Do you...

Pray?





Or are you the fingers and toes crossing type? Doesn't matter, really. I know a little boy and his family that could use either or...





All my IS friends reading know exactly who I'm referring to...





One of THE cutest, most adorable kids on the IS block. Bennett.

He's scheduled for a temporal lobectomy tomorrow, Aug. 27th. And, it's not cut and dry. There's more to it than just a surgical resection. Not that a surgical resection is ever just a simple surgery. When you're talking matters of the brain...Nothing is simple. But, he has a unique case. And it's scary beyond description for his family.



Not only does little Bennett need our prayers and support...But his mommy and daddy, too. They need all the strength, encouragement, and comfort right now to be solid for their brave little guy.



So, if you could designate some fingers and toes for crossing...or remember him and his family while saying a prayer...

Every little bit helps!

Pardon me...

I must be the crazy one!



I think the exhaustion is clouding my judgement.







Because if...IF I were in my right mind...





I would not have attempted a mall visit on the last weekend before school starts!



Not to mention TX tax free weekend at that!!



Can you imagine the craziness?!!



Even the toddler section at TCP was crawling with bargain hunters!



Much MUCH fun, I tell you. Much fun. But, leave it to procrastinator-me to wait until the day before our nephew's 1st birthday party to get a gift. I like getting clothes for 1st birthdays...it's like the last time you can get them clothes for an occasion without getting that sad look of disappointment!







Oh, and by the way...there is an upside to the Felbatol although you'd never know it by reading my previous post.



Between all the fits and nap protesting...I get to see this!

Sleep is for the weak...

Whoever came up with that phrase had serious issues.


Some of you might have stumbled upon my desperate plea for help on the IS board. Some of you reading may have heard me on the phone. Or had the pleasure (not) of witnessing it firsthand.



THIS BOY IS CRAZY!!!!


Thank you, Felbatol. Thanks a ton.


No, really, I forgot what sleep deprivation was like. I needed a reminder.



All joking aside. This boy does not want to sleep anymore. I have no choice but to blame, you got it, Felbatol.

My trusty standbys and old sleep tactics were no match for Austin and Felbatol last night.

While Chad was waking at 4:30 am to start his day...

I had just ended my previous one.

I've long suspected that he was programmed with some type of sensor that alerts him when I'm drifting off to sleep. He can be as still as the night, but as soon as my eyelids start getting heavy and the groggy state sets in...the stirs and squeals begin.

Case in point...last night.


My poor little restless boy. I had one of those breaking moments around 2 am. That nasty anger brewing that results from a weary mommy on the brink of a meltdown. Not angry at Austin. No way. Sad for Austin. Angry at life in general. The kind of moment that reminds me of what we are living. A life where I am so desperate for seizure relief that I have subjected my baby to this hell. Felbatol.

A vivid reminder of the no nap/no sleep days of ACTH.

Counting down...Three and a half days until we reach our so-called treatable dose. Then maybe another week or two after that to know whether or not this could possibly be beneficial for Austin.

Something's gotta give here! Either these side effects must subside...or we've got...I mean GOT to find a remedy.

I did get a good suggestion (thanks, Elaine). It's called Neurontin. I read up on it. Not a technical sleep aid, but typically does the trick. It's got dozens of uses (most being off-label uses). It can be prescribed for anything from the treatment of seizures, to bipolar disorder, to treatment for withdrawal symptoms.

Of course, I don't want Austin on any more meds than necessary. So, I will keep up my sad attempt of getting him settled the old fashion way for now. For now being the key words here. Too many days of this can't be healthy for either of us.

Coasting on fumes here!

Hoping...hoping...that these are the type of side effects that disappear or lessen over time.

He's not just not sleeping. He's gets in a slightly agitated state. He's biting his fists with ferocity again. The callous that was finally improving is constantly red again and spotted with baby teeth marks. He's been moaning and crying at times. For those of you that really know Austin, you know he's always been a go with the flow, laid back kid -except, of course, those newborn days . For the past year or so, he's rarely been the type to cry without an obvious cause. Not so now. This is not the baby I've grown accustomed to. My days of swaddling and shushing and rocking have been gone for over a year now. All of my old previous tricks are being squashed now. DOA.

