overdue update

Both Kim & I realize we've been quite remiss in updates, so I wanted to try & get a quick one in. Hopefully I can also get some pictures of Evie's Christmas Party at the Center for the Visually Impaired up soon, too. However, we've been so remiss, I'll not wait for that to get an update posted, since I've been so slack lately.

Overall, we feel like Evelyn's been doing pretty well, lately, and that we're seeing some significant improvement from the ketogenic diet. Her seizures come much more clustered now, which makes for a rough time while they're occurring, but gives her much more "non-seizing" time throughout the day when she's very alert and we can work on development. Lately, we're seeing about 7-8 clusters per day. Of those, most seem to be 5 or less individual seizures per cluster, but there seems to occasionally be one or two rough clusters of 15-20 seizures; typically around 6:00pm (I get cranky around then, too).

The hardest part, lately, has been at night - Evelyn's still usually up at least once in the middle of the night with some seizures, but in 3 out of the last 5 days or so, she was up 3 times, so Kim & I were both kind of running on empty headed into the weekend, here. But - it's the weekend. :-)

We're also seeing that she's much more "aware" of her seizures. Which all the therapists & doctors tell us is a good thing - it means Evelyn's "with it" and "present" enough to know that "something's going on that shouldn't" - but it can kind of break your heart sometimes. Typically, with her "longer" clusters, she'll cry out and/or start crying during each seize, which can really just kick you square in the gut. But then it's absolutely amazing to see how quickly she can bounce back once the cluster's over.

One more bit of seizure-related good news: In conference with Evie's neurologist we have decided to start taking Evelyn off of Klonopin. This is an anti-convulsant / relaxant that whose effects are quite similar to Valium. It is a slow reduction process; thankfully she's on a teeny tiny dose - but she'll be done on Christmas day. This should provide her with a bit more awareness, alertness and perhaps more muscle control. Of course the weaning process is the likely culprit for these sporadic sleepless nights lately. :)

Evelyn's physical therapist has said she's been seeing some marked improvements in the past few weeks, particularly as far as head control goes. Evie still can't hold her head up on her own for more than about 30 seconds, but we're hopeful that she's working her way toward full control. :-)

In general, the "dust is still settling" from the move, but it's settling, nonetheless. Kim & Evelyn might actually drive more miles per week than I do (which given my commute to midtown from Lilburn is no small feat!) - twice a week, they head over to therapies in Alpharetta/John's Creek. These will eventually take place at our home (which will be nice), once we finish buying & moving. We opted to "do it the hard way" in this interim period to maintain some level of continuity with therapists, so my aunt & uncle over in John's Creek graciously offered the use of their home for the therapy sessions until we get settled into our place over there. Then once or twice a week, Kim & Evie head downtown (midtown) to the aforementioned-CVI for therapy and the BEGIN program. It actually works out pretty nicely, as I'm working 2 buildings down from the CVI, so I can usually meet them for lunch. :-)

As noted, we are hopefully very near to closing on a house over in Alpharetta. We had a pretty hard-to-meet set of criteria for a house there:

• We wanted the Alpharetta area (and a specific subset of that area) for some schools that have excellent special-needs programs & resources
• We wanted a single-story house, just in case Evie doesn't end up walking well (most places in north Atlanta are 2-story, owing to squeezing more square footage out of the uneven terrain)
  
• Of course we had some price-constraints :-)

But, we managed to find a place that we think will meet all those needs quite well, and are currently set to close next Friday (14-Dec-2007). So, barring any unforeseen difficulties (which in real-estate transactions are just about omnipresent!) - we should be good-to-go.

Anyhow, that's about the latest. Apologies again for the slacking in posting - and apologies in advance, as it will likely continue to be a bit sporadic until we've completed the final leg of this move - but thank you again for your continued interest in Miss Evie. :-)

the big syringe, redux

Well, this time - we remembered the big syringe. So we passed that crucial check-point.

The last Sunday in October, we went to church with my mom & dad. They're fairly recent members at that church, but are still reasonably-well plugged in. But, for context - "they're new there". Anyway, their service starts at 10am. This is also Evelyn's mid-morning meal-time.

I figured it would probably be easiest to just use the Big Syringe to push food in while sitting in back, instead of breaking out the whole feeding pump & bag apparatus. So, thusly prepared, we headed off to church.

Mom & dad went ahead and saved us some seats at the end of a row in the back, so we could get out easily if need be. This later proved to be a fortuitous decision, but at any rate, we went in, got settled, and the service began. We sang some songs & hymns, and many of the normal "churchly" things, and then about 10:15, settled in to begin feeding Evelyn.

It all seemed too easy - Evelyn was sitting quietly & contently in Kim's arms; I was pushing the food in with the syringe; 2.5 mL every 30 seconds or so. This Big Syringe was an even bigger syringe than that last Big Syringe (see the image above; contrasting it with a regular 5 mL syringe) - this one holds 2 oz (60 mL), instead of 1 oz like the old Big Syringe. It was a little tougher to push in, but I attributed that to maybe a slightly-narrower NG-tube (Evie has a new type of tube), and/or the fact that we were using an adapter for the syringe, as the syringe's tip was a bit too large for this new NG-tube.

