Tag Archives: ophthalmologist

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

I’ve been meaning to write this post for months—since September of last year, really. I’m only getting to it now because I’ve had some horror dentist experiences in the past few weeks (there is nothing wrong with my teeth, I might add, other than one front tooth was busted in my youth and poorly fixed. I do mean to get around to fixing that, but it’s just not a top priority). But because I’m all too quick to rant about our (american) health care system, I’m going to start by relating a few good experiences.

Last fall I noticed floaters in my vision. I only have sight, for the most part, in my right eye because of a birth defect called persistent hyperplastic primary vitreous (PHPV) in my left. It’s fairly rare. It happens in utero, beginning around the third month. The eye forms with a gel that turns into vitreous as it develops. With this condition, for some unknown reason, the hyaloid artery does not recede and the gel instead keeps growing and becomes scarred. My doctor explained that it hardens into a stalk, which causes all sorts of structural problems. A few include the stalk pulling on the retina, elevated eye pressure, a smaller size (microphthalmia) and orientation of the affected eye, band keratopathy (calcium deposits on the cornea), cataracts, and extreme light sensitivity, though the scarring prevents enough light from getting to the back of the retina. Because the eye is so malformed, there isn’t much that can be done for it other than to protect the vision of the good eye.

I saw a lot of ophthalmologists in my childhood, and wore a patch for awhile in hopes of strengthening my left eye. It didn’t work. Now, I can see a bit in the left eye, but it’s mostly peripheral vision on the far left. Because of the calcium deposits on the right side of the left eye (which appear to be on the left side of the eye on the right in the photo), I see more out of the left side. The right is just fuzzy. My brain has trained itself to see the periphery with this eye. If I focus hard and try to get full vision with both eyes, I see double, as the left eye is tilted up a bit, as you can see in the picture.

I adored my childhood ophthalmologist, or my adolescent ophthalmologist I should say (Dr. Todd Woodruff MD), who looks quite a bit like David Letterman. I was transferred to him when I was about 11 to be treated for elevated eye pressure, which was occasionally called glaucoma. I was always the only patient in the office under 70, as it’s not a young person’s condition. Regardless, he was much more patient with me than the pediatric ophthalmologist I’d suffered for my first decade of life. After a horror experience with an eye doctor in Oakland during my college years who told me I needed surgery because of a detached retina (I didn’t), and suggested cosmetic work to make my eyes look more alike (it’s fairly risky to even wear a contact lens when one has vision in only one eye), I didn’t trust anyone else with my eyes for a long time. Though inconvenient, I would try to make appointments with Woody, as we called him, when I visited my mother.

This got a bit tiresome scheduling wise, especially as my insurance didn’t cover him. And when my mother started visiting me, I stopped going to the eye doctor. Bad. So, in September, when I started seeing floaters, and coincidentally read in an unrelated blog that it can signal retinal detachment and potential blindness if untreated, I logged on my health insurer’s website to find an ophthalmologist I might trust. Oh dear.

This is getting a bit long. I’ve not even gotten to my point. Even so, I’m going to continue tomorrow.

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