1996 - The Island of the Colorblind (21 page)

It is now a third of a century since he and his colleagues delineated progressive supranuclear palsy in the early 1960
s
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.
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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 1940
s
and ‘50
s
,’ 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.

W
hen 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.
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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.
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1 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.
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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.