Technical Difficulties

So I was looking for information on ocular albinism last night because I was trying to think of ways to condense the explanation (I find it more than a little tedious, and am always worried that the person I'm explaining to is also finding it tedious), and I discovered something.

I don't have ocular albinism.

Your actual ocular albinism is an X-linked condition, which means only men have it. Women can be carriers, and [corrected per marsdejahthoris's comment] which means very very few women ever have it. If my dimly resurrected ninth-grade biology is right, the only way for a woman to have it would be for her father to have ocular albinism and her mother to be a carrier--and for her to get her mother's albinist X. This is not my situation. Women who carry ocular albinism may have mottled retinas. This is not my situation, either.

No, what I have is albinism: oculocutaneous albinism (OCA), an autosomal recessive condition, the same thing that produces the albino of popular culture, with white skin and white hair and pink eyes. There are different types of OCA, with differing degrees of ability to produce melanin. The fact that I'm a blonde in a family of redheads and brunettes is caused by albinism, exactly the same way the absence of pigment in my retinas is.

Research has clearly taken a step forward since the last time I looked at NOAH's website--which was, in point of fact, several years ago. And it's taken big steps forward since the 1970s. (I remember having hair pulled out for the test described on their What Is Albinism? page; from the results, the doctors decided I didn't have albinism, which shows you that the test is, as NOAH says, not real reliable.)

NOAH also has a page about nomenclature. For the record, I will not be offended by the word "albino." I don't have any negative childhood associations with the word (although I can completely understand why people whose albinism is more visually apparent than mine might have big honking issues), and for me, it doesn't carry a lot of freight. (I wasn't diagnosed with "ocular albinism" until I was 22, and only discovered YESTERDAY that I am, in fact, entitled to use the word "albino" without immediate qualification and clarification. So, yeah. I've got my share of baggage, but not about that.) Other persons with albinism may feel differently; I do not pretend to speak for them.

In some ways this is completely trivial--it's not telling me anything about my own personal albinism that I didn't already know--but it's still kind of, I don't know, unsettling. So yeah, this is me, being unsettled and readjusting. Onward.

Albinos in Tanzania are being murdered for their body parts.

Also, a quite interesting Q&A about albinism itself. And how much do I love the SciAm writers for remarking on the popular culture Evil Albino stereotype?

(N.b., the type of albinism I have is not the same as the albinism being discussed in the article and Q&A. I have no pigment in my retinas, but my pupils and irises are normal. I am also, as people who have met me can testify, extremely pale--among my extended family of brunet(te)s and redheads, I am the only blonde, and my skin is also noticeable for its pallor. (See icon.) This is typical of ocular albinism, which seems to decrease pigmentation in hair and skin, but doesn't remove it entirely, as oculocutaneous albinism does. I also have nystagmus, and a lot of trouble with light levels, and the ocular albinism is the reason I started wearing bifocals at the age of thirty-two. I'm lucky, though, in that my problems are not as severe as they would most likely be if I had oculocutaneous albinism--I'm not legally blind, as long as I'm wearing my glasses.)

If you're interested in finding out more about any type of albinism, NOAH (the National Organization for Albinism and Hypopigmentation) has a lovely and informative website.

There's an article here that makes a valiant stab at describing how people with albinism see the world. Literally.

Since, obviously, I've never seen the world WITHOUT albinism, I'd be curious to hear what people who do not have albinism make of it.