Maybe if he doesn't adjust to the meds...I'm hoping I will soon. Re-adjust to our new sleep schedule (or lack of). I lived through life with a newborn. Lived through ACTH. Vigabatrin. I just need a refresher I guess.

Too SWEET!!!

Sweet is the word!

A basket full of yummy sweets came to my door at 5 pm.

Seems my dear sweet Mommy and sister thought it may cheer me up a bit after my insanely frantic day yesterday...

They were right!

Bless y'all!! Love you both!! And thanks a bunch! You know I'm a sucker for anything sweet!

tornadoes and seizures?

Did any of you happen to watch The Weather Channel yesterday? Man...our neighbors in Beaumont had a little excitement. An F2 ( I think, might have been a strong F1) tornado blew through some of the largest retailers in town (right before the big TX tax free weekend for school shopping)...the mall, Wal Mart, and Kohls. I tell ya, if it's not a hurricane it's something else. They were determined to make the news again this summer! No one was seriously hurt though, thank goodness. Even the woman that was in her car when an SUV landed upside down on top it. Not injured, just a little in shock.

Completely unaware, and smack dab in the middle of fixing Austin's lunch, I got a call from my mom who'd heard that there was a funnel cloud just a few miles from MY house!! There's no type of weather I fear more than tornadoes. And our house...not exactly tornado ready. I ended up piling a mattress, pillows, Austin's helmet and my cell phone in the bathroom...you know..just in case! Can't you just see us all (me, Austin, and the Bailey) all camped out in the bathtub?!!

That certainly didn't come at the best time, although it's not like it was planned. Seizures were the priority of my day. Back up again. Way up from the weekend.

Without getting into too much detail, I concluded from the phone conversation with the epi nurse, that they think I'm a total nutcase. She said there was noooo way that the Felbatol could be causing the increase in seizures. According to them, it was too soon to see this type of effect. I asked her if it was a coincidence that they got dramatically worse the day after the increase...and if it was a coincidence that they got dramatically better the day after the decrease.

Oh, well, what would I know? I'm just his mother. The only person who sees him all day everyday. I guess she would know more since she sits here and counts seizures with me and knows everything about him and seizure meds for that matter. Her having her MD and all.

Not really. But, seriously, I still don't understand why I was under attack for adjusting his meds. I told them in a message something needed to be done. They didn't bother to call me. For four days. A five minute phone call was all it would have took. She pointed the finger at me for disrupting the increase schedule.

I could be wrong, but it seems like I might have mentioned wanting to avoid this type of confusion. It's exactly what I feared would happen if we went adjusting more than one med at a time. Guess my mommy's instinct should be obeyed from now on.

Anyway, thanks everyone for the support. I never feel comfortable adjusting his meds on my own, and to know that I'm not the only one who would have done that restores my confidence a bit.

Why no update?

Because I have STILL yet to hear back from the epi. Believe it or not.

It's been more like a circus ring with clowns jumping through hoops than anything else.

Quite the disaster. There's no explaining it. No making sense of it. They are nuts. Plain and simple.

By Thursday's end, I was desperate for some sort of relief for Austin. Since no one else seemed to give a flip, I adjusted his Felbatol myself (after much debate and a heads up message to the epi clinic). We had just increased on Wednesday so I brought it back down to that dose of 1 ml. And, whatdaya know...he improved. I would never ever typically adjust his meds on my own. Buuut....since no one would return my frantic calls...and he had just been on that dose one day prior...I did it. And it's okay. Better even.

Sadly, I have still not received any further instructions. It would be so nice to be able to discuss this with our epi and feel like I'm getting genuine feedback. That has so not been the case. They are too busy for us. Too busy for Austin.

And that infuriates me to no end!!!

It's a shame. Really is. I've called over and over. My child was seizing like crazy. Now I'm stuck with a med that I suspect is worsening his seizures...and they won't CALL ME BACK!!!


