VNS

Well, Evs is now the proud owner of a Cyberonics Demipulse Model 103 VNS (Vagus Nerve Stimulator) implant. In very short, it's essentially a pacemaker for the brain.

Her surgery was Monday morning (19-Jul) - technically neurosurgery, and thus performed by a neurosurgeon, but they never had to touch her cute little head. ;-) It's 2 small incisions; one in her neck, and the other in her chest (see illustration).

More information can be found on Cyberonics' website - but very basically, the device sends electrical pulses to the brain via the left vagus nerve.  As with most seizure treatments (anti-convulsant drugs, ketogenic diet - etc) - "they don't quite know exactly how it works", but it can both help prevent seizures, by sending electrical pulses through the vagus nerve to her brain, as well as when she has a seizure - it can be used to "interrupt" it, by a magnet-activated switch.

At any rate, the surgery was relatively easy, as far as surgeries go - normally, it's actually an outpatient procedure, or doesn't typically require a stay overnight.  However, due to Evelyn's recent respiratory issues, we had already planned with her doctors to keep her a night.  After the procedure, she did have a hard time coming off the ventilator - The anesthesiologist & recovery team brought me back to help try her on her Bi-PAP for a little bit (fitting the mask is a bit of a learned art), but between irritation, agitation, and leftover anesthesia keeping her airway a little floppy, she had to be re-intubated for the next day.

She (and Kim & I) spent the rest of Monday in the PICU.  Around 10AM the following day (Tue 20-Jul), Evs was extubated without much issue, and had a pretty relaxed day (as relaxing as the PICU can be) - mostly involving sleeping to Tinkerbell.  ;-)  We were discharged Wednesday morning, also without much event.

The rest of the week was a relaxing one for Evs.  As far as the VNS goes, we'll have a post-op follow-up late this coming week, and about 2 weeks later, we'll go in to actually have the device turned on.  Then it will be kind of a like starting a new medication - we'll slowly ramp-up the settings, tweaking both intensity and frequency until we find the right settings for Evs.  It will be a process over several weeks (or even months), but we're hopeful this will be another helpful addition towards helping keeping seizures a smaller & smaller part of Evs' day.  :-)

Out of the PICU; in to the TICU!

Well, so far - so good!

Today, we were moved from the PICU to the TICU.  The TICU is the "Technology-Dependent ICU" - normally reserved for kids requiring ... technology, to live.  E.g. Ventilators, Trachs, etc.  No, Evs thankfully does not fit into that category (though a number of her Aicardi sisters do, so we are most sympathetic).  While both do have "ICU" in them - the used the TICU as a "step-down" (or as I jokingly referred to it, a "halfway-house") from the PICU.  To get there, Evs had to be off of a high-flow oxygen cannula - which means she's down to 2L of oxygen, which - considering her peak of 15L - is good progress!

Friday & Saturday were overall pretty good days for Evs - her right lung looked a little hazy again, but just as a result of some settling mucus, and not infection.  The doctor was actually encouraged by this; as she had some mild "gunk" to work through to breathe, but she had not really exhibited any outward issue - e.g. her oxygen saturation was remaining high, she wasn't struggling, etc - so she was able to "work through it", which is good.  Anyway, after turning her every 2 hours over the course of the day & overnight - her lung looked much better by morning.

Today (Sunday) was a little "up-and-down" - respiratorily, she continued to do well, leading to her move "downstairs".  On the downside, she had 2 seizures, both of which required Diastat.  Boo.  Our hopeful theory is that maybe we're seeing as similar trend as when she was teething - very few seizures "while she's hurting", and then when she's strongly on the mend - whamm-o.

At any rate, we still think we're on-track for a late-week jail-break - so we're keeping our fingers crossed for that!

Spasticity & Seizures

Hi all -

I thought I'd drop a brief update on Evs, since we're about 3 months overdue. Evs' last round of hospitalizations ended up - finally - with a diagnosis of spasticity, which is essentially uncontrolled tightening of her muscles. It's not uncommon with neurological disorders, but it really took us a long time to get to the diagnosis - we worked through her entire GI system; checked the her intracranial pressure; did a full-body scan for broken bones... you name it. We even, actually, asked about dystonia or spasticity, but were assured it was not - after seeing a doctor specializing in it, he diagnosed & started treating for it - and Evs started feeling a lot better. SO - frustrating that it took us so long to get to that answer - but, happily, we finally got one.

