Checkup… and weaning OFF another medication!

It has been a glorious 2 months since our last neuro appointment.  It’s the longest we’ve gone without seeing a doc or going to the hospital since this all began late last June, which is a very, very, very good thing!  We didn’t even have to do an EEG this time since we haven’t seen any seizure activity.

Z was highly entertaining in the doctor’s office.  I was going to say he was in rare form, but that’s not true these days.  He was up and down, wiggling and giggling, and had an absolute ball crinkling the paper on the bed.  After a long wait in the room, and discussions with the resident, he was even still awake and going strong when Dr. V. came in.  That was fun, because so often by the time she gets in the room, he has long since crashed and gone to sleep.  She talked to him, held him, and seemed excited about his progress, as are we!

She even decided to move forward weaning him off his last IS medication… the Sabril.  He is officially on only one anti-epileptic drug now, which is a huge feat considering at one point he was on about 7 different mediations and treatments at once for the IS and it’s related symptoms.  He did have some small focal seizures during his last EEG, so he will remain on topiramate indefinitely… well, until he is 2 years seizure free on the medicine.

He will go back to the hospital for a 23-hour EEG in a couple of months, just to check everything out.  Seems weird that it’s a 23-hour EEG not 24-hours, right?  That’s just a loophole with insurance because of how they charge for a full day (ie. 24  hours).

Anyway… things are going well for baby Z at this point.  He’s still behind, and even our extended family doesn’t seem to quite comprehend that, judging from some of the questions and comments I’ve seen in email or on fb, etc.  It’s hard, and getting harder as he grows, to explain to strangers in a relatively short manner why he won’t hold his own bottle or why he’s not crawling or walking, etc.  But, none of that even matters to us after all he’s been through.  We’re just so happy to be able to look in his eyes and see a smile on his face!



Update on Ketogenic Diet

Baby Z was still having some slight seizures on the 3:1 ratio, so we went up to the 3.5:1 ratio.  Unfortunately, over a day or two, he became lethargic and his ketones were too high (meaning his blood sugar was too low).  So, we had to give him a tiny bit of apple juice, and went back to the 3:1 ratio.
I do think the diet helped, but it didn’t resolve the spasms completely.  So, we decided to proceed to ACTH.  We can always go back to the diet, but we need to get rid of the spasms (and hypsarrhythmia on EEG) completely if possible, and the ACTH is our best chance.

2nd day of Keto Diet

It seems like it should have been an easy day, but I think in general when you’re in the hospital, no day is an easy day.  Last night, baby Z was poked every 2 hours for blood sugar.  They just prick the fingers (or in his case, the toes), like you see on the commercials for diabetes.  He was so exhausted from getting the IV and getting his blood drawn yesterday, that he actually slept through the pokes during the night.  But, he was up at 2 a.m. for a feeding (which may become our new normal because he’ll need to eat more often on this diet).

Today though, his IV came out, so they had to put another back in, and they needed another vial of blood too.  At least the nurses learned from yesterday that he really is a hard stick, so they called the transport team to come do it.  Even they had a hard time, but they finally got it in, and they actually listened when I told them NOT to pull pressure to draw the blood, so they let it drip into the vial and that worked much better.  image

Baby Z took the whole thing well.  In fact, he took it too “well”.  He didn’t cry when they put the band around his arm before the poke, nor did he cry during the actual poke, and she had to push the needle in, pull it partially out, re-push it in, pull it out, over and over, and still didn’t get the IV in.  So then her partner found another spot and did the same thing, but they finally got it.  But, baby Z didn’t cry during any of it.  It was actually quite depressing, because it was like he lost the will to cry because he couldn’t control the situation, he couldn’t get away, and his own mother wouldn’t help him.  UGH.  My poor, sweet baby.  I try to tell him over and over how I am trying to help him, and that I’m so sorry when they poke him.  I wish he could understand just how much I love him and want to help him.  But, all he knows is that *I* am the one who is always here when bad stuff happens to him.  He won’t even look at me after.  😦  But, I just try to snuggle and hold him and talk to him about what is going on, and tell him over and over how much I love him.

Even though he didn’t cry during the blood work, he had a rough day.  He cried a lot.  A LOT.  He didn’t want his food, his paci, or anything.  But, he did want to be held a lot, so that’s what we did most of the day.

The neurologist told us that he is having less clinical seizures (the ones we can see) and is having more sub-clinical seizures (the ones that aren’t noticeable).  He is still showing hypsarrhythmia in the background of the EEG. But, the neuro said it is moving in the right direction.  So, I guess that’s something.  


