We had that all important appointment with our epilepsy specialist this past Friday. Unfortunately, I didn't get the news I wanted to hear.
We talked past, present, and future. I did get the information I was seeking, just not what I was wanting.
The first burning question was WHY WHY WHY things aren't playing out the way they had originally thought immediately after surgery.
Let me start by saying that we are dealing with lesional epilepsy. There was a clear lesion on Austin's last three MRI scans. In cases of surgical intervention with lesional epilepsy, the most common predictor of a successful or favorable outcome is the ability to remove all affected areas depicted on imaging (or MRI). Failure to resect the entire area of abnormal tissue shown is the most common reason for the surgery to fail or not produce a favorable outcome.
Austin had an incomplete frontal lobectomy -meaning the entire lesion as seen on the MRI was not removed. They could not safely remove the entire area without causing harm (right-sided paralysis). At the back of the frontal lobe lies the motor strip (this is where the motor function controlling his right side resides). Removing that removes his ability to move his right side. Even though that area was clearly spiking (favorable for seizure production) during electrode placement in surgery, they felt it better to leave it alone. Austin is quite delayed in every area. However, his mobility is one of his stronger areas. Even with a clearly defined lesion (abnormal area) on his MRI, a deficit such as paralysis would be devastating to a child with so many other "problems". They wanted to leave him with this function and see if removing the other area would be sufficient. They also did not want to take the chance that there could be more extensive abnormalities causing seizures that MRI was not showing, and removing that would mean causing paralysis and still not stopping seizures. I did not even consider this scenario, and it was not explained to me until I asked him point blank, Why? What is more devastating...a motor deficit involving one side, or daily seizures affecting his development? He definitely answered my question. More devastating would be paralysis on one side AND seizures.
They also felt that the areas that were removed were so significant that they anticipated a huge improvement. They felt it was enough to make a huge impact, and in the case that it didn't, we could always go back and take another look. The areas removed could have been enough to suppress any other abnormal areas from producing seizures -or it could have the opposite effect...the removal could make any other areas take over and produce seizures. This is what he feels happened.
We talked about other scenarios as well. Austin has confirmed Focal Cortical Dysplasia Type II (or FCD Type II). This type tends to be more extensive and widespread. It is the leading cause for intractable epilepsy. It could be that the only remaining area is the motor strip and removing that (causing paralysis on his right side) would stop the seizures. Or, it could be multilobar (involving other lobes of his left side) in which a total hemispherectomy (complete removal of the left side of his brain) would work. However, there is also the possibility that it is in multiple areas ALL over his brain -right hemisphere as well. That is the worse case scenario because that would mean surgery would never be an option.
Not that we are talking surgery again. I did want to know ALL our future options though. It's not completely out of the question later on, but there would have to be more convincing evidence ruling out anything that suggests his dysplasia is bilateral (both side of the brain). Right now, though, all we have is the MRI which showed the lesion to be contained to the frontal lobe. It may or may not be in other areas and is just not showing up (like his previous "normal" MRIs). We just can't take the chance that it is far more extensive in which case another surgery could be unsuccessful. My heart could not take that again.
In my heart, I do not feel it is bilateral. Although my eyes are clearly not enough to validate this, I truly believe it is only the left side. I could be wrong. What do I know? But, if seizure appearance really does play an important role in determining which side is affected, it is clearly only his left. I don't think I have ever seen a left sided seizure. Sure, he has generalized seizures, but almost all the seizures I see definitely have more of a right sided appearance. I've pointed it out numerous times. And when I had no clue there was left frontal lobe dysplasia, I knew something was going on with the left side. Even when there was absolutely no other evidence to suggest this. They are more right sided now than ever. And he has a strange new behavior that involves picking at his right thumb (always associated with either seizures or seizure behavior, aka the zoning) in addition to still rubbing his right hand before/during/after seizures and/or clusters. I feel just as confident as I did before, but I'm leaving it at that for now. It has only been one month since surgery and we are in no place to be considering it again. Our wounds are still too fresh to even really go there. Not to mention Austin is still healing and will need much more time before another surgery is even an option anyway.
We discussed our present options. We don't have any new meds to try. We didn't get a good start on the LGIT (low glycemic index treatment), thanks to the steroids wreaking havoc on everything. Which reminds me...the steroids, although showing some success (drops are down and there are extended periods with no seizures at all), cannot be a long term treatment. There are too many health risks and side effects associated with them. When treating seizures, they are only meant to be short term. Either they will stop the seizures or they won't. Improvement is not enough. The only thing worse than a med not helping is a med that helps that has to be withdrawn. That is where we are.
Since we have no more meds to trial, we start over. He asked me which ones I would like to give another try -ones that I thought might have helped. He said there is a possibility they will help now -due to the time that has passed and the areas left from surgery may be more responsive. The only ones that came to mind were zonisamide (Zonegran) and Depakene. We have a long history with the Zonegran and I really didn't want to go back there (we finally got him off after two years of trying last fall). He was on Depakene for about six months back in 2009. During that time he had no drops at all. I always blamed the vigabatrin for the drops since they started when he was first given it and stopped when he got off. During the vigabatrin wean, we added the Depakene. It wasn't until a month after stopping Depakene that the drops came back. Our lives were so much different before the drops came back. I really leaned more toward starting Depakene again first, but he settled on Zonegran. We started him back on a decent dosage. No wasting time on this trial because I was adamant that I didn't want to draw it out again. If there is not significant improvement within a couple weeks, we will go to Depakene. Hopefully, we will start the clonazepam wean sometime soon when we're not making other changes. I'm dreading that one, but there's absolutely no reason to have him on it. The only reason he is still on it is because it will be a very long and difficult wean and there have always been other changes taking place. I'm going to ask if we can maybe to a little at a time in between other changes. The key is to not make simultaneous changes. Which is why we decided against the LGIT for the moment. He said we should keep it in our back pocket for now and rethink it if these other two meds fail. There's also the ketogenic diet we can consider again since it did have an effect. No comment on that one for now.
We will go in April for another follow up and an MRI to check that his brain is healing properly. Then, we will have an EEG.