, the extremes of furious resistance and total surrender – the antipodes of the subcortical will. After Jose and Estella, I examined Claudia and her two brothers briefly, but none of them, it was clear, had any sign of the disease. Nor did they seem to have any fear of getting it, despite the fact that both their parents, and many older relatives, were affected. John contrasted their confidence with the great anxiety felt by members of the older generation, who often feared – especially if they had relatives with the disease – that they might have it already latent in their bodies. These folk attitudes, John pointed out, were entirely appropriate, given the fact that no one born after 1952 had been known to contract the disease.
Jose’s sister, who lives with them, showed yet another form of the disease, one marked by a severe and progressive dementia. She was at first frightened by our presence – she had lunged at me, and tried to scratch me, when we first entered the house. She became angry, and perhaps jealous, as we talked to the others, and now she came across the room, pointing to herself, saying ‘Me, me, me – ME.’ She was also quite aphasic, and very restless, given to bursts of screaming and giggling – but music calmed and cohered her to an amazing extent. This too had been discovered by the family; the traditional knowledge of these disorders, and ways of dealing with them, is very considerable. To calm her, the family started to sing an old folk song, and the old lady, so demented, so fragmented, most of the time, joined in, singing fluently along with the others. She seemed to get all the words, all the feeling, of the song, and to be composed, restored to herself, as long as she sang. John and I slipped out quietly while they were singing, suddenly feeling, at this point, that neurology was irrelevant.
‘You can’t see a family like this,’ said John as we set out the next morning, ‘without wondering what causes so many of them to be affected. You see Jose and his sister, and you think, this must be hereditary. You see Estella and her husband, who are not blood relatives, though their lives are intertwined: Is the lytico-bodig due to something in the environment they share, or has one passed the disease to the other? You look at their children, born in the 1960s, free of the disease, like all their contemporaries, and infer that the cause of the disease, whatever it was, vanished or became inoperative in the late forties or fifties.’
These were some of the clues and contradictions, John continued, which faced Kurland and Mulder when they came here in the 1950s – and which are so difficult to reconcile by any single theory. Kurland was at first inclined to think in terms of a genetic origin. He looked at the early history of the island, and how a near genocide reduced the population from 100,000 to a few hundred – the sort of situation which disposes to the spread of an abnormal trait or gene (as with the achromatopsia on Pin-gelap) – yet there was no simple Mendelian pattern linking those with the disease. He wondered, in the absence of such a pattern, whether this was a gene with ‘incomplete penetrance.’ (He wondered too if a genetic predisposition to lytico-bodig might also have a paradoxical, selective advantage – perhaps increasing fertility or conferring immunity to other diseases.) But he had to wonder whether there might not be some environmental factor, in addition to a genetic susceptibility, a ‘necessary adjunct,’ as he put it, to developing the disease.
In the late 1950s he extended his studies to the very large population of Chamorros who had migrated to California. They had, he observed, the same incidence of lytico-bodig as the Chamorros in Guam, but the disease might only develop ten or twenty years after they had left Guam. There were, on the other hand, a few non-Chamorro immigrants who seemed to have developed the disease a decade or two after moving to Guam and adopting a Chamorro lifestyle.
Could the environmental factor, if there was one, be an infectious agent, a virus, perhaps? The disease did not appear to be contagious or transmissible in any of the usual ways, and no infectious agent could be found in the tissues of those affected. And if there was such an agent, it would have to be one of a very unusual sort, one which might act as a ‘slow fuse’ – John repeated the phrase for emphasis – a slow fuse in the body, setting off a cascade of events which only later might manifest as clinical disease. As John said this, I thought of various post-viral neurodegenerative syndromes, and especially again of my postencephalitic patients, who in some cases only started to show symptoms decades after the initial encephalitis lethargica – sometimes as much as forty-five years later.
At this point in the story, John started pointing emphatically through the window. ‘Look!’ he said. ‘Look! Look! Cycads!’ Indeed I saw cycads all round, some growing wild but many, I now saw, cultivated in gardens, as we drove to Talofofo to visit another patient of John’s, a former mayor of the village, whom everyone called the Commissioner.
Cycads only grow in tropical or semitropical regions and were new, alien, to the early European explorers when they first saw them. At first glance, cycads bring to mind palms – indeed the cycad is sometimes called a sago palm – but the resemblance is superficial. Cycads are a much more ancient form of life, which arose a hundred million years or more before there were palms or any other flowering plants.
There was a huge native cycad, at least a century old, growing in the Commissioner’s yard, and I stopped to gaze at this splendid tree, fondled a stiff, glossy frond, then caught up with John at the front door. He knocked on the door, and it was opened by the Commissioner’s wife, who ushered us into the main room where her husband sat. Sitting in a massive chair – rigid, immobile, and parkinsonian, but with a sort of monumental quality, the Commissioner looked younger than his seventy-eight years and still exuded a sense of authority and power. Besides his wife, there were his two daughters and a grandchild – he was still, for all his parkinsonism, very much the patriarch of the house.
In a deep musical voice, as yet scarcely touched by parkinsonism, the Commissioner told us of his life in the village. He had at first been a cattle rancher, and the village strongman, able to bend horseshoes with his bare hands (his hands, gnarled now, and slightly tremulous, still looked powerful enough to crush stones). Later he had been a teacher in the village school; and then, after the war, he had been drawn more and more into village affairs – very complex and unsettled after the Japanese occupation, and with all the new pressures of Americanization on the island, trying (without being ‘backward’) to preserve the traditional Chamorro ways and myths and customs – finally becoming mayor. His symptoms had begun eighteen months ago, at first with a strange immobility, a loss of initiative and spontaneity; he found he had to make a huge effort to walk, to stand, to make the least movement – his body was disobedient, seemed disconnected from his will. His family and friends, who had known him as a driving, energetic man, first took this as aging, a natural slowing down after a life of intense activity. Only by degrees did it become clear to them, and him, that this was an organic malady, an all-too-familiar one, the bodig. This fearful, thick immobility advanced with frightening speed: within a year he had become unable to get up alone; once up, unable to sense or control the posture of his body, he might fall suddenly and heavily, without warning, to either side. He now had to have a son-in-law, a daughter, with him all the while, at least if he wanted to get up and go anywhere. He must have found this humiliating in a way, I thought, but he seemed to have no sense of being a burden, imposing on them, at all. On the contrary, it seemed natural that his family should come to his aid; when he was younger he too had had to help others – his uncle, his grandfather, two neighbors in his village who had also contracted the strange disease he himself now had. I saw no resentment in his children’s faces or their behavior; their helping seemed entirely spontaneous and natural.
I asked, a little diffidently, if I might examine him. I still thought of him as a powerful authority figure, not someone to lay hands on. And I was still not quite certain of local customs: Would he see a neurological examination as an indignity? Something to be done, if at all, behind closed doors, out of sight of the family? The Commissioner seemed to read my mind, and nodded. ‘You can examine me here,’ he said, ‘with my family.’
When I examined him, testing his muscle tone and balance, I found fairly advanced parkinsonism, despite the fact that his first symptoms had begun little more than a year before. He had little tremor or rigidity, but an overwhelming akinesia – an insuperable difficulty in initiating movement, greatly increased salivation, and profound impairment of his postural sense and reflexes. It was a picture somewhat unlike that of ‘ordinary’ Parkinson’s disease, but more suggestive of the much rarer post-encephalitic form.
When I asked the Commissioner his thoughts on what might have caused his illness, he shrugged. ‘They say it is fadang,’ he said. ‘Our own people sometimes thought this, and then the doctors too.’
‘Do you eat much of it?’ I asked.
‘Well, I liked it when I was young, but when they announced it was the cause of lytico-bodig I quit, we all did.’ Despite concerns about the eating of fadang as far back as the 1850s (which Kurland had reiterated in the 1960s), the notion that it might be dangerous was only widely publicized in the late 1980s, so this quitting, for the Commissioner, must have been relatively recent – and he was evidently nostalgic for the stuff. ‘It has a special taste,’ he said, ‘strong, pungent. Ordinary flour has no taste at all.’ Then he motioned to his wife, and she brought out a huge bottle of cycad chips – obviously the family supply, and one which they had not thrown away, but were still at pains to keep, despite the decision to ‘quit.’ They looked delicious – like thick corn chips – and I was strongly tempted to nibble them, but refrained.
The old man suggested we all go outside for a photograph before we left, and we lined up – his wife, himself, and me in the middle – in front of the giant cycad. Then he walked slowly back to the house, a regal figure, a parkinsonian Lear, on the arm of his youngest daughter – not merely dignified in spite of his parkinsonism, but somehow gaining a strange dignity from it.
There had been some controversy about the local cycads for two centuries or more. John was interested in the history of Guam and had copies of documents from the early missionaries and explorers, including a Spanish document from 1793, which praised fadang or federico as ‘a divine providence,’ and Frey-cinet’s 1819 Voyage Autour du Monde, in which he described seeing this harvested on a large scale in Guam.[48] He described the elaborate process of soaking and washing the seeds, and drying and grinding them to make a thick flour ideal for tortillas and tamales and a soup or porridge called atole – all this is illustrated in his account. It was well known, Freycinet remarked, that if the seeds were not washed sufficiently, they might still be highly poisonous:
A bird, goat, sheep or hog that drinks from the first water in which the federico has been soaked is apt to die. This does not happen with the second, much less the third, which can be consumed without danger.
Although this washing of the seeds was supposed to be effective in leaching out their poisons, several governors of Guam were to express reservations, especially when federico became the main article of the diet (as happened, typically, after typhoons, when all the vegetation was destroyed except for the tough cycads).
Thus Governor Pablo Perez wrote, in the famine of 1848, that
…not having sweet potatoes, yams and taro, food staples destroyed by the storm, [the Chamorros] have to betake themselves to the woods to seek there the few fruits which are left, which, though noxious, they use as a last resource…This is now their chief staple of food; and notwithstanding the precautions with which they prepare it, all believe that it is injurious to health.
This was echoed by his successor, Don Felipe de la Corte, seven years later, who singled out federico as the most dangerous of all the ‘fruits…of the forest.’[49]
Kurland, a century later, having found no clear evidence for an infectious or genetic origin for the lytico-bodig, now wondered whether some element of the Chamorro diet might be the pathogen he sought; and he invited Marjorie Whiting, a nutritionist working on Pohnpei, to come to Guam to investigate this. Whiting had a special interest in indigenous plants and cultures of the Pacific islands, and as soon as Kurland outlined the problem to her, she was fascinated and agreed to come. On her first visit to Guam, in 1954, she spent time in two very different communities – Yigo, which is close to Agana, and part of the Westernized, administrative center of the island; and Umatac, where she lived in a traditional Chamorro household. She became very close to the Chamorro family, the Quinatas, with whom she lived, and often joined Mrs. Quinata and the women of the village in preparing special dishes for Umatac’s frequent fiestas.
Cycads had never particularly attracted her attention before (there are no cycads on Pohnpei) – but now everything she encountered seemed to direct her attention to the local species, so common on Guam and the neighboring island of Rota. Cycas circinalis was indigenous, it grew wild, it was free, it required only the labor of collection and preparation.
I had met Marjorie in Hawaii, on my way to Micronesia, and she had told me some vividly personal anecdotes from her time on Guam. She had gone out to do field studies each day for six months, coming back each evening to her Chamorro family – she only discovered later, somewhat to her chagrin, that the rich soups she had been served every day had all been thickened with fadang. People were well aware of its toxic properties and the need for very elaborate washing, but enjoyed the taste of fadang, and especially prized it for making tortillas and thickening soup, ‘because of its peculiar mucilaginous quality.’ The Chamorros sometimes chewed the green outer seed husks to relieve thirst; when dried, the husks were considered a tasty sweet.
Whiting’s experience in Guam initiated an entire decade of research in which, collaborating at times with the botanist F.R. Fosberg, she made an encyclopedic investigation of cycads around the world, and their use by dozens of different cultures as foods, medicines, and poisons.[50] She undertook historical research, exhuming incidents of cycad poisoning among explorers as far back as the eighteenth century. She put together the scattered but voluminous evidence on the neurotoxic effects of cycads in various animals. Finally, in 1963, she published a detailed monograph on her work in the journal Economic Botany.
There were approximately a hundred species of cycad around the world, and nine genera,[51] she noted, and most of these had been used as sources of food, containing as they did large quantities of edible starch (sago), which could be extracted variously from the root, stem, or nut.[52] Cycads were eaten not merely, Whiting noted, as a reserve during times of shortage, but as a food with ‘a special prestige and popularity.’ They were used on Melville Island for first-fruit rites; among the Karawa in Australia for initiation ceremonies; and in Fiji, where they were a special food reserved only for the use of chiefs. The kernels were often roasted in Australia, where settlers referred to them as ‘blackfellows’ potatoes.’ Every part of the cycad had been used for food: the leaves could be eaten as tender young shoots; the seeds, when green, could be ‘boiled to edible softness; the white meat has a flavor and texture…compared to that of a roasted chestnut.’
Like Freycinet, Whiting described the lengthy process of detoxification: slicing the seeds, soaking them for days or weeks, drying and then pounding them, and, in some cultures, fermenting them too. (‘Westerners have compared the flavor of fermented cycad seeds with that of some of the best-known European cheeses.’) Stems of Encephalartos septimus had been used in parts of Africa to make a delicious cycad beer, she wrote, while the seeds of Cycas revoluta were used, in the Ryukyu Islands, to prepare a form of sake.[53] Fermented Zamia starch was regarded as a delicacy throughout the Caribbean, where it was consumed in the form of large alcoholic balls.
Every culture which uses cycads has recognized their toxic potential, and this was implied, she added, by some of the native names given to them, like ‘devil’s coconut’ and ‘ricket fern.’ In some cultures, they were deliberately used as poisons. Rumphius (the Dutch naturalist whose name is now attached to the widespread Pacific species Cycas rumphii ) recorded that in the Celebes ‘the sap from the kernels…was given to children to drink in order to kill them so that the parents would not be hampered when they went to follow their roving life in the wilderness of the forest.’[54] Other accounts, from Honduras and Costa Rica, suggested that Zamia root might be used to dispose of criminals or political enemies.
Nonetheless many cultures also regarded cycads as having healing or medicinal properties; Whiting instanced the Chamorro use of grated fresh seeds of Cycas circinalis as a poultice for tropical leg ulcers.
The use of cycads as food had been independently discovered in many cultures; and each had devised their own ways of detoxifying them. There had been, of course, innumerable individual accidents, especially among explorers and their crews without this cultural knowledge. Members of Cook’s crew became violently ill after eating unprepared cycad seeds at the Endeavour River in Australia, and in 1788 members of the La Perouse expedition became ill after merely nibbling the seeds of Macroza-mia communis at Botany Bay – the attractive, fleshy sarcotesta of these are loaded with toxic macrozamin.[55] But there had never been, Whiting thought, a cultural accident, where an entire culture had hurt itself by cycad eating.
There were, however, examples of animals poisoning themselves en masse, unprotected by any ‘instinctive’ knowledge. Cattle which browse on bracken may come down with a neurological disorder which resembles beriberi or thiamine deficiency – this is caused by an enzyme in bracken which destroys the body’s thiamine. Horses in the Central Valley of California have come down with parkinsonism after eating the toxic star thistle. But the example which Whiting especially remarks is that of sheep and cattle, which are extremely fond of cycads; indeed, the term ‘addiction’ has been used in Australia, where some animals will travel great distances for the plants. Outbreaks of neurocycadism, she noted, had been recorded in Australian cattle since the mid-nineteenth century. Some animals, browsing on the fresh young cycad shoots (this would especially occur in dry seasons, when other plants had died off, or after fires, when cycads would be the first plants to reshoot new leaves) would get a brief, acute gastrointestinal illness, with vomiting and diarrhea – this, if not fatal, would be followed by complete recovery, as with acute cycad poisoning in man. But with continued browsing on the plants, neurocycadism would develop; this would begin as a staggering or weaving gait (hence the colloquial name ‘zamia staggers’), a tendency to cross the hind legs while walking, and finally complete and permanent paralysis of the hind limbs. Removing the animals from the cycads at this stage was of no use; once the staggers had set in, the damage was irreversible.
