What Is

We tried to take the weekend to rest, to regroup, to catch up. I'm not entirely sure we succeeded, but we tried. We spent time with friends, attended church and Olivia's field hockey game, caught up on work and homework, and we got as much sleep as we could manage.

Brian and I tried to talk about how we are coping with our new situation. We are worried about each other. We acknowledged the cosmic sense of unfairness we feel -- Simon does not deserve to be faced with so many challenges. He does not deserve to go through all that he already has, much less all he yet has to face. In his sweetness and innocence he has done nothing to warrant being subjected to so much frightening medical intervention, tests, drugs, and surgeries. He should not need to spend so much of his precious childhood in hospitals and doctors' offices. He does not deserve to have his hard-won abilities threatened by this monstrous disorder. He does not deserve to have his young life endangered.

We don't deserve to go through this. No one does. We are frightened, and angry, and overwhelmed. We tried to comfort each other, but in the end the best we could do was sit beside one another silently crying. Which, in its own way, was comfort enough for now.

But, being me, I can't sit still for long. Maybe one day I will fall back, but not yet. Now is a time for doing. So I am spending a lot of my time researching, and talking to multiple neurologists. We learned a lot in our meeting with Dr. Riviello and I need to figure out how to act upon it.

We learned that Simon's ESES is pretty severe and has most likely built gradually over time (how much time no one knows). I actually saw surprise register on the kindly doctor's face when he scrolled through the EEG and saw the unceasingly wild and wavering lines. He diagnosed Simon as having CSWS, a syndrome within the syndrome of ESES. Based on this, and on Simon's repeated status seizures (which the doctor was also very worried about), he feels we need to proceed aggressively with treatment.

This is what a normal EEG looks like during sleep

This is what Simon's EEG looks like during sleep.

The first thing is to try to control the epilepsy with more aggressive drugs and to frequently test their efficacy with overnight EEGs. If the more standard epilepsy drugs do not work, we move to high-dose steroids that are so heavy-duty they have to be given inpatient via IV for 3 days at a time. Ultimately, however, based on Simon's history of refractory (drug resistant) epilepsy and Dr. Riviello's experience with kids like Simon, the projection is that the effect of the drugs will not last indefinitely and we will most likely need to pursue a surgical solution.

Regarding surgery, we got more bad news when the doctor reviewed the MRI of Simon's brain. The best case senario would be to pinpoint exactly where the seizures are originating on the EEG, and then in reviewing the MRI find a corresponding area of damaged tissue which could be isolated and surgically removed without risking further harm. We do not have a best case senario. Although we know from the EEG that Simon's seizures are primarily coming from the right side of his brain, and though we can clearly see the damage done from the stroke, it is diffuse throughout the right side. Dead tissue is mingled with healthy tissue and we can't easily tell what Simon is using and what could be removed to help him without creating further injury.

So.... what do we do now? First, we start with drugs to try to control the syndrome as quickly as possible. We started this last week and are continuing to make adjustments and get bloodwork to test levels. Once Simon is at a therapeutic level we will get another EEG. If this works for now, great. If not, or if it stops working, we move to the steroids. I have already discussed setting this up locally with our neuro.

In the meantime, for the past 3 days I have been calling and emailing with multiple neurologists to try to find ANOTHER doctor to help us with the extensive and highly specialized testing that will need to occur (and which can take a very long time to do) to determine if Simon's epilepsy is operable and, if it is, to "map" that surgery for us and work with a neurosurgeon to implement it. Currently, I don't know where we will end up for that. We are discussing options in New York, Washington, Philadelphia, and Cleveland.

Right now, I don't have time to sit and cry silently. Though I think I have always been tough, and for a long time I have been trying to be a super mom (for both my kids) and an impassioned advocate for my special needs son; over the past two weeks I have felt myself evolving into something else entirely. Harder, more intense, more determined. A dragon mom? A warrior mom? I don't know yet. I don't quite recognize this more ferocious version of myself. Though I am reluctant to embrace her, I am also glad she is there. I think she will hold me together, and I hope she has enough strength to hold Simon, and Brian, and Olivia, too. I am frightened of what is to come, and how our world may change (has already changed), but I know I cannot stop it or change it or wish it away. "It is what it is" (a phrase I have a long history with) but also I know that I am who I am, and I am going to kick It's ass.

I hope.