***********************************************************************************

Update:

I finally received a call back from the epi clinic. According to the epi, the increase in seizures was most likely due to the decrease in zonisamide rather than the increase in Felbatol. After much discussion with the epi nurse, I agreed to restart the zonisamide (his last dose was Sunday night) and keep with the Felbatol increase until he reaches the treatable dose of 2 ml 3x daily. I'm hestitant, of course, because I am leary of the Felbatol. I have only seen negative effects since we started. However, we were adjusting the zonisamide at the same time. Time will tell I suppose. I have to try something. Maybe this will help determine which is responsible -if either. Like I've said many times before, Austin's seizures tend to go up and down regardless of med adjustments.

Lost it

Where do I begin???

As if the anticipation and prep work weren't enough for this week (and the therapy in half an hour followed by a trip to the lab), I'm frazzled beyond words right now.

Last night I lost it. Lost my patience in med trialing/weaning, lost my cool exterior, lost my friggin' mind!!

After a loooong nap (which I would expect to see a few clusters), Austin proceeded to have nearly 8 clusters in a span of six hours. One right after another. Between 8 & 10 pm he had four. What gives?

What's more...that smiley, glowing boy is gradually fading again. Sometimes turning away from me and avoiding us. Walking around aimlessly. Falling again. Gnawing on his fist again. Irritated. Whiny.

Let me add even more to that. The real zinger.

I have absolutely NO clue what the heck is going on!!! Or even what kinds of seizures he's having for that matter!

I've been exploring the possibility that Austin may not even be having atonics. At least not right now. Long and confusing story...but, bottom line, he's jerking his arm up with the seizures I've always thought to be atonics. Atonics, by description of our epi, should not include a movement like this. It looks to me like a spasm (myoclonic). Maybe they were all along, just not with strong intensity?

I feel like we're back where we were exactly a year ago. Only with 100 more seizures a day. August 13, 2008. The last injection of ACTH. Exactly three months into this hell. Times were so confusing then, but slightly looking up and I swore I was seeing the light at the end of the previously pitch dark tunnel. The ACTH wean was finally over and Austin was having less than 20 seizures a day. On maybe 50mg of Zonegran (but with intentions of going up). Exactly what he's on now (with the intention of going down). Only now it's accompanied by two others -Felbatol and Klonopin. Those were the best seizure days he's had since this all began. And the seizures were not occurring in clusters. I was just starting to suspect that they were atonics. Today? I'm suspecting that they may not be. Ironic, isn't it?

I find it so incredibly strange that we are in this position today.

Plus, that old gut feeling is coming back to haunt me. The one I had when the discussion of adding Felbatol arose. Somehow I knew we would find ourselves questioning everything again.

And, here we are. Clueless. Was Zonegran really helping? Is Felbatol making it worse? Are the hyps back? Are we losing him to this Felbatol? The one that's supposed to be making our lives better?

I only hope I am able to form actual, complete & coherent sentences when we get our call back. At least enough to get my point across!

I'll try to update if I get the chance before we leave...providing I hear something back before then!

Time for some firsts

We've made the trip up to the lake several times this summer...but always minus Austin. The summer heat has just been too much for him to handle with his previous dosages of zonisamide (one of the many side effects is decrease in sweat production and high risk of heat stroke). Until just recently, he could stay outside in our intense SE Texas sun for a maximum of 20 minutes at a time -maybe, providing he has a cool wash cloth, fans, and plenty of ice water. Our temps here are a bit much for the average person, much less a toddler who's on a med that can cause heat complications. Very little warnings to even know when he's getting overheated. No sweat and he's non-verbal. His skin would get warm to the touch and his little face would get flush. Other than that...no clue as to how he's tolerating the heat. Which is obviously dangerous.

But, fortunately, the baby sweat is picking up these days thanks to our steady decreasing of the med.

So...

To the lake we will go...with Austin in tow this time!

All the firsts this weekend has in store! His first trip to the lake, his first boat ride, the first full-size pool experience, first real getaway with the family (that doesn't include running from a hurricane or hospitals).

And talk about impeccable timing. The recent decrease in his meds mean an increase in his alertness. And curiosity. And interaction. And happy giggles not just seizure giggles (yes, he sometimes smiles and laughs between seizures -it's beyond me why he does this).

It happens to be coincidental that all this is occurring at the same time. We made reservations months ago...well before we knew of this med schedule.