After that episode, Evs had a reasonably-good run of it, until mid-June or so - including a week at the beach, which of course she enjoyed. ;-) Partly owing to higher-tone from the spasticity, her ankle tone has been very high, so we got some new AFO's made. But, all in all, she had a good May - mid-June; doing therapies, and generally being a happy kid. :-)

Around mid-June, we believe that she started working on more teeth, and - as is her habit with teeth - had some prolonged seizures, that none of her emergency meds would touch. So, she landed back in the hospital for just shy of a week in late June. She was discharged on a short-term regimen of Dilantin, which we'll keep for a week or two to "bridge the gap" while ramping up her existing Lamictal a bit more. That's kept her a little drowsier, lately, but we're hoping that will prove short-lived - shorter-so than the Dilantin regimen itself.

Anyway - that's about the latest, here! Happy 4th of July!

a year already!?!?

If I was a bit more "on" this blog, I would've had a whole series of posts with the same title, starting from about mid-March. I actually started drafting several, way-back, but just didn't get around to polishing them off. So I'll keep this one brief and "un-polished", to not let it fall into the usual trap of my over-engineering. ;-)

At any rate, I was on the bus home, and was chatting with one of my "bus buddies". I'd told him a little bit about Evelyn before; that "she has a seizure disorder"; so on and so forth. At one point, the conversation turned to "How's the family", and he asked a little bit more about Evelyn, including when her seizures started.

Then I stopped and realized - a year ago today, we were about half-way through our 11- or 12-day hospital stay, inaugurating us into the grand and exciting world of seizure management. Evelyn's seizures started in earnest in maybe early July 2007, but by mid- to later-July, they were continuing to get worse, and we began our stay at PCH.

It's all still a bit surreal, really - "Have we really only been dealing with this stuff for a year?" I guess precise measuring and grinding pills and discussing doses and logging seizures and considering the omnipresent "next step" has become a legitimate part of "normal life".

Heh. Strange; the memory of not doing that seems so long ago, yet a year seems way too short.

Now, as-promised, such as to not keep this post in "Draft" limbo while I over-engineer it - I'm just going to post it; un-refined thoughts and all. ;-)

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. :-)

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! :-)

one med, two meds, red med, yellow med...

Okay, that title was a stretch, but hopefully you got the reference. ;-) Evelyn hasn't had a blue med (yet; and yes, she's had a red one), so we had to substitute in "yellow".

Just a brief update, because we realized we've been quite remiss! I can't believe it's been over 3 weeks - almost 4 - since we got out of the hospital. Most of that time has been dabbling with seizure medications, still trying to get Evelyn's seizures under control, to one degree or another.

It's been a bit of a trying time, to be perfectly honest. Very up-and-down. As noted earlier, Evelyn was discharged on a regimen of Topamax and Depakene, and Klonopin for acute seizing. Topamax brings with it the unfortunate side-effect of a loss of appetite, so Evelyn's been fed largely by NG-tube ever since. After a few weeks on that, it was evident that it wasn't really doing much, so Dr. Bernes began ramping down the Topamax, still keeping the Depakene, just to have something there. We then began ramping up Keppra, still working our way through the more "benign" medicines first (insofar as side effects go). After a while on that, Evelyn's seizures were still largely unaffected (50-60/day; 10-30s each). So, we began taking down the Depakene and ramping up Zonegran. Which is pretty much where we are today.

Generally-speaking, we've been very optimistic in the early days of a new medication, but then as we see no improvement, get kind of down, down, down.... so on and so forth. Evelyn's very much subdued, compared to your average almost-4-month-old(!), between some of the seizures and the drugs. Keppra and Zonegran are both (supposed to be) non-sedative, but Klonopin is a definite sedative. Thus, we try quite hard to be judicious with the Klonopin, but if Evie starts on a sustained (~10 minutes) string of seizures approaching 1 per minute (as opposed to 1 per 5 minutes), we usually administer it.

