Thanks so much for sharing our journey… Elihu and I are in a fantastic mood tonight as we’ve now had over 1000 visits to our young blog. I am so grateful to our friends and passersby for coming along with us on our journey. Who knows what lies ahead? Right now, who cares? We just feel so happy right now, and much less alone than we have in the past. Thanks everyone. And don’t forget to say hello sometime. (It will be easier to do that when I get this silly guest book figured out. Soon…)
My son has Achromatopsia. It’s a congential disorder of the retina, which is to say he’s had it since birth, and will have it for the rest of his life. The best metaphor I can find to describe Achromatopsia is this: his eyes have the hardware to see, but he’s missing the software, or the app. Essentially, he is missing a protein in his retina which delivers the visual information from the cone cells to the brain. I do realize that my explanation may be rather simplistic, and those who’ve spent hours upon hours learning about this might find fault with my presentation or correct my understanding about it, yet for our intents and purposes the metaphor works well. All I know is that his experience of the world is much different from ours, and as his mother, his number one advocate, I am always mindful of it. Thankfully, when the technology to deliver the missing protein to his eye is perfected, there is hope that he will one day have the possibility of correcting it.
Elihu is considered legally blind, which is a strange, nether-world in which to live. Yes, he can see. No, he cannot see color – any color at all. Most people are amazed at this fact alone – and in order to demystify it, I usually tell kids to take the color out of there TVs in order to see the world as he does. For the over 30 set I just tell them to envision black and white films, or perhaps even and Ansel Adams photograph. Yet that’s not the end of it. Elihu sees very little detail beyond 20 feet. He also is virtually blinded by light and must wear dark red glasses in order to function. Why red? Because that is the spectrum that sops up most of the light for him. Since he can’t distinguish color, the fact that the lenses are red means nothing besides the comfort they afford him. Essentially, he sees using only rod cells – the ones that kick into gear for us at twilight. The glasses he wears must dim light down to that sort of level. And as those who’ve studied candle power can attest – the sun is not just a whole lot brighter than artificial light, but rather exponentially brighter, so finding ‘comfort’ in outside light is a tall order.
Elihu can’t recognize friends in the hall at school when they call to him. He can’t always follow kids running in the playground. Depth perception is tricky for him; he’s been tripping over curbs all his life. However, as with anything, he’s become adept at living with it, and the older he gets the fewer things he’s surprised by. He’s learned to be gracious when people approach him. He’s learned in part from mom and dad, who being performers are often greeted by people whom they can’t always recall meeting. Being polite is all that’s called for. “I’m sorry, I’m really bad with names, can you please tell me your name again?” or “I’m sorry, can you please tell me again how we know each other?” In his case, I’m encouraging him to tell folks he can’t quite make them out until they’re fairly close up, so they know he’s not being aloof. Elihu is now beginning to feel fairly self-conscious about being different (like he needed a retinal problem to set him apart!) and so he’s going to face some challenges in the next few years. I just keep telling him that a sense of humor helps. This he knows well.
There is an island in the middle of the Pacific – it’s in the Federated States of Micronesia to be more specific – called Pingelap, on which many of the current residents are Achromats. Apparently, hundreds of years ago several of Captain Cook’s crew, shipwrecked on the tiny island, carried the recessive gene for Achromatopsia. They stayed on the island to live, to raise families and ultimately created a gene pool heavily populated with the gene for A. Elihu and I have a dream to one day visit this place, and bring with us dark glasses for all the residents who need them. I cannot imagine having Achromatopsia and living on a sun-drenched island, and it makes my heart lift to think of the relief we might one day bring to them.
John Kay of Steppenwolf is an Acrhromat. (Check out some pictures of them and note John’s dark glasses. Not a costume choice, but a necessity.) I think it’s absolutely ironic that the man who penned the iconic “Born To Be Wild” doesn’t even have a driver’s license. It’s even more ironic that today he produces nature videos. I’ve heard his wife does the color correction for him. This always makes me smile. Go John! Go Elihu! Go As!
Even though Achromatopsia will not prevent my son from realizing his full potential, it is still my single greatest hope that one day Elihu will have the option to choose for himself whether or not to change his vision. Ultimately, it will something that only he can choose for himself. ‘Til that time, we have many adventures awaiting…