Earlier this week was J.’s second vEEG (video electroencephalograph). We were placed in the PICU overnight and she was hooked up to all the wires all over again (I was bummed we didn’t get to stay in the same wing of the hospital--we really miss our nurses!). The hope was that there would be no sign of hypsarrhythmia.
Hypsarrhythmia is pretty unique to Infantile Spasms, though it can occur in a few other conditions. It is perhaps the most important key to diagnosing Infantile Spasms, as the spasms themselves can be hard to detect. If you think your child is having spasms, consult with your pediatrician immediately about whether an EEG should be ordered. When J. was hooked up to the EEG the first time, her hypsarrhythmia was immediately apparent. Even if we’d never seen an EEG before (which we had in our research) it would have been obvious that hers was a mess. Hypsarrhythmia can be described as “chaotic brain waves,” and they absolutely look chaotic. The Acthar website shows you the difference between a normal pediatric EEG and an EEG of a child with hypsarrhythmia.
Leading up to this appointment, I was sick with nerves. Our first week home with J. (her second week on the ACTH) was amazing. She tried to roll over for the first time ever and in just a couple of days she was able to roll to the right from back to belly. She was so happy, and smiley, and babbling non-stop. She was a completely different baby. It was crystal clear she felt a million times better and we were thrilled. She didn’t even cry when we gave her the ACTH injections.
After that week, however, as time progressed, she seemed to be doing a little less amazing. A zillion times better than pre-diagnosis . . . but she gained some more weight and kind of stopped rolling over, and wasn’t holding her head up as reliably. She wasn’t babbling as much. She wasn’t smiling as much.
On the other hand, she was gaining ground on sitting up by herself (still not quite there) and she has learned how to hold her own bottle by herself, which she only started trying to do since we brought her home.
Despite the progress, I was really concerned that the treatment wasn’t working. I had really noticed a difference in her behavior when we tapered her dose from everyday to every other day, and then to half a dose (.2 mL) every other day. I thought maybe the taper schedule was too dramatic. It's difficult to know when you're seeing something concerning, though, versus when you're just hyper aware of everything your child is or is not doing.
I should add here that some of the side effects of the ACTH that we were told to expect were finally starting show. As I mentioned before, J. has put on some weight (just a little, and since she’s such a good gainer it’s hard to say if she would have anyway), she is having a LOT of trouble sleeping--some days she only takes two 30 minute naps around 10am and 4pm, then goes to bed around 9pm and sleeps only until 5am or 6am. J. was always an excellent sleeper before, so this is really terrible for her and it’s obvious she’s tired. Plus, another side effect we hadn’t really seen is grouchiness. Pre-diagnosis J. was in a bad mood or asleep about 22 or 23 hours of the day (not an exaggeration). So if she is irritable now, and she can be, it doesn’t phase us. She’s still 400% better than before, so it seems like nothing, but if she’s in a bad mood because of the meds, that could explain why she’s not doing certain things. She may not have the patience to try.
So, anyway, back to the vEEG. I had planned on not looking at the vEEG at all, being that I was so nervous about it. Our first stay in the hospital, the screen was right there between her crib and the couch, so it was impossible not to look at it. It’s bright, white glow pried at our eyes at all hours of the day. Naturally, when I realized that the screen this time was on the back of a column of power outlets, I thought I’d won the lottery.
But the tech who came to attach J.’s leads needed my help finding a good position for the crib in relation to the camera, so she asked me to watch the screen. I couldn’t help but glance at the EEG, too. I had already steeled myself to defend against the debilitating disappointment that threatened when I saw exactly what I’d expected which was, while J.’s EEG looked inarguably much better, it was still nowhere near normal. At least, that’s what I thought. All I could do at that point was tell myself: “You are not an EEG tech, nor are you a neurologist, so you basically have no idea what you’re looking at.”
Which is true. Still, I braced for bad news from the neuro team when they visited the next morning. Imagine my surprise when our previously very grave epileptologist offered us the first smile of hers we’d ever seen. She said that J.’s scan looked great and that she had no spasms overnight (though she did have an episode of eye-rubbing that corresponded with spikes).
We’ve grown accustomed to receiving bad news and good news is now foreign to us. I pressed her. I asked about the spikes, and pointed out that maybe she’s not having spasms, but her EEG isn’t "normal." And what about all the regressions?
The neurologist said that if “normal” is 100%, then J. is at 90%. J.’s regressions are likely a side effect of the ACTH and she’d be concerned only if that pattern continued after the ACTH injections stopped.
Wow. 90 percent. 90% normal.
She also said (after I asked) that, yes, this kind of response to the medication after only 4 weeks is a very good sign.
Finally, a little luck.
You may be picturing us jumping up and down and celebrating. I doubt we even smiled. T. and I are “cautiously encouraged.” We don’t dare get too excited. We still don’t know what is causing J.’s Infantile Spasms and while they are now controlled (at least for the time being), another diagnosis is looming and we have no way of knowing how severe it will be. So far, J. has beat the odds and that excites us--don’t get me wrong--but the higher you get, the further to fall. We’ll celebrate after we have all the facts, if it’s appropriate to do so.
The neuro team told us that they feel they are close to uncovering the cause of J.’s IS which is both good and not good. No discernible cause would be better (cryptogenic IS), of course, though unsatisfying. A diagnosis will be more satisfying, but could go either way as far as being good news or bad news. They believe it’s metabolic, based on some abnormal levels of something-or-others (they weren’t that specific because they want to have concrete information to give us). One thing we know, is that J.’s carnitine levels are low. I made the mistake of Googling metabolic disorders that are associated with that, but after reading some terrifying stuff, I have decided to wait for the blood test results to come back before conducting more research.
So, today is J.’s last dose of ACTH. Hooray! Starting tomorrow, she’ll be on Topamax until she’s 8-10 months old, to keep her seizure free. Topamax has it’s own slew of terrible possible side effects but most of them are relatively uncommon. I’ll write more about Topomax in a later post.
Next time I’ll talk a little more about how J. (and T. and I) are doing 1 month post-diagnosis.
In the meantime, as always, if you have questions please e-mail me, or leave a comment!
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