1996 - The Island of the Colorblind

by Oliver Sacks

  Pohnpei was one of the first of the Carolines to be colonized by humans – Nan Madol is much older than anything to be found on any of the outlying atolls – and with its high terrain, its size, and rich natural resources, it is still the ultimate refuge when disaster strikes the smaller islands. The atolls, smaller, more fragile, are intensely vulnerable to typhoons, droughts, and famines – Oroluk, according to legend, was once a thriving atoll, until most of it washed away in a typhoon; it now consists of a fifth of a square mile.26 Moreover, all of these islands, with their limited size and resources, are liable sooner or later to reach a Malthusian crisis of overpopulation, which must lead to disaster, unless there can be emigration. Throughout the Pacific, as O’Connell observed, islanders are periodically forced to emigrate, setting out in their canoes, as their ancestors did centuries before, not knowing what they will find, or where they will go, and hoping against hope that they may find a new and benign island to resettle.27

  But Pohnpei’s satellite atolls are able to turn to the mother island in such times, and thus there are separate enclaves in the town of Kolonia, Pohnpei’s capital, of refugees from other islands – Sapwuahfik, Mwoakil, Oroluk, and even the Mortlock Islands, in the neighboring state of Chuuk. There are two sizeable Pingelapese enclaves on Pohnpei, one in Sokehs province, the other in Kolonia, first established when Pingelap was devastated by the 1905 typhoon, and enlarged by subsequent emigrations. In the 1950s there came yet another emigration from Pingelap, this time in consequence of extreme overcrowding, and a new enclave was established by six hundred Pingelapese in the remote Pohnpeian mountain valley of Mand. Since then the village has burgeoned to a population of more than two thousand Pingelapese – three times the population of Pingelap itself.

  Mand is isolated geographically, but even more ethnically and culturally – so that forty years after the original settlers migrated here from Pingelap, their descendants have avoided, largely, any contact or marriage with those outside the village, and have maintained, in effect, an island on an island, as homogenous genetically and culturally as Pingelap itself – and the maskun is, if anything, even more prevalent here than on Pingelap.

  The road to Mand is very rough – we had to travel in a jeep, often slowing down to little more than a walking rate – and the journey took more than two hours. Outside Kolonia, we saw occasional houses and thatched sakau pubs, but as we climbed, all signs of habitation disappeared. A separate trail – traversable only by foot or by four-wheel drive – led off from the main road, climbing steeply up to the village itself. As we got higher, the temperature and humidity diminished, a delightful change after the heat of the lowlands.

  Though isolated, Mand is a good deal more sophisticated than Pingelap, with electricity, telephones, and access to university-trained teachers. We stopped first at the community center, a spacious, airy building with a large central hall used for village meetings, parties, dances. Here we could spread out our equipment and meet some of the achromatopes of the community, and distribute sunglasses and visors. Here, as on Pingelap, there was a certain amount of formal testing, and we explored the details of daily life in this very different environment, and how much this might be helped with proper visual aids. But, as in Pingelap, it was Knut, quietly open about himself, who could do the deepest, most sympathetic probing and counselling. He spent a good deal of time with the mother of two achromatopic children, five years and eighteen months old, who was deeply anxious that they might go completely blind – fearful too that their eye condition might have been her fault, that it was something she had done during pregnancy. Knut did his best to explain to her the mechanisms of heredity, to reassure her that her daughters would not go blind, that there was nothing wrong with her as a wife or a mother, that the maskun was not necessarily a barrier to receiving an education and holding a job, and that with the proper optical aids and eye protection, the proper understanding, her daughters could do as well as any other child. But it was only when he made clear that he himself had the maskun – she suddenly stared at him in a new way at this point – that his words seemed to take on a solid reality for her.28

  We moved on to the school, where a busy day was in progress. There were twenty or thirty children in each class, and, in each, two or three were colorblind. There were a number of excellent, well-trained teachers here, and the level of education, sophistication, was clearly far better than on Pingelap; some of the classes were in English, others in Pohnpeian or Pingelapese. In one class of teenagers, we sat in on a lesson in astronomy – this included pictures of earthrise from the moon and close-ups of the planets from the Hubble space telescope. But admixed with the latest astronomy and geology, the secular history of the world, a mythical or sacred history was given equal force. If the students were taught about shuttle flights, plate tectonics and submarine volcanoes, they were also immersed in the traditional myths of their culture – the ancient story, for example, of how the island of Pohnpei had been built under the direction of a mystical octopus, Lidakika. (I was fascinated by this, for it was the only cephalopod creation myth I had ever heard.)

  Watching two little achromatopic girls doing their arithmetic lessons with their noses virtually touching the pages of the book, Knut was reminded powerfully of his own school days, before he had any optical aids. He pulled out his pocket magnifying loupe to show them – but it is not easy, unpracticed, to use a high-power magnifying glass to read with.

  We stayed longest in a class of five- and six-year-olds, who were just learning to read. There were three achromatopic children in this class – they had not been placed, as they should have been, in the front row; and it was immediately apparent that they could not see the letters on the blackboard where the teacher was printing, which the other children could see easily. ‘What’s this word?’ the teacher would ask – everyone’s hands would shoot up, including the achromatopes’, and when another child gave the answer, they echoed it in unison. If they were asked first, though, they could not answer – they were just imitating the other children, pretending to know. But the achromatopic children seemed to have developed very acute auditory and factual memories, precisely as Knut had developed in his own childhood:

  Since I could not actually discern the individual letters even in ordinary book print…I had developed a very keen memory It was usually enough if a class-mate or someone in the household read my home-assignment to me once or twice, in order for me to remember and reproduce it, and to perform a rather convincing reading behaviour in class.

  The achromatopic children were oddly knowledgeable too about the colors of people’s clothing, and various objects around them – and often seemed to know what colors ‘went’ with what. Here again Knut was reminded of his own childhood strategies:

  A constantly recurring harassment throughout my childhood, and later on too, was having to name colors on scarves, ties, plaid skirts, tartans, and all kinds of multicolored pieces of clothing, for people who found my inability to do so rather amusing and quite entertaining. As a small child I could not easily escape these situations. As a pure defence measure, I always memorized the colours of my own clothes and of other things around me, and eventually I learned some of the ‘rules’ for ‘correct’ use of colours and the most probable colours of various things.

  Thus we could already observe in these achromatopic children in Mand how a sort of theoretical knowledge and know-how, a compensatory hypertrophy of curiosity and memory, were rapidly developing in reaction to their perceptual problems. They were learning to compensate cognitively for what they could not directly perceive or comprehend.29

  ‘I know that colors carry importance for other people,’ Knut said later. ‘So I will use color names when necessary to communicate with them. But the colors as such carry no meaning for me. As a kid, I used to think that it would be nice to see colors, because then I would be able to have a driver’s license and to do things that people with normal color vision can do. And if there were some way of acquiring color vi
sion, I suppose it might open a new world, as if one were tone deaf and suddenly became able to hear melodies. It would probably be a very interesting thing, but it would also be very confusing. Color is something you have to grow up with, to mature with – your brain, the whole system, the way you react to the world. Bringing in color as a sort of add-on later in life would be overwhelming, a lot of information I might not be able to cope with. It would give new qualities to everything that might throw me off completely. Or maybe color would be disappointing, not what I expected – who knows?’30

  We met Jacob Robert, an achromatope who works at the school, in charge of ordering books and supplies. He was born in Pingelap, but emigrated to Mand in 1958 to finish high school. In 1969, he told us, he had been flown, with Entis Edward and a few others, to the National Institutes of Health in Washington for special genetic studies associated with achromatopsia – this was his first glimpse of life outside Micronesia. He was particularly intrigued, when he was there, to hear about the island of Fuur, in Denmark. He had not known there were any other islands of the colorblind in the world, and when he returned to Pohnpei, his fellow achromatopes were fascinated too. ‘It made us feel less alone,’ he said. ‘It made us feel we had brothers somewhere in the big world.’ It also started a new myth, that there was ‘a place in Finland, which gave us the achromatopsia.’ When we had heard this myth in Pingelap, we had assumed it was a new one, generated by Knut’s presence; now, as we listened to Jacob, and how he had brought back news of a place in the far north with the maskun, it became evident that the myth had arisen twenty-five years earlier and, perhaps now half forgotten, had been reanimated, given a new form and force, with Knut’s arrival.

  He was intrigued to hear the story of Knut’s own childhood in Norway, so similar in many ways to his – and yet different, too. Jacob had grown up surrounded by others with the maskun and by a culture which recognized this; most achromatopes around the world grow up in complete isolation, never knowing (or even knowing of) another of their kind. Yet Knut and his brother and sister, by a rare genetic chance, had each other – they lived on an island, a colorblind island, of three.

  The three of them, as adults, all achromatopic, all highly gifted, have reacted and adapted to their achromatopsia in very different ways. Knut was the firstborn, and his achromatopsia was diagnosed before he started school – but it was felt that he would never be able to see well enough to learn to read, and recommended that he (and his siblings, later) be sent to the local school for the blind. Knut rebelled at being regarded as disabled, and refused to learn Braille by touch, instead using his sight to read the raised dots, which cast tiny shadows on the page. He was severely punished for this and forced to wear a blindfold in classes. Soon after, Knut ran away from the school, but, determined to read normal print, taught himself to read at home. Finally, having convinced the school administrators that he would never make a willing student, Knut was allowed to return to regular school.

  Knut’s sister, Britt, dealt with her loneliness and isolation as a child by identifying with, becoming a member of, the blind community. She flourished at the school for the blind as much as Knut hated it, becoming fluent in Braille; and she has spent her professional life as an intermediary between the blind and sighted worlds, supervising the transcription and production of books into Braille at the Norwegian Library for the Blind. Like Knut, Britt is intensely musical and auditory and loves to close her eyes and surrender herself to the nonvisual domain of music; but equally, she relaxes by doing needlework, using a jeweller’s loupe attached to her glasses, to keep her hands free.

  It was now three in the afternoon – time to set back for Kolo-nia – and despite our altitude, burningly hot. While Knut sat under a shade tree to cool off, Bob and I decided to dive into the beautifully clear stream which ran nearby. Finding a flat rock under the surface, shaded by ferns, I clung onto this and let the cool waters stream over me. Downstream, a quarter of a mile or so, some of the women were washing dark, heavy clothes – the formal Sunday wear of Mand.

  Refreshed by our swim, Bob and I decided to walk down the trail from the village; the others would meet us at the road below in the jeep. In the afternoon light, we were dazzled by the brilliance of oranges hanging in the trees – they seemed almost alight in the dark green foliage, like Marvell’s oranges in his poem ‘Bermudas’:

  He hangs in shades the Orange bright,

  Like golden Lamps in a green Night.

  I felt a sudden sadness that Knut, that the achromatopes around us, could not share this startling Marvellian vision.

  We had gone a couple of hundred yards when we were overtaken by a twelve-year-old boy running at top speed, fearlessly, looking like a young knight with his new sun visor. He had been squinting, looking down, avoiding the light when we saw him earlier, but now he was running in broad daylight, confidently making his way down the steep trail. He pointed to the dark visor and gave a big smile. ‘I can see, I can see!’ and then he added, ‘Come back soon!’

  Dusk descended as we drove back slowly to Kolonia, and we began to see occasional bats, then great numbers of them, rising from the trees, taking off on their nighttime forays, emitting shrill cries (and doubtless sonar too). Bats are often the only mammals that manage to make it to distant islands (they were the only mammals on Pohnpei and Guam, until rats and others were introduced from sailing ships), and one feels they ought to be more respected, more loved, than they are. They are considered fancy eating on Guam, and exported by the thousand to the Marianas. But they are an essential part of the island’s ecology, eating many types of fruit and distributing the seeds, and one hopes their delicious taste does not lead to their extinction.

  Greg Dever, director of the Pacific Basin Medical Officers Training Program in Kolonia, has a brusque surface, but underneath this is deeply romantic and dedicated to his work. He had gone to Palau as a young man in the Peace Corps, and had been shocked at what he saw – a fearful incidence of treatable diseases, combined with a drastic shortage of doctors – and this decided him on a career in medicine, so that he could return to Micronesia as a doctor. He trained as a pediatrician at the University of Hawaii and moved to the Carolines fifteen years ago. Here on Pohnpei he has established a small hospital, a clinic and outreach service stretching to the outlying atolls, and a medical program aimed at training indigenous students from all the archipelagoes, in the hope that when they graduate as doctors they will stay and practice and teach in the islands (although some, now that their degrees are accepted in the States, have gone on to more lucrative careers on the mainland).31

  He had asked us, as visiting scientists, to give a presentation on the maskun. We felt odd, as visitors, talking to these doctors, mostly native, about problems they themselves presumably had lived with and knew intimately. Yet we thought that our very naivete, coming at the subject from another angle, might have some value for the audience – and we hoped we might learn more from them as well. But it became increasingly clear – as Bob spoke about the genetics and the retinal basis of the maskun; I about adapting neurologically to such a condition; and Knut about the challenges of actually living with it – that many of those in the audience had never actually encountered the maskun. We found this extraordinary. Even though there are half a dozen papers in the scientific literature on the maskun, here in the capital of achromatopsia there was almost no local medical awareness of the problem.

  One reason for this, perhaps, had to do with the simple act of recognizing and naming the phenomenon. Everyone with the maskun has behaviors and strategies which are obvious once one is attuned to them: the squinting, the blinking, the avoidance of bright light. It was these which allowed an instant mutual recognition between Knut and the affected children the moment he landed on Pingelap. But before one has assigned a meaning to these behaviors, categorized them, one may just overlook them.

  And there is also a medical attitude, enforced by necessity, which militates against proper recognition of the ma
skun. Greg and many others have worked incessantly to train good doctors in under-doctored Micronesia. But their hands are constantly full with critical conditions demanding immediate attention. Amebiasis and other parasitic infections are rife (there were four patients with amebic liver abscesses in the hospital while we were there). There are constant outbreaks of measles and other infectious diseases, partly because there are not enough resources to vaccinate the children. Tuberculosis is endemic in the islands, as leprosy once was.32 Widespread chronic vitamin-A deficiency, probably linked to the shift to a Western diet, can cause severe ear and eye problems (including night blindness), lower resistance to infection, and lead to potentially fatal malabsorption syndromes. Though almost every form of venereal disease is seen, AIDS has not yet appeared in this remote place, but Greg worries about the inevitable: ‘All hell will break loose when we get AIDS,’ he said. ‘We just don’t have the manpower or the resources to deal with it.’

  This is the stuff of medicine, the acute medicine which must be the first priority in the islands. There is little time or energy left over for something like the maskun, a congenital, nonprogressive condition which one can live with. There is no time for an existential medicine which enquires into what it might mean to be blind or colorblind or deaf, how those affected might react and adapt, how they might be helped – technologically, psychologically, culturally – to lead fuller lives. ‘You are lucky,’ said Greg. ‘You have the time. We’re too harried here, we don’t have the time.’