Today’s surgery will add a device on the surface of my daughter's eye to control her persistent high pressure, or glaucoma. The device will be permanent and will remain in her eye indefinitely. It will help drain fluids since her left eye is unable to do so naturally. The implant's job is to allow fluids produced by my daughter's ciliary bodies to drain via that shunt, if and when her pressure is high. It intelligently drains fluid as needed and will keep her eye at normal pressure. In normal eye function, the aqueous bodies produce fluid continually and the eye maintains normal pressure by draining fluid over time. Not unlike how we produce saliva continually and swallow to maintain a comfortable level in our mouths.
In the fall of 2015, my daughter got an infection in her eyes that triggered a series of catastrophic events. Dr. Trese theorizes that the infection caused damage to the ciliary bodies, causing them to stop producing aqueous fluid. Both of her eyes dropped to zero pressure, her retinas collapsed inward and fluid filled in behind them. Her retinas sustained significant damage over the next several years. The detached retinas weren’t the typical detachment and couldn’t just be reattached. It took a long time, but eventually most of the retinas reattached. That’s just the retinas. There were other things going on in her eyes due to malformations that contributed to her loss of vision In all of this, the one thing that seemed to recover in her eyes were those fluid producing ciliary bodies. They’re now happily producing fluid, but unfortunately her eye is no longer able to drain it out in a normal fashion.
In a way, that could be a good sign, since something in her eyes recovered and regained function, maybe other areas of her eyes could as well. The ciliary bodies are only one small piece in the overall puzzle that is my daughter’s eyes and many, many more things would have to occur for her to have significant or even minor vision to return.
Today’s procedure is called a Pars Plana Implant in which a device, in this case an Ahmed Glaucoma Valve with Pars Plana Clip is inserted under a flap of the sclera, or outer, white area in the eye. The Pars Plana part refers to the area of the eye it will be implanted, Dr. Freedman will create the flap and insert the device in an area above and behind her pupil. Then Dr. Freedman will insert a shunt into center portion of her eye so fluid will have a way to drain out. The device looks like this:
It will look like this when inserted in her eye:
Dr. Freedman recommended my daughter stay home from school for the remainder of the week and stay quiet and calm if possible. She also said she didn’t think my daughter would be in much discomfort, which is good, because I thought this would be worse than the cataract surgery. When I mentioned it, Dr. Freedman said cataract surgery stitches were the worst and that this will be higher up and further back and likely won’t be as uncomfortable to her. She’s still in surgery as I write this part of the post so we won’t know until later today.
Restrictions for my daughter are significantly less than I expected. She only has to wear eye shields at night for a week and after two weeks she can resume normal activity. For two weeks she’ll skip P.E. and will need to sit on the side during recess to make sure she isn’t run into by other children that might jar her eye. Dr. Freedman said she doesn’t expect her much in the way of vision change from the procedure as she's working on the exterior of her eye aside from the shunt. My daughter has been using the minimal vision she has a good bit lately and relies on it to move in space and avoid obstacles (which she is able to discern some of the time) so keeping that vision intact would be very helpful.
My daughter is out of surgery now and we’ve had a meeting with Dr. Freewman on how it went. The implant went well. Her pressure was a high 47, which is in the range we had been consistently seeing for months now. Dr. Freedman opened a flap in the far back of my daughter's eye in such a way that the only stitch would be at the far back of her eye, in the center of the c-shaped incision. Because my daughter's eye was extra small, she had to trim the shunt. My daughter has microphthalmia which literally means 'small eye’. Dr. Freedman has a very precise way to measure and shorten the shunt and made sure it wasn’t close to the optic nerve when inserted.
We’ll see tomorrow if there is any discomfort or change in vision when my the bandages come off after staying on overnight. Dr. Freedman added healon to her eye. Healon is thick, sort of like honey in consistency and will permeate out over the next day or two. She also added a bubble of air to her eye but that, too, will dissipate quickly.
There are some questions we have to wait to find out the answers to. First of all, will the shunt be able to drain fluid fast enough to keep my daughter’s pressure in normal range? The tube is tiny, but the biggest that could go in. Since my daughter had fluid production problems in the past, we’re hoping the shunt will be enough keep her eye at normal pressure. If not, we’ll have to continue some of the drops we’ve been doing which will slow fluid production in the eye.
Tomorrow we go in to have the bandages removed. Her pressure could vary wildly from two to forty-two as the first day there is a lot in play with the added fluids and the process of surgery. So question one is have we solved the glaucoma problem? We won’t know tomorrow and likely won’t know for sure until we have some data collected over the next month or two. Fortunately we’re in a study at Duke and have a tonometer at home and will measure her pressure regularly.
The second question is will she have any return of vision. This question is the one I don’t want to write about because I’m fairly upset about it. Early last spring my daughter started having a decline in vision. And by last spring I mean 2018, not 2019. Since then we’ve been slowly doing things to try and address it. In retrospect I don’t think we moved quickly enough when we realized there was a problem. Some of this is because it’s hard to get a pressure reading from my daughter but not all of it. We knew something was going on. We didn’t realize the decline in vision was truly pressure-related until much later. We tried medication, hoping, I think, that it was temporary and would resolve with time, especially given that she’d always had low pressure. When that didn’t work we added more drops, then we increased the frequency of the drops. Then we added a pill. Then we increased the frequency of the pill. And we kept on, until it was clear—far too long later upon reflection, that nothing was working and my daughter had a silent vision killer—glaucoma—that was taking the precious little vision she had left.
