Tag Archives: eyes

PHPV: the eye, vision, and how I see

[for other posts in the category, click ‘older posts’ at bottom]

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This is part iii of my perfect deformity. It stands alone, but part i and part ii are informative as well.

PHPV (Persistent Hyperplastic Primary Vitreous) is a rare, congenital eye disease that begins around the third month in utero. I have it in my left eye (right to you) and have written about it before. In short, the primary vitreous and hyaloid artery of the developing eye do not become clear and recede (they’re persistent), but instead grow even more (hyperplastic), scar, and form a stalk. Sometimes this is in the front of the eye. Sometimes the back. Mine runs from the cornea in the front all the way back to the retina. This, my ophthalmologist calls “classic,” “amazing,” “beautiful,” and “textbook” when describing it to her residents, whom she will pull off lunch break to view because it’s so rare to see such a case. Also rare because the cataracts and calcium deposits that can develop on the cornea often make it impossible to see into the eye. Not so for me. Mine fog up only the right side of my eye, so you can see straight in.

It is oddly comforting to have my deformity so appreciated. And since I’m a huge advocate of real world education, I’m happy to let the apprenticing doctors take a look, painful as it may be.

When I was little, as in the photo above, the deposits gave the eye more of a blue cast, so I appeared to have one brown eye and one blue (no, not like your cat). Now the coloration isn’t as extreme, but the eye is smaller (microphthalmia) and doesn’t track with the right. Other side effects are the retina peeling off a bit and elevated eye pressure (glaucoma). I have both, though both are pretty stable.

adult-phpvPhoto: Far right, adult eye with PHPV doesn’t track with normal eye.

I have yet to meet someone with PHPV. There’s a facebook group called “People with Persistent Hyperplastic Vitreous Unite” but it should be called “Parents of Babies & Toddlers with PHPV Support and Discuss.” I’ve chatted online with someone upstate (we’re FB friends now), and a few people here who have read my other posts, but I have never met another person with this disease. And before the internet (most of my life), the only information I got was from my ophthalmologist. There’s only so much one can absorb in a visit.

That’s why I write this. There’s very little info out there, and nothing about what it’s like to have PHPV.

Even so, I’ve known I see differently since I was young. My pediatric ophthalmologist (he was mean. Parents of Small Children with PHPV, please do not send your child to a mean eye doctor. Traumatown) gave me a slew of tests. One was a fly coming off a board, and I was meant to say if it was 3-D or not. It was the 70s, and this was the “Titmus Fly Stereotest.” Oh, I found a picture. What a horror.

I knew there was a correct answer to the question and I was pretty sure it was not what I saw. So instead of answering as such, I guessed. I don’t remember if I guessed right. I remember the doctor, the scariness, the stress, the tests, and trying to guess what I was supposed to see and say. I was perhaps four or five, and my dad was there in the dark doctor’s office, so I knew it was serious business.

Titmus Fly StereotestI do not have stereopsis, or, what most people take for granted as three-dimensional vision. Stereopsis requires that both eyes track together, so that the brain can use the perfect disparity between the right and left eyes to judge depth. A few inches apart, they see a slightly different image and the visual cortex uses that difference to create the third dimension. It is a trick of the mind. The cells in the visual cortex of the brain that do this develop quite early, and they rely on sight from two properly aligned eyes.

What does this mean to a kid? I sucked at ball games, because judging the distance of a ball moving through the blue sky is pretty much the pinnacle of three-dimensional sight. I loved photography since before I can remember, and got my first camera for Christmas at age ten. I first thought I was trying to freeze and memorize images, just in case I went blind. Later I realized that using one eye to make two-dimensional images is my reality, so of course it comes naturally. Though I do wonder how others see photographs. While your two-dimension vision is no different than mine, it differs from your regular, three-dimensional vision. Mine does not. All the tricks my brain uses to judge depth are pretty much there in a photograph. So perhaps I’m good at relaying the third-dimension in only two. I can’t know.

I also realized in high school that I could play tennis, as long as there were no lobs, because my brain used the lines on the court to judge where the ball was. I liked that. I did not like 3-D movies, because they didn’t work. I saw a lot of lines. I didn’t and don’t like many movies because the brightness hurts my eyes, which are ultra-sensitive to light. Especially in a pitch black room.

