Lily had her 24-hr EEG on Tuesday. I haven't said much about it because I wasn't worked up about it at all. However, it was WAY harder than I anticipated. I thought it would be relatively easy, I mean, it's just hagning out in a hospital room w/leads attached, right?? Not so much. Keeping the leads & cords untangled while lily was crawling around EVERYWHERE was a challenge! plus, she wanted to take the stocking off all the time whcih was a pain in the arse. Then they wouldn't let Hank spend the night, so he had to go to my mom's. It was the first night the twins have EVER spent apart. THey did OK...I think it was harder on me than anything. Lily had a very rough night, sleeping little. I guess it's hard to sleep w/metal leads all over your head. But, a sleep deprived Lily + stress of the situation = lots of seizures. I think she had over a dozen clusters in teh 24 hours (normally she only has 3 or 4), with many individual spasms in each cluster. THe good news is that this provided GREAT data on the EEG.
We don't have a final report, but what we do know is that these spasms are indeed spasms/seizures, and not some other kind of movement disorder or something. This wasn't surprising at all. What was surprising was that she doesn't have hypsarrhythmia anymore. She still has a very chaotic pattern, but it is no longer considered hyps. I can't remember the name he gave to the pattern she has now. The majority of the activity occurs on the right hemisphere, with a small concentration on the occipital lobe. This correlates with the findings of the PET scan, however her seizures do appear to be generalized.
We're in a bit of a tricky spot. We see Dr. Red Sox Fan, a pediatric neurologist. We've seen him since Lily was dx'd, and we love him. He is warm & gentle with Lily, and genuinely seems to be interested & caring of her outcome. He has come down to the hospital on days he's not normally there just to see us. However, he is not an epileptologist. Epileptologists are also neurologists, but they specialize in epilepsy (thus the name, natch).
The epileptologist who was doing telemetry while Lily was in was Dr. Bow Tie. Dr. Bow Tie is quite familiar w/Lily's case. He's the doc who confirmed that her MRI changes were likely due to Vigabatrin and not that awful mito disease, Leigh's (a terminal illness for anyone who wasn't here, or doesn't remember). Dr. Bow Tie is a an epileptologist, and he also specializes in metabolic/mitochondrial disorders. He's read many of Lily's EEG's & is also helping to write the paper on the vigabatrin side effects. So, that's his background.
This week was the first time we've ever talked/met face to face. And we LOVED him. He was VERY smart, knows A TON about epilepsy & infantile spasms. After we talked w/him on Tuesday afternoon, Todd & I were thinking, "hmm...why aren't we seeing an epileptologist?" Not that we don't adore & respect Dr. Red Sox Fan, but Lily's case is OBVIOUSLY complex &; difficult to manage; why aren't we seeing someone who specializes in this?
We talked w/Dr. Bow Tie more about Lily on Wednesday, and I finally asked why we didn't see him. He was cautious in choosing his words as he didn't want to disparage Dr. Red Sox Fan, or reduce our confidence in him, but it seemed pretty clear that he (& Nurse Wonderful, the epilepsy nurse practioner [who we also ADORE]) kind of thought we should be seeing an epileptologist, even if it wasn't him.
So, I think we'll be switching. I have to craft an email to Dr. Red Sox Fan because I'm SO BAD at face to face confrontation that I just can't bring myself to "break up" with him in person. I want to talk with Nurse Wonderful a bit more about how the transition will work, but I'm pretty sure we're switching.
In terms of what is next for Lily, I think we're still going to get the 3T MRI. Children's up here just got the 3T magnet so we won't have to travel to Portland for that. We're also considering a SPECT scan, but I think we'll discuss that more after we've made the change to Dr. Bow Tie. Both tests are really just more ways to search out the cause of the spasms. And to confirm what we all suspect is there -- some structural abnormality in the right hemisphere, likely in the temporal or occiptal lobe, of her brain. It may or may not make her a surgical candidate...that just remains to be seen. Dr. Bow Tie seems to think that if she were a surgical candidate, we'd be looking at a total hemispherectomy (where they'd remove HALF of her brain, YIKES), as that is what most kids w/generalized seizures have (meaning there is no one origination point; they start from the entire half).
We see surgery, especially a hemispherectomy, as being a last ditch effort. As such, we've put travelling to Detroit to see Dr. Detroit on hold. Dr. Bow Tie has also told us about a new drug that is in clinical trials that he thinks Lily is a perfect candidate for. Childrens will likely be one of the primary test sites too. It's a new neurosteroid that acts similarly to ACTH but without the side effects of steroids. So she could theoretically stay on it longer (she can only be on ACTH for ~8ish weeks before the s/e become too bad). Dr. Bow Tie feels it is a good fit because she responded SO well to the Israeli Protocol (long time ACTH high dose, followed by slow wean & prednisone). He didn't particularly like the rate/dosing of Lily's wean or prednisone when she did it. He thought it was too fast. Which, while he didn't say it specifically, implies that if we had stretched it out longer, she might still be seizure free. Argh. (and the schedule was drawn up by our beloved Dr. Young Neuro, who lives on a pedestal in my mind, so it must have been fine).
Attempting the Israeli Protocol again (UGH! ACTH for a THIRD time!!) is not out of the question either.
Basically, Dr. Bow Tie had lots of ideas of things to try, but seemed unwilling to reccommend/direct us to do any of them because Lily wasn't his pt.
Dr. Red Sox Fan told us to increase the topamax a bit, until our scheduled appointment w/him on Tuesday. However, I'll probably cxl the appointment if we're switching, depending on when we can get in to see someone else. Dr. Red Sox Fan doesn't have a lot of faith that a topamax increase will help, but Nurse Wonderful & Dr. Bow Tie seem to think it is a logical choice, with a decent chance of working.