1996 - The Island of the Colorblind (2 page)

Ordinary colorblindness, arising from a defect in the retinal cells, is almost always partial, and some forms are very common: red-green colorblindness occurs to some degree in one in twenty men (it is much rarer in women). But total congenital colorblindness, or achromatopsia, is surpassingly rare, affecting perhaps only one person in thirty or forty thousand. What, I wondered, would the visual world be like for those born totally colorblind? Would they, perhaps, lacking any sense of something missing, have a world no less dense and vibrant than our own? Might they even have developed heightened perceptions of visual tone and texture and movement and depth, and live in a world in some ways more intense than our own, a world of heightened reality – one that we can only glimpse echoes of in the work of the great black-and-white photographers? Might they indeed see
us
as peculiar, distracted by trivial or irrelevant aspects of the visual world, and insufficiently sensitive to its real visual essence? I could only guess, as I had never met anyone born completely colorblind.

Many of H.G. Wells’ short stories, it seems to me, fantastical as they are, can be seen as metaphors for certain neurological and psychological realities. One of my favorites is ‘The Country of the Blind,’ in which a lost traveller, stumbling into an isolated valley in South America, is struck by the strange ‘parti-coloured’ houses that he sees. The men who built these, he thinks, must have been as blind as bats – and soon he discovers that this
is
the case, and indeed that he has come across an entire blind society. He finds that their blindness is due to a disease contracted three hundred years before, and that over the course of time, the very concept of seeing has vanished:

For fourteen generations these people had been blind and cut off from all the seeing world; the names for all the things of sight had faded and changed…Much of their imagination had shrivelled with their eyes, and they had made for themselves new imaginations with their ever more sensitive ears and finger-tips.

Wells’ traveller is at first contemptuous of the blind, seeing them as pitiful, disabled – but soon the tables are reversed, and he finds that they see
him
as demented, subject to hallucinations produced by the irritable, mobile organs in his face (which the blind, with their atrophied eyes, can conceive only as a source of delusion). When he falls in love with a girl in the valley and wants to stay there and marry her, the elders, after much thought, agree to this, provided he consent to the removal of those irritable organs, his eyes.

Forty years after I first read this story, I read another book, by Nora Ellen Groce, about deafness on the island of Martha’s Vineyard. A sea captain and his brother from Kent, it seems, had settled there in the 1690
s
; both had normal hearing, but both brought with them a recessive gene for deafness. In time, with the isolation of the Vineyard, and the intermarriage of its close community, this gene was carried by the majority of their descendants; by the mid-nineteenth century, in some of the up-island villages, a quarter or more of the inhabitants were born totally deaf.

Hearing people were not so much discriminated against here as assimilated – in this visual culture, everyone in the community, deaf and hearing alike, had come to use sign language. They would chat in Sign (it was much better than spoken language in many ways: for communicating across a distance, for instance, from one fishing boat to another, or for gossiping in church), debate in Sign, teach in Sign, think and dream in Sign. Martha’s Vineyard was an island where everyone spoke sign language, a veritable country of the deaf. Alexander Graham Bell, visiting in the 1870
s
, wondered indeed whether it might not come to harbor an entire ‘deaf variety of the human race,’ which might then spread throughout the world.

And knowing that congenital achromatopsia, like this form of deafness, is also hereditary, I could not help wondering whether there might also be, somewhere on the planet, an island, a village, a valley of the colorblind.

When I visited Guam early in 1993, some impulse made me put this question to my friend John Steele, who has practiced neurology all over Micronesia. Unexpectedly, I received an immediate, positive answer: there
was
just such an isolate, John said, on the island of Pingelap – it was relatively close, ‘barely twelve hundred miles from here,’ he added. Just a few days earlier, he had seen an achromatopic boy on Guam, who had journeyed there with his parents from Pingelap. ‘Fascinating,’ he said. ‘Classical congenital achromatopsia, with nystagmus, and avoidance of bright light – and the incidence on Pingelap is extraordinarily high, almost ten percent of the population.’ I was intrigued by what John told me, and resolved that – sometime – I would come back to the South Seas and visit Pingelap.