In any event, other than that, all seemed well and Kim and I were both having an "aren't we a happy little family" moment. The singing wound down, and the preacher had just begun a prayer. Another 30 seconds had elapsed, so I casually began pushing in another 2.5 mL.

"Pssssssfffffffffffft".

Recall, this is in the middle of a prayer at a church service, so it was like the "Psssssfffffft" heard 'round the world, as far as we were concerned.

We watched in horror, as in slow-motion, the adapter broke free from the tube input. However, motion & time were not, sadly, slow enough for me to release my fairly stiff pressure on the plunger of the Big Syringe. As one might imagine, this course of events ended up sending a big, fat stream of KetoCal shooting across Evelyn, Kim, my dad, and an innocent bystander seated diagonally in front of us. I'm pretty sure we also misted some other bystanders.

Taking an aside, to help you comprehend the magnitude of this Rain of KetoCal - I ran a small test at home, after all events had transpired. I loaded the Big Syringe up with water, put the adapter tip on, and shot the water from my parent's back deck clear across their back yard; a healthy 20 or 30 feet. So there's some power there.

Needless to say, back in church - time began to speed back up. Quickly. Dad, dripping with KetoCal, started to hee-haw (and grin like an animal that hee-haws) from down the row, while the poor lady in front began to process that she'd just been splattered with an ample barrage of formula. "That's strange... I'd swear I was just hit with a fire-hose. Only one that dispenses baby formula. Except I'm in church. Wrapping up a prayer. Interesting." Luckily, she was also the mom of a 1-year-old, so "she understood" - most graciously. Perhaps even more luckily, she was wearing a water-resistant jacket, so most of the would-be damage was easily wiped off.

Needless to say, after many apologies and some quick clean-up, we hightailed it out of the sanctuary to complete Evie's meal (told you that back seat was fortuitous!). While outside of the sanctuary, we ran into another kind lady we'd chatted with the prior week, who worked as a chaplain at the children's hospital. As we red-facedly relayed the chain of recent events, she advised perhaps Evelyn was just taking the preacher's sermon to heart, and sharing what she had with the world? ;-)

Anyway, being as Evelyn apparently learned last week's lesson a little too well - we skipped church this week. ;-)

ketogenic diet

As noted in earlier posts, we recently started Evelyn on the Ketogenic Diet. This diet is (yet another) treatment to try and reduce seizures.

At this point, we've worked our way through about.... 7 different medications (phenobarbital, fosphenytoin, Topamax, Klonopin, Depakene, Keppra, Zonegran), without significant impact on Evelyn's seizures. These are largely the more benign medicines with respect to side effects. As those haven't worked, before we go on to the "less benign" medications, we're giving "the diet" a shot.

It's essentially the Atkins diet on a whole different level - low-carb/high-fat/high-protein to the extreme. It's so low-carb that we even had to replace our baby wipes with ones that specifically did not contain glucose, and ditto for any other commonly-used "baby product" (lip balm, lotion, soap - you name it).

Anyway, the theory is it puts your body into ketosis (just like the Atkins diet), which causes your body to produce ketones. The theory (as I best understand it, and may be incorrect) is that these ketones bind with amino acids in the brain when the brain begins using them for energy (in place of glucose), and... does something to the chemical makeup of the brain that reduces seizures in about 50% of people, with about 30% of people showing a 90% reduction or more.

Part of what we have to keep track of is Evelyn's ketone levels, to assure they're not too much, and not too little. These are measured through a urine test.

Now, if you stop & recall that Evelyn is 5 months old, you may be asking yourself, "How in the world do you collect a urine sample from a baby?!?!?" ;-) Well, we're happy to report it doesn't actually involve holding Evelyn over a cup for hours on end (as was the initial picture we had in our heads!). We just slap a small cotton makeup-remover pad in her diaper, and squeeze the resultant output a few hours later onto a test strip. So, if you've ever wondered how to test the urine of your 5-month-old - there ya go. Aaaah, but one of the many things we never thought we'd know. ;-)

At any rate, as Evelyn's a baby, the diet's actually easier (hah) to maintain, as for her, it's just a special formula; KetoCal. For older kids, you can imagine that it gets much more difficult to find "regular food" that meets the fat & protein needs of the diet.

As of this writing, Evelyn's been on the diet for right at a week. She's just now hitting the full level of ketones. With any luck, we should be seeing results (if they're going to be seen) within a few weeks, or maybe a month or two. And if so, we can begin ramping down some of her other current meds, which would be nice. But, as with everything Aicardi... Time will tell!

Generally-speaking, we're all doing pretty good. Evelyn's seizures are still running anywhere from 30-50 a day (again; typically 30s each), and we have been seeing some new types of seizures - but she's been much more alert, overall, which has been nice.