As far as the diet, today he started having more of the ketogenic formula than the regular formula.  He’s still not making ketones, but maybe in a day or two.  His blood sugar checks and specific gravity have been fine.  Tomorrow afternoon he’ll transition to all ketogenic formula, and we’ll learn when and how to prepare real food at a 3:1 ratio.  We’ll also get a list of approved products to use for pain/fever medicine, teething medicine, lotion, shampoo, toothpaste, etc.  His real food will consist of 3 parts fat (butter, cream, etc) to 1 part protein+carbs, so it should make for some unique recipes.  It requires a very precise digital scale, not just to 1 gram, but to 0.1 grams.  I ordered it from the Charlie Foundation, per the recommendation of our dietitian.  If you need one, look for it half way down the page here:

Abby and Joel came by tonight, and it was good to see them both.  I wish we could see them more, but it’s just so hard to get Abby enough sleep coming down here each night, with Joel in traffic to get home, pick her up and then head all the way into the city, stay a bit, then head home.  She’s been through a lot, and I’m not sure if it’s the stress, the new daycare, having a really busy weekend, or just being in school (or all of the above), but I’m crossing fingers that she stays healthy, because tonight she was complaining about her ear hurting, and she sounds a little congested.  Joel really needs to be at his “new” job, but obviously I can’t take her to the Dr., so hopefully she stays healthy and kicks whatever she’s getting.  Needless to say, I didn’t let her hug and kiss on her brother, which is really hard for her, but at least she understands!

Here’s our less than homey digs. lol



Surprise, Surprise. The Dr. didn’t listen.

We went to our appointment with the neurologist today.  But, it didn’t go as planned.

I brought up some of my questions about Infantile Spasms, how his seizures have changed, the video I had as evidence, and the fact that he isn’t responding to any of 3 medications.  She explained that the EEG doesn’t show the hypsarrhythmia, and that we just don’t have the right dose of the right medication yet.  She was nice, but at the same time, dismissive of me.  I get it.  I mean, she’s a neurologist, and I’m just the mom.  But, you know what?  She is *just* a neurologist.  I AM THE MOM.

Something isn’t right.  I wish she would listen.

Hmmm. Epilepsy or Infantile Spasms?

Our appointment with the neurologist (Dr. H) is this morning, and since I know that as often as he does have seizures, that he probably won’t have one during the time we are with the neurologist, so I decided to prepare by waiting and watching and getting a good video.  His seizures have changed, so I want to be able to show the doc.  I have also been doing my own research, just trying to understand a little bit of what is happening and trying to figure out how to help my little guy.  I’ve filled a 2-inch binder full of print-outs, notes, etc.  I hope I’m wrong, but I really want her to re-consider her diagnosis.  He isn’t responding to any of the three anti-seizure medications he is on (Trileptal, Clonazepam, & Keppra), and he continues to have more and more seizures.  From the research I’ve read, I’m concerned that he might have Infantile Spasms, rather than just epilepsy.  If you don’t know anything about epilepsy or Infantile Spasms, you might not realize the difference, but it is significant.  Basically, Infantile Spasms can cause the little one to regress (become mentally retarded).  But, if it’s caught and treated early enough, there’s a good chance the baby can progress and develop normally.

Why am I (just a mom) questioning a doctor (a neurologist, no less)?  Well.  I don’t know.  I wish I didn’t feel the need to.  But, I feel like all signs are pointing me toward this diagnosis and she’s just not seeing it.  She’s fixated on the EEG he had 2 weeks ago and the 30-minute worthless repeat.

Okay.  I need to tell you that I have been doing a LOT of research.  I mean, a LOT!  I realize that Infantile Spasms has a very specific pattern on the EEG called Hypsarrhythmia.  But, I’ve also read that an IS diagnosis is based on three criteria, but only 2 of 3 need to be met.  He most definitely meets the other two.

I just want her to watch the video and see the seizure activity.

Here’s the seizure from last night.  It starts about 16 seconds into the video.

I also stumbled upon a youtube video of a baby with IS that had seizures that are almost identical to my little one’s.  So, then I started searching Youtube for Infantile Spasms, and I started seeing LOTS of videos of babies who had seizures like my little guy.  I know that this particular “research” might not sound very reliable, but all these babies had seizures just like my baby’s, and they all had an IS diagnosis.  I just really hope she listens to me and watches the video.