It was at that point that we all agreed it was time to consult Dr. Freedman, a pediatric glaucoma specialist at Duke. With one visit, we had a plan to put in this implant but that, too, took a long time with one surgery with Dr. Trese first, then our vacations and finally, today with the implant. It’s taken it’s toll though. I clearly remember Dr. Trese saying her optic nerve looked healthy (for the most part). I’m not going back through my surgical notes right now, I have too many people who want to hear how today went, but he never said the optic nerve was significantly damaged. Today, that’s what Dr. Freedman told us.
She also told us it was almost without question because of the glaucoma. I knew glaucoma caused irreparable damage. I knew she had ongoing, unmitigated significantly high pressure. So why didn’t I push for more to be done? Our doctors are wonderful, I don’t fault them. My daughter’s healthcare is in my hands, I must be her advocate. But I didn’t advocate fast enough in my opinion. That damage is irreversible. The vision she’s lost is gone. There is very little chance we’re going to get anything better vision-wise than we have today—and what she has is so very, very little. My daughter didn’t need to lose more. Possibly we could have saved some if I’d taken more action, been more insistent we look into other options, not wasted time hoping things would just get better. Possibly.
Dr. Freedman is a realist, and I like that. She said observed a dip in the optic nerve area, sort of a cup-shaped indentation. Think of the optic nerve as a pipe filled with a million wires. When any of the wires are damaged, they shrivel up and die. And a lot of hers have died. The indentation indicates a lot of the optic nerve has shriveled up and died. I mentioned Dr. Trese’s regenerative medicine and she said that indeed, there could possibly be hope, but that’s not today. The regenerative medicine is also more for the rods and cones of the retina, not for the optic nerve. Our optic nerves are a part of our spinal cord and when the spinal cord is damaged, it doesn’t recover. Is there a chance? Yes, she said there’s always a chance some healing might occur, but realistically, the vision she has today will probably be the best she’ll ever have again.
We don’t talk to my daughter any more about possibly getting vision back, because with her eyes, it’s not likely. We talk about not losing more vision. Dr. Freedman said this was definitely the right decision, going with the implant. I hope it works and we have no more pressure issues in her left eye.
Some good news though was that she encountered very little scar tissue when she made the “Scleral Patch Graph” which means lifting up a piece of the sclera, or white part of the eye, and putting the implant under the flap before stitching it back down. The vitrectomy Dr. Trese did in which he removed all her vitreous also looked good. Although there is a small bit of vitreous he didn’t remove. She understood why he didn’t when she saw it as it looks like removing it might cause the retina to detach in that area. If, however, that piece of vitreous ever breaks off (and this can happen) it could possibly block the shunt and prevent it from doing its job of draining excess aqueous fluid. It’s a low probability, but something to look out for in the years to come. One thing that’s good is my daughter’s cornea. She has a malformation where some of the outermost edges are shortened, but overall the cornea is in good shape.
We talked about my daughter’s right eye, what with the left eye looking like it has no chance for a return of vision. In its current state, my daughter’s right eye as internal scar tissue that completely occludes her ability to see anything. We don’t think her retina is functioning so opening that scar tissue again (Dr. Trese has done this three times only to have it grow back) would be moot and is a risk not worth taking. One thing I did find out though was that if there is ever a chance for her right eye to gain some vision back, possibly with the regenerative medicine Dr. Trese is working on or stem cell implantation, there is hope she will be able to have her brain understand what her eye is seeing. A child born blind, or a child that looses vision very early misses out on the brain mapping of vision, but because my daughter had sight up until four-years-old, her brain has mapped those visual pathways and would be able to interpret what her eye sees. It’s a huge long shot with a lot of variables, but I’m not completely, totally writing off the right eye forever. For now though, her right eye sees nothing, including we think even light perception.
Dr. Freedman was finishing up talking to us when we both got a phone call. My son fell from four feet and had suffered hyper flexion of his wrist at parkour camp. They put him on the phone and he sounded scared and about to cry so I left my husband with my daughter and I headed off to get my son.
I had him checked out and he doesn’t have a any dislocated bones in his wrist and all signs appear nothing is broken. It’s bothering him badly though. My chiropractor recommended having him put his wrist in a bucket of ice water for two minutes every few hours to reduce the already visible inflammation. My son hated it. It hurts—a lot—but he made the two minutes. I think we’re going to have both children home tomorrow, convalescing.
Back to my daughter. She woke up after surgery and you wouldn’t know anything had been done to her eye. It’s bandaged, but otherwise she’s home and is her normal self. I’m asking her to remain calm and not do anything vigorous, which is hard for her. We’re going to make some rubber band bracelets I think in a few minutes when she wants to take a break from her audio book.
Long, long post. Thanks to everyone for the words of encouragement and well-wishes. As my son told me in the car today when I explained to him about his sister’s vision outlook for the future, “I think Reese is a better person because she’s blind. I think she looks great with her glasses on. And she’s smart. She’ll overcome a lot and can do so many things, even though she’s blind. She even found me the other day when we were playing hide and seek even though she couldn’t see."
The Big Boy Update: As my daughter and husband left to go to the hospital this morning my son uncharacteristically said, “Goodbye Reese. I love you."
The Tiny Girl Chronicles: My daughter likes to sing on the phone to Mimi. She makes up songs and tunes as she goes and many times it’s a way to find out how she feels about things or how she sees the world. My mother told me the other day some of the words she sang were, “I can do difficult things, even though I’m blind.” “I am a wonderful girl.” “I am beautiful.” It warms my heart to know she doesn’t dislike herself and thinks she’s capable, despite the blindness.
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