So, I may not be blogging much, if any, until next week. The rest of this week is packed full with therapies, labs, chores, and of course the preparations for the big weekend. My only regret is that it's not all week! To get away from typical day to day life for a week would do us all justice!

Is it just me, or do you almost feel relaxed just by looking at these pictures?!

Oh, another first...the slide!

Med update

Our first morning in over a year where zonisamide (Zonegran) wasn't a part of the medicine mix.
That was definitely different! I'm so used to the routine where the applesauce is always present with breakfast. No matter what he's having, it's always been accompanied by a side of applesauce -blueberry, strawberry, mixed berry, or any berry to spice it up...containing our old standby...zonisamide. This morning there was none. Just good ole Cinnamon Roll oatmeal -one of Austin's faves. It felt like I was forgetting something. I even reached for it when I opened the fridge out of habit.

We are one week away from completing the wean. Now, he'll only be receiving 50mg at night until next Monday. We're currently at 1ml of Felbatol three times daily & 0.25mg of clonazepam (Klonopin) twice daily.

There have certainly been some differences, but whether or not it's related to the wean, I don't know for sure. Like I've said before, multiple changes at once throw my judgement off. I see the changes, but I can't pinpoint the reason. The stopping of Depakote, the wean off zonisamide, the addition of Felbatol...and now the improved EEG. Who knows what's responsible for what. I hate that. I thought the medicine changes were spaced far enough apart to be able to clearly assess any notable differences -good or bad. NOT!

Never fails. There seems to always be questions surrounding any difference in Austin. I remember last year when the seizures drastically started increasing. First, I thought it was the late and short dosing of meds (Keppra and zonisamide) and the stress of our hurried hurricane evacuation. Then, we found out he had an eye and ear infection that week. That had to have been it. But, the seizures never decreased. After that, he contracted a bacterial infection from the antibiotics. That was it. Once that cleared up and the seizures didn't, I was clueless. I wanted to point my finger at something. Anything is better than the seizures increasing for no reason. And the idea of pinpointing the culprit (even if I don't know what it is yet), seems much easier than accepting that it's all out my control. If I even think there's a logical explanation, and it's just a matter of finding it, I have more peace of mind. Maybe that's not a rational thought process, but...what can I say? This is not an easy life. We all find have to find our own coping mechanisms. That's one of mine.

I got a little off topic there...sorry! My mind is bouncing all over the place...

Getting back on track, I've been seeing some great new skills trying to emerge. I've mentioned before that Austin figured out how to crawl over pillows and such. Now, he's trying to climb the walls...literally! Or the cabinets, or the sides of his play yard... I got him one of those stuffed fold-out couches last week, and I found him standing on top of it, facing the wall, with his little leg propped up as if he were Spiderman about to make his way up. If you know me, I freaked and ran over there thinking it was only a matter of time before he lost his balance and fell backward! He did. But, not quite fulfilling that elaborate mental picture of mine!

By the way, Step 2 is still going strong. No major boo-boos yet. The day after that post, he started having some spontaneous drop seizures again, which made me a little apprehensive about letting him go again. But, thankfully, he's been sitting again the majority of the time. And, if he doesn't, I can usually spot the changes in his mood which is my cue that something's coming. Crossing my fingers that this continues as long as the seizures do.

Article: Single gene mutation responsible for 'catastrophic epilepsy'

A link to the following article was recently posted on my infantile spasms message board.


It's from Children's Neurobiological Solutions Foundation (cnsfoundation.org)


**side notes**

The university that conducted this study was Baylor College of Medicine. That's the same one affiliated with the hospital where Austin receives treatment, Texas Children's Hospital in Houston, TX. Austin has been checked for this gene mutation already, but it's an interesting read for me nonetheless. It gives a little more hope that there is research going on into his disorder...and treatments are being tested.



Single gene mutation responsible for 'catastrophic epilepsy'

July 8, 2009

Source: Baylor College of Medicine

Catastrophic epilepsy characterized by severe muscle spasms, persistent seizures, mental retardation and sometimes autism results from a mutation in a single gene, said Baylor College of Medicine researchers in a report that appears in the current issue of the Journal of Neuroscience.