Could this, Whiting and Kurland wondered, be a model for lytico? The idea was intriguing: fadang had been a common food before the war and, during the Japanese occupation, was used in much larger quantities, as other crops were requisitioned or destroyed. After the war, fadang consumption declined sharply because of the greater availability of imported wheat and corn flour – this, it seemed to them, could provide a very plausible scenario for the disease, why it had peaked immediately following the war, and steadily declined thereafter, an incidence which ran parallel to the use of fadang.
But the cycad theory was problematic on several grounds. First, there were no other known examples, outside Guam, of a chronic human illness ascribable to the use of cycads, despite their very wide and long use throughout the world. It was, of course, possible that there was something special about the Guam cycad, or some special vulnerability to it among the Chamorros. Second, the period of decades which might elapse between exposure to the cycads and the onset of lytico-bodig, if indeed the two were connected, was something which had no precedent in poisonings of the nervous system. All known neurotoxins acted immediately or within a few weeks, the time needed to accumulate to toxic levels in the body or for neurological damage to reach critical, symptomatic levels – this was so with heavy metal poisoning, as had occurred in the notorious Minamata Bay paralysis, with the neurolathyrism in India caused by eating the toxic chickling pea, and with the neurocy-cadism in cattle.[56] But these seemed quite different from a poison which, while causing no immediate effects, might lead to a progressive degeneration of particular nerve cells starting many years later. No such delayed toxic effect had ever been described – the very concept strained belief.
We set off again, to return to Umatac; John had more patients he wanted me to meet. He loved showing me patients, he said, taking me on house calls with him – I also loved this, seeing his energy, his neurological skill, and, even more, the delicate feeling, the caring, he showed for his patients. It took me back to my own growing up, when I would go out on house calls with my father, a general practitioner – I had always been fascinated by his technical skills, his elicitation of subtle symptoms and signs, his knack for making diagnoses, but also by the warm feeling which manifestly flowed between him and his patients. It was similar, I felt, with John; he too is a sort of GP – a neurological GP, an island GP – for his hundreds of patients with lytico-bodig. He is not just a physician to a group of individuals, but physician to a whole community – the community of the afflicted Chamorros and their relatives who live in Umatac, Me-rizo, Yona, Talofofo, Agat, Dededo, in the nineteen villages which are scattered over Guam.
Juan, another of John’s patients, has a very unusual form of the disease, John had told me. ‘Not like ALS, not like parkinsonism, not like any of the typical forms of lytico-bodig. What he does have is a peculiar tremor which I have never seen before in lytico-bodig – but I am sure this is the beginning of the disease in him.’ Juan was fifty-eight, very powerfully built, deeply sunburned, looked much younger than his years. His own symptoms had come on a couple of years ago, and he noticed them first when he was writing a letter. The act of writing brought on a shaking, and within a year it was no longer possible to write, at least with his right hand. But he had no other symptoms at all.
I examined him and was puzzled by the tremor. It looked nothing like the resting (‘pill-rolling’) tremor one usually sees in parkinsonism, for it came on with action or intention (which suppress the resting tremor). Nor did it resemble the ‘intention tremor’ which one may see (with incoordination and other cerebellar signs) if there is damage in the cerebellum or its connections. It resembled instead what neurologists gaily call essential or benign tremor. ‘Essential’ because it seems to arise without any demonstrable lesion in the brain, and ‘benign’ because it is usually self-limiting, responds well to medication, and does not interfere with life too much.
Usually this is the case. But there are a certain number of people who go on from such a ‘benign’ tremor to develop fullblown parkinsonism or other neurodegenerative disease. I thought of one patient of mine, an elderly woman in New York, who, when she developed such a tremor, in her seventies, was severely incommoded by it. She burst into tremor whatever she did, and could only prevent this by sitting stock-still. ‘They call it benign,’ she said, ‘what’s so benign about it?’ In her case, it was intensely malignant, not only in the way it interfered with her life, but in the fact that it proved to be the first symptom of a rare corticobasal degeneration, going on to rigidity, spasticity, and dementia, and, within two years, death.
There was no reason to suppose that Juan had anything like this. What he probably had, John felt – and I trusted his intuition – was an extremely mild form of bodig, so mild that he would probably be able to work and live independently for the rest of his life. Progressive and disabling as the lytico-bodig usually is, there are some, like Juan, who are only touched by it lightly, and who, after a sometimes rapid development of symptoms over a year or two, seem to show little further advance of the disease (though I have recently heard from John that Juan has developed some parkinsonian rigidity now).[57]
Had I let him, John would have driven straight on to the next patient, and the next. He was eager to show me everything in the few days I would be on Guam, and his energy and enthusiasm seemed to know no limits. But I had had enough for one day, and needed a break, needed a swim. ‘Yes, you’re right, Oliver,’ said John. ‘Let’s take a break – let’s go snorkelling with Alma!’
Alma van der Velde has a charming, sloping house, covered by vines, perhaps held together by them, surrounded by ferns and cycads, right by the water’s edge in Merizo. She herself is a water creature, who spends half her days swimming in the reef – badly arthritic, she moves painfully on land, but she is a graceful, strong, and tireless swimmer. She came to Micronesia as a young woman, fell in love with it, has never left. She has swum among these reefs daily for thirty years; she knows where to find the best chitons, cowries, and top shells, she knows the caves where octopuses hide, the underhangs of the reef where the rarest corals are found. When she is not swimming, she sits on her verandah, painting the sea, the clouds, the rocky out-croppings by the reef – or reading, or writing, completely self-sufficient. She and John are close friends, so close they hardly need to talk when they are together; they sit, they watch the waves thundering on the reef, and John is able, briefly, to forget the lytico-bodig.
Alma greeted us, and smiled when she saw I had brought my own fins and snorkel. John wanted to stay on the verandah and read; Alma and I would go to the reef together. She gave me a stick to help me walk over the shallow coral shelf with its razor-sharp branches, and then led the way – following a path which I could not have discerned, but which she clearly knew intimately, out to the clear waters beyond. As soon as the water was more than a couple of feet deep, Alma dived in, and, following her, I dived in too.
We moved past great coral canyons, with their endless forms and colors and their gnarled branches – some shaped like mushrooms, some like trees, being nibbled at by tetrodons and file-fish. Clouds of tiny zebra fish and fish of an iridescent blue swam through them, and around me, between my arms, between my legs, unstartled by my movements.
We swam through shoals of wrasse and parrot fish and damsels, and saw turkey fish, with rusty feather fans, hovering beneath us. I reached out my hand to touch one as it hovered, but Alma shook her head violently (later she told me the ‘feathers’ were quite poisonous to touch). We saw flatworms waving like tiny scarves in the water and plump polychaetes with iridescent bristles. Large starfish, startlingly blue, crawled slowly on the bottom, and spiny sea urchins made me glad my feet were protected by fins.
Another few yards and we were suddenly in a deep channel, the bottom forty feet below us, but the water so clear and transparent that we could see every detail as if it were at arm’s length. Alma made some gesture I could not understand as we swam in this channel; and then we turned back, to the shallower waters of the reef. I saw hundreds of sea cucumbers, some nearly a yard long, making their cylindrical way slowly across the ocean floor, and found these enchanting – but Alma, to my surprise, made a grimace, shook her head.
‘They’re bad news,’ she said, after we had come in and showered and were eating fresh tuna and a salad with John on the porch. ‘Bottom feeders! They go with pollution – you saw how pale the reef was today.’ Indeed, the corals were varied and beautiful, but not quite as brilliant as I had hoped, not as brilliant as they had been when I snorkelled off Pohnpei. ‘Each year it gets paler,’ Alma continued, ‘and the sea cucumbers multiply. Unless they do something, it’ll be the end of the reef.’[58]
‘Why did you gesture when we were in the channel?’ I asked.
‘That means it’s a shark channel – that is their highway. They have their own schedules and times, times I would never dream of going near it. But it was a safe time today.’
We decided to rest and read for a while, in companionable silence on the verandah. Wandering inside to Alma’s comfortable living room, I spotted a large book on her shelf entitled The Useful Plants of the Island of Guam, by W.E. Safford. I pulled it out – gingerly, as it was starting to fall apart. I had thought, from the title, that it was going to be a narrow, rather technical book on rice and yams, though I hoped it would have some interesting drawings of cycads as well. But its title was deceptively modest, for it seemed to contain, in its four hundred densely packed pages, a detailed account not only of the plants, the animals, the geology of Guam, but a deeply sympathetic account of Chamorro life and culture, from their foods, their crafts, their boats, their houses, to their language, their myths and rituals, their philosophical and religious beliefs.
Safford quoted detailed accounts of the island and its people from various explorers – Pigafetta, Magellan’s historian, writing in 1521; Legazpi in 1565; Garcia in 1683; and half a dozen others.[59] These all concurred in portraying the Chamorros as exceptionally vigorous, healthy, and long-lived. In the first year of the Spanish mission, Garcia recorded, there were more than 120 centenarians baptized – a longevity he ascribed to the ruggedness of their constitutions, the naturalness of their food, and the absence of vice or worries. All of the Chamorros, noted Legazpi, were excellent swimmers and could catch fish in their bare hands; indeed, he remarked, they sometimes seemed to him ‘more like fish than human beings.’ The Chamorros were skilled as well in navigation and agriculture, maintained an active trade with other islands, and had a vital society and culture. Romantic exaggeration is not absent in these early accounts, which sometimes seem to portray Guam as an earthly paradise; but there is no doubt that the island was able to support a very large community – the estimates all fall between 60,000 and 100,000 – in conditions of cultural and ecological stability.
Though there were occasional visitors in the century and a half that followed Magellan’s landing, there was to be no massive change until the arrival of Spanish missionaries in 1668, in a concerted effort to Christianize the population. Resistance to this – to forced baptism, in the first place – led to savage retaliation, in which whole villages would be punished for the act of a single man, and from this to a horrifying war of extermination.
On top of this, there now came a series of epidemics introduced by the colonists – above all smallpox, measles, and tuberculosis, with leprosy as a special, slowly smoldering gift.[60] And in addition to actual extermination and disease, there were the moral effects of a forced colonization and Christianization – the attempted soul murder, in effect, of an entire culture.
This…weighed so heavily upon phem]…that some even sacrified their lives in despair; and some women either purposely sterilized themselves or cast into the waters their new-born infants, believing them happy to die thus early, saved from the toils of a life gloomy, painful, and miserable…they judge that subjection is the worst misery in the world.
By 1710, there were virtually no Chamorro men left on Guam, and only about a thousand women and children remained. In the space of forty years, ninety-nine percent of the population had been wiped out. Now that the resistance was over, the missionaries sought to help the all-but-exterminated Chamorros to survive – to survive, that is, on their own, Christian and Western, terms – to adopt clothing, to learn the catechism, to give up their own myths and gods and habits. As time passed, new generations were increasingly hybridized, as mestizo children were born to women who were married to, or raped by, the soldiers who had come to subdue their nation. Antoine-Alfred Marche, who travelled the Marianas between 1887 and 1889, felt there were no longer any pure-blooded Chamorros in Guam – or at most a few families on the neighboring island of Rota, where they had fled two centuries before. Their bold seafaring skills, once renowned throughout the Pacific, were lost. The Chamorro language became creolized, admixed with much Spanish.
As the nineteenth century progressed, Guam, once a prized Spanish colony on the galleon route, fell into deepening neglect and oblivion; Spain herself was in decline, had problems at home, other interests, and all but forgot her colonies in the western Pacific. This period, for the Chamorros, was a mixed one: if they were less persecuted, less actively under the heel of their conquerors, their land, their diet, their economy, had become more and more impoverished. Trade and shipping continued to decline, and the island became a distant backwater, whose governors had neither the money nor the influence to change things.
The final sign of this decline was the farcical way in which Spanish rule was officially ended, by a single American gunboat, the USS Charleston, in 1898. There had been no ships for two months, and when the Charleston and its three companion vessels appeared off Guam, a pleasurable excitement swept the island. What news, what novelties, the ships might bring! When the Charleston fired, Juan Marina, the governor, was pleased – this must be, he assumed, a formal salute. He was stunned to discover that it was not a greeting, but war – he had no idea that there was a war going on between America and Spain – and he now found himself led in chains aboard the Charleston, a prisoner of war. Thus ended three centuries of Spanish rule.
It was at this point that Safford himself entered the history of Guam. He was a navy lieutenant at the time, an aide to Captain Richard Leary, the first American governor – but Leary, for reasons of his own, elected not to leave his ship, which was moored in the harbor, and sent Safford to act in his stead. Safford soon ‘gained a working knowledge of the Chamorro language and customs, and his respect for the people, his courtesy, his curiosity, made him an essential bridgehead between the islanders and their new masters.[61] The new American administration, though not quite as out of touch as the Spanish one it replaced, did not institute too many changes in Guam. It did, however, open schools and English classes – the first of which were conducted by Safford in 1899 – and greatly improved medical observation and care. The first medical reports of ‘hereditary paralysis’ and its unusual incidence date from 1900; the more specific term, ‘ALS,’ was used as early as 1904.
Life in Guam remained much the same as it had been for the past two centuries. The population had gradually increased since the genocide of 1670-1700; a census in 1901 found 9,676 people, of whom all but forty-six considered themselves to be Chamorros. Nearly 7,000 of them lived in the capital of Agana or its adjaacent villages. Roads were very poor, and the villages in the south, like Umatac, were almost inaccessible in the rainy parts of the year, and could only reliably be reached by sea.
Nevertheless, Guam was deemed important from a military point of view, because of its size and crucial position in the Pacific. During the First World War, Japan was one of America’s allies, and Guam was not drawn into the conflict. But there was great tension on December 8, 1941, as Guam got news of the attack on Pearl Harbor; within hours, it too found itself under attack as Mitsubishis from Saipan, just a hundred miles to the north, suddenly appeared in the sky above Agana, spitting machine-gun fire. Two days later, Japanese infantry, which had been massing on Rota, landed, and Guam could offer little resistance.
The Japanese occupation was a time of great cruelty and hardship, reminiscent of the conquistadores. Many Chamorros were killed, many were tortured or enslaved for war work, and others fled their villages and farms to live out the occupation, as best they could, in the hills and jungle. Families and villages were broken up, fields and food supplies were taken over, and famine ensued. Cycad seeds had been an important part of their diet for two hundred years at least; now they became a near-exclusive diet for some. Many more Chamorros were brutally murdered near the end of the war, especially when it became clear that the Japanese days were numbered, and that the island would soon be ‘liberated’ by the Americans. The Chamorros had suffered appallingly during the war, and welcomed the American soldiers, when they came, with jubilation.
The real Americanization of Guam came after 1945. Agana, which had housed half of Guam’s population before the war, had been levelled in the recapture of the island and had to be totally rebuilt; the rebuilding transformed it from a small town of low, traditional houses to an American city with concrete roads, gas stations, supermarkets, and ever-higher high-rise apartments. There was massive immigration, mostly of servicemen and their dependents, and the population of the island swelled from its prewar 22,000 to more than 100,000.
Guam remained closed to visitors and immigrants, under military restriction, until 1960. The entire north and north-eastern portions, which contained the best beaches on the island, and the beautiful and ancient village of Sumay (taken over by the Japanese in 1941, and finally flattened by the Americans in 1944), were appropriated for new military bases, and closed even to the Chamorros who had once lived there. Since the 1960s, huge numbers of tourists and immigrants have arrived – Filipino workers by the tens of thousands, and Japanese tourists by the million, requiring ever vaster golf courses and luxury hotels.