But, that's about the size if it - plugging away, trying to find what (if anything) will give her a little more seizure control. We're still feeding through the NG-tube, but Evelyn's showing a little more interest in the bottle, so we're still hopeful we can be done with that sometime or another. Again, absolute seizure control is not the goal - seizures will very likely be a part of Evelyn's life ad infinitum - but just some level of control of them so we can do other things, like play. :-)

Anyway, we're hanging in there, and we'll keep at it until we get there. Thanks again for all the thoughts, prayers & considerations - they really, really, really help. :-)

Evie still at PCH

Today was kind of a long - but slow - day, with all of the action at PCH.

We got up early (5:30am), mainly because... it was less painful to be awake than asleep. ;-) The main activities for the day were, basically, to consult with Dr. Bernes (Evie's neurologist), get an EEG, and get these blasted seizures figured out! Daniel ended up taking most of the day off.

In brief - for any that are unaware, seizures are a very expected symptom of Aicardi Syndrome - perhaps the signature outward symptom. Typically, the seizures initially present around 2-3 months (which... Evie is), when most kids are diagnosed, as all of a sudden, they start seizing noticeably. It's really more an issue of controlling the seizures, on an ongoing basis, rather than preventing them absolutely.

Anyhow, late the previous night, Evie was administered a dose of fosphenytoin, to try and take the edge off the seizures "now" (the "faster-acting anti-convulsant referenced earlier). This seemed to work until.... maybe 8 or 9am, until (naturally) right after Dr. Bernes paid Evelyn a visit.

Shortly after Dr. Bernes departed, Evie began continuing her seizures. They weren't drastically different from the previous night, at this point; typically around 6 distinct ~20 second episodes in the 20-30 minutes following waking up. Anyhow, we essentially waited & Evie slept most of the morning until the EEG around noon. Naturally, most of her seizures occurred while not hooked up to the EEG, but they did manage to capture one.

Kim left for the house about 1:30pm to go grab a shower and a change or two of clothes (Daniel stayed); returning later in the afternoon. Dr. Bernes came by around 2:00pm or so to discuss "where to go from here". In short, he wasn't comfortable to let her go home without getting her seizures under a little more control (and frankly, neither were/are we). It's Daniel's own suspicion that Dr. Bernes was a bit surprised the fosphenytoin didn't completely knock the seizures down.

Anyhow, we went ahead & re-started Topamax, this time at 10mg twice a day (instead of 15mg once a day) - the theory, I think, being to build it up in her system faster, through a slightly higher, but mostly more constant, flow. Most of these seizure medications "build up" in your system, so it takes some time to ramp up (and down) when changing medications. Topamax has had reasonably good success, as reported by a few Aicardi parents, so we're hopeful once it gets built up, we'll see good results.

Evie continued to have worsening episodes of seizures into the afternoon & evening, transitioning to a few more pronounced & somewhat longer seizures (the longest around 45-60 seconds), interspersed by more occasional but drawn out "semi-seizures", or "waves" as we've taken to calling them. It's as if you can see her fighting with herself, starting to seize, but then not, so on and so forth. Really not the easiest thing to watch, but we're just hopeful that in the end it confirms what we've thought all along; that she's stubborn & a fighter. ;-) The past two wake-up cycles - when she seems to experience the most, lately - we've counted about 8-10 distinct seizures over the hour after she wakes up, some of those with the "waves" in between.

At this point, Evie is continuing on phenobarbital and Topamax "regularly" (twice daily ongoing), and will have at least one more fosphenytoin dose overnight.

At any rate, both because "official" policy at the hospital is only one parent staying overnight, and because Daniel needs to get to work, we decided to send Daniel home tonight, to take care of poor Rocky & Rufus, with Kim staying at PCH with Evie. He'll swing by the hospital tomorrow morning on his way in to work. If Evie continues in the hospital tomorrow night (which is at least 50-50 odds), we'll probably switch.

So, that leads us to the present: Daniel drafting this post at the kitchen table when he should be sleeping, and Kim sleeping fitfully on a hospital chair-sleeper next to Evie's crib at PCH. Hopefully good - or at least better - news tomorrow! :-)