When I returned to New York, the thought receded to the back of my mind. Then, some months later, I got a long letter from Frances Futterman, a woman in Berkeley who was herself born completely colorblind. She had read my original essay on the colorblind painter and was at pains to contrast her situation with his, and to emphasize that she herself, never having known color, had no sense of loss, no sense of being chromatically defective. But congenital achromatopsia, she pointed out, involved far more than colorblindness as such. What was far more disabling was the painful hypersensitivity to light and poor visual acuity which also affect congenital achromatopes. She had grown up in a relatively shadeless part of Texas, with a constant squint, and preferred to go outside only at night. She was intrigued by the notion of an island of the colorblind, but had not heard of one in the Pacific. Was this a fantasy, a myth, a daydream generated by lonely achromatopes? But she had read, she told me, about another island mentioned in a book on achromatopsia – the little island of Fuur, in a Jutland fjord – where there were a large number of congenital achromatopes. She wondered if I knew of this book, called
Night Vision
 – one of its editors, she added, was an achromatope too, a Norwegian scientist named Knut Nordby; perhaps he could tell me more.

Astounded at this – in a short time, I had learned of not one but
two
islands of the colorblind – I tried to find out more. Knut Nordby was a physiologist and psychophysicist, I read, a vision researcher at the University of Oslo and, partly by virtue of his own condition, an expert on colorblindness. This was surely a unique, and important, combination of personal and formal knowledge; I had also sensed a warm, open quality in his brief autobiographical memoir, which forms a chapter of
Night Vision
, and this emboldened me to write to him in Norway. ‘I would like to meet you,’ I wrote. ‘I would also like to visit the island of Fuur. And, ideally, to visit the island
with
you.’

Having fired off this letter impulsively, to a complete stranger, I was surprised and relieved by his reaction, which arrived within a few days: ‘I should be delighted to accompany you there for a couple of days,’ he wrote. Since the original studies on Fuur had been done in the 1940
s
and ‘50
s
, he added, he would get some more up-to-date information. A month later, he contacted me again:

I have just spoken to the key specialist on achromatopsia in Denmark, and he told me that there are no known achro-mats left on the island of Fuur. All of the cases in the original studies are either dead…or have long since migrated. I am sorry – I hate to bring you such disappointing news, as I would much have fancied travelling with you to Fuur in search of the last surviving achromat there.

I too was disappointed, but wondered whether we should go nonetheless. I imagined finding strange residues, ghosts, of the achromatopes who had once lived there – parti-colored houses, black-and-white vegetation, documents, drawings, memories and stories of the colorblind by those who once knew them. But there was still Pingelap to think of; I had been assured there were still ‘plenty’ of achromatopes there. I wrote to Knut again, asking how he might feel about coming with me on a ten-thousand-mile journey, a sort of scientific adventure to Pingelap, and he replied yes, he would love to come, and could take off a few weeks in August.

Colorblindness had existed on both Fuur and Pingelap for a century or more, and though both islands had been the subject of extensive genetic studies, there had been no human (so to speak, Wellsian) explorations of them, of what it might be like to be an achromatope in an achromatopic community – to be not only totally colorblind oneself, but to have, perhaps, colorblind parents and grandparents, neighbors and teachers, to be part of a culture where the entire concept of color might be missing, but where, instead, other forms of perception, of attention, might be amplified in compensation. I had a vision, only half fantastic, of an entire achromatopic culture with its own singular tastes, arts, cooking, and clothing – a culture where the sensorium, the imagination, took quite different forms from our own, and where ‘color’ was so totally devoid of referents or meaning that there were no color names, no color metaphors, no language to express it; but (perhaps) a heightened language for the subtlest variations of texture and tone, all that the rest of us dismiss as ‘grey.’