The traditional Chamorro ways of life are dwindling and vanishing, receding to pockets in the remotest southern villages, like Umatac.[62]
John normally goes on his rounds with Phil Roberto, a young Chamorro man who has had some medical training, and who acts also as his interpreter and assistant. Like Greg Dever in Pohnpei, John feels strongly that Micronesia has been far too dominated by America and American doctors, imposing their own attitudes and values, and that it is crucial to train indigenous people – doctors, nurses, paramedics, technicians – to have an autonomous health-care system. John hopes that Phil will succeed him, completing his medical degree and taking over his practice when John retires, for Phil, as a Chamorro himself, will be an integral part of the community in a way that John can never fully be.
Over the years there has been increasing resentment among the Chamorros in regard to Western doctors. The Chamorros have given their stories, their time, their blood, and finally their brains – often feeling that they themselves are no more than specimens or subjects, and that the doctors who visit and test them are not concerned with them. ‘For people to admit that their family has this disease is a big step,’ Phil said. ‘And then to let medical people come into their homes is another big step. Yet in terms of treatment or care, health care, home care, they’re really not given enough assistance. Visiting doctors come and go, with their forms and research protocols, but they don’t know the people. John and I go into people’s houses regularly, and we come to know the families, their histories, and how they’ve come to this point in their lives. John has known many of his patients for ten or twelve years. We have videotaped hundreds of hours of interviews with patients. They have come to trust us, and are more open in terms of calling for assistance – saying, ‘So-and-so is looking rather pale, what should I do?’ They know we are here for them.
‘We are the ones who go back to their homes weeks after the researchers have been here and taken their samples back to the States. The patients ask us, ‘So what happened to those tests performed on us?’ But we have no answers for them, because they’re not our tests.’
The next morning John and Phil picked me up early. ‘You saw a little of the parkinsonism and dementia – the bodig – yesterday,’ he said. ‘Kurland felt this form of the disease was replacing ALS in the 1970s – but you must not imagine the ALS is extinct. I have lytico patients I have been following for years, and new cases as well – we’ll see some today.’ He paused, and added, ‘There is something unbearable about ALS; I’m sure you have felt it, Oliver – every neurologist does. To see the strength go and the muscles wasting, people unable to move their mouths to speak, people who choke to death because they can’t swallow…to see all this and feel you can do nothing, absolutely nothing, to help them. Sometimes it seems especially horrible because their minds remain absolutely clear until the end – they know what is happening to them.’
We were on our way to see Tomasa, whom John has known ever since he came to Guam. She had already had lytico for fifteen years when he met her; it has advanced steadily since, paralyzing not only her limbs but the muscles of breathing, speech, and swallowing. She is now near the end, but has continued to bear it with fortitude, to tolerate a nasogastric tube, frequent choking and aspiration, total dependence, with a calm, unfright-ened fatalism. Indeed a fatality hangs over her entire family – her father suffered from lytico, as did two of her sisters, while two of her brothers have parkinsonism and dementia. Out of eight children in her generation, five have been afflicted by the lytico-bodig.
When we entered her room, Tomasa looked wasted, paralyzed, but alert. With a cheery ‘Hello, Tomasa, how is everything today?’ John walked over to the couch where she was lying. He leaned over and touched her shoulder, and she followed his hand with her eyes, which were bright and attentive. She followed everything, with an occasional (perhaps sometimes reflexive, pseudobulbar) smile, and a slight groaning as she exhaled. She was dying in full consciousness, after twenty-five years of an implacable disease, in a bright sunlit room. John introduced me to Tomasa and to her daugher, Angie, who was with her. When I asked her date of birth, Tomasa produced a string of (to me) unintelligible sounds, but her daughter interpreted this as April 12, 1933. Tomasa could open her mouth on request, and put out her tongue. It was fearfully wasted, fissured, fasciculating, like a bag of worms. She made another unintelligible sound. ‘She wants me to bring you and Dr. Steele something to drink,’ Angie said. Tomasa’s manners have not deserted her, even at this point. ‘She has taught countless people about the disease on Guam,’ said John; Tomasa smiled. ‘Don’t worry, Tomasa – Angie will not get the lytico. No one in the younger generation gets this, thank God,’ he added softly.
Family, friends, neighbors, come in at all hours, read the papers to her, tell her the news, give her all the local gossip. At Christmas, the Christmas tree is put by her couch; if there are local fiestas or picnics, people gather in her room. She may scarcely be able to move or speak, but she is still, in their eyes, a total person, still part of the family and community. She will remain at home, in the bosom of her family and community, in total consciousness and dignity and personhood, up to the day of her death, a death which cannot, now, be too far off.
Seeing Tomasa surrounded by her large family made me think of a 1602 description of the Chamorros by an early missionary, Fray Juan Pobre, which I had seen while browsing in John’s office:
They are naturally very compassionate people… The day when the master of the house, or his wife, or a child falls ill, all the relatives in the village will take dinner and supper to them, which will be prepared from the best food they have in the house. This is continued until the patient dies or recovers.
This acceptance of the sick person as a person, a living part of the community, extends to those with chronic and incurable illness, who may, like Tomasa, have years of invalidism. I thought of my own patients with advanced ALS in New York, all in hospitals or nursing homes, with nasogastric tubes, suction apparatus, sometimes respirators, every sort of technical support – but very much alone, deliberately or unconsciously avoided by their relatives, who cannot bear to see them in this state, and almost prefer to think of them (as the hospital does) not as human beings, but as terminal medical cases on full ‘life support,’ getting the best of modern medical care. Such patients are often avoided by doctors too, written, even by them, out of the book of life. But John has stayed close to Tomasa, and will be with her, with the family, the day she finally dies.
From Tomasa’s house, we drove north across the island, up through the cycad-dappled hills, and past placid Lake Fena, Guam’s only reservoir of fresh water.[63] Everything looked very dry on the plateau; at one point, John pointed out the charred trees and large areas of blackened ground which were the legacy of a great forest fire the previous summer. And yet here, even in these blackened areas, were new shoots of green – shoots which came from the stumps of cycads.
Dededo is a more modern village, now the largest on Guam after Agana. It has a somewhat suburban look, with each house set at a little distance from the others, so there is more sense of ‘privacy’ (though this seems to be more a Western concept than a Chamorro one). It is in one of these houses that Roque lives. He is a strong, muscular man in his early fifties – robust, covered with tattoos from his tour of duty in the army – in perfect health, apparently, until fourteen months ago, when he started to complain of something blocking his throat. He soon noticed symptoms in his voice, his face, his hands, and it became clear that he had a rapidly progressive, almost fulminating, form of lytico. While he is not too disabled at this point, he knows he will be dead in a few months. ‘You can talk to me about it,’ he said, seeing my reluctance. ‘I have no secrets from myself.’ Part of the problem, he said, was the mealymouthed doctors in Agana, who were evasive, who wished to convey hope and reassurance – an optimistic, false view of the lytico, which might prevent him from coming to terms with it, with his rapidly narrowing life and the certainty of death. But his body told him the truth – and John did too.
‘I was a very athletic man, and now the disease has pulled me down,’ he told us. ‘I accept it, but sometimes I feel so depressed that I feel like doing something drastic…To commit suicide is no good. It’s not right. But I wish the Lord would take me rather than wait for no result or no cure. If there’s no cure, I would have the Lord take me.’
Roque was deeply sad, he said, that he would not see his children grow up, and that his youngest son (just two now) might not retain any memories of him; he was sad that he would be leaving his wife a widow, and his old parents, still in good health, bereaved.
What will happen with him, I asked John – will he die at home, like Tomasa, or will he go to a hospital? ‘That depends,’ said John, ‘on what he wants, what the family wants, the course of the illness. If you have complete bulbar paralysis, and respiration is affected, you have to have assisted breathing, a respirator, or you die. Some people want this, some do not. I have a couple of patients on respirators at St. Dominic’s – we’ll see them tomorrow.’
Later in the afternoon, Phil and I had planned to go down to the beach at Sumay, said to be the finest for snorkelling in Guam. This was on the military base, so Phil had arranged permission for us to go. We arrived around four, and presented our papers. But our reception at the gate was surly and suspicious, especially when the guards saw that Phil was a Chamorro.
When I tried to put a good-natured, genial spin on things, I was met by a blank, faceless stare – I was reminded, unavoidably, of the hateful episode on Kwajalein, the helplessness of civilians, civility, in the face of military bureaucracy. Phil had warned me that I had best say nothing, that we both had to behave in the most deferential, abject manner, or they would find reason to deny us entrance at all. I had thought, at the time, that his advice was a little overstated – but now I saw that it was not. In the event, we were kept waiting at the gate for an hour, while the guards phoned for various permissions and confirmations. At five o’clock, we were told that our admittance had been approved – but also that it was too late, because the base was now closed. At this point, fortunately (for I was about to explode with rage), a senior officer came along; we could override the regulations this time, he said – we could enter and have our swim, but we would have to be accompanied by military police as long as we were on the base.
Phil choked at this, and the feeling of supervision made me furious, but having come this far, we decided to go ahead and have our swim. Changing into our swimming gear in full sight of a jeep with four police was slightly unnerving, and an antino-mian part of me wanted to do something outrageous – but I controlled myself, with some regret, tried to put the police out of mind, and surrendered to the water.
It was, indeed, exquisite. There are more than three hundred species of coral native to Guam, and the colors of these at Sumay seemed far richer than those at Alma’s, or even those of the glorious corals off Pohnpei. A little farther from shore, we could see the outlines of the wreck of a Japanese warship, richly and strangely metamorphosed by a crust of barnacles and corals – but it would take more time, and scuba gear, to examine it properly. As we swam back in, I could see the shape of the waiting jeep shivering through the transparent waters, and the stiff figures of the MPs, distorted by their shifting refraction. As we dried ourselves in the gloaming, I seethed to think that this perfect reef was denied to the people of Guam, hoarded and locked up by institutional order.
But Phil’s anger had a deeper layer. This was the site of the old village of Sumay, he said as we drove back to the entrance of the base. ‘It was the most beautiful village in the whole of Guam. It was bombed by the Japanese, the first day they attacked Guam; then all the inhabitants were evicted or killed. When the Allies came, the Japanese retreated to those caves in the cliffs you can see, and trying to get them out, the Americans bombed the whole place into dust. That fragment of the church and the graveyard – that’s the only thing left. My grandparents were born here,’ he added, ‘and they are buried here too. Many of us have ancestors in the graveyard here, and we want to visit the graves, pay our respects – but then we have to go through the bureaucratic process you’ve seen. It is a great indignity.’
The next day, John and I set out for St. Dominic’s, a beautiful new hospital, or, as the nuns prefer to call it, Home, with gardens, patios, a tranquil chapel, perched on Mount Barrigada, overlooking Agana. Here were two more patients of John’s – both, like Roque, still in their fifties, and stricken by lytico in its most virulent form. Both had been in perfect health, seemingly, eighteen months before; both had now reached a point where the muscles of respiration were paralyzed, and mechanical ventilation was needed to help them breathe. As we approached their rooms, I heard the heavy, animal-like breathing of their respirators, and the unpleasant sucking sounds made as their throats were suctioned dry (for they could no longer swallow their own secretions and had to have these sucked out mechanically, lest they be aspirated into the trachea and lungs). I could not help wondering whether life was worth it under these conditions, but both patients had children with them – an adult son in one case, an adult daughter in the other – with whom some contact and simple communication was still possible; they could still be read to, watch television, listen to the radio. Their minds were still alive and active, even if their muscles were not, and both had indicated that they wanted to go on, to stay alive as long as they could, even if this meant being maintained on a machine. Both were surrounded by religious pictures and icons, which they gazed at with unblinking eyes. Their faces, I wanted to think, seemed to be at peace, despite the heaving, gurgling bodies below.
Many patients with very advanced bodig come to St. Dominic’s too, in some cases suffering not only from parkinsonism, but from a severe dementia and spasticity as well. In such patients, in the final stages, the mouth hangs open, drooling with saliva; the palate hangs motionless, so that speech and swallowing are impossible; and the arms and legs, severely spastic, become bent in immovable flexion contractures. Patients in this state can hardly be looked after at home by even the most devoted families, and are usually brought to St. Dominic’s, where the nuns are devoted to their care. I was deeply moved by the dedication of the nuns who undertook this care; they reminded me of the Little Sisters of the Poor, an order of nuns I work with in New York. Unlike what one sees in most hospitals, the Sisters’ first care, and continuing concern, is with the dignity and state of mind of each patient. There is always a sense of the patient as a total individual, not just a medical problem, a body, a ‘case.’ And here, where family and communal ties are so close, the patients’ rooms, the corridors, the patios, the gardens of St. Dominic’s, are always thronged with family and neighbors – the family, the village, the community, of each patient is reconstituted here in miniature. Going to St. Dominic’s does not mean a removal from all that is dear and familiar, but rather a translocation of all this, as much as is possible, into the medical milieu of the hospital.
I felt drained by seeing these patients with lytico and bodig in their final, terrible stages, and I wanted desperately to get away, to lie down and collapse on my bed, or swim again in a pristine reef. I am not sure why I was so overwhelmed; much of my practice in New York involves working amid the incurable and disabled, but ALS is rare – I may see only one case every two or three years.
I wondered how John, who has forty or more patients with advanced lytico-bodig, dealt with his feelings. When he was with patients, I noted, he often adopted his booming, professional voice, and an optimistic, bracing, cheery manner – but this was only a surface, behind which he remained intensely sensitive and vulnerable. Phil later told me that when John is alone, or thinks he is alone, he may weep at the plight of his patients, and at his impotence, our impotence, to do anything about it.
After lunch we visited a different part of St. Dominic’s – a pleasant, open room looking onto a garden, where some of the day patients had collected for their afternoon session. St. Dominic’s is not just a chronic-care hospital, but also has an active day program for ambulatory patients who come from all over the island. It is a place where they can meet, enjoy meals together, walk in the gardens, or work in a workshop, and receive therapy of all sorts – physiotherapy, speech therapy, arts and music therapy. It was here that John brought me to see Euphrasia, another patient of his. She is seventy, but looks much younger, and has had a parkinsonian form of bodig for twenty-four years, though not the least memory impairment or dementia. She had moved to California as a young bride soon after the war, and did not revisit Guam for many years. Nevertheless, she came down with bodig in 1969, despite having lived out of Guam for twenty-two years.
Seeing Euphrasia brought home to me the immense lag which might exist between exposure to whatever it is (or was) on Guam, and the subsequent development of lytico-bodig. John told me, indeed, that he had heard of one patient in whom the gap between leaving Guam and developing the disease was more than forty years – and that there might be similar lags in those who came to Guam. No Caucasian, as far as he knew, had ever contracted the disease, but he knew of a few Japanese and Filipino patients who had come to Guam, married Chamorros, entered the culture completely, and then come down with apparent lytico or bodig many years later.[64]
This, for him, was the most convincing clinical evidence of the extraordinary ‘silent’ period in which the lytico-bodig must, in some sense, be present – but subclinical or latent. Was it burning away slowly beneath the surface, all through these years? Or did there have to be a new event, which might ignite a previously harmless, perhaps arrested, process and turn it into an active one? Sometimes he favored the first thought, John said; sometimes the second – though seeing a patient such as Roque, in whom there had been so explosive an onset of disease, erupting in the midst of seemingly perfect health, one had less sense of a steady, ongoing process finally surfacing than of a sudden, lethal transformation.