Anyhow - that's it for now - more later! :-)

600 grams

This is an amusing ... story; anecdote; something I thought y'all would appreciate. ;-)

So, we're starting the ketogenic diet with Evelyn. I'll detail that in a separate post. Particularly because she's a baby, we have to be very precise in how much food (a special formula - KetoCal) we give her on the diet, as it's a delicate balance of ketosis to be maintained, plus we don't want her to actually lose weight. Thus, a precise scale was recommended by the dietitian; one that can measure in tenths (0.1) of a gram.

While these can be procured online, brick-and-mortar stores carrying them are harder to find. We didn't want to wait for a shipment, as we wanted to go ahead & get things going. Moreover, Kim wanted a smaller-sized scale - e.g. easily portable or what-not - so if we had to feed Evie "on the road" - it could be done. So, I was tasked with procuring one from one of few the stores known to carry very precise scales (and recommended for this reason by the dietitian!) - a "Department Store" which shall remain nameless. I couldn't even find a website for them, but they're a local chain here in Phoenix, as best I can tell. And they're not your "usual" department store - no lady's and housewares (though there might've been a jewelry section!). They are a ... "pipe store". Or "smoke shop". Or "counter-cultural and related paraphenalia store".

For those not catching the drift, it is a store which sells drug-related paraphernalia. Bongs. Incense. "Cigarette" rolling paper. And, yes - very precise scales. In the event you wanted to weigh something "small and expensive" in precise quantities. Like KetoCal! ;-)

So, I walk in on my lunch break (e.g. dressed for work), and was immediately on sensory overload between the burning incense, giant wall o' bongs, tye-dye everywhere and the Dead playing not-so-softly in the background. So I asked to be directed to the scales, to which I was gladly pointed. I ask the kind "salesman" for help in choosing a scale that was precise to the tenth of a gram, was reasonably small, but also wasn't too pricey. He recommended a few models, but then he posed a question (or a point) that we hadn't considered: How much weight would we need to weigh? E.g. what was the upper-limit of the scale?

To complicate things, because it was all quite precise, we're going to be mixing Evelyn's food up all at once. So, we're talking about a whole day's worth of food, not a single feeding's worth. The model otherwise seeming to most meet our needs only went up to 600 grams. I had no idea what 600 grams looked like or felt like; I'm not a metric guy! ;-) The "salesman" pulled out some calibration weights, in 200 gram increments. This helped a little, but was deceiving, since they're quite dense, as compared to the KetoCal formula.

While I was pondering this and calling Kim to ask how much food we'd have to weigh at once for Evie, my first "salesman" got preoccupied with another fine & upstanding customer. I got off the phone with Kim, and was still pondering the scale, when another "salesman" walked up. Now, I hate to call anybody a dirty hippie, because I know plenty of good, upstanding & most importantly clean hippie-types, but... this guy was a dirty hippie; dread-locked & just... dirty (literally). ;-) I labor at all this description only so you'll fully appreciate the hippie "dude" expression, said as only a hippie can say it.

He sees me really quite consternated over this scale, so I explain that "I'm just not sure if I'll need to measure more than 600 grams or not."

Him: "DUDE - it's 600 GRAMS!" [as if to say, "unless you're starting your own cartel - that's plenty, man!"]

Me: "Oh, yeah... well... I'm not trying to measure what you guys... normally might measure with this."

Him: [look on his face of "yeah-if-I-only-had-a-dollar-for-every-yuppie-looking- white-boy-that-comes-in-here-looking-for-a-scale-that-measures-to-the- tenth-of-a-gram-that-will-fit-in-his-pocket-that-wants-to-weigh-'other stuff'-with it"]

Me: [ignores look] "Yeah; I'm trying to measure food with it."

Him: [look of "Yeah.... 'food'... whatever helps you sleep at night, buddy"]

[awkward pause]

Him: [finally asks, gesturing with his hands an approximate size] "So... like... how much are you trying to measure?"

Me: "Well, it's baby formula - so it's ...." [I stop short as I realize I'm about to describe it as "a fine white powder"] "...well, see, my 4-month-old daughter has a seizure disorder. So there's this special diet that she has to go on, but we have to be very precise with how much she gets, as it's a delicate balance of the diet controlling the seizures and keeping her from losing weight. See, the diet puts your body into ketosis - just like the Atkins diet - which produces amino acids which bind with certain proteins in the brain, which - in about 30-50% of the general population with seizures - helps control seizures."

Him: [look of "oh-wow-either-that's-cool-or-this-guy-really- put-some-effort-into-coming-up-with-his-excuse"] "Wow... so it, like, stops the seizures?"

Me: "Yep; about 30-50% of time."

Him: "Cool, man... cooool."

So, long story short, we are now the proud owners of our Jennings JS-600V pocket-sized ultra-precise scale for measuring out our expensive fine white powder.

And that's the honest truth, Your Honor. ;-)