1996 - The Island of the Colorblind (13 page)

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 1960
s
, 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 1950
s
 – 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.