Steroid

EEG Update and Some Thanks

On April 20th, Paige had another 24 hour Video EEG.  I’ve been documenting her recent seizure activity on the blog and this particular EEG was to hopefully “catch” a seizure in action to determine an appropriate medicinal course to keep them at bay.

**Quick version** If you haven’t been keeping up, or are just tuning in, we’re pretty sure Paige is having tonic seizures induced by startles and being woken up.

As I predicted, our girl didn’t have one “episode” during our whole hospital stay!  Believe me, we tried to induce one.  A nurse did the light test where they flash blinking lights in her face, I woke abruptly while she was napping, and I tried to scare her a bit.  (Her neurologist got a pretty good laugh watching me try to induce a seizure when they looked over the footage…)  Nothing worked.  Not. One. Thing!  So, even though we didn’t really get what we were looking for, I ultimately took it as a good thing that she wasn’t having them and figured I’d get a call from the neurologist saying, “No change!”.

Wrong.

Well, maybe.

Paige’s neurologist called me this morning and told me that while they did not see any seizure activity her brain waves looked “scary”.  I’ve explained this before, but Paige’s brain activity will never look normal because her brain structure is not normal.  They can get a baseline for “Paige’s Normal” and still detect dangerous or problematic activity going on.

So, what does this mean?  We don’t really know.  Paige is acting as happy as ever and even had a burst of development!  She has been rolling over unassisted, appropriately says “yeah” (in the cutest voice, might I add), shakes her head no (mostly when she doesn’t like a food or get a diaper change), and has been reaching and playing more and more.  All the while, her brain is looking “scary” and we don’t know why.

What I do know is that she has been having some sort of seizure activity.  In what capacity?  We’re not sure but we’ve had to give her rescue med, Klonopin, a number of times and, while it’s worked, the fact hat we needed to use it means… something.  We just don’t know what.  We also know that seizure activity tends to change around age two and she’ll be that age in June.  We know that we’ll have to deal with seizures in some way, shape, or form for the rest of her life.  A leading Lissencephaly doctor at Boston Children’s told us that she has a 100% chance of having a seizure disorder, so there’s that.

Our options:

1.  Another round of ACTH injections.  Paige has had a successful round of ACTH during her bout with Infantile Spasms when she was 6 months old.  This is a heavy duty steroid that I had to inject in her thighs twice a day for a month-long course.  She gained weight, was completely miserable, and didn’t smile for weeks.  However, after she weaned, we had ourselves a happy baby with a “near normal” EEG.  It worked and it worked for a long time (yes, a year is a long time in the Liss world).

2.  Another oral steroid.  Month-long course.  Probably the same as above aside from the needle pricks and pain.

Let me add, Paige was a CHAMPION during her ACTH treatment!

3.  Try another epilepsy drug on top of the one she takes twice a day to see if that helps.  Most kids that I know with Liss are on more than one.  Sometimes a handful or more.

4.  Wait and see.  Since her behavior and development is great, this sounds like a no brainer but there is a chance that the “scary” brain waves can mean trouble’s a-brewin’ and she can have a really BAD seizure and complications that follow.

I don’t even want to think about the complications that can follow.

I’m currently waiting for another neurologist in Pittsburgh to give their opinion as well as the previous doctor’s I mentioned in Boston.  From there, Andrew and I will make the most educated decision for Paige and hope that it works.  We’ve always told ourselves that we’re going for quality of life over quantity but it doesn’t make these decisions suck any less.  We shall see.

Andrew and I are doing okay after the information we were given today.  We’re happy that she’s so happy and there’s still a lot of facts and options that we need to acquire for her doctors.  All we can do is consider this a plot twist in our lives and do what is best for our girl.  We’ll definitely keep you updated!

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On another note, we want to give a HUGE thanks from the bottom of our hearts to those who have supported our t-shirt fundraiser.  We met and exceeded our goal in less than 24 hours..  24 HOURS!!  

The amount of support, well wishes, likes, shares, and donations brought me to tears and we’re so thankful!  So, thank YOU!  We can’t wait to rock our PAIGE PATROL t-shirts!

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NO MORE STEROIDS!