These things I had figured out on my own. In the last few years, I’ve noticed even more. Partly due to technology, and perhaps partly due to yoga and meditation, and simply being more aware of my experience. This is getting a bit long, so I’ll save more on how I actually see for the next post.

other posts on phpv:
my perfect deformity
my perfect deformity, part ii

PHPV: my perfect deformity (p2)

01-thanks-20110608So, I started calling eye doctors. The first few either no longer took my insurance or couldn’t get me in until October. I found, in my contacts, an eye doctor I’d met while bartending a party for the Columbia Presbyterian Ophthalmology Department way back when I was an undergrad. They were so fascinated by my eye that one gave me a card and said to come in sometime. Years later, I still had his contact info—James D. Auran, MD. He came up as one of NY’s best doctors, according to NY Magazine. I told Bij that maybe I’d just spring for him, out-of-network, until his office told me a first time visit was $375 + any tests. Happily enough, I went to their website, as Bij said she knew of someone else good there. That’s how I found the big-hearted Amilia Schrier, M.D. She takes my ghetto insurance because she thinks that everyone deserves to see (what a socialist)—even if they barely pay her anything. The person making her appointments was alarmed enough by my having floaters in my only good eye that she talked to the doctor and got me in the next day. Wow. I was scared but happy.

Note my left eye, which is on the right. It’s smaller, a bit higher, a slightly different color, and has cloudy bits on/in it. Refer to the last post for more details.

After too many years without seeing the eye doctor, I go. When I signed  in, I was asked to sign a form stating that most insurance does not cover a refraction test because they do not see it as necessary health care, and that I would be responsible for this cost, about $75. Of course I asked about this. A refraction test? Not essential? As in, the test to see what prescription a near- or far-sighted person’s lenses should be? A test that says if you need specs and how strong they should be—not essential? The DMV doesn’t think so. How very strange.

The nurse agreed with me. We complained a bit about insurance insanity as she dilated my eyes. Then she took me back to wait for the doctor.

Eyes dilated, I met the doctor, who wore a knitted sweater, the style befitting a kindly grandmother. It was comforting indeed. She heard my story and looked at my eyes. Right eye, good. Left eye, wow! Amazing! A beautiful case of PHPV! “My word, you can see everything!” She enthused. “Mittendorf’s dot!” I was pretty familiar with the discourse around my disease, but this was new. She drew me a picture of my left eye, and explained that Mittendorf’s dot is the hyaloid body. “It’s the embryologic remnant of the hyaloid artery as it joins the capillaries of the lens.” (Source, Mission for Vision.)

She explained that I had a perfect, textbook case of PHPV, exciting because usually the band keratopathy and haziness obstructs the view, especially of the back of the eye. After she told me that my right eye is okay, but I shouldn’t do inversions or jump-backs in yoga :(, she asked me if she could call the residents (her students) to look at my left eye. I said sure. Even though it’s torture to have lights shined into my eye, I like aiding the cause of education, and hey, it’s not often I’m perfect, even if it is a deformity.

She called down to the residents on the phone and told them, “You might never have a chance to see this again in your entire careers.” So up they came, four or five of them. Dr Schrier was very considerate, asking again and again if it was okay, and if I was okay. I was fine. I enjoyed listening to her tell them about my eye, “No, there’s no point in treatment. Nothing to be done. It’s just a matter of making sure the right eye stays healthy,” as well as other logistical info I’d heard before, though she told them differently than I’d heard it. One of the residents said, “Cool!” after peering into my eye with a slit lamp (at left), a contraption I’ve known all my life. It was both bizarre and amusing. Dr. Woodruff (who did his residency at Columbia Presbyterian, once upon a time) never told me what an oddity I was—but then, perhaps that’s not patient etiquette, if you don’t have a bunch of residents around to teach. The residents also seemed to appreciate Dr Schrier’s dedication. When they thanked me, I wanted to make them promise to be as caring practitioners as their teacher, but I kept my mouth shut.

Dr. Schrier thanked me again and again for giving them the chance to look. She took the time (not to mention my insurance) to tell me more about my condition. You might guess I ask a lot of questions, and she took the time to answer them. Being a textbook case, I was curious about what the textbooks said about PHPV, but I didn’t want to press her further. She did tell me to look it up on the internet, which not all doctors will do.

Of course, that’s the first thing I did once my eyes recovered from dilation. Mission for Vision has good info, with pictures. It appears that Dobermans get PHPV too, and you can get certificates to prove your show dog is PHPV-free. And, of course, there’s a facebook group: People with Persistent Hyperplastic Primary Vitreous (PHPV) Unite! In looking at all this, I realize that I’m quite lucky, as many people have more severe (& severe looking) cases. How strange that I can chat online with people who also have this, when I’ve lived my whole life without knowing another soul with the same condition. It also seems that doctors are doing more surgery on infants now, with little affect, which seems quite traumatic to me.

Looking at all the different problems associated with PHPV, I was hugely grateful that Dr. Schrier both took my insurance and got me in quickly—not many top doctors do. The doctors I saw in Oakland (there were two, in the same office) didn’t know my condition and thought that the retina was detached and needed surgery. The retina isn’t exactly detached. It’s not exactly attached, either, but it’s typical of the condition. Seeing docs who don’t recognize PHPV is painful, as well as a waste of time. Thank heavens that wasn’t the case this time around. And lovely I could be educative, as well.

other posts on phpv:
my perfect deformity
my perfect deformity, part ii
PHPV: the eye, vision, and how I see