1996 - The Island of the Colorblind (12 page)

But
was
Pingelap an island of the colorblind after all, an island of the Wellsian sort I had fantasied or hoped for? Such a place, in the full sense, would have to consist of achromatopes only, and to have been cut off from the rest of the world for generations. This was manifestly not the case with the island of Pingelap or the Pingelapese ghetto of Mand, where the achro-matopes were diffused amid a larger population of color-normals.
44

Yet there was an obvious kinship – not just familial, but perceptual, cognitive – among the achromatopes we met on Pingelap and Pohnpei. There was an immediate understanding and sharing between them, a commonality of language and perception, which instantly extended to Knut as well. And everyone on Pingelap, colorblind or color-normal, knows about the maskun, knows that it is not only colorblindness that those affected must live with, but a painful intolerance of bright light and inability to see fine detail. When a Pingelapese baby starts to squint and turn away from the light, there is at least a cultural knowledge of his perceptual world, his special needs and strengths, even a mythology to explain it. In this sense, then, Pingelap is an island of the colorblind. No one born here with the maskun finds himself wholly isolated or misunderstood, which is the almost universal lot of people with congenital achromatopsia elsewhere in the world.

Knut and I each stopped in Berkeley, separately, on our way back from Pohnpei, to visit our achromatopic correspondent, Frances Futterman, and tell her what we had found on the island of the colorblind. She and Knut were especially excited to meet one another finally; Knut told me later that it was ‘an unforgettable and very stimulating experience – we had so much to talk about and so much to share with each other that we talked incessantly like excited children for several hours.’

Like many achromatopes in our society, Frances grew up with a severe degree of disability, for although her condition was diagnosed relatively early, good visual aids were not available to her, and she was forced to remain indoors as much as possible, avoiding any situation with bright light. She had to contend with a great deal of misunderstanding, and isolation, from her peers. And perhaps most important, she had no contact with others of her kind, with anyone who could share and understand her experience of the world.

Did such isolation have to exist? Could there not be a sort of community of achromatopes who (even though geographically separated) were bound together by commonalities of experience, of knowledge, of sensibility, of perspective? Was it possible that even if there was no actual island of the colorblind, there might be a conceptual or metaphoric one? This was the vision which haunted Frances Futterman and inspired her, in 1993, to start an Achromatopsia Network, publishing monthly newsletters so that achromatopes all over the country – and potentially all over the world – could find each other, communicate, share their thoughts and experiences.

Her network and newsletter – and now a Web site on the Internet – have indeed been very successful, have done much to annul geographical distance and apartness. There are hundreds of members spread around the world – in New Zealand, Wales, Saudi Arabia, Canada, and now in Pohnpei too – and Frances is in contact with them all, by phone, fax, mail, Internet. Perhaps this new network, this island in cyberspace, is the true Island of the Colorblind.

CYCAD ISLAND

I
t 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 1950
s
and ‘60
s
, 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 1960
s
and ‘70
s
, 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 1960
s
, 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.