The Seven-Year Itch to Destroy

It turns out that if you don't visit a doctor for seven years, that doctor is well within his rights to destroy all evidence of your existence... at least, when it comes to his own files. For those of you with long-term, slowly-progressing diseases, this is a handy thing for you to keep in mind. I kinda wish I had known it.

When I was first diagnosed, I went to visit some researchers doing state-of-the-art stuff in Boston. It was an international crew of rogue retina enthusiasts. They were entertaining and only made me want to puke a couple of times. They played with lasers and radioactive dye.

Within five years, that practice had dissolved. I still went for follow-up visits, heading up to Boston every four or five years to see the guy who had been in charge. This, quirky, larger-than-life gentleman tried to speak as quickly as he thought, but it was usually a losing battle. He spoke many words to me but rarely, if ever, told me anything super-useful.

At the end of each visit, he would casually suggest that I come back in four or five years. If I felt like it. He didn't imply that it was all that important. From the usefulness o each visit, and the travel costs of getting to and from Boston, I didn't really disagree.

My last visit there was in the summer of 2002. And then, in February, I got the news about my change in prognosis. I asked the current experts if the old tests results might be helpful to them, and they were interested in seeing them.

Now, we get back to that seven-year issue. I tracked down that retina doctor, the one who had been so casual about my ever coming back. His office, it seems, has dutifully destroyed everything that had my name on it. They must have done so promptly at the seven-year mark. I called twice, and even brought up the fact that I had sent him a letter, at his office, in mid-2003. Nothing.

I called my current, regular ophthalmologist, hoping they had sent him my records back in the day. They sent him a letter. No hard data, no images: just a letter.

It's amazing to me that someone would engage in a field that involves long-term study, watching cases over years and decades, and still would have no clue about the importance o archives. Sure, the law allows you to get rid of your files after a while... but the law also allows banks to foreclose on people who have filed for medical bankruptcy. It allows men to walk out on their families and do nothing but pay child support. It doesn't make either one the right thing to do.

So much for my latest contribution to science. On the bright side, I now have a reason to hold on to all my bank statements from 1998. Someone might need them for science.

A New View of My Future Vision

Back when I saw the experts in September, they wanted to do just one more thing: take my picture. Really, they wanted a picture of my eyes, but same difference. It shouldn't have taken very long, but since I had been there for more than seven hours ,they really didn't want to keep me, and suggested that I just come back another time.

Well, I never really got around to calling them. With my condition not being very severe, I couldn't really tell if I was any use to them, and it's a decent commute. More to the point, they were so disorganized and inefficient about everything that I wasn't eager to go back.

So, needless to say, I was a little surprised to get a call from one of the research doctors. He asked if I could come back and go through a few more tests. And, yes, they would also take my picture.

The day I went back happened to be one of the big snow days, and they were a little surprised to see me.

"I don't live that far from the subway," I explained.

They looked at me blankly. I think they had forgotten that, while most of their patients come from all over the world, I come from Brooklyn.

The doctor assured me that they would be much more efficient this time. Apparently, I had been one of the first patients to visit this particular group, and they were still figuring out their process. That's what they told me, anyway. My impression was that the doctor himself had taken over a lot of the testing, just because he didn't really trust other people to do it.

True to the doctor's word, things went very smoothly, and after a lot of sitting in dim rooms, followed by trying not to blink with bright lights being shone in my face, I sat back and blinked. A lot.

The doctor looked at his screen and said, "Yes, this looks like classic Stargardt's."

I nodded. I've heard that before; no one has ever questioned my diagnosis, not since it came twenty years ago.

"Just out of curiosity," I said, "on the range of mild to severe among Stargardt's people, where do you think I fall?"

I had asked this question the last time, but he hadn't had a chance to answer it. This time, he did.

"Actually," he started, "I think you fall into the more severe category."

I'm not sure how I reacted in the moment. Experts in facial expressions and lying say that you give tiny little twitches to indicate how you feel about something, most of the time that you don't even realize yourself. I don't recall being shocked. I really should have been. But, then again, that's what "being in shock" means:: not really feeling anything at first.

The doctor explained that there are three general categories of my disease:
1. (mild) Only the very center of the vision is ever affected.
2. (moderate) The center and the rods (little bits that deal with light and darkness) are affected.
3. (severe) The center is affected, and much of the surrounding rods and cones eventually go as well.

I had always gotten the impression that I was in category 1, or possibly category 2. He felt that I was "somewhere between 2 and 3." Since Stargardt's patients generally don't change from one group to the other, this meant that I was in group 3. A few days later, he checked with the resident super-expert, who confirmed that I was indeed in that most severe category.

Group 3 is a pretty bad prognosis. The ultimate result, over time, is that I would no longer be independent: I'd need a guide dog. Now, I've always wanted a dog, but there's a difference between wanting one and needing one, and I'd rather not need one. I'd also rather be able to, um, see stuff.

After about half an hour of sitting in the dark (necessary for one of the tests), I ate the lunch I had brought (seemed like a good idea, after last time) and ate what felt a bit like comfort food. The doctor came in and let me know that we would have to wait a little while longer for the test, since there was a line.

"While you're here," I started, "what kind of progress should I expect? I mean, if I'm supposed to go functionally blind in forty years, what about the next ten or twenty?"

First, he assured me that I would not go completely blind, that I would still be able to perceive light and dark and some color. This was not all that reassuring; I'd like to have better vision than my future guide dog, if possible. But he also said that while he couldn't say what would happen in ten or twenty years, he could say that the disease tends to move slowly, and that my vision probably would not seem too different twenty years from now.

Now, in research terms, twenty years really is a long time. Unlike the empty assurances twenty years ago, when I was first diagnosed, there are some real possibilities for cures now. Actual studies are underway in gene therapy (which would halt the progress of the disease) and stem cell treatment (which might even reverse it). At the point that I'm at now, no one would want to experiment on my eyes because of the risk of actual blindness, but I would probably be a candidate down the road.

So, really, this hasn't changed my life too much. I had always expected the vision to get worse in old age, but I understood that to mean that I'd need magnification glasses. This will definitely be a bigger deal than that. But, hey, I've never been one to take the easy path.

Monkey See, Monkey See Color

Apparently, male monkeys are red-green colorblind. In this sense (and this alone), I do not resemble a monkey.

In recent research, scientists seem to have found a cure for colorblindness in monkeys. The method is gene therapy, which has often been floated as a possible cure for my condition. After about five months of gene therapy treatment, male monkeys were able to see the difference between red and green.

The adorable story is on NPR:

http://www.npr.org/templates/story/story.php?storyId=112897277&sc=emaf

The scientists in this story ask the same question that I have often asked of myself: namely, if you were to cure the physical eye, would the brain synapses still exist to process the image? In the case of the monkeys, the answer was a resounding "yes," despite the nay-sayers. I wonder if the same would happen for me.

Well, for the moment, this research is still monkey business. But, hey, in another five years... you never know.