Winter time!

Well, I'm a bad blogging mom. But it's time for a Lily Update as there are some big things going on!

First off, HI JEAN!

Ok, now that's out of the way...

Lily came off the Ketogenic Diet over Christmas. It wasn't planned, and I think our neurologist is not super thrilled, but it was the right thing for us to do. Lily had a little stomach flu and couldn't keep anything Keto Friendly in her body. So we initiated the B.R.A.T. diet: Bananas, Rice, Applesauce, Toast. We stuck with bananas and dry toast, which aren't exactly Keto foods. And she was SO SO SO happy. Seriously, we haven't seen her that happy since before starting the diet. We did 24 hours of BRAT, then tried some Keto Foods. And they just went right through her, so back to BRAT we went. And she was so happy, not exhibiting ANY side effects of "normal" food, that we slowly introduced other normal stuff: turkey, beef, milk, cheese, yogurt, etc. We didn't "carb load" her, we tried to stick to higher protein stuff for a few days.

All in all, it has made a WORLD of difference for her disposition. She is having fewer seizures than before her illness. We don't know if they will eventually creep back up, but it just became clear that the diet wasn't helping her enough to make the trouble and hassle worth it. And putting LIly back on a regular diet opens up all kinds of teaching/therapy possibilites because food is a major motivator for Lily. We can use it as a reward for performing certain tasks and activites.

We couldn't be happier with our decision, despite it being an unconventional move and not what we had originally talked about with our neurologist.

Lily is also going to be weaned off of Ganaxolone, the study drug she takes. Marinus, the drug company sponsoring the study, has found that Ganaxolone doesn't provide the kinds of seizure control results they wanted so they're pulling the study & will not be pursuing FDA approval (at least for use in Infantile Spasms...it could be approved for other siezure types). So, we start the wean next Monday and she'll be off by early February.

This leaves us in a bit of a pickle as we're waiting on some genetic testing results before we start the next medication that we want to try (Valproic Acid). We'll set up the plan next week, but we may add a different med to bridge the time before our genetic results are back.

Lily was officially diagnosed with autism this summer. Normally parents don't cheer this diagnosis, but we did! This opens up a benefit in our insurance that will cover the majority of ABA therapy for Lily. I'm working on getting that set up for her now. I'm so excited about this! I just know it's going to do wonders for Lily and that she'll really start to learn. Our friends Cody and Sophie have both exploded in development with the use of ABA.

Lily is 1/3 the way through her first year of school. We really like her teacher and her 1:1 aide, and are happy with how things are going. We're going to revise some of her IEP goals soon now that we can use food as a motivator/teaching tool. There's just so much more we can do with Lily without the burden of food restrictions!

Finally we are working on arranging a trip out to consult with Dr. Harry Chugani at Children's Hospital of Michigan. Dr. Chugani is considered "the" doctor to see among IS parents. He has pioneered the use of PET scans to treat Infantile Spasms and determine if kiddos are surgical candidates or not. It's a path we've considered for a long time, since Lily was 6 months old at least. We haven't been able to pursue it this past year since Lily's been on the diet, but now that she's off we are planning a trip. It won't be easy -- it involves a 24 hour EEG, a PET scan (with sedation) and theoretically, a second kind of PET scan that Dr. Chugani is studying. Then on the fourth day we actually get to meet with Dr. Chugani and discuss all the results.

We'd probably take the whole family to go. It's very important for Hank & Lily to be together as they really depend on each other. It's important for Hank to be a part of Lily's world as it is, and will be, his world for his whole life. He's such a natural caretaker of her -- he is always checking on her, loves to snuggle her, and nwo that she's off-diet, FEED HER! LOL! Anyhow, since we'd be taking all four of us, we'll probably drive down to Maumee, OH to visit Todd's grandma. Hopefully Todd's mom will come up to visit, along with his sister & her kids. The four cousins haven't been together since before Lily was diagnosed -- October, 2005! I can't wait for these kids to be together and play!!

Here are the twins on Christmas Eve:

Started Phase 2

Well, we're now 60 hours into Phase Two of the Ganaxolone trial. I should probably explain how this works.

The study is a double-blind, placebo controlled trial. The way it is set up is this:
The subject (Lily) is randomized into the control group, or the study group. The results of this are not known to anyone. During week one, the subject starts out on "Bottle A" of medication. This bottle could have Ganaxolone, or it could be placebo. This medication is titrated up to the full dose (meaning we start at a small dose, and slowly work up to full strength) over a couple days.

After a 24-hr EEG, Phase 2 begins. The subject remains on Bottle A, and Bottle B is added. Bottle B is titrated just like Bottle A was. At this point, ALL subjects in the study are getting Ganaxolone. You just don't know what bottle has the med. Some people get it starting with Bottle A. Others don't get it until Phase 2 with Bottle B.

So. At the end of Phase One, Lily was having a rough go of it. She was having tons of spasms, all through the day. I felt like she wasn't making as much eye contact or responding to her name as well (and since I'm the mom & around her most of the time, I get to make that call. :) ). And her EEG was a COMPLETE mess. NOTHING even remotely resembling normal. Tons of high volt spikes & waves, complete chaos. Total hypsarrhythmia (although Dr. LA called it only hyps while she was sleeping; looked like hyps to my untrained EEG reading eye).