I thought of how von Economo, the physician who had first identified the encephalitis lethargica, had spoken of postencephalitic patients as ‘extinct volcanoes.’ This seemed an apt comparison until L-DOPA came along, when I began to think of them as sleeping volcanoes, which might suddenly (sometimes dangerously) erupt with this new drug. But these patients were already manifestly ill – frozen, catatonic; whereas the lytico-bodig patients, seemingly, were perfectly well and active before their symptoms began. ‘But you can’t be sure of that on purely clinical grounds,’ said John. ‘You have no way of judging what may be going on at the cellular level.’ What had been going on, we wondered, in Euphrasia during those twenty-two years after she had left Guam?
Euphrasia was started on L-DOPA by her doctor in California in 1969 (this intrigued me, as it was the same year I had started my own post-encephalitic patients on L-DOPA). In ordinary Parkinson’s disease, the initial effects of the drug are smooth and steady and last for many hours, though sooner or later, its effects may become unstable, giving patients a brief period of fluidity, sometimes accompanied by chorea and other involuntary movements, followed in an hour or so by an intense immobility – a so-called on-off effect. Such on-off effects, I had found, tended to set in much earlier with my post-encephalitic patients – sometimes, indeed, from the very start, and Euphrasia too, John said, had shown reactions which were extreme and hyperbolic from the beginning. And yet despite its ups and downs, she continued to get a crucial benefit from L-DOPA, for it allowed her a few hours of relatively good function each day.
She had not had any medication for several hours when we stopped by, and she was in an ‘off’ state, sitting completely motionless in a chair, her head bent, almost jammed, on her chest, only her eyes still capable of any movement. There was extreme rigidity in all her limbs. Her voice was very soft, flat, almost inaudible, and devoid of any animation or expression. She drooled constantly.
John introduced us, and I took her hand and squeezed it gently She could not speak, but she smiled back, her eyes crinkling, and I could feel a faint squeeze in response.
With a conspiratorial wink to Euphrasia, I said to John, ‘I’ll show you something – or Euphrasia will.’ I managed, with some difficulty, to get her to her feet. Walking backward in front of her, holding her gnarled hands, cueing her all the time, I was able to guide her, with tiny, tottering steps, to the garden just outside. There was a rock garden in the form of a little hill, with irregular ledges and slopes. ‘Okay,’ I said to Euphrasia, pointing to a rock, ‘climb over this, you’re on your own – go!’ To John’s horror, and the nuns’, I took my hands off her, and let her go. But Euphrasia, who had been almost incapable of movement on the flat, featureless floor of the dayroom, lifted her leg high, and stepped boldly over the rock, and then over another one, and another, up to the top of the rock garden, without difficulty. She smiled, and climbed down again, as surefootedly as she had gone up. As soon as she reached the level ground, she was as helpless as before. John looked rather stunned at this, but Euphrasia still had a ghost of a smile on her lips – she was not in the least surprised at all. And had she been capable of speaking, she might have said, like so many of my post-encephalitic patients, ‘If only the world consisted of stairs!’
It was two o’clock, the nun said, time for her medicine. She brought Euphrasia, now sitting in the dayroom once again, a tiny white pill with some water. Fourteen minutes after receiving her L-DOPA – we timed this, as if waiting for a chemical reaction, or explosion – she suddenly jumped to her feet with such energy her chair fell over backward, hurtled along the corridor, and burst into lively, even rambunctious, conversation, bursting with all the things she had wanted to say, but could not while she was frozen. This was not just a disappearance of her parkinsonism, her motor problems, but a transformation of her senses, her feelings, her whole demeanor. I had not seen anything like this in more than twenty years, and was both stunned (though I had half expected her to show such a reaction) and a bit nostalgic – Euphrasia especially reminded me of my postencephalitic patient Hester, in whom there was a similar, instantaneous transformation, with no intermediary state, no warming-up period whatever.
But it was not a wholly simple ‘awakening’ for Euphrasia, any more than it had been for hyperbolic Hester. For along with the motor animation, the liveliness, the playfulness, which suddenly came on her, there came a tendency to wisecrack, to tic, to sudden lookings and touchings, to tossing and darting, to jabbing and lunging – a dozen strange impulsions, a drivenness of body and mind. There was this tremendous rush of life, of extravagant activation, both healthy and pathological, and then, twenty minutes later, a re-descent into her original state, coupled with repeated yawning, a sudden complete lethargy.
‘What do you think of that, eh?’ asked John, at my side, eagerly. ‘Remind you of anything?’
When he is not seeing patients, John teaches at the Guam Memorial Hospital in Tamuning and does research in his laboratory there. He has lobbied hard for more research funding to be put into local facilities, and would like to establish a complete center on the island for investigating the lytico-bodig, with sophisticated neuropathology equipment and facilities for MRI scans and other brain imaging. At present, many of these studies have to be done on the mainland, while much of the epidemiological work – interviewing patients and piecing together extensive family trees – as well as basic clinical and lab work of various kinds, is done here on the island.
He showed me into his lab; he had something special he wanted me to see. ‘Let me show you these slides, Oliver,’ said John, waving me over to a microscope. I looked through the eyepiece, under low power first, and saw pigmented cells, symmetrically arranged in a V.
‘Substantia nigra,’ I said. ‘Many of the cells are pale and depigmented. There’s a lot of glial reaction, and bits of loose pigment.’ I shifted to a higher power, and saw a huge number of neurofibrillary tangles, densely staining, convoluted masses, harshly evident within the destroyed nerve cells. ‘Do you have samples of cortex, hypothalamus, spinal cord?’ John handed these to me, I looked at them one after another – all were full of neurofibrillary tangles.
‘So this is what lytico-bodig looks like,’ I said, ‘neurofibrillary degeneration everywhere!’
‘Yes,’ said John, ‘that’s very typical. Here’s another case – have a look at this.’ I went over it as before; the findings were very similar, and there was much the same distribution of tangles.
‘All the lytico-bodig cases look like this?’ I asked.
‘Actually, Oliver’ – John smiled broadly – ’what you’re looking at now isn’t lytico-bodig at all. It’s your disease, it’s postencephalitic parkinsonism – these slides were sent to me by Sue Daniel in London.’
‘I haven’t done much pathology since I was a resident,’ I said, ‘and I’m no expert – but I can’t tell them apart.’
John grinned, pleased. ‘Here, I have some more slides for you.’ I looked at this new series, starting with the substantia nigra, the midbrain, moving up and down from there.
‘I give up,’ I said, ‘I can’t tell whether it’s lytico-bodig or post-encephalitic parkinsonism.’
‘Neither,’ said John. ‘This is my disease, progressive supranuclear palsy. In fact, it’s from one of the original cases we described in 1963 – even then we wondered about its similarity to post-encephalitic parkinsonism. And now we look at the Guam disease…and all three look virtually the same.
‘Sue Daniel and Andrew Lees and their colleagues at the Parkinson’s Brain Bank have wondered whether these diseases are, in fact, related – perhaps even the same disease, a viral one, which could take three different forms.
‘These are very similar to the neurofibrillary tangles to be found in Alzheimer’s disease,’ John went on, ‘though in Alzheimer’s there are not as many, and they occur in a different distribution. So we have tangles – like little tombstones in the nervous system – in four major neurodegenerative diseases. Perhaps the tangles contain vital clues to the process of neu-rodegeneration, or perhaps they are relatively nonspecific neural reactions to disease – we don’t know.’
As we got back in his car to return to Umatac, John continued to sketch the history of the lytico-bodig. Another dimension was added to the problem as the 1960s advanced, and a curious change was observed in the natural history of the disease: cases of bodig, which had been much rarer than cases of lytico in the 1940s and early ‘50s, now came more and more to outnumber them. And the age of onset was also increasing – there were no more teenage cases (like the nineteen-year-old youth with lytico whom Kurland had seen), and almost no cases in their twenties.
But why should a single disease present itself chiefly as lytico in one decade, and then predominantly as bodig the next? Did this have something to do with age? – bodig patients, by and large, were a decade older than those with lytico. Did it have something to do with dose – could it be that the most severely exposed patients had their motor neurons knocked out in the 1950s, producing an ALS-like syndrome; whereas those exposed to less of the agent (whatever it was) were then caught by the slower effects of this on the brain, which might cause parkinsonism or dementia? Would most patients with lytico, were they to survive long enough, go on to develop bodig years later? (This, of course, was an impossible question, because lytico in its acute form cuts short the course of life. But Tomasa, still alive after twenty-five years of lytico, showed not a trace of bodig.) All of these questions were posed – but none of them could be answered.
Kurland had always felt that the possibility of cycad toxicity, however odd it seemed, should be investigated as carefully as possible, and to this end, he had organized, with Whiting, a series of major conferences starting in 1963 and continuing for a decade. The first of these were full of excitement, hopes of a breakthrough, and brought together botanists, nutritionists, toxicologists, neurologists, pathologists, and anthropologists to present research from all over the world. One constituent of cycad seeds was cycasin, a glycoside which had been isolated in the 1950s, and this was now reported to have a remarkable range of toxic effects. Large doses caused death from acute liver failure; smaller doses might be tolerated by the liver, but later gave rise to a variety of cancers. While cycasin did not seem to be toxic to adult nerve cells, it was one of the most potent carcinogens known.
There was renewed excitement when another compound found in cycad seeds was isolated – an amino acid, beta-2V-methylamino-levoalanine (BMAA), very similar in structure to the neurotoxic amino acid beta-N-oxalylamino-levoalanine (BOAA), which was known to cause the paralysis of neuro-lathyrism. Was BMAA, then, the cause of lytico-bodig? It had been administered in many animal experiments, John said, but none of the animals developed anything like lytico-bodig.
Meanwhile there were two further discoveries of an epidemiological sort. In 1962 Carleton Gajdusek, who had been working on the cause of kuru, a fatal neurological disease in eastern New Guinea (work for which he was later awarded a Nobel Prize), now found an endemic lytico-bodig – like condition among the Auyu and Jakai people on the southern coastal plain of western New Guinea.[65] This proved indeed to be an extraordinarily ‘hot’ focus, for the incidence of disease here was more than 1,300 per 100,000, and thirty percent of those affected were under the age of thirty. At about the same time, in Japan, Kiyoshi Kimura and Yoshiro Yase discovered a third focus of a lytico-bodig – like disease on the Kii Peninsula of the island of Honshu. But in neither of these places did they find any cycads.
With these new findings, and the inability to produce an animal model of the disease, the plausibility of the cycad hypothesis seemed to fade. ‘The cycad proponents thought they had it,’ said John, somewhat wistfully. ‘They thought that they’d cracked the lytico-bodig, and it was a real loss to let the cycad hypothesis go. Especially as they had nothing to replace it; they were left with a sort of conceptual vacuum.’ By 1972 only Kurland continued to consider it a possibility, but for most of the researchers, the cycad hypothesis had died, and attention turned elsewhere.
John had arranged to take me that evening to a Japanese restaurant in Agana. With our huge tourist trade, he said, we get the best Japanese food in the world here, outside Japan. As we sat down and studied the enormous, exotic menus before us, I was interested to see fugu, puffer fish, listed; it was ten times as expensive as anything else on the menu.
‘Don’t try it!’ said John, adamantly. ‘You have a one in two hundred chance of being poisoned – the chefs are highly trained, but sometimes they make a mistake, leave a trace of skin or viscera on the fish. People like to play Russian roulette with the stuff, but I think there are better ways to die. Tetrodotoxin – a ghastly way to go!’
On Guam, John continued, warming to his theme, the most common form of toxic seafood illness was ciguatera poisoning – ’It’s so common here, we just call it fish poisoning.’ Ciguatoxin is a powerful neurotoxin produced by a tiny organism, a dinoflagellate called Gambierdiscus toxicus, which lives among the algae that grow in channels on the coral reefs. Herbivorous fish feed on the algae, and carnivorous fish in turn feed on them, so the toxin accumulates in large, predatory fish like snapper, grouper, surgeonfish, and jack (all of which I saw on the menu). The ciguatoxin causes no illness in fish – they seem to thrive on it – but it is very dangerous to mammals, and to man. John is something of an expert on this. ‘I first saw it when I was working in the Marshall Islands twenty years ago – a fourteen-year-old boy, who became totally paralyzed, with respiratory paralysis as well, after eating a grouper. I saw hundreds of cases in those days. There were fifty-five different species of fish we found which could carry the ciguatoxin. There is no way a fisherman can tell whether a particular fish is toxic, and no way of preparing or cooking it that will deactivate the toxin.
‘At one point,’ he added, ‘people wondered if the lytico might be caused by some similar kind of fish poisoning – but we’ve never found any evidence of this.’
Thinking of the delectable sushi I had looked forward to all day, I was conscious of a horripilation rippling up my spine. ‘I’ll have chicken teriyaki, maybe an avocado roll – no fish today,’ I said.
‘A wise choice, Oliver,’ said John. ‘I’ll have the same.’
We had just started eating when the lights went out. A groan – ’Not again!’ – went around the restaurant, and the waiters quickly produced candles, which they lit. ‘They seem very well prepared for power outages,’ I said.
‘Sure,’ said John, ‘we have them all the time, Oliver. They’re caused by the snakes.’
‘What?’ I said. Did I mishear? Was he mad? I was startled, and for an instant wondered if he had somehow eaten some poison fish after all, and was beginning to hallucinate.
‘Sounds odd, doesn’t it? We have millions of these brown, tree-climbing snakes everywhere – the whole island is overrun by them. They climb the telephone poles, get into the substations, through the ducts, into the transformers, and then, pfft! We have another outage. The blackouts can happen two or three times a day, and so everyone is prepared for them – we call them snakeouts. Of course, the actual times are quite unpredictable.
‘How have you been sleeping?’ he added, inconsequentially.
‘Rather well,’ I said. ‘Better than usual. At home, I tend to be woken by the birds at dawn.’
‘And here?’ John prompted.
‘Well, now you mention it, I haven’t heard any birds at dawn. Or any other time, It’s strange; I hadn’t realized it until you asked.’
‘There is no birdsong on Guam – the island is silent,’ John said. ‘We used to have many birds, but all of them are gone – there is not a single one left. All of them have been eaten by the tree-climbing snakes.’ John had a prankish sense of humor, and I was not quite sure whether to believe this story. But when I got back to my hotel that evening, and pulled out my trusty Micronesia Handbook, I found confirmation of all that he had said. The tree-climbing snake had made its way to Guam in the hold of a navy ship toward the end of the Second World War and, finding little competition among the native fauna, had rapidly multiplied. The snakes were nocturnal, I read, and could reach six feet in length, ‘but are no danger to adults as their fangs are far back in their jaws.’ They did, however, feed on all manner of small mammals, birds, and eggs; it was this which had led to the extinction of all the birds on Guam, including a number of species unique to the island. The remaining Guam fruit bats are now in danger of vanishing. The electrical outages, I read, cost millions of dollars in damages each year.[66]
The next morning I had arranged to spend some time hunting for ferns in the Guamanian jungle. I had heard of Lynn Raulerson, a botanist, from my friends at the American Fern Society in New York. She and another colleague, Agnes Rine-hart, both work at the herbarium at the University of Guam and had published, among other things, a delightful book on Ferns and Orchids of the Mariana Islands (its frontispiece, a representation of the life cycle of a fern, was drawn by Alma). I met Lynn at the university, and we set off for the jungle, accompanied by one of her students, Alex, who was equipped with a machete. Alex remarked on the denseness of the forest in places. ‘You can still get completely lost, even with a good sense of direction,’ he said. ‘You go five yards in, and it’s so thick, you’re already dislocated.’
