2 Weeks Left on Sabril

Rayleigh Bug is now down to her last 2 weeks on Sabril (Vigabatrin). She will be on this medicine for a total of 2 months, give or take during the weaning process, when it's all said and done.

Last week Bug had her mandatory vision test. Eye exams are required by the FDA for any patient taking Sabril. For Bug, because she's only on the medication for 2 months, she was required to have one during the medication period and then she will have to have a second one done in August - a few months after she's done with Sabril. These eye exams are required because Sabril has several different side effects on vision.

The most common side effect on the eyes from Sabril is the loss or decrease of peripheral vision. If there is a decrease or loss to the peripheral vision due to the Sabril then it is permanent damage and will not be restored.

Other side effects include damage to the rods and the cones in the retina (that read light and darkness, black & white and color), overall decrease in vision and total loss of vision.

So, back to Bug! Last Tuesday she had an ERG (electroretinagram) on her eyes. Her pediatric neuro-opthomologist had her under anesthesia and placed small electrodes on her eyeballs. She sits in a dark room for 30 minutes to get a base reading on her rods and to calm the entire retina. Then they have a red light on to see how her eyes process that. Next is a soft white light, then a brighter white light, then a strobe light.

The test itself was really easy on Bug. She wasn't sore or anything for the electrodes and she wasn't nearly as groggy throughout the day like we thought she would be.

The ERG has to be evaluated by a trained person, computers cannot read it - yet, and it takes a while so we had a follow up appointment with her ped neuro-opthomologist (what a mouthful!) for that Friday.

During the exam Dr. Mike and his nurse used different lights and contrast tools for Bug to recognize and track and she did better than she usually does so that was enlightening! They also noted that her nystagmus has really calmed down, her eyes didn't shake once during the exam. It's very occasional now.

Dr. Mike explained that Bug's rods in her retina are responsible for seeing in the dark and seeing black and white images. Her rods are at 100%, so they're perfect and not affected by the medicine at all!

Then he said that her cones, responsible for seeing light and bright objects, are 1/3 to 1/2 depleted. He said that this could absolutely be caused by the medicine. He's not able to tell us for sure because she didn't have an ERG prior to starting Sabril so he doesn't have a previous test to compare these results to and see if it's something new or was always there. We really hope that the depletion was caused by Sabril because if it is then there is a good chance they will heal back to 100% after she's done taking Sabril.

We have an appointment with her ped neurologist this week and our main question to him is this: Bug has had increased headdrops, at least one a day, for the past week now. She is also cutting teeth bad and getting a small fever about every other day that we are treating with Advil. That being said, should we stay at this level of Sabril for the last 2 weeks or wean it off in case it damages her eyes further, or should we increase the dose for the last 2 weeks to control these head drops?

I Spy With My Big Blue Eyes

Today Rayleigh saw a pediatric neuro opthomologist. In short, his job is to link any eye problems to neurological issues. There are only a couple in our state so we have had this in our calendar for a while now. Previously, we had been excited and ready for the day to arrive, but all this week I just got more and more nervous! You just never want anything to be wrong with your child, and on the flip side, you also want to be able to fix whatever is wrong!

So we get there and the technician takes us back to the exam room and asks a few questions (was she full term?, what concerns do you have?, etc) and she starts some small tests on Bug. For instance, she has a rectangular board that is half painted solid gray and the other half has black and white contrast stripes. Rayleigh Bug had just woken up from a nap and wasn't participating in this at all. Then the technician pulls out this large spinning wheel and the entire thing is black and white stripes. Bug would look at it and look away. I started getting nervous again for my baby girl :(

Then Dr. Mike comes in and does the same tests and uses a light also. Then he and a nurse get some drops in Bug's eyes to dilate them. After about 20 minutes her eyes are dilated and we go back to the exam room to see Dr. Mike. He looks in her eyes with a light and he held little glasses lenses up to her eyes.

Dr. Mike finishes with Bug and we soothe her and Dr. Mike lets us know that she has a slight astigmatism that he prescribed glasses for her to wear only a couple of hours a day to help correct that over time.

Then he lets us know that Rayleigh's vision issues derive from her seizure activity. They are starting in the focal region of her brain causing delays in vision. He is confident that she will improve a lot if/when we can get her seizures under control.

She has Cortical Vision Impairment (CVI).

Her eyes are just fine, it's just the part of the brain that reads what she sees that is having the issue. This is good and bad news. Good, because there's nothing REALLY WRONG with her but bad because this isn't just something we can fix.

We'll have to work even more with Rayleigh when it comes to looking at us and things around her. A lot of high contrast is great for her. I should try and get her to notice me and track toys without making noise to follow or let her know where I am.

We go back for a check-up with Dr. Mike in 6 months. He said he will be very surprised if her vision hasn't improved by then!