Wednesday, January 24, 2007

Early Intervention

Today we had our annual IFSP (Individualized Family Serivce Plan) for Lily's Birth to Three program. We're required to meet annually to review Lily's progress & reassess her goals. In preparation for our meeting, I had Michelle do a screening test on Lily to get an idea of how big her delay is, and to see how far she'd progressed since we last did it (last May).

When Lily was first diagnosed & enrolled in EI, she was six months old and tested at about a 4-month old level (which made sense, because she was four months old when she started regressing & was diagnosed). Last May, when she was in really bad shape we did the screening she was at a 1-2 month old level.

Now, she tests between a 9 & 14 month old level, depending on the skill. She's highest in her adaptive skills (mostly due to self-feeding), and lowest in her motor and...something else, I can't remember.

Anyhow, that laid the ground work for today's meeting. The main points coming out of today's meeting are that we're adding occupational therapy to Lily's treatment, every other week. We're going to try & add a 2nd physical therapy every other week (while continuing weekly PT), but they have to get approval for funding on that one. We'll continue with speech therapy weekly.

Also, we had a great discussion about which group was best for Lily. The baby group is moving from 9:00am Fridays to 10:30am Fridays. The toddler group is moving to 9:00am on Wednesdays. It is really hard for us to be up & somewhere by 9:00am. I have late sleepers. Plus, my mom has Fridays off & generally helps with playgroup. Also, two other mom's in the baby group have babies with epilepsy (one is even another IS baby), and they are obvious good support & connections for me. However, in the fall Lily will be eligible for the preschool through EI & I want her to participate in that, and if moving to the toddler group, as a prepartory step for preschool, is the best for her then that's what we'll do.

Leah, who is our (new!) OT and also runs the preschool & groups said that she didn't think there was that much of a huge benefit to moving up to the toddler group especially considering the logistics & practicalities of me getting to a 9:00am group by myself, and the social support for me. So we decided to go with the Friday baby group and reevaluate if Lily needs more "challenging" group play around Spring Break time.

Overall it was a good meeting. I'll be able to post more about our specific goals once I get my copy of the IFSP.

About the 24hr EEG

I wanted to share a few pictures of Lily's 24hr EEG.

First, Lily has the leads placed on her head & they're "glued" on with this foul-smelling glue. Lily was wrapped up snug as a bug in blankets so she wouldn't rip the leads off. She loves being wrapped up like this & nearly fell asleep a couple times.

Then, her entire head is wrapped with gauze (to keep tiny fingers away from the leads!), and then a "stocking" is placed over the gauze which protects even more & keeps the wires in a tidy bundle.

They take a baseline EEG in the prep lab (where they do the short EEG's), and then they moved us up to the telemetry unit where we settled in for the night.

Managing Lily with all the leads attached was much more difficult than I thought it would be. Not only did we have to monitor the leads, but we had to make sure she stayed on the camera the whole time (they video the entire test so they can correlate her clinical seizures with the EEG activity).

For the most part, Lily tolerated it just fine.
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I was able to capture a picture of Lily's EEG, to give you an idea of what it looks like:

In the picture above, you can see the squiggly lines behind the video picture of Lily (she's laying down in the middle of the bed). Those are the lines showing the electrical activity in her brain. (For those of you who are used to seeing EEG's, this picture was about 30 minutes before the end of the test & Lily had managed to take of two leads, thus the flat lines in the middle of the screen). The red line across the bottom is EKG. Notice how there is no pattern at all to the lines; it's total chaos.

In comparison, here's a picture of a normal EEG:

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Taking the leads off made Lily very sad:

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But juice makes it all better:
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And just for fun, here are a couple pictures of Lily REALLY enjoying her dinner:
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Monday, January 22, 2007

It's official!

I let Dr. Red Sox Fan know today that we are switching Lily's care to Dr. Bow Tie. I sent him a nice email, and he responded just as kindly. I didn't expect anything less, but it still is hard to do.

I spoke with Nurse Wonderful just a moment ago & she, with out saying as much, agreed that it was the right move to make. They have to be careful, you know, about not stealing patients from colleauges & stuff.

So we have a clinic visit with Nurse Wonderful next week & then we'll see Dr. Bow Tie sometime in the next month.

I feel good about this move. It was clearly the right choice for us, and for Lily.

Sunday, January 21, 2007

Trying to protect a tiny bit of privacy

In an effort to protect our privacy a little bit, I've changed the names of Lily's care providers. I may abandon this effort in the future, however it seems to make good sense for now.

Lily's 24 Hour EEG

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.


Welcome to Lily's blog!! We are so blessed to have so many people interested in Lily's progress & development, and are grateful for your continued support.

After forwarding too many emails to too many people, I finally decided that Lily was popular enough, at 18 months old, to warrant her own blog. Such a cutting edge girl!

I will do my very best to update this blog at least once each week with what is going on in Lily-Land, and of course will update after any doctor appointment or major change.

Over the coming days, I will try & get some very helpful links about Infantile Spasms available to anyone who wishes to learn more.

If you are a parent of a child who was recently diagnosed with Infantile Spasms, know that you are not alone! IS is a terrifying disease, and the period following your child's diagnosis is a very, very difficult one. Please consider joining the Yahoo Group for Infantile Spasms. The group is an amazing wealth of information, support, and love. I would be walking a very different path were it not for the knowledge & support of the members.

Please feel free to forward this page on to anyone who may be interested in learning about Lily and her journey with Infantile Spasms & epilepsy.