The road itself was soon surrounded by an ocean of very large, bright-green sword ferns. Hundreds, thousands, of them pointed straight up into the air, almost as far as we could see. Nephrolepis biserrata, at least the variety we saw, is not your ordinary, humble sword-fern, but a species indigenous to the Marianas, with huge fronds sometimes as much as ten feet long. Once we had waded through these, we were into the jungle, with its great pandanus and ficus trees, and a canopy so dense it closed over our heads. It was a jungle as rich, as green, as any I had ever seen, the trunks of every tree blanketed with a dozen epiphytes, every available inch crowded with plants. Alex walked a few yards ahead of us, clearing a path with his machete. We saw huge bird’s-nest ferns – the Chamorros, Alex told us, call them galak – and a smaller ‘bird’s-nest’ fern which looked like a close relative, but was actually, Lynn told me, a different genus, a Polypodium indigenous to the Marianas.
I was delighted to see ferns of all shapes and sizes, from the lacy, triangular fronds of Davallia, and bristly Pyrrosia sheathing the trunks of the pandanus, to the gleaming shoestring fern, Vittaria, which seemed to hang everywhere. In moist, protected areas we saw a filmy fern, Trichomanes, which excited me, not just because of its delicacy and beauty, but because Safford, in an uncharacteristic error, had written that there were no filmy ferns on Guam (there are actually three species, said Lynn). We came upon the rare Ophioglossum pendulum, an immense ribbon fern with great succulent fronds, rippling and forking as they descended from the crotch of a tree.[67] I had never seen this species before, and even Lynn was excited to find it. We took pictures of it, with ourselves standing by – as one might photograph oneself with a marlin one had caught, or a tiger. But we were careful not to disturb the plant – and glad to think that our path to it would close itself up within days.
‘There is one more fern, over here,’ said Lynn, ‘you’ll want to see. Take a look at this fellow, with its two different types of leaves. The divided fronds are the fertile ones; the spearlike ones are sterile. Its name is Humata heterophylla, and it is named after Umatac (or Humatag) where it was found in the 1790s, by the first botanical expedition to Guam – you might call it the national fern of Guam.’
John and I made some more housecalls in the afternoon. We drove to the village of Yona, and stopped at the first house, where John’s patient, Jesus, was sitting on the porch; now that he had become almost petrified with the bodig, this was where he loved, above all, to sit all day. I was told he had ‘man-man’ – the Chamorro word for staring blankly into space – though this was not a blank staring, a staring at nothing, but an almost painfully engrossed, wistful staring, staring out at the children who played in the road, staring at the occasional passing cars and carts, staring at the neighbors leaving for work each morning, and returning late in the day. Jesus sat on his porch, unblinking, unmoving, motionless as a tortoise, from sunrise till midnight (except on the rare days when high winds or rain lashed across it), forever gazing at a constantly varying spectacle of life before him, an enraptured spectator, no longer able to take part.[68] I was reminded of a description of the aged Ibsen after his stroke, aphasic, partly paralyzed, no longer able to go out or write or talk – but insistent, always, that he be allowed to stand by the tall windows in his room, looking out on the harbor, the streets, the vivid spectacle of the city. ‘I see everything,’ he had once murmured, years before, to a young colleague; and there was still this passion to see, to be an observer, when all else was gone. So it was, it seemed to me, with old Jesus on his porch.
When John and I greeted Jesus, he answered in a small, flat voice, devoid of inflection or intonation, but his answers were precise and full of detail. He spoke of Agana, where he had been born in 1913, and how pleasant and tranquil it was then (‘Not like now – it’s completely changed since the war’), of coming to Umatac with his parents when he was eight, and of a long life devoted to fishing and farming. He spoke of his wife, who had been half Japanese and half Chamorro; she had died of bodig fifteen years ago. Many people in her family had lytico or bodig; but his own children and grandchildren, fortunately, seemed free of it.
We had been told that Jesus might pass the whole day with scarcely a word. And yet he spoke well, even volubly, when we engaged him in conversation; though, it soon became apparent, he waited for our questions. He could respond quite readily, but could not initiate a sentence. Nor, it seemed, a movement either – he might sit totally motionless for hours, unless something or someone called him to move. I was again strongly reminded of my post-encephalitic patients and how they were crucially dependent on the initiative of others, calling them to speech or action. I tore a page out of my notebook, balled it up, and threw the balled paper at Jesus. He had been sitting, seemingly incapable of movement, but now his arm shot up in a flash, and he caught the paper ball precisely. One of his little grandsons was standing by, and his eyes widened with astonishment when he saw this. I continued playing ball, and then asked Jesus to throw the ball at his grandson, and then to another child, and another. Soon we had the entire family playing ball, and akinetic Jesus, no longer akinetic, kept it going between us all. The children had not realized that their ‘paralyzed’ grandfather could move by himself at all, much less that he could catch a ball, aim it accurately, bluff, throw it in different styles and directions, and improvise a fast ball game among them.
For his grandchildren it was a discovery, and one, I thought, which might transform his relationship with them – but this calling-into-action was well known to his old friends in the community. Once a week, he would go to the senior center – he would have to be picked up and lifted (‘like a corpse,’ he said) into the car; but once there, and seated at a card table, he could play a fast and hard game of gin rummy. He could not start the play – someone else had to do this – but once the first card was slapped down, he would suddenly come to life, respond, pick up another card, and continue the game. The people of Umatac, Merizo, Dededo, and Santa Rita may have little scientific knowledge of parkinsonism, but they have a great deal of informal knowledge, a folk neurology based on decades of close observation of the bodig in their midst. They know well how to unfreeze or unlock patients if they get frozen, by initiating speech or action for them – this may require another person walking with the patient or the rhythmic pulse of music. They know how patterns on the floor or the ground can help the parkinsonian to organize his walking; how patients scarcely able to walk on a flat surface can negotiate complex obstacles, rough terrain, easily (and indeed, fare oddly well with these); how the mute and motionless parkinsonian can respond beautifully to music, singing and dancing, when speech and motion had previously seemed impossible.
But what was it that had caused the lytico-bodig, what was it that had come and gone? There had been a sort of conceptual vacuum, John said, when the cycad hypothesis had collapsed in the early seventies. The disease continued to claim more Chamorros, and patients were treated, when possible, for their symptoms – but there was a marked lull in research for a while, at least in Guam.
And yet in the seventies there was a discovery of great importance. Two pathologists, Frank Anderson and Leung Chen, performed autopsies on two hundred Chamorros, many of whom had died suddenly in traffic accidents. (Agana had been a small, slow-moving town before the war, and transport was leisurely – usually by carts pulled by the big-horned carabao, along the rutted and frequently flooded roads. But following the war, there was a sudden increase in population, especially American military, who brought along with them fast roads and cars; this caused a sudden rise in traffic fatalities among the Chamorros, who were wholly unused to this rapid pace.) None of these people had ever shown any neurological symptoms; yet seventy percent of those born before 1940 showed clear pathological changes in the nervous system similar to the neurofibrillary tangles which Hirano had found in patients with lytico-bodig. The occurrence of these neurofibrillary tangles fell off sharply in those born in the 1940s, and they were not seen at all in anyone born after 1952. This extraordinary finding suggested that the lytico-bodig might have been almost universal among the Chamorros at one time – even though only a small proportion went on to develop overt neurological symptoms. It suggested, moreover, that the risk of contracting the disease was now very much reduced – and that even though cases continued to occur, these had probably been contracted many years before, and were only now becoming symptomatic. ‘What we are now seeing, Oliver,’ said John, pounding the steering wheel for emphasis, ‘are the late effects of something that happened long ago.’[69]
When Yoshiro Yase, an ardent sport-fisherman as well as a neurologist, went to study the newly identified disease focus on the Kii Peninsula, he was told there were scarcely any fish in the local rivers, and this prompted him – memories of the Minamata tragedy still being vivid – to analyze their waters. Though these were free of infectious agents or toxins, they were oddly low in calcium and magnesium. Could this, he wondered, be the cause of the disease?
Gajdusek was fascinated by Yase’s findings, the more so as he had been struck by the red soil, rich in iron and bauxite, in the swamp lands around the Auyu and Jakai villages. When he was able to return in 1974 – Western New Guinea having become Irian Jaya in the intervening upheavals – he now tested the water from the shallow wells which the villagers dug in the red soil, and found unusually low levels of calcium and magnesium, as well as elevated levels of iron, aluminum, and other metals.
At this point, Kurland moved to the Mayo Clinic to pursue other research, feeling that the cycad hypothesis, though valid, could not be proven. His place at the NIH was taken by Gajdusek, who was now intrigued and excited at the notion of a mineral etiology of the Western Pacific disease. Gajdusek enlisted Yase, and together they examined well water from Guam and found that this too was low in calcium and magnesium. This triple coincidence seemed definitive:
Comparison of the Western New Guinea focus with the foci of ALS and Parkinson’s disease on Guam and the Kii Peninsula of Japan is inescapable [Gajdusek wrote], and the close association of parkinsonism and motor neuron symptoms in yet another non-Chamorro population group should not only dispel most doubts about the probably close relationship between the two syndromes, but also point to an aetiologic role of some unknown environmental factor.
The unknown environmental factor, it seemed likely, had to do with low calcium and magnesium levels in the drinking water, and the consequences of these on the nervous system. Such low levels, he speculated, might trigger a compensatory reaction in the parathyroid glands, leading in turn to excessive absorption of calcium, aluminum, and manganese ions. The deposition of these in the nervous system, he felt, might result in the premature neuronal aging and death seen with the lytico-bodig.
It was John’s hope, in 1983, that he might join Gajdusek’s team and help crack the disease at last. But Gajdusek told him he was too late – the cause of the lytico-bodig had now been established, and in any case the disease had almost vanished, because of the shift to a Western diet, which was high in calcium – there was not much left to do, and his team would be pulling out of there soon. John was surprised to hear Gajdusek express himself so forcefully, he told me, and disappointed, because he had hoped to work with him. But he decided to come to Guam nonetheless, if only to take care of patients as a physician, and not as an investigator.
But the very day after John arrived on Guam, he had an experience comparable to Zimmerman’s nearly forty years before: working in the naval hospital in Agana, he saw a dozen patients with the lytico-bodig in his first clinic. And one of them also had a supranuclear palsy – a complex disturbance of gaze, in which the patient can look sideways, but not up or down. This had never previously been reported in lytico-bodig, but it was the hallmark of the syndrome John and his Toronto colleagues had delineated nearly twenty years before. This convinced him that lytico-bodig was neither extinct nor comprehensively described, and that there was still time and opportunity for its further investigation.
Guam had superb medical facilities on the naval base, but in the outlying villages, basic medical care was very inadequate, and neurological care scarce – there was only one overworked neurologist, Dr. Kwang-Ming Chen, to care for 50,000 Chamor-ros, and 100,000 other residents of the island as well. Not only were there still many hundreds of Chamorro people with lytico-bodig, Chen told John, but new cases kept appearing – several dozen a year, he thought, and these new cases sometimes took forms different from either the classic lytico or bodig; the man with supranuclear palsy was a case in point.
In particular, John observed, he began to see increasing numbers of elderly people, women especially, who had severe memory disturbances, amnesic syndromes, without any dementia; catatonia without parkinsonism (like Estella); dementia without parkinsonism (like her sister-in-law); arousal disorders (like Euphrasia); or unclassifiable syndromes (like Juan’s), novel forms of the disease never described before.
John was still excited by the mineral hypothesis, and he wanted to pursue it, to gather more conclusive evidence. He invited an old friend and colleague from Toronto, Donald Crapper McLachlan (a neurologist and chemist who had shown elevated levels of aluminum in Alzheimer’s brains as far back as 1973), to join him on Guam, and working with colleagues from the University of Guam, they compared soil samples from Umatac with soil from fifty-five other sites on Guam and reexamined mineral levels in samples of well water all over the island.
Their results, to their surprise, differed greatly from Gaj-dusek and Yase’s – indeed it seemed that the one water source in Umatac, the Piga spring, which the early investigators had found to have low calcium, was quite atypical. Every other water source and all the soils they sampled had high levels of calcium, as might be expected on a limestone island. Further analysis of the soils and of vegetables grown in them found adequate levels of calcium and magnesium and normal levels of aluminum, which seemed to shake the notion of a mineral deficiency or aluminum excess as the cause of lytico-bodig (without, however, excluding it completely).
John is of a passionate disposition and tends to get strongly invested in theories and ideas. He had a huge respect for Gaj-dusek’s intuition, and was greatly taken by the mineral hypothesis; John had hoped to confirm, and perhaps elucidate, this with his own investigations. He had been elated by these hopes, and the promise of Gajdusek and Yase’s hypothesis – and now, suddenly, all this had collapsed. He was back to where Kurland had been a decade earlier, in a conceptual void.
Then, in 1986, his eye was caught by a letter in the Lancet which resurrected the cycad hypothesis. Peter Spencer, a neurotoxicologist, using a purified form of the amino acid BMAA from cycad seeds, found that it could induce a neurological syndrome in monkeys, conceivably analogous to human lytico.
Spencer’s work in this realm went back to the 1970s, when, with his colleague Herb Schaumburg, he had travelled to India to investigate the neurolathyrism there. It had been known for centuries that a spastic paralysis of the legs could follow continued eating of the chickling pea; that this was due to the neurotoxic amino acid BOAA, which damaged the cortical motor cells and their descending connections in the spinal cord, had been known since the 1960s. Spencer’s new studies made clear how BOAA heightened sensitivity to glutamate, one of the neurotransmitters involved in the motor system, and simulated its action as well. BOAA intoxication, therefore, could push the glutamate receptor cells into a sort of overdrive, until they literally died of overexcitation and exhaustion. BOAA was an ex-citotoxin – this was the new term. Could BMAA, he wondered, so similar in structure to BOAA, also act as an excitotoxin and produce a disorder like lytico?
There had been attempts to induce such disorders in animal experiments during the sixties, but the results were inconclusive, and this line of research had been dropped. Now, using cynomolgus monkeys and repeated administrations of BMAA, Spencer succeeded, after eight weeks, in inducing ‘a degenerative motor system disease’ associated with damage to the motor cells in the cerebral cortex and spinal cord.[70] He further observed that BMAA might have two distinct effects: given in high doses, it caused an ALS-like condition to develop rapidly; but smaller doses seemed to cause, after a considerably longer period, a parkinsonian condition – a double action reminiscent of the Guam disease.
These results seemed to refute the first criticism made in the 1960s of the cycad hypothesis – that there existed no animal model. Now Spencer, with a characteristic burst of energy, set about refuting the other, seemingly lethal criticism of the cycad hypothesis – that there were no cycads in the Kii Peninsula or Irian Jaya. Like Gajdusek had before him, he trekked into the jungles of Irian Jaya with his colleague Valerie Palmer – and they discovered that there were cycads here (though they seemed to be a different species from the Guam one). They found, moreover, that cycads were treated as veritable medicine cabinets by the local people, who used raw seeds (like the Chamorros) as poultices on open wounds. On the Kii peninsula, they went on to discover, cycads were also used medicinally, as tonics. With these two discoveries, in the lab and the field, the cycad hypothesis, discarded fifteen years earlier, was now revivified.
John could not contain his excitement at these new thoughts and findings – everything seemed to fit together perfectly. He would phone Spencer in New York, and the two would have excited conversations for hours, sometimes nightly, discussing clinical data, and bringing out more and more ‘coincidences’ of cycads and disease in the Marianas. With his colleague Tomasa Guzman, John now embarked on reexamining the whole question of cycad distribution and use in the Marianas. They observed that while lytico-bodig was common among the Chamorros on Guam and Rota, where cycads were plentiful, there was no lytico-bodig reported on the island of Saipan (at least none in the previous seventy years – it remained uncertain whether the Saipanese Chamorros had been prone to it before this).[71] But they pointed out that the cycad forests of Saipan had been cut down by the Japanese in 1914, to clear land for sugar cane, and that the use of fadang had ceased soon after this. And that on lytico-bodig – free Tinian, where there were forests of cycads, the Chamorros had never made use of them. They proposed that the family clusters of disease found on Guam, which did not follow any known genetic distribution, could be related to differences in the way each family prepared their fadang – some family recipes involved soaking the seeds overnight, some for three weeks; some would use seawater, some fresh; some would shorten the washing process so that the flour would have a stronger taste. Steele and Guzman ended their paper with some striking accounts of people who had developed lytico-bodig as long as twenty years after a single exposure to fadang.
