Saturday, July 28, 2007

Video update!

Well - since mom & dad sent me a web-cam for my birthday, of course I had to play with it "right now". Further - when I was setting it up - I just now realized PCH has wi-fi available!! Just now I find this - arrrgh! That's how you know a nerd is off his game; if he's in a place for a week before he checks for wi-fi, and then, only incidentally.

Anyway, I'll leave most of it to the video. We're still here, and anticipated to be here until Sunday or Monday. Her seizures are better than when we came in - much less intense and much shorter - but they're not quite as good as they were a day or two ago - still seeing them as much as every 5 minutes for a while. A fairly typical example of the current seizures is caught "on tape". As can be seen, they're not that dramatic (unless you're holding her & feel how stiff she gets :-( ...), but it should give you some idea - it's not as dramatic as a big grand-mal foaming-at-the-mouth-and-jerking episode (though she does have some where she jerks a bit; thankfully not as much), but it's still not what you want. The plan is still to build Topamax, and wean the fosphenytoin, and continue the phenobarbital.

Good Christian Boy

We're in a shared room, and have (sadly) outlasted all our roommates thus far (for the morbid or cynical, in a they-get-discharged-first kind of way). Usually we get new roommates in the mid- to late-evening; I guess after surgeries are done.

Anyhow, we heard a pretty funny exchange over the curtain tonight; in the "from the mouths of babes" department.

A teenage boy (the patient; about 16) had just come from surgery, and had a small entourage there, including his mom, little brother (about 8?), and grandmother. He was still largely under the influence of painkillers, and an admin was there, going through the usual admitting rigamarole...

, boringly: "Are there any religious or spiritual beliefs you want noted or followed?"

Doting mom
, confidently: "He's a good Christian boy."

Little brother
, quickly & directly: "Is his little brother?"

We're still not entirely sure if it was a genuine or a loaded question. Moreover, we never heard the answer. ;-)

Thursday, July 26, 2007

oh yeah - more pics!

I totally forgot - I (finally) got a few new sets of pictures up last week before all this got started!

a few more days....

Well, I'll try to re-cap things since my last post, and cover what we know now & where things are going from here!

recent events

The road to the hospital is a long one (about 45 minutes, if no traffic), but one we got used to driving when Evelyn was in the NICU. Yesterday (Tuesday 24-Jul-2007), I got up early & visited "the girls" at the hospital before heading in to work. Evie was scheduled for an MRI, mainly because "she's here & it's easy", just to rule out building fluid or other more physiological issues. Though we still haven't gotten the official blessing by the neurosurgeon, preliminarily, everything looks fine.

Anyhow, Tuesday was otherwise a largely slow day at the hospital; "status quo" from Monday, which of course was pretty frustrating. Evelyn was still having strings of seizures run over the course of an hour. They're difficult to "typify", but roughly-speaking, she would wake up, and begin seizing with a very distinct, distinguishable & definable seizure lasting about 20 seconds, once every 5-6 minutes or so, over the course of an hour. More troubling, perhaps, to Kim and I, was that in between seizures, she was just "not herself". Some nurses suggested it was perhaps just Evelyn being postictal (the "altered state of consciousness" after a seizure), which was likely a large part of it - but we also kept seeing many small "almost-seizures", which was a bit unnerving, with it dragging on for up to an hour, many times leaving her too tired to eat.

At any rate, Evelyn's fosphenytoin level was checked, and found to still be quite low - not in the "therapeutic range" - she apparently metabolizes it quite quickly! ;-) So, they gave her additional larger doses of it to move it up, in addition to a phenobarb booster for the short-term.

Kim begrudgingly agreed to spend the night at home Tuesday night, and I stayed with Evie at the hospital. It was a bit of a rough night for Evelyn; she was awake from about 10:30pm-2:30am - quite a long stretch for her - with at least one of those hours seizure-filled as described above, with other smaller stretches sprinkled around. She finally got to sleep about 2:30am, and slept hard until 6:30,am when she woke up - and only had 3 seizures! Now we're getting somewhere! ;-)

what we know & where we're going

Evelyn's levels were checked again this morning (Wednesday 25-Jul-2007), and finally, the fosphenytoin was in the therapeutic range. Her phenobarb was also on the high end, but not at a worrisome level. The theory would now appear to be that the drugs are effectively blocking more of the seizures, which has been the plan since Sunday, to allow the slower-building Topamax to take effect.

Kim arrived around 7am or so, and I left for work around 9am. Evelyn has continued to do pretty well today - still definite seizures, but not the hour-long on-and-off episodes from Tuesday, which is quite a relief. The only slight concern is she's been particularly tired - likely due to both the seizures and the medicine - which is reducing her eating a little, but not tremendously at this point. She's been seen by the neurologist on-call (hers will be in Thursday), who did indicate that he expected she would likely remain in the hospital until Friday. This is fine with Kim and I, at this point. We don't really want to be there any longer than necessary, but we don't want to leave unsure, with things not at a "steady state".

It's a bit of a fine line to walk - We feel fortunate that Evelyn's "regularly scheduled neurologist" sees another child with Aicardi, so he has some idea of what to expect - namely that the seizures, even with medication, will likely persist at some level. We've heard from other Aicardi families to be wary of any neurologist who wants to make them all go away - "perfectionism" being an otherwise-desirable trait in a doctor - as it's likely a futile effort with serious quality-of-life ramifications. You really don't want your child to become a chemical experiment for the sake of the experiment. It's really a question of finding that "manageable and consistent" level of seizures that doesn't have a serious detractor to other quality-of-life areas, as many of the meds can cause drowsiness, lethargy, etc. And if Evelyn was too tired to smile - well, that just wouldn't work at all, now, would it? :-)

Anyhow, Kim is back on the hospital shift tonight, and myself bach'ing it at home with the boys (Rocky & Rufus), after swinging by the hospital after work until 7:30 or so - I wanted to stay longer, but was really feeling the late night/lack of sleep, and with a 45 min drive ahead, felt it best to head on. I'll swing back by the hospital early tomorrow morning (Thursday 26-Jul-2007). We're hopeful that from here, it's a very quiet stay thru Friday (27-Jul-2007), with continued improvement, followed by a discharge. :-)

Tuesday, July 24, 2007

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! :-)

Monday, July 23, 2007

Happy birthday Kim!

Happy birthday, to you!
In a hos-pital, it's true!
But what can ya do-oo?

Seizures & the hospital

Well- