The Island of the Colorblind

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.

Book II CYCAD ISLAND

Guam

It 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 1950s and ‘60s, 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 1960s and ‘70s, 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 1960s, 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.