But many researchers felt, after the first flush of enthusiasm, that the amounts of BMAA Spencer was feeding his monkeys were completely unphysiological – more than the most devoted fadang eater could consume in a lifetime. Indeed, Gajdusek calculated, to reproduce Spencer’s experiment in a human being, the subject would have to eat a ton and a half of unprocessed cycad seeds in twelve weeks. This in itself was not an annihilating criticism – experimental toxicology often uses massive doses of materials in its initial experiments in order to increase the chance of getting results within a reasonable time. But now John, knowing how meticulously the seeds were detoxified before the production of fadang, set about measuring the amount of BMAA the flour actually contained; he started sending samples out for analysis, and was surprised to find that many of these had very low levels of BMAA, and some almost none at all. With this he turned against the cycad hypothesis, which had so exercised him for more than three years – turned against it with the vehemence with which he had once espoused it.
Gajdusek and his group, meanwhile, had also been trying to produce an animal model for lytico-bodig and had been maintaining a number of macaques on a low-calcium, high-aluminum diet. The monkeys developed no clinical symptoms in the four years of the trial, but autopsies showed many neurofibrillary tangles, as well as degenerative changes in the motor neurons, throughout the neuraxis. These changes seemed to resemble those of lytico-bodig or the presymptomatic changes described by Anderson and Chen, and it was speculated that a longer period of calcium deficiency, or higher doses of toxic metals, might have led to overt clinical disease. And though Gajdusek had told John in 1983 that he thought the lytico-bodig was dying out in Guam, he has continued to investigate it in Irian Jaya, where in 1993 he found it still had a remarkably high incidence. He and his colleagues continue to see aluminum neurotoxicity as the cause of lytico-bodig and indeed of a wide range of other conditions.
While Spencer, for his part, was greatly encouraged by his own success in inducing neurological disorders in primates with BMAA, he soon developed reservations. The disorders shown by his monkeys were dose-related, came on promptly, and were acute and nonprogressive (they resembled, in this way, the neu-rocycadism of cattle); whereas human lytico-bodig, it was abundantly clear, had a very long latency or incubation period, but once it had become symptomatic, was almost invariably progressive. Was it possible, Spencer speculated, that another factor was involved besides the BMAA, which might not predispose to overt disorder for many years? Slow viruses had been described by Gajdusek; could there not be, analogously, a slow toxin? Spencer did not have any clear idea, at this stage, of how such a toxin might work, or any way of validating the concept.
Though Gajdusek might have been expected to be sympathetic to the idea of a slow toxin, he argued passionately against it in a sternly titled paper, ‘Cycad Toxicity Not the Cause of High-Incidence ALS⁄Parkinsonism-Dementia on Guam, Kii Peninsula of Japan, or in West New Guinea,’ asserting that such a hypothesis was, first, redundant; second, without precedent; third, without support; and fourth, impossible:
No neurotoxin has been demonstrated to give rise to fatal central nervous system disease, neurological signs and symptoms of which first start to be detectable years after exposure to the neurotoxin has ceased. In fact, we have no example of any toxin producing progressive damage to any organ years after last exposure to the substance…Only hypersensitivity disorders, slow infections, and genetically-timed disorders have given rise to this pattern of long delay.
Spencer, undeterred, saw Gajdusek’s words as a challenge (indeed he has cited them in several of his own papers), and continued to see his task as the search for a new kind of toxin, a new kind of toxic mechanism, hitherto unrecognized in medicine. A great deal of attention was focused, in the sixties and seventies, on carcinogenesis, the appearance of cancers, in some cases, years after an initial exposure to the carcinogen, whether radioactivity, a toxin, or a virus. It had been established, in Kur-land’s original cycad conferences, what a potent carcinogen cycasin was, capable of inducing liver cancers and colon and kidney malformations. It had been observed, moreover, that if infant rats were fed cycasin-high diets, the still-dividing Purkinje cells of the cerebellum might develop bizarre multinucleated forms and ectopic ‘nests,’ and such findings had also been reported, on occasion, in cases of human lytico-bodig.
What then might be the effect of cycasin, Spencer wondered, on adult nerve cells, which are no longer capable of dividing?
He and Glen Kisby have postulated recently that cycasin (or its component, MAM, or methazoxymethanol) may be able to form stable compounds with the DNA in nerve cells (such adduct formation is believed to underlie the overt carcinogenic and teratogenic effects of cycasin elsewhere in the body). This aberrant DNA in the nerve cells, he thinks, could lead to subtly but persistently altered metabolic functions, the nerve cells finally becoming oversensitive to their own neurotransmitters, their own glutamate, so that this itself could act as an excitotoxin. No external agent would be needed to provoke a neurological disaster at this point, for in this pathologically sensitized state, even normal neural functioning would now overexcite neurotransmitter receptor cells and push them toward their own destruction.
The notion of such a gene toxin is not as outlandish as it seemed a decade ago, and Spencer and Kisby have now observed DNA changes in tissue cultures of cells exposed to cycasin which suggest that such a mechanism may be at work in lytico-bodig. Such a gene toxin would actually alter the genetic character of the nerve cells it affected, producing, in effect, a genetically-based form of hypersensitivity disorder.
Now that Spencer was pondering the possible effects of cycasin on adult nerve cells, he had new analyses made of traditionally prepared cycad flours and found (contrary to what John had found earlier) that the Guam samples, even though low in BMAA, contained substantial amounts of cycasin. The highest levels of cycasin, indeed, were found in samples from villages with the greatest prevalence of lytico-bodig, lending strong circumstantial support to the hypothesis of cycasin toxicity.[72]
John is a very vivid storyteller, and as he told me this story – a story not just of a scientific odyssey, but of his own most passionate hopes and disappointments – he seemed to relive it with almost unbearable intensity. He had enjoyed a cordial relationship with Kurland and Gajdusek, he thought, and a passionate one with Spencer – but when, in 1990, he gave up on the cycad hypothesis (as four years earlier he had given up on the mineral hypothesis), a sense of intense isolation gripped him; he felt he was out in the cold, seen as an apostate by them all. In the early 1990s, he toyed with a viral hypothesis (this was very much in his mind when we first met, in 1993). But as a primary physician, a general practitioner, living and working amidst the whole affected community in Umatac, he has been forced to think in terms of the entire families or clans with lytico-bodig under his care – no external cause alone, it seemed clear, could adequately account for such a pattern of distribution. Had a genetic theory been thrown out prematurely? Much had changed since Kurland and Mulder first considered, then rejected, this in the 1950s. The classic Mendelian patterns of inheritance had now been joined by concepts of complex inheritance involving the presence of multiple genetic abnormalities and their interactions with each other and with environmental factors. Further, it was now possible to directly examine the genetic material with molecular biology, using technologies and concepts not available to the early investigators.
Working with Verena Keck, an anthropologist, John started to collect pedigrees of every patient he had seen – pedigrees of unprecedented accuracy and detail, including medical histories going back fifty years. The more pedigrees he obtained, the more he became convinced that there had to be some genetic predisposition, or perhaps several predispositions – for it looked as though the lytico and the bodig had different patterns in different families. Sometimes one saw a family in whom the affected members had only the lytico, sometimes a family in which the clinical expression was always bodig, and sometimes, more rarely, a family with both. The similarity of the pathological pictures in lytico and bodig, he started to feel, might have been misleading them all; genealogically, they seemed to be two separate diseases.
Recently John has embarked on a new series of studies, collecting DNA samples from all of his patients and sending them out for genetic analysis. He has been very excited by preliminary results indicating the presence of a genetic marker in several cases of bodig – a marker which seems to be absent in lytico and normal controls. His immediate reaction has been one of exuberance: ‘I feel the excitement coming again, and it’s a feeling I have not had since ‘86, when I was captivated by Spencer’s hypothesis.’ But it is an exuberance tempered by considerable caution (‘I don’t quite know what it means’). The search for genetic markers is extraordinarily laborious and difficult – it took more than a decade of incessant work to find a marker for Huntington’s chorea – and John is not sure whether these preliminary results will be borne out. (And even if a clear genetic basis is established for lytico and for bodig, John feels, this will indicate no more than a vulnerability or disposition; he has never doubted that some external agent is also necessary.)
It is now a third of a century since he and his colleagues delineated progressive supranuclear palsy in the early 1960s and perceived it as a unique yet exemplary disease which might shed some light on neurodegenerative disease in general. The similarity of the clinical picture of lytico-bodig and postencephalitic parkinsonism to PSP continues to intrigue him. He had been struck from the start by the fact that supranuclear palsies could also be observed in some patients with lytico-bodig, and on occasion in those with post-encephalitic syndromes too (on a recent trip to New York, he was intrigued to meet one of my post-encephalitic patients who has had a supranuclear palsy for more than thirty years). But he is not yet sure how to interpret these affinities.
He has been fascinated, as well, by the similarities of the neurofibrillary tangles which are so characteristic of lytico-bodig, post-encephalitic parkinsonism, and PSP with those of classic Alzheimer’s disease and has been investigating this with Patrick McGeer, a neuropathologist in Vancouver. The tangles themselves are virtually identical, as are the areas of inflammatory reaction about them (though there are other features of Alzheimer’s, most notably the presence of so-called ‘plaques,’ which one does not see at all in the other three diseases). At an immediate and practical level, the presence of these inflammatory reactions around the tangles makes him wonder whether anti-inflammatory agents can be helpful in lytico-bodig. Their use in Alzheimer’s disease is under study, and John is eager to see if they can help his own patients, if only to retard the course of a fatal disease. This is one of the few thoughts which gives him a brief sense of therapeutic optimism or hope, as he does his daily rounds among chronically ill and ever-deteriorating patients. And he is concerned by the steadily rising incidence of classic Alzheimer’s and Parkinson’s disease – which rarely if ever occurred on Guam before the Second World War – even as the native disease, the lytico-bodig, declines.
After forty years of research, then, we have four (or more) seemingly divergent lines of thought and research – genetic, cycadic, mineral, viral (Alma’s money is on prions) – each with some support, but with no overwhelming evidence for any of them. The answer will not be a simple one, John now feels, but a complex interaction of a variety of genetic and environmental factors, as seems to be the case in many diseases.[73]
Or perhaps it is something else, as Ulla Craig, one of John’s research colleagues, muses. ‘I’m not sure what we are looking for – though, like John, I have the feeling of some sort of virus that came and went. Some mutant virus, perhaps, with no immediate effect, but affecting people later, as their immune systems responded. But I am not sure. I am afraid we are missing something – this is the value of a fresh mind, seeing things in a new way, someone who may ask the question we have not asked. We are looking now for something complex, but it could be something we have overlooked, something very simple.’
‘Back in the 1940s and ‘50s,’ John mused, ‘there was a sense that we would find the cause of lytico-bodig in a matter of months. When Donald Mulder came here in ‘53, he thought he might have the problem solved by the time Kurland arrived six weeks later – but after forty-five years, it remains a complete enigma. Sometimes I wonder if we will ever decipher it. But time is running out: the disease may vanish before we can understand it… This disease has become my passion, Oliver, and my identity.’ If it is John’s passion and identity, it is Kurland’s, and Spencer’s, and many others’ as well. A colleague of mine, who knows and respects them all, says, ‘Guam has been a tar baby for all of them – once they get stuck, they can never let go.’
The disease is indeed dying out at last, and the researchers who seek its cause grow more pressured, more vexed, by the day: Will the quarry, hotly pursued for forty years now, with all the resources that science can bring, elude them finally, tantalizingly, by disappearing at the moment they are about to grasp it?
‘We’re on our way to see Felipe,’ John said as we climbed into his car once more. ‘You’ll like him, he’s a very sweet man. And he’s been touched by at least four different forms of lytico-bodig.’ He shook his head slowly.
Felipe was sitting on the patio at the back of his house, as he does most days, staring out, with a faint fixed smile, at his garden. It was a lovely garden, full of native plants, and the patio itself was shaded by banana trees. He has spent most of his life in Umatac, fishing and farming. He raises cockerels, and has a dozen of them, gorgeously colored, and all very tame. My neurological examination of Felipe was punctuated by the crowings of cockerels, a sound which he imitated, very loudly, to perfection (this was in striking contrast to his poor vocal volume when talking), by their perching on both of us during the exam, and by the affectionate nuzzling and occasional barking of his black dog. This was all delightful, I thought – rustic neurology, rural neurology, in the backwoods of Guam.
Felipe spoke movingly of his life and the past. He enjoyed fadang occasionally (‘we all did’), but he was not, like many other Chamorros, forced to subsist on it during the war. On the contrary, he spent the war as a sailor with the U.S. Navy, stationed part of the time in Portsmouth, Virginia (hence his excellent English), and he was part of the Navy force which retook Guam. He himself had to take part in the bombardment of Agana, a heartbreaking business, for it was the destruction of his native town. He spoke movingly of friends and family with lytico-bodig. ‘And now,’ he said, ‘I have it too.’ He said this quietly, simply, without a hint of self-pity or drama. He is sixty-nine.
His memory, intact for the past, has become severely eroded for recent events. We had in fact passed his house and stopped to say hello the previous day – but he had no memory of this, showed no recognition now we had come to visit again. When John told him the Chamorran version of his name (John Steele translates as ‘Juan Lulac’), he would laugh, repeat it, and forget it within a minute.
Though Felipe had an inability to register current events, to transfer them from short-term to permanent memory, he had no other cognitive deficits – his use of language, his perceptual powers, his powers of judgment, were all fine. His memory problem had worsened, very slowly, for about ten years. Then he had developed some muscular wasting – the thinning of his once thick and powerful farmer’s hands was striking when we examined him. Finally, a couple of years ago, he had developed parkinsonism. It was this, in the end, which had so slowed him down, taken him out of active life, made him a retiree in his garden. When John had examined him last, a few months before, the parkinsonism was entirely confined to one side, but it had progressed apace, and now affected both sides. There was very little tremor, just an overall immobility, a lack of motor initiative. And now, John showed me, there were the beginnings of a gaze palsy too, an indication of yet a fourth form of lytico-bodig. Felipe’s civility, his character, was perfectly preserved despite his disease, along with a sense of rueful insight and humor. When I turned to wave goodbye, Felipe had a cockerel perched on each arm. ‘Come again soon,’ he said, cheerfully. ‘I won’t remember you, so I’ll have the pleasure of meeting you all over again.’
We returned to Umatac, this time stopping at the old graveyard on the hillside above the village. One of John’s neighbors, Benny, who tends the graveyard – he cuts the grass, acts as a sexton in the little church, and as grave digger when needed – showed us around. Benny’s family, John told me, is one of the most afflicted in Umatac and one of the three families which especially caught Kurland’s attention when he came here forty years ago. It was one of his forebears, in fact, at the end of the eighteenth century, who was cursed after stealing some mangoes from the local priest, and told that his family would contract fatal paralyses generation after generation, until the end of time. This, at least, is the story, the myth, in Umatac.