Excitedly, I began making plans for the voyage to Pingelap. I phoned up my old friend Eric Korn – Eric is a writer, zoologist, and antiquarian bookseller – and asked him if he knew anything about Pingelap or the Caroline Islands. A couple of weeks later, I received a parcel in the post; in it was a slim leather-bound volume entitled
A Residence of Eleven Tears in New Holland and the Caroline Islands, being the Adventures of James F. O’Connell
. The book was published, I saw, in Boston in 1836; it was a little dilapidated (and stained, I wanted to think, by heavy Pacific seas). Sailing from McQuarrietown in Tasmania, O’Connell had visited many of the Pacific islands, but his ship, the
John Bull
, had come to grief in the Carolines, in a group of islands which he calls Bonabee. His description of life there filled me with delight – we would be visiting some of the most remote and least-known islands in the world, probably not much changed from O’Connell’s time.

I asked my friend and colleague Robert Wasserman if he would join us as well. As an ophthalmologist, Bob sees many partially colorblind people in his practice. Like myself, he had never met anyone born totally colorblind; but we had worked together on several cases involving vision, including that of the colorblind painter, Mr. I. As young doctors, we had done fellowships in neuropathology together, back in the 1960
s
, and I remembered him telling me then of his four-year-old son, Eric, as they drove up to Maine one summer, exclaiming, ‘Look at the beautiful orange grass!’ No, Bob told him, it’s not orange – ’orange’ is the color of an orange. Yes, cried Eric, it’s orange like an orange! This was Bob’s first intimation of his son’s colorblindness. Later, when he was six, Eric had painted a picture he called
The Battle of Grey Rock
, but had used pink pigment for the rock.

Bob, as I had hoped, was fascinated by the prospect of meeting Knut and voyaging to Pingelap. An ardent windsurfer and sailor, he has a passion for oceans and islands and is reconditely knowledgeable about the evolution of outrigger canoes and proas in the Pacific; he longed to see these in action, to sail one himself. Along with Knut, we would form a team, an expedition at once neurological, scientific, and romantic, to the Caroline archipelago and the island of the colorblind.

We converged in Hawaii: Bob looked completely at home in his purple shorts and bright tropical shirt, but Knut looked distinctly less so in the dazzling sun of Waikiki – he was wearing two pairs of dark glasses over his normal glasses: a pair of Polaroid clip-ons, and over these a large pair of wraparound sunglasses – a darkened visor such as a cataract patient might wear. Even so, he tended to blink and squint almost continuously, and behind the dark glasses we could see that his eyes showed a continual jerking movement, a nystagmus. He was much more comfortable when we repaired to a quiet (and, to my eyes, rather dimly lit) little café on a side street, where he could take off his visor, and his clip-ons, and cease squinting and blinking. I found the café much too dark at first, and groped and blundered, knocking down a chair as we went in – but Knut, already dark adapted from wearing his double dark glasses, and more adept at night vision to begin with, was perfectly at ease in the dim lighting, and led us to a table.

Knut’s eyes, like those of other congenital achromatopes, have no cones (at least no functional cones): these are the cells which, in the rest of us, fill the fovea – the tiny sensitive area in the center of the retina – and are specialized for the perception of fine detail, as well as color. He is forced to rely on the more meager visual input of the rods, which, in achromatopes as in the rest of us, are distributed around the periphery of the retina, and though these cannot discriminate color, they are much more sensitive to light. It is the rods which we all use for low-light, or scotopic, vision (as, for instance, walking at night). It is the rods which provide Knut with the vision he has. But without the mediating influence of cones, his rods quickly blanch out in bright light, becoming almost nonfunctional; thus Knut is dazzled by daylight, and literally blinded in bright sunlight – his visual fields contract immediately, shrinking to almost nothing – unless he shields his eyes from the intense light.

His visual acuity, without a cone-filled fovea, is only about a tenth of normal – when we were given menus, he had to take out a four-power magnifying glass and, for the special items chalked on a blackboard on the opposite wall, an eight-power monocular (it looked like a miniature telescope); without these, he would barely be able to read small or distant print. His magnifying glass and monocular are always on his person, and like the dark glasses and visors, they are essential visual aids. And, with no functioning fovea, he has difficulty fixating, holding his gaze on target, especially in bright light – hence his eyes make groping, nystagmic jerks.