The BCM department of neurology team replicated the defect in mice, developing a mouse model of the disease that could help researchers figure out effective treatments for and new approaches to curing the disease, said Dr. Jeffrey Noebels, professor of neurology, neuroscience and molecular and human genetics at BCM and director of the Blue Bird Circle Developmental Neurogenetics Laboratory at BCM, where the research was performed.


"While many genes underlying various forms of childhood epilepsy have been identified in the past decade, most cause a disorder of 'pure' seizures," said Noebels. Why some children have a more complicated set of disorders beginning with major motor spasms in infancy followed by cognitive dysfunction and developmental disorders such as autism remained a mystery until the discovery by the BCM team that a mutation in only a single gene explains all four features of catastrophic epilepsy.


A gene known as Aristaless-related homeobox or ARX has a specific mutation called a triplet repeat, which means that a particular genetic (in this case, GCG) is repeated many times in the gene. When the researchers duplicated this particular mutation in specially bred mice, the animals had motor spasm similar to those seen in human infants. Recordings of their brain waves showed that they had several kinds of seizes, included absence epilepsy and general convulsion. They also had learning disabilities and were four times more likely to avoid contact with other mice than their normal counterparts. This behavior is similar to that seen in children with autism or similar disorders in the same spectrum.


"The new model is an essential tool to find a cure for the disorder," said Noebels.


"Mutation of the ARX gene was previously known to affect interneurons, a class of cells that inhibit electrical activity in the brain," said Dr. Maureen Price, the report's lead author and an instructor in neurology at BCM.


When researchers evaluated the brains of the adult mice with the mutated gene, they found that a special class of interneurons had never developed in specific brain regions.


"Further study will allow use to pinpoint which brain region is liked to the autistic-like behavior," said Price.


Two members of the research team Dr. James Frost, professor of neurology at BCM, who developed the concept of the special mouse, and Dr. Richard Hrachovy, also a professor of neurology at BCM are pioneers in the study of human infantile spasms.


"At present there is no proven cure to offer children with this specific epilepsy", said Noebels. "We now have new clues into the mechanism and have already initiated studies with a new class of drugs not previously explored for this disorder." The new drug testing is supported by the private foundation People Against Childhood Epilepsy.

Step 2 in full swing!

You may recall a post I made a while back about my new step program for Austin.

We (or I, rather) have been stuck on Step 1 ever since.

But, I'm happy to announce that Step 2 (which consists of giving Austin freedom to roam certain, non-carpeted areas of the house) is now a part of our day! I decided to take that step for several reasons...

1. Austin seems more in tune with his seizures. He's starting to have a pre-seizure/cluster behavior. Aura maybe? Dunno. But, it's definitely becoming more evident to me. What's more, he sits well in advance for some as well. So...I thought maybe he would be okay on the non-carpeted areas.

2. There's no keeping this boy behind make-shift barriers made of pillows and blankets anymore! He will climb a mound nearly his height!!

3. His balance has improved tremendously. He used to take the hardest spills that would leave him crying on carpet. I couldn't imagine him falling on the hard floor...without knee and elbow pads! It's still a bit unsteady...but very much improved.

So, what I've done is added a gate in one hallway and I put his play yard in another doorway that leads into the other living area where the fireplace is. Now, he has free reign over his play room and the kitchen, and I can see him everywhere.

It has made a world of difference. In me. And in him. He gets to see areas of the house from a new prospective...a 2' 10" prospective to be exact!! Instead of from my hip! I do let him walk freely in other areas, but not without me. The gate and play yard barriers create a space for roaming. Just his other area expanded a bit. More to see. More to burn that energy!!

Another change I've made is slowly making some of his toys accessible again. The ones that made me crazy before. Some of the ones made of hard plastic that haven't seen the light of day in months are now a major interest to him. It was like buying new stuff...without spending a penny! Some were either blocked by safety guards (pillows, stuffed animals, or his ballpit was my favorite way to block the bigger ones) or simply put away in his room. Anything hard or tall...forget it. I just kept fearing he'd whack his little noggin from a seizure. But since re-introducing them, I'm happy to say there have been no injuries. I don't really expect there to be either (although one is still driving me nuts -but he is loving it way too much for me to take it away again). His helmet actually gives him ample protection. It's just taken me so long to work up the courage to let it happen.