We walked slowly with Benny among the limestone grave markers, the older ones crooked and sunken with time, the newer ones in the shape of simple white crosses, often embellished by plastic statues of the Virgin Mary or photographs of the deceased, some with fresh flowers on them. As Benny led the way, he pointed out individual stones: ‘Here’s Herman, he passed away from it…and my cousin, that one here…another cousin is the one down here. And one of the couple here, the wife, passed away from that…yeah, they all passed away from the lytico-bodig. And up here – my sister’s father-in-law passed from the same disease…my cousin and her dad and mom, the same thing…the mayor’s sister, same problem…got a cousin here passed away too. Yes, here’s another cousin, Juanita, and her dad, they both had it. My uncle Simon, right here – he was the oldest in the family who passed away from the lytico-bodig…and another cousin, he just passed away a couple of months ago. Another uncle, same problem – and the wife, same disease; I forget his first name. I didn’t really know him, he just passed away before I got to know him.’
Benny went on, leading us from one grave to another, continuing his endless, tragic litany – here’s my uncle, here’s my cousin, and his wife; here’s my sister, and here’s my brother…and here (one seemed to hear, intimated in his voice, necessitated by the tragic logic of it all), here too I will lie, among all my family, my community of Umatac, dead of the lytico-bodig, in this graveyard by the sea. Seeing the same names again and again, I felt that the entire graveyard was devoted to lytico-bodig, and that everyone here belonged to a single family, or perhaps two or three interrelated families, which all shared the same curse.
As we walked slowly among the stones, I remembered another graveyard, also by the sea, which I had visited in up-island Martha’s Vineyard. It was a very old one, going back to the end of the seventeenth century, and there I also saw the same names again and again. In Martha’s Vineyard, this was a graveyard of the congenitally deaf; here in Umatac, it was a graveyard of the lytico-bodig.
When I visited Martha’s Vineyard, there were no longer any deaf people left – the last had died in 1952 – and with this, the strange deaf culture which had been such a part of the island’s history and community for more than two hundred years had come to an end, as such isolates do. So it was with Fuur, the little Danish island of the colorblind; so, most probably, it will be with Pingelap; and so, perhaps, it will be with Guam – odd genetic anomalies, swirls, transients, given a brief possibility, existence, by the nature of islands and isolation. But islands open up, people die or intermarry; genetic attenuation sets in, and the condition disappears. The life of such a genetic disease in an isolate tends to be six or eight generations, two hundred years perhaps, and then it vanishes, as do its memories and traces, lost in the ongoing stream of time.
When I was five, our garden in London was full of ferns, a great jungle of them rising high above my head (though these were all uprooted at the start of the Second World War to make room for Jerusalem artichokes, which we were encouraged to grow for the war effort). My mother and a favorite aunt adored gardening, and were botanically inclined, and some of my earliest memories are of seeing them working side by side in the garden, often pausing to look at the young fronds, the baby fiddle-heads, with great tenderness and delight. The memory of these ferns and of a quiet, idyllic botanizing became associated for me with the sense of childhood, of innocence, of a time before the war.
One of my mother’s heroines, Marie Stopes (a lecturer in fossil botany before she turned to crusading for contraception), had written a book called Ancient Plants, which excited me strangely.[74] For it was here, when she spoke of ‘the seven ages’ of plant life, that I got my first glimpse of deep time, of the millions of years, the hundreds of millions, which separated the most ancient plants from our own. ‘The human mind,’ Stopes wrote, ‘cannot comprehend the significance of vast numbers, of immense space, or of aeons of time’; but her book, illustrating the enormous range of plants which had once lived on the earth – the vast majority long extinct – gave me my first intimation of such eons.[75] I would gaze at the book for hours, skipping over the flowering plants and going straight to the earliest ones – ginkgos, cycads, ferns, lycopods, horsetails. Their very names held magic for me: Bennettitales, Sphenophyllales, I would say to myself, and the words would repeat themselves internally, like a spell, like a mantra.
During the war years, my aunt was headmistress of a school in Cheshire, a ‘fresh-air school,’ as it was called, in the depths of Delamere Forest. It was she who first showed me living horsetails in the woods, growing a foot or two high in the wet ground by the sides of streams. She had me feel their stiff, jointed stems, and told me that they were among the most ancient of living plants – and that their ancestors had grown to gigantic size, forming dense thickets of huge, bamboolike trees, twice as tall as the trees which now surrounded us. They had once covered the earth, hundreds of millions of years ago, when giant amphibians ploshed through the primordial swamps. She would show me how the horsetails were anchored by a network of roots, the pliant rhizomes which sent out runners to each stalk.[76]
Then she would find tiny lycopods to show me – club mosses or tassel ferns with their scaly leaves; these too, she told me, once took the form of immensely tall trees, more than a hundred feet high, with huge scaly trunks supporting tasselled foliage, and cones at their summits. At night I dreamed of these silent, towering giant horsetails and club mosses, the peaceful, swampy landscapes of 350 million years ago, a Paleozoic Eden – and I would wake with a sense of exhilaration, and loss.
I think these dreams, this passion to regain the past, had something to do with being separated from my family and evacuated from London (like thousands of other children) during the war years. But the Eden of lost childhood, childhood imagined, became transformed by some legerdemain of the unconscious to an Eden of the remote past, a magical ‘once,’ rendered wholly benign by the omission, the editing out, of all change, all movement. For there was a peculiar static, pictorial quality in these dreams, with at most a slight wind rustling the trees or rippling the water. They neither evolved nor changed, nothing ever happened in them; they were encapsulated as in amber. Nor was I myself, I think, ever present in these scenes, but gazed on them as one gazes at a diorama. I longed to enter them, to touch the trees, to be part of their world – but they allowed no access, were as shut off as the past.
My aunt often took me to the Natural History Museum in London, where there was a fossil garden full of ancient lycopod trees, Lepidodendra, their trunks covered with rugged rhomboid scales like crocodiles, and the slender trunks of tree horsetails, Calamites. Inside the museum, she took me to see the dioramas of the Paleozoic (they had titles like ‘Life in a Devonian Swamp’) – I loved these even more than the pictures in Marie Stopes’ book, and they became my new dreamscapes. I wanted to see these giant plants alive, straightaway, and felt heartbroken when she told me that there were no more tree horsetails, no more club-moss trees, the old giant flora was all gone, vanished – though much of it, she added, had sunk into the swamps, where it had been compressed and transformed into coal over the eons (once, at home, she split a coal ball and showed me the fossils inside).
Then we would move ahead 100 million years, to the dioramas of the Jurassic (‘The Age of Cycads’), and she would show me these great robust trees, so different from the Paleozoic ones. The cycads had huge cones and massive fronds at their tops – they were the dominant plant form once, she would say; pterodactyls flew among them, they were what the giant dinosaurs munched on. Although I had never seen a living cycad, these great trees with their thick, solid trunks seemed more believable, less alien, than the unimaginable Calamites and Cor-daites which had preceded them – they looked like a cross between ferns and palms.[77]
On summer Sundays, we would take the old District Line to Kew – the line had been opened in 1877, and many of the original electric carriages were still in use. It cost 1d. to enter, and for this one had the whole sweep of the Garden, its broad walks, its dells, the eighteenth-century Pagoda, and my favorites, the great glass and iron conservatories.
A taste for the exotic was fostered by visits to the giant water lily Victoria regia, in its own special house – its vast leaves, my aunt told me, could easily bear the weight of a child. It had been discovered in the wilds of Guyana, she said, and given its name in honor of the young queen.[78]
I was even more taken by the grotesque Weiwitschia mirabilis, with its two long, leathery, writhingly coiled leaves – it looked, to my eyes, like some strange vegetable octopus. Weiwitschia is not easy to grow outside its natural habitat in the Namibian desert, and the large specimen at Kew was one of the few which had been successfully cultivated, a very special treasure. (Joseph Hooker, who named it and obtained the original material from the euphonious Welwitsch, thought it the most interesting, though ugliest, plant ever brought into Britain; and Darwin, fascinated by its mixture of advanced and primitive characteristics, called it ‘the vegetable Ornithorhynchus,’ the platypus of the plant kingdom.)[79]
My aunt especially loved the smaller fern houses, the ferneries. We had ordinary ferns in our garden, but here, for the first time, I saw tree ferns, rearing themselves twenty or thirty feet up in the air, with lacy arching fronds at their summits, their trunks buttressed by thick cably roots – vigorous and alive, and yet hardly different from the ones of the Paleozoic.
And it was at Kew that I finally saw living cycads, clustered as they had been for a century or more in a corner of the great Palm House.[80] They too were survivors from a long-distant past, and the stamp of their ancientness was manifest in every part of them – in their huge cones, their sharp, spiny leaves, their heavy columnar trunks, reinforced like medieval armor, by persistent leaf bases. If the tree ferns had grace, these cycads had grandeur and, to my boyish mind, a sort of moral dimension too. Widespread once, reduced now to a few genera – I could not help thinking of them as both tragic and heroic. Tragic in that they had lost the premodern world they had grown up in: all the plants they were intimately related to – the seed ferns, the Bennettites, the Cordaites of the Paleozoic – had long ago vanished from the earth, and now they found themselves rare, odd, singular, anomalous, in a world of little, noisy, fast-moving animals and fast-growing, brightly colored flowers, out of synch with their own dignified and monumental timescale. But heroic too, in that they had survived the catastrophe which destroyed the dinosaurs, adapted to different climates and conditions (not least to the hegemony of birds and mammals, which the cycads now exploited to disperse their seeds).
The sense of their enduringness, their great phylogenetic age, was amplified for me by the age of some of the individual plants – one, an African Encephalartos longifolius, was said to be the oldest potted plant in Kew and had been brought here in 1775. If these wonders could be grown at Kew, I thought, why should I not grow them at home? When I was twelve (the war had just ended) I took the bus to a nursery in Edmonton, in north London, and bought two plants – a woolly tree fern, a Ci-botium, and a small cycad, a Zamia.[81] I tried to grow them in our little glassed-in conservatory at the back of the house – but the house was too cold, and they withered and died.
When I was older, and first visited Amsterdam, I discovered the beautiful little triangular Hortus Botanicus there – it was very old, and still had a medieval air, an echo of the herb gardens, the monastery gardens, from which botanical gardens had sprung. There was a conservatory which was particularly rich in cycads, including one ancient, gnarled specimen, contorted with age (or perhaps from its confinement in a pot and a small space), which was (also) said to be the oldest potted plant in the world. It was called the Spinoza cycad (though I have no idea whether Spinoza ever saw it), and it had been potted, if the information was reliable, near the middle of the seventeenth century; it vied, in this way, with the ancient cycad at Kew.[82]
But there is an infinite difference between a garden, however grand, and the wild, where one can get a feeling of the actual complexities and dynamics of life, the forces that press to evolution and extinction. And I yearned to see cycads in their own context, not planted, not labelled, not isolated for viewing, but growing side by side with banyans and screw pines and ferns all about them, the whole harmony and complexity of a full-scale cycad jungle – the living reality of my childhood dreamscape.
Rota is Guam’s closest companion in the Marianas chain and is geologically similar, with a complex history of risings and fallings, reef makings and destructions, going back forty million years or so. The two islands are inhabited by similar vegetation and animals – but Rota, lacking Guam’s size, its grand harbors, its commercial and agricultural potential, has been far less modernized. Rota has been largely left to itself, biologically and culturally, and it can perhaps give one some idea of how Guam looked in the sixteenth century, when it was still covered by dense forests of cycads, and this was why I wanted to come here.[83]
I would be meeting one of the island’s few remaining medicine women, Beata Mendiola – John Steele had known her, and her son Tommy, for many years. ‘They know more about cycads, about all the primitive plants and foods and natural medicines and poisons here,’ he said, ‘than anyone I know.’ They met me at the landing strip – Tommy is an engaging, intelligent man in his late twenties or early thirties, fluent in Chamorro and English. Beata, lean, dark, with an aura of power, was born during the Japanese occupation, and speaks Chamorro and Japanese only, so Tommy had to interpret for us.
We drove a few miles down a dirt road to the edge of the jungle and then went on by foot, Tommy and his mother with machetes, leading the way. The jungle was so dense in places that light could hardly filter through, and I had the sense, at times, of a fairy wood, with every tree trunk, every branch, wreathed in epiphytic mosses and ferns.
I had seen only isolated cycads on Guam, perhaps two or three close together – but here there were hundreds, dominating the jungle. They grew everywhere, some in clumps, some as isolated trunks reaching, here and there, twelve or fifteen feet in height. Most, though, were relatively low – five or six feet tall, perhaps – and surrounded by a thick carpet of ferns. Thickened and strengthened with the scars of old leaves, leaf scales, these trunks looked mighty as locomotives or stegosaurs. High winds and typhoons beat through these islands regularly, and the trunks of some of them were bent at all angles, sometimes even prostrate on the ground. But this, if anything, seemed to increase their vitality, for where they were bent, especially at the base, new growths, bulbils, had erupted in scores, bearing their own crowns of young leaves, still pale green and soft. While most of the cycads around us were tall, unbranched ones whose life force seemed to be pouring upward to the sky, there were others, almost monstrous, which seemed to be running riot, exploding in all directions, full of anarchic vitality, sheer vegetable exuberance, hubris.
Beata pointed out the stiff reinforcing leaf bases which ringed each tree trunk – as each new crown of leaves had sprouted at the top, the older leaves had died off, but their bases remained. ‘We can estimate the age of a cycad by counting these leaf scales,’ said Beata. I started to do this, with one huge prostrate tree, but Tommy and Beata smiled as I did so. ‘It is easier,’ she said, ‘if you look at the trunks – many of the older ones have a very thin ring in 1900, because that was the year of the great typhoon; and another thin ring in 1973, when we had very strong winds.’
‘Yeah,’ inserted Tommy, ‘those winds got to two hundred miles per hour, they say.’
‘The typhoon strips all the leaves off the plant,’ Beata explained, ‘so they can’t grow as much as usual.’ Some of the oldest trees, she thought, were more than a thousand years old.[84]
A cycad forest is not lofty, like a pine or oak forest. A cycad forest is low, with short stumpy trees – but the trees give an impression of immense solidity and strength. They are heavy-duty models, one feels – not tall, not flashy, not capable of rapid growth, like modern trees, but built to last, to withstand a typhoon or a drought. Heavy, armored, slow growing, gigantic – they seem to bear, like dinosaurs, the imprint of the Mesozoic, the ‘style’ of 200 million years ago.
Male and female cycads are impossible to tell apart until they mature and produce their spectacular cones. The male Cycas has an enormous upright cone, a foot or more in length and weighing perhaps thirty pounds, like a monstrous pinecone, tessellated, with great chunky cone scales sweeping round the axis of the cone in elegant spiral curves.[85] The female of the Cycas genus, in contrast, lacks a proper cone, but produces a great central cluster of soft woolly leaves instead – megasporophylls, specialized for reproduction – orange in color, velvety, notched; and hanging below each leaf, eight or ten slate-colored ovules – microscopic structures in most organisms, but here the size of juniper berries.
We stopped by one cone, half a yard high, ripe and full of pollen. Tommy shook it, and a cloud of pollen came out; it had a powerful, pungent smell and set me tearing and sneezing. (The cycad woods must be thick with pollen in the windy season, I thought, and some researchers have even wondered whether the lytico-bodig could be caused by inhaling it.) The smell of the male cones is generally rather unpleasant for human beings – as far back as 1795, there were ordinances in Agana requiring inhabitants to remove the cones if they grew male plants in their garden. But, of course, the smell is not for us. Ants are drawn by the powerful smell, said Tommy; sometimes a horde of tiny, biting ones will fly out as the tree is poked. ‘Look!’ he said. ‘See this little spider? We call him paras ranas in Chamorro, ‘the one that weaves the web.’ This type of spider is mostly found on the cycad; it eats the ants. When the cycad is young and green, the spider is green too. When the cycad starts to become brown, the spider takes that color too. I am glad when I see the spiders, because it means there will be no ants to bite me when I pick the fruits.’