This led me (and [my] dad, he was here this week) to believe hope that she was on placebo last week.

So, Lily started Bottle B (and continued on Bottle A, remember) on Wednesday afternoon. Since then, she has had drastically reduced clusters of spasms. In fact, I think since then she's only had six total clusters. Yesterday she had no spasms after 10:30am. She had one cluster this morning (15 spasms) after she woke up (which, all things considered, was to be expected because she got the nighttime dose early last night [at 6:00pm instead of 8:00pm] so we could be discharged from the hospital a little early). So she'd gone 14+ hours without a dose of the med. Then she had two small spasms when she woke up from her nap this afternoon, again when she was due for the next dose.

And I think (and dad concurs) that her eye contact & responsiveness is better.

So, this reinforces my thought hope that she was on placebo last week and is now getting the med.

(The worry is, of course, that she was getting Ganaxolone in Bottle A & it just didn't work for her. That's the risk you take with any epilepsy med; we just have/had high hopes for this one.)

We bump up to the highest dose of Bottle B tomorrow afternoon. The next few days should be telling. And of course, hopefully her EEG will be much clearer than it was last week. That sucked. A lot.

Dad leaves tomorrow (BOO!!!!), so the twins & I will be on our own for a few days. My mom is coming down on Wednesday with Very Good Friend Donna, and Toddy arrives back on Friday night. My 30th Birthday is Saturday and I think we're going to make the trek over to Disneyland. Fun times, and plenty of photo ops.

Home from the hospital, WHEW

Well, we made it through our first admission & 24-hr vEEG. It actually went better than her first 24hr EEG went back in January. We originally asked for a crib, but once we got to the room, we realized that a regular bed would be much easier as then we could sit on the bed & hold her on our lap.

Turns out that was the right choice, and she did really, really well. Her EEG is still abnormal, however she doesn't have constant hypsarrhythmia. She does have a modified hyps while sleeping. It was tough that Lily didn't go to sleep until around 11:00pm on the 1st night (her normal bedtime is 7:00pm), and then she was awake from about 2:30-5:00am.


playing on the bed (you can really see how flexible she is in this picture!)


all smiles!

resting on mama


the hospital has a program where volunteers bring in their dogs for visits. We visited with a lovely woman & her chihuahua, Robert Henri (pronounced rho-bear, Ahn-ree)

On Wed, Thu & this morning, Lily was just in for observation during the start of the study. We received her 1st dose on Wednesday, mid-day. We don't know if it is placebo. We haven't noticed any huge changes in Lily, but she maybe just isn't titrated up to the appropriate dose yet.

Oh, and two days before we left, Lily got her DAFO's (orthotics). She's still trying to figure them out (and we haven't been great about working wiht her in them, given the road trip & hospital stay), but she looks wicked cute in them:


And a couple pictures of the roadtrip (and big-twin-brother, Hank):



My dad arrives tomorrow & Todd leaves on Sunday (boo hoo! We will miss him!). Lily doesn't have to be back at the hospital until Wednesday, and that admission is for only two nights. Hopefully we'll see some seizure improvement over the next few days, or if she's on placebo see some good improvement next week!

BIG news on the study!

Well, after hearing that the Ganaxolone study won't be at CHOS until July, it dawned on me that Lily might age out of the study before it even gets going up here (the age limit is 2, & her 2nd birhtday is July 8).

So, last week we started scrambling to get Lily into a study elsewhere. After much consideration, we decided to go to Los Angeles & get Lily in the study at Children's Hospital Los Angeles.

Our original plan was to leave on June 16, however the coordinator called this afternoon & they've changed their dates & want us there on TUESDAY. AKK! So we're leaving THIS Saturday (6/9, yes in three days!) for a month in Sunny Southern California!!!

Lily will have three hospital admissions; the first will be for three nights beginning on 6/12, the second for three nights on 6/19, and the third for one night on 7/2. We'll leave to come home on 7/4 or 7/5.

We'll be staying in short term housing in a 2 bedroom apartment a few miles from the hospital. We're driving down so that we'll have our own car (renting a car is expensive!). Todd will be with us for the first and last weeks, and we're working on who will be with me the 2nd week. The 3rd week we don't have any clinic visits/admissions so I could be alone if we can't find anyone else, but we're working on a few other options for some help for at least a few days.

We're so, so grateful to have this opportunity to get a medication for Lily that will hopefully be a really good fit for her. I have to say, my hopes are somewhat high that it will work for her. Of course, all those parents of IS kids know that "high hopes" is a relative term; getting hopes up too high doesn't ever really happen.

Wow. So much to do in the next 72 hours!!!

PS -- Lily's DAFO's will be here on Thursday when our PT comes!! I really think having some AFO's will help Lily's walking IMMENSELY!!

*sigh*

Just heard from our neuro...Ganaxolone study won't be starting until LATE June or early July.

So. Very. Frustrating.