A big fist pump to the air was given yesterday after I gave Paige her last steroid injection!  She was an absolute trooper throughout all of them the past four weeks and we’re so thankful that it did it’s job.  Paige is still not exhibiting any infantile spasms and that is still a huge thing to be positive about.  She’s also back to the happy baby we knew and it’s such a relief.  

The first two weeks during her course consisted of a high dose of the steroid (ACTHar) in her thighs twice a day.  With every dose she was given vitamin B6 and Zantac for acid reflux.  During this time, she was a completely different baby.  Her bright eyes became heavy, she never smiled, and she just looked miserable.  You can tell it totally wiped her out and made her pretty zombie-ish.  It was heartbreaking.  Even though we were told that it was temporary, we still feared that it wouldn’t be and we missed our happy little snuggler pretty quickly.  She also ate like a horse and gained about four pounds in these two weeks!  Yikes!

The third week started the weaning process with only one injection a day at a lower dose.  Pretty easy right?!  Well, this was actually the most miserable and exhausting week. Sleep didn’t happen and Paige was up every hour and was at her most ravenous and was very fussy – we called this her ‘roid rage.  She was still pretty miserable and didn’t want to be moved around a lot.  The notes from her therapists said that she was: not alert, not engaged, not totally responsive, and rigid.  Our saving grace was actually a pacifier, that we dug up in her sock drawer, and it provided a lot of comfort to her and a lot of sanity for all of us!

And then HOORAY! The last and final week!  This past week, Paige only received a low dose every other day with yesterday being the final day.  I’m not exaggerating when I say that her personality changed almost over night early in the week.  Andrew was getting ready for work on Monday and I was making coffee (or something) when he called me over and yelled, “She’s SMILING!”.  

IT. WAS. AWESOME!  

Andrew and Paige always have had a special bond and it was really touching to see how excited she was when her daddy was talking to her.  She was just sooo smiley and alert!!  She hammed it up for her therapists and the adjectives in their notes turned to: active, engaged, pleasant, and feisty.  DEFINITELY easier to read than the previous week’s notes from them.  Her sleep even got back to normal and she was back to snoozing through the night!  Paige also seemed to be stronger and also rolled over from tummy to back with very little assistance. This was HUGE and we let ourselves be happy about this.  Celebrating the good times is definitely more fun then worrying and fretting.

Buuuutttt, it happens…

As a family, I’d say we’re doing pretty well considering it’s only been a month and a half since diagnosis.  However, since appointments and hospital visits have been slowing down I’ve had some more time to think and I’ve had some pretty bad days.  For so long, we’ve tried to look on the bright side and keep the negative thoughts at the back of our minds but this past week I couldn’t handle it anymore and I let it out.  

I let myself feel.  Whatever emotion I had, I let it out.  To be honest, I’m not proud about a lot of the things that came out of my mouth or in my head.  I can’t tell you how many times I screamed “IT’S NOT FAIR!” and asked myself “why MY baby?”.  I felt sorry for myself because I tried to do everything right during my pregnancy and my baby has a terrible diagnosis while some women do drugs during theirs and have completely healthy kids.  I know it’s not right, but I’m being honest and it’s how I felt at the time.  Why any baby, really?  Why any family?  Acceptance with the diagnosis comes in waves and it’s been a struggle.  I’ve always accepted Paige but I’m having trouble with the “why”.  While I can go about my day and have genuinely happy moments, there is a constant heaviness in my heart that won’t go away.  I’m not sure it ever will.  I’m hoping and praying I get to a point where acceptance isn’t such a struggle and our “new normal” becomes more natural.  

Thankfully, Andrew doesn’t really let myself feel this way for too long.  He has been such a beacon of strength for me (and everyone, really) during this time and encourages me to snap out of it.  While I have been pretty faith-based and look above for guidance, Andrew turns to science and medicine for answers.  In that way, I think we balance each other and it’s how we keep each other grounded, hopeful, and strong.  I think I definitely needed to cry it out, but after a while it’s not productive and it certainly isn’t good for Will to see in a puddle of my own tears for days on end.  We are very mindful that this whole situation affects him and we want every transition to go as smoothly as possible for our family. 

If there’s anything I’ve learned, it’s that we must live in the now.  Right now, we are okay.  Right now, we have a lot to be thankful for.  And that makes me feel so much better.

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