Brilliantly colored fungi sprouted in the wet earth – Beata knew them all, which were poisonous, and what remedy to use if poisoned; which were hallucinogenic; which were good to eat. Some of them, Tommy told me, were luminous at night – and this was also true of some of the ferns. Looking down among the ferns, I spotted a low, whisklike plant, Psilotum nudum – inconspicuous, with stiff leafless stems the diameter of a pencil lead, forking every few inches like a miniature tree, bifurcating its way through the undergrowth. I bent down to examine it, and saw that each tiny fractal branch was capped with a yellow three-lobed sporangium no bigger than a pinhead, containing all the spores. Psilotum grows all over Guam and Rota – on riverbanks, in the savannah, around buildings, and often on trees, as an epiphyte drooping like Spanish moss from their branches – and seeing it in its natural habitat gave me a peculiar thrill. No one notices Psilotum, no one collects it, esteems it, respects it – small, plain, leafless, rootless, it has none of the spectacular features which attract collectors. But for me it is one of the most exciting plants in the world, for its ancestors, the psilophytes of the Silurian, were the first plants to develop a vascular system, to free themselves from the need to live in water. From these pioneers had come the club mosses, the ferns, the now-extinct seed ferns, the cycads, the conifers, and the vast range of flowering plants which subsequently spread all over the earth. But this originator, this dawn-plant, still lives on, humbly, inconspicuously coexisting with the innumerable species it has spawned – had Goethe seen it, he would have called this his Ur-pflanze.[86]
If the cycads conjured up for me the lush forests of the Jurassic, a very different, much older vision rose before me with the Psilotum: the bare rocks of the Silurian – a quarter of a billion years earlier, when the seas teemed with great cephalopods and armored fish and eurypterids and trilobites, but the land, apart from a few mosses and lichens, was still uninhabited and empty.[87] Psilophytes, stiff stemmed as no alga had ever been, were among the first colonizers of the bare land. In the dioramas of ‘The First Life on Land’ I so loved as a child, one could see panting lungfish and amphibious tetrapods emerging from the primordial waters, climbing aboard the now-green margins of the land. Psilophytes, and other early land plants, provided the soil, the moisture, the cover, the pasture, without which no animal could have survived on land.
A little farther on, I was startled to see a large accumulation of empty, broken coconut shells on the ground, but when I looked around, there were no coconut palms to be seen, only cycads and pandanus. Filthy tourists, I thought – must have come in and thrown these husks here; but there were few tourists on Rota. It seemed odd that the Chamorros, who are so respectful of the jungle, would leave a pile of refuse here. ‘What is this?’ I asked Tommy. ‘Who brought all these shells here?’
‘Crabs,’ he said. Seeing my confusion, he elaborated. ‘These large coconut crabs come in. The coconut trees are over there.’ He gestured toward the beach, a few hundred yards away, where we could just see a grove of palm trees. ‘The crabs know they will be disturbed if they eat them by the beach, so they bring them over here to eat.’[88]
One shell had a huge hole, as if it had been bitten in half. ‘This must have been a real big crab to do this,’ Tommy observed, ‘a monster! The crab hunters know when they find coconut shells like this that there are coconut crabs all around, and then we search, and then we eat them – I would like to catch the crab that did this!
‘Coconut crabs love the cycads, too. So when I come out to gather the cycad fruit, I bring along a bag for crabs too.’ With his machete, Tommy cut through the undergrowth, making a path. ‘This is good for the cycads – it gives them room to grow.’
‘Feel this cone!’ Tommy said, as we came to a large male plant – I was surprised to find it warm to the touch. ‘It is like a furnace,’ said Tommy. ‘Making the pollen gives it heat – you can really feel it as the day cools, in the evening.’ Botanists have known for about a century (and cycad gatherers, of course, for much longer) that the cones may generate heat – sometimes twenty degrees or more above the ambient temperature – as they ready for pollination. The mature cones produce heat for several hours each day by breaking down lipids and starches within the cone scales; it is thought that the heat increases the release of insect-attracting odors, and thus helps in the distribution of pollen. Intrigued by the almost-animal warmth of the cone, I hugged it, impulsively, and almost vanished in a huge cloud of pollen.
In his Useful Plants of the Island of Guam Safford has much to say about Cycas circinalis – its role in Chamorro culture, its use as food; but ‘its chief interest,’ he adds (one remembers that he is a botanist here), ‘lies in the structure of its inflorescence and the manner of its fructification.’ At this point he cannot suppress a special enthusiasm and excitement. He describes how the pollen settles on the naked ovules and sends a tube down into them, within which the male germ cells, the spermato-zooids, are produced. The mature spermatozooids are ‘the largest known to occur in any animal or plant. They are even visible to the naked eye.’ He goes on to describe how the spermatozooids, which are motile, powered by cilia, enter the egg cell and fuse with it totally, ‘cytoplasm with cytoplasm, nucleus with nucleus.’
These observations were quite new at the time he was writing; for though cycads had been described by Europeans in the seventeenth century, there had been much confusion as to their origins and place in the vegetable kingdom. It was only the discovery of their motile spermatozooids, by Japanese botanists in 1896, that afforded the first absolutely clear evidence of their kinship (and thus of their whole group, the gymnosperms) with ferns and other ‘lower’ spore-bearing plants (which also have motile spermatozooids). The importance of these discoveries, made only a few years before he wrote, is strong and fresh for Safford, and enriches his account with a feeling of intellectual fervor. Longing to see this visible act of fecundation for myself, I pulled out my hand lens and peered into the male cone, then into the notched ovules, as if the whole drama might be enacted before my eyes.
Tommy and Beata seemed amused by my barmy enthusiasm, and burst out laughing – for them, basically, cycads are food. Their interest is not in the male plant, its pollen, or the giant spermatozooids which are produced within the ovules – these, so far as they are concerned, are just instrumental in getting the female plants fertilized, so that they may bear their great, glossy, plum-sized seeds. These they will gather, and slice, and wash, and wash again, and finally dry and grind to form the finest fadang flour. Like connoisseurs, choosing only the best, Tommy and his mother went from tree to tree – this one was unfertilized, that one unripe, but there was a carpophyll of heavy ripe seeds, a cluster of a dozen or more. Tommy sliced the machete, and caught the cluster as it fell. He poked another cluster, too high to chop, with a stick he was carrying, and asked me to catch the seeds as they fell. I found my fingers covered with sticky white sap. ‘That’s really poisonous,’ said Tommy. ‘Don’t lick your fingers.’
It was not just the reproductive structures of cycads which so fascinated me as a boy, or the sheer gigantism that seemed characteristic of the group (the biggest spermatozooids, the biggest egg cells, the biggest growing apices, the biggest cones, the biggest everything in the vegetable world) – though (I could not deny it) these had a certain appeal. It was rather the sense that cycads were brilliantly adaptable and resourceful life-forms, full of unusual capacities and developments which had enabled them to survive for a quarter of a billion years, when so many of their contemporaries had fallen by the way. (Maybe they had been so poisonous to fend off the dinosaurs which ate them, I used to speculate as a child – maybe they had been responsible for the dinosaurs’ extinction!)
It was true that cycads had the largest growing apices of any vascular plant, but, equally to the point, these delicate apices were beautifully protected by persistent leaf bases, enabling the plants to be fire resistant, everything resistant, to an unusual degree, and to reshoot new fronds, after a catastrophe, sooner than anything else. And if something did nonetheless befall the growing apices, the plants had an alternative, bulbils, which they could fall back on. Cycads could be pollinated by wind – or insects, they were not choosy: they had avoided the path of overspecialization which had done in so many species over the last half-billion years.[89] In the absence of fertilization, they could propagate asexually, by offsets and suckers (there was a suggestion too that some plants were able to spontaneously change sex). Many cycad species had developed unique ‘cor-raloid’ roots, where they symbiosed with blue-green algae, which could fix atmospheric nitrogen for them, rather than relying solely on organic nitrogen from the soil. This struck me as particularly brilliant – and highly adaptive should the seeds fall on impoverished soils; it had taken legumes, flowering plants, another hundred million years to achieve a similar trick.[90]
Cycads had huge seeds, so strongly constructed and so packed with nourishment that they had a very good chance of surviving and germinating. And they could call on not just one but a variety of vectors for their dispersal. All sorts of smaller animals – from bats to birds to marsupials to rodents – attracted by the brightly colored, nutritious outer coat, would carry them off, nibble at them, and then discard the seed proper, the essential inner core, unharmed. Some rodents would squirrel them away, bury them – in effect, plant them – increasing their chances of successful germination. Large mammals might eat the entire seed – monkeys eating individual seeds, elephants entire cones – and void the endosperm, in its tough nut, unharmed in their dung, often in quite far-removed places.
Beata was examining another cycad plant, speaking softly in Chamorro to her son. When the rains come, she was saying, the seeds can float. You can tell where they float to in the jungle, because new cycad plants sprout up all along the little rivers and streams. She thinks they float in the sea as well, and that this is how they get to other islands. As she spoke she split open a seed, and showed me the spongy flotation layer just beneath the seed coat – a feature peculiar to the Marianas cycad and the other littoral species of Cycas, which grow in coastal and near-coastal forests.
Cycads have spread to many different ecoclimes, from the humid tropical zones they flourished in during the Jurassic, to near-deserts, savannahs, mountains, and seashores. It is the littoral species which have achieved the widest distribution, for their seeds can float and travel great distances on ocean currents. One of these species, Cycas thouarsii, has spread from the east coast of Africa to Madagascar, to the Comoros and the Seychelles. The other littoral species, C. circinalis and C. rumphii, seem to have originated in the coastal plains of India and Southeast Asia. From here their seeds, borne on ocean currents, have fanned out across the Pacific, colonizing New Guinea, the Moluccas, Fiji, the Solomon Islands, Palau, Yap, some of the Carolines and Marshalls – and, of course, Guam and Rota. And as the buoyant seeds of the ancestral species have settled on different islands, they have begotten striking variants, some of which have diverged now, in a manner which would have delighted Darwin, to half a dozen new species or more.[91]
Although cycads vary greatly in size and character, from sixty-foot trees to delicate plants with underground rhizomes, many of the sixty-odd species of Cycas do not look that different (as opposed, say, to the species of Zamia, which vary so widely, and wildly, in appearance that one has difficulty believing they all belong to the same genus) – and that one of these species should be mistaken for another is very understandable. Indeed, I had been surprised, after my Guam visit, when I went into a nursery in San Francisco, thinking to buy a Cycas circinalis for a wedding present – and was shown a plant which was clearly different from the Guam one. When I queried the nursery owner, she indignantly insisted that it was a circinalis, and suggested that perhaps what I had seen in Guam was not. It seemed astonishing that there should be such confusion even among plant experts – but David Jones, in his Cycads of the World, speaks of the complexities of identifying the island cycads:
The plants adapt over generations in various small ways to their own particular environmental circumstances and local climate.…The situation is further complicated by new arrivals being regularly carried on ocean currents. On reaching maturity these recent plants can hybridize with existing plants and the resulting complex range of variation may defy taxonomic separation. Thus C. circinalis must be regarded as an extremely variable species.
And indeed, since I returned from Guam, I have learned that the cycad peculiar to Guam and Rota, regarded for centuries as a variety of C. circinalis, has recently been reclassified as a distinct species within the C. rumphii ‘complex,’ and renamed C. micronesica.[92]
C. micronesica, it seems, is distinctive not only morphologically, but chemically and physiologically too – with a notably higher content of carcinogenic and toxic substances (in particular, of cycasin and BMAA) than any other cycad which has been analyzed. Thus cycad eating, relatively benign elsewhere, may be peculiarly dangerous on Guam and Rota – and the Darwinian process which has brought a new species into the world may also, conceivably, be contributing to a new human disease.
I find myself walking softly on the rich undergrowth beneath the trees, not wanting to crack a twig, to crush or disturb anything in the least – for there is such a sense of stillness and peace that the wrong sort of movement, even one’s very presence, might be felt as an intrusion, and, so to speak, anger the woods. Tommy’s words, earlier, came back to me now. ‘All my life,’ he said, ‘I was taught to walk backwards in the jungle, and not to destroy anything…I have the attitude that these plants are alive. They have powers. They can invoke some kind of a disease to you if you do not respect them…’ The beauty of the forest is extraordinary – but ‘beauty’ is too simple a word, for being here is not just an esthetic experience, but one steeped with mystery, and awe.
I would have similar feelings as a child, when I lay beneath the ferns, and later, when I entered through the massive iron gates at Kew – a place which was not just botanical for me, but had an element of the mystical, the religious too. My father once told me that the very word ‘paradise’ meant garden, spelling out for me the four letters (pe resh dalet samech ) of pardes, the Hebrew word for garden. But gardens, Eden or Kew, are not the right metaphors here, for the primeval has nothing to do with the human, but has to do with the ancient, the aboriginal, the beginning of all things. The primeval, the sublime, are much better words here – for they indicate realms remote from the moral or the human, realms which force us to gaze into immense vistas of space and time, where the beginnings and originations of all things lie hidden. Now, as I wandered in the cycad forest on Rota, it seemed as if my senses were actually enlarging, as if a new sense, a time sense, was opening within me, something which might allow me to appreciate millennia or eons as directly as I had experienced seconds or minutes.[93]
I live on an island – City Island in New York – surrounded by the brilliant transient artifacts of man. And yet each June, without fail, horseshoe crabs come up from the sea, crawl on the beach, mate, deposit eggs, and then slowly swim away again. I love to swim in the bay alongside them; they permit this, indifferently. They have crawled up to the shores and mated every summer as their ancestors have done since the Silurian, 400 million years ago. Like the cycads, the horseshoe crabs are rugged models, great survivors which have endured. When he saw the giant tortoises of the Galapagos, Melville wrote (in The Encantadas ):
These mystic creatures…affected me in a manner not easy to unfold. They seemed newly crawled forth from beneath the foundations of the world.…The great feeling inspired by these creatures was that of age – dateless, indefinite endurance.
Such is the feeling inspired, for me, by the horseshoe crabs each June.
The sense of deep time brings a deep peace with it, a detachment from the timescale, the urgencies, of daily life. Seeing these volcanic islands and coral atolls, and wandering, above all, through this cycad forest on Rota, has given me an intimate feeling of the antiquity of the earth, and the slow, continuous processes by which different forms of life evolve and come into being. Standing here in the jungle, I feel part of a larger, calmer identity; I feel a profound sense of being at home, a sort of companionship with the earth.[94]
It is evening now, and as Tommy and Beata go off to gather some medicinal plants, I sit on the beach, looking out to sea. Cycads come down almost to the water’s edge, and the strand is littered with their gigantic seeds, along with the tough egg cases of sharks and rays, which are shaped like bizarre fortune cookies. A light wind has sprung up, rustling the leaves of the cycads, blowing up little ripples on the water. Ghost crabs and fiddler crabs, hidden in the heat of the day, have emerged and are darting to and fro. The chief sound is the lapping of waves on the shore, lapping as they have done for billions of years, ever since land rose out of the water – an ancient, soothing, hypnotic sound.
I look at the cycad seeds curiously, thinking of Beata’s words, how they float and can perhaps survive long immersion in sea-water. Most, no doubt, have dropped from the trees above me, but some, perhaps, are nomads, brought here across the sea from Guam, or more distant islands – perhaps even Yap or Palau, or beyond.
A large wave comes in, lifts a couple of the seeds, and they float, bobbing, by the shore. Five minutes later, one of the seeds has been cast up again on the shore, but the other is still bobbing atop the waves, a few feet from land. I wonder where it will go, whether it will survive, will be cast back here on Rota, or taken hundreds, perhaps thousands, of miles to another island in the Pacific. Ten minutes more and I can no longer see it – it is launched, like a little ship, on its journey on the high seas.