Category Archives: phpv

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

top doctors

So, I went on a bit in the last two posts. I want to hit a few points that might have been lost in the story. One, I’ve noticed that most of the people in online forums talking about PHPV are parents of young children who are horrified by this condition. Of course, we all want our kids to be healthy. There aren’t that many people who actually have the disease chatting about it, and that’s because we are used to it. It really isn’t that big a deal. I see that docs are doing surgery on babies’ eyes now, and the only advice I can give a parent is to be really, really sure that it’s necessary, because often I don’t think it does any good, and honestly, other than some fear around having only one good eye, and having to wear glasses (which I don’t even think about, really), the worst part of the condition as a child was all of the doctor visits (before Woody, of course). My eyes are extremely sensitive to light, and having light blasted into them is very, very unpleasant. (This is why my prescription shades are on even in winter.) Not to mention the doctor talking to the parents in a grave, hushed tone. Scary.

As for my last last few check-ups, Dr. Amilia Schrier (I’ve seen it spelled Amelia, too) explained that if I saw bright or flashing lights, or worse, a very obvious darkness, as if a veil of gray has been pulled over my eyes, I should get to her immediately because the retina is starting to detach. So, if that should happen to you, make haste.

My other great doctor experience was with an orthopaedic guy I found over a year ago, believe it or not. (According to my doc friends, orthopaedic surgeons are the most egotistical and jock-like of all MDs). Dr. Dermksian, however, listens, is patient, explains everything without condescending, has never offered or prescribed me drugs (always a plus in my book), and is the most prompt doctor I’ve ever seen. And he takes my insurance. I’ve seen him on different occasions for a foot condition (sesamoiditis) and patellar tendonitis. When I asked him about wearing a knee brace, he told me it’s probably more helpful psychologically than anything. I appreciate that. Why my inflammation occurs in the tendons holding my sesamoid bones (the patella is a sesamoid bone, as well as the sesamoids in the feet), we’ve no idea.

Yes, sesamoid does come from sesame seed.

There’s also an amazing dermatologist in that building (1090 Amsterdam), Dr. Robin Buchholz M.D. I saw her years ago because of a weird spot on my leg I feared was skin cancer (it wasn’t). She was recommended by a friend, for good reason. She’s just lovely.

So there. The good stuff. None of the horror stories so easy to come by. Some doctors out there do care about our health, though the system does make that difficult for them.

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

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