Today my daughter had another long day at the hospital. It’s tough selling these visits to a four-year-old as beneficial, helpful, or anything other than something she hates and wants to avoid, but she does her best and we’re grateful most of the time when she’s wailing it’s when she’s partially sedated and doesn’t remember it.
Overall, I would call today a win. I would love to tell you we left the hospital elated to hear great news that her eyes are vastly improved and she should be catching flies with chopsticks in no time, but I can’t. What we did get was some good news and a possible direction towards a diagnosis. But let me start at the beginning of the day and get to that.
First, I want to say scheduling is no fun. Over the past several days I’ve spent time talking to four schedulers, sending emails to three doctors and being both frustrated and grateful at the same time for what we were hopeful would happen today. My daughter needed a brain MRI to confirm there have been no changes in since her September hospitalization scan. Some white spots had been present, which could be normal, but to rule out anything eye-related, they wanted to do a re-scan. We wanted that scan before we went to Detroit and we only had one time radiology could do it—this morning first thing.
Then there were our eye doctors. We have two main doctors who have to coordinate their OR schedules to begin with but this time they wanted to add a third specialist. The only way they could coordinate this (since different procedures take different times) was to have my daughter as their first cases.
Both of these things needed to happen before we went to Detroit on Sunday and it looked like it wasn’t going to happen. I can’t tell you what went on behind the scenes but I’m happy to say it did all happen today. When we got to the OR after my daughter’s MRI we found out that an operating room had been reserved for her since the beginning of the morning. That OR had sat empty for three hours while they waited for her arrival from radiology. I can not tell you how grateful we are for everyone at Duke who made today work.
After the procedure we spoke with two of her doctors. Here’s what we learned:
Remember that Anterior Segment Dysgenesis or Persistent Fetal Vasculation? It’s those vessels in the front of her eye that brought us to the hospital in March and gave us a “normal” baseline for everything else. They were causing her to see 20/60 distant vision. After all this, those little vessels have shrunk to mostly nothing. If nothing else were happening in her eyes, she’s see fine at distance now.
Her eyes are still significantly detached. The right eye is detached more than the left and the left eye is about 50% detached. There is improvement in the detachment percentage overall though, mostly in the peripheral areas. The macula (main focal point) is detached in both eyes and in quite wrinkly which makes seeing anything of detail very, very hard. (Which we observe daily.) The reduction in detachment is largely due to the improvement in the amount of fluid behind her retinas. In addition, the tractional detachment at the far edges of the retinas appears to be almost resolved.
We have pictures of her eyes in which we can see the “folds” of the detachment. It is quite dramatic (scary) looking. These aren’t little wrinkles like crows feet around your eyes. They’re more like a a Shar Pei dog has folds in his skin. How she’s seeing anything at all is amazing. Serous detached retinas aren’t flat, but the amount of folding she has more than would normally be expected. It could be in part due to the low ocular pressure she’s had.
So the question remains, what is acting on her eyes? Is it immunological in nature? There is a new idea the doctors are considering and will be working with Dr. Trese (pronounced “Tracy”) in Detroit. Could the issue be mechanical in nature?
We had been told before that my daughter has small eyes. The name for this condition is Micropthalmia. There are all sorts of other things that will scare the pants off you if you go reading about Micropthalmia on the Internet, so don’t. Just read on here because we’re not going to have all that nonsense with my daughter.
Today for the first time they measured my daughter’s eyes to get a good understanding of how her eyes are not normal. She meets the criteria of abnormal in three ways: 1) her corneas are small, 2) her lenses are round instead of elliptical and 3) her eyes themselves are small. To what degree or percentage below normal ranges I don’t know but the amounts are measurable.
Then there’s the issue of ocular pressure. It’s been low—too low. The eye needs structure and there needs to be a certain amount of pressure in the eye to hold it up, just as you need enough air in a balloon to keep it firm. If the fluid producing cells aren’t producing enough fluid, the normal draining process in the eye can remove the fluid faster than it’s created. Today our doctors looked at those fluid producing cells again. The good news is they look normal and intact.
So what does this all possibly add up to mean? One theory is that there is a leak. Fluid that should be staying in the eye is leaking through the choroid layer which could have possibly caused the detachments and is continuing, preventing her eyes from healing. One of our doctors told us today that human eyes start from a single cell that divides initially divides and that her onset happening at almost the same time in both eyes could make sense even. It’s a theory, but it’s one they’re going to investigate more, including treatment paths to help get her eyes back to where they can manage themselves.
Overall, a good day I think. I am excited as I write this because I believe we’re getting close to an answer and a solution.
The Big Boy Update: My son informed everyone at the table, “when I go potty by myself I just don’t wash my hands.”
The Tiny Girl Chronicles: Hospital day. My daughter had three different sedation medications and she didn’t wake up better at the end of it as a result. Let me tell you something: an incoherent, screaming, floppy child who demands to be put down is quite the challenge. Today my daughter moved on to other, more aggressive ways to tell me she’s angry other than “you’re poopy!” When I didn’t comply with her irrational demands today she slurred at me, “put me down or I’ll punch you in the face!”
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