Curious about…Achromatopsia.
As someone who lives with a colorblind person, I have been interested in this subject for ages. I first read about Achromatopsia in Oliver Sack’s seminal 1997 book: “The Island of The Colorblind,” about the colorblind people living on the Micronesian atoll of Pingelap.
Achromatopsia (ACHM) is a recessive congenital color vision disorder, the inability to perceive color and to achieve satisfactory visual acuity at high light levels (typically exterior daylight). As illustrated in The Island of the Colorblind some achromats cannot see color, only black, white, and shades of grey. With 5 different genes currently known to cause similar symptoms, it may be that some do see marginal levels of color differentiation due to different gene characteristics.
Achromatopsia is a relatively uncommon disorder, with a prevalence of 1 in 30,000 people (0.0033%). However, in Pingelap approximately 5% of the atoll’s 3000 inhabitants are afflicted. In 1775, a catastrophic typhoon, Typhoon Liengkieki, swept through the island, killing 90% of the inhabitants and leaving only approximately 20 people. It is believed that one of the survivors, namely Nahnmwarki Mwanenised (the ruler at that time), was a carrier for complete achromatopsia (known on the island as maskun, meaning literally “not see” in Pingelapese), a recessive genetic disorder which causes total color-blindness in sufferers. All Achromats on this island nowadays can trace their ancestry to this male survivor. However, the achromatopsia disorder did not appear until the fourth generation after the typhoon, where 2.70% of the Pingelapese were affected.
What Of It? I may have imagined this, but one memory I have of the book is that in a color-challenged world, pattern suddenly takes on new significance. Elaborate textiles, tattoos and surface decoration become a way for people to decorate, celebrate and navigate the world around them. Imagine this: if you can’t see gold, how do you know that a material has special significance?
I am Curious to understand how the modification of one sense affects the others, how a physical design restraint can open-up opportunities for new ways to see things, in this case, literally.
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