Monday, February 14, 2011

Information Overload

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.

The answer?

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.

So, that's it. That's the plan for now.


GB's Mom said...

Sorry the news was not good. Still praying for healing.

JSmith5780 said...

I hurt for you, but it seems like the neuro has a plan for now and that's good.

I know I have asked before, but why can they not (why did they not) do an MST on the motor strip? This would keep the motor skills intact but *should* stop any seizures from spreading from there. I think you answered but I don't remmeber.

I am thinking of you all.

Holli said...

Jen -I don't know if you check back for comments, but...

I never did mention that on here. It was actually something I had planned on asking him when we went, but it totally slipped my mind (Austin was screaming the entire visit). It will likely be one of my first questions at our neurosurgeon follow up in March. They've never even mentioned it to me.

Jonathan said...

Not that ANY of this is really ANY of my business. But.

I'm PISSED that they didn't do an MST on the motor strip while they were still in his head. It was spiking. They should have been more aggressive. And I'm PISSED.

Also...get a MEG scan. Some neurologists believe it's as reliable as an invasive EEG. So instead of opening him up again and placing electrodes over ALL of his brain (which I think needs to be considered at this point to rule out right sided involvement) why not see what a MEG scan reveals and go from there?

Lastly...Trevor also has a type of dysplasia which is known to be wide spread. AND he has a right sided lesion which Chugani warned us could cause problems in the future. It was worth the risk to us. I know it sounds easy to say now that we're on this side of things and Trevor is doing good. But we took the leap blind. We had no assurances before surgery. The only thing we knew for sure was that meds weren't working and the odds of them ever working was slim to none. It made it worth the risk. I'm not saying you should go radical with Austin like we did. I really don't know what I'm saying. Other than I'm sorry. And my heart wants you to keep fighting for Austin. Especially if your heart is convinced it's all left involved.


IDK. And like I said...not that it's even ANY of my business.



Holli said...

Danielle -You hit the nail on the head. I'm BEYOND PISSED about this. I was told before surgery that everything could be safely removed. Maybe they didn't know the motor strip was involved until the electrode placement (maybe it wasn't actually showing that area on the MRI). But, I fully expected them to update me with any surprises during surgery -which they didn't. So I didn't know any of this until AFTER the fact. He was definitely having seizures while we were still in the hospital, but no one was concerned about it. They kept telling me it would settle down -and what he was having was still about an 80% improvement. It never even occurred to me they probably should have done the MST while we were there and could have had it addressed. It wasn't until the seizures started increasing that I started googling and read about it.

This definitely isn't over. I'm not settling for this. I KNOW he could be so much better...I saw that potential with my own eyes. I will never stop!

And thank you..for being pissed too! Now that two of you have brought it up, I feel a little more validated. They're supposedly #4 in neurology and neurosurgery in the country. Who woulda thought? They're obviously not considering epilepsy surgery.

Sophie's Story by Elaine said...

Ya know what...I am in the same boat you are with the MST. I'm not sure why they didn't do it on Sophie. It's a fine line. I wish they would have been more aggressive. But I am happy that they aren't so aggressive that they did a full hemispherectomy. So, it is kind of like...where do you draw the line. They always can always go back in and do more surgery but you can't put it back in. As with EVERYTHING with IS, it is always a guessing game. But with all that said, it doesn't make it easier. I'm still upset. Because I am so afraid that if they decide to do another surgery, they aren't going to just want to do the MST but take the whole motor strip. And I can't bear to think that. (BTW, it wasn't until our 19 months post-op visit that I found out there was rare spiking in the motor cortex that was picked up during the monitoring during surgery...I was under the assumption there was none.)

blogzilly said...

This post, and the comments by D and E, are infuriating to me. Why doctors do not inform patients of every conceivable detail and angle of every single contingency and every single aspect of this very complex thing is beyond my ability to comprehend.

We had docs like this, at Nationwide here in Columbus, who at least to me didn't know the difference between a seizure and someone being too cold.

I have to fight against two reactions...burying my head in the sand or burying a bat in someone's head. Neither are good. Or productive.

Life is pretty much sucking ass right now. My mom is the 'holy shit' kind of sick. Carter's 'problem'? They don't know yet if it's as simple as post Strep Glomerulonephritis or Henoch–Schönlein purpura (WTF!?!) or even...get this...LUPUS.

Last night, a rough one...a tooth in the back of my mouth cracked apart. Second time this happened. Dental insurance sucks, of course, and it hurts like a bitch. Can my unemployed, struggling ass even risk spending the money to fix it? Should I?

Then Bennett walks up to me and for no reason at all whacks me on the side of the head with one of his heavier toys. A part of me, I shit you not, just wanted to deck him. But it is not his fault. I was scared I destined to turn into one of my two fathers? The one that ran away or the one that beat the holy hell right out of me?

I should have lost it last night. Many nights. Wanted to.

In those moments I often think about you, D, Elaine, Sinead, Craig, Liz and all the guys are really the only glue I have left.

Your strength is MY strength. Your fight is MY fight. Any time I think I can't keep going into tomorrow, I read one of your blogs. It grounds me.

Hang in there kiddo. I've got so much pent up anger in me though, if you need me to come down there and break someone's kneecaps, lemme know.

JSmith5780 said...

First- Ken- I am so sorry things are so rough right now. I truly hope things turn around.

Second- Holli, Danielle, Sinead- MST is not new, they've been doing it for about 5 years. I think it just sucks that more neurosurgeons aren't using it. It's like the "perfect surgery". You make the vertical slices, not removing it. It leaves the brain function intact but stops the seizures from spreading across the region. Brain function(learning) passes vertically through the brain, seizures spread horizontally.

I'll be offline for a week or so, but I'll be thinking of all my friends and their little ones while I am away.

Holli said...

Elaine -Ugh! All this time I was thinking they DID perform the MST with Sophie. Have you ever asked them why they didn't? I just don't understand why neither did it or at least proposed it. Makes me wonder if there's more to it than it seems. I read it has a 70% success rate...seems to me it was worth trying the first time around since it doesn't involve actual removal.

Ken -I don't even know where to start! Doesn't it seem like just when you get to a manageable stress level, it starts pouring crap again? I'm so sorry to hear about Carter's health problems and that he too may be facing a rare diagnosis. WTF is right. It all just sucks.

By the way, I'm not far from wanting to bust some kneecaps myself. One more surprise and I'll be at my limit on the crap I can take.

Jen -Thanks for the extra info. I've read about it but didn't realize the differences in learning/seizures etc. It makes total sense.

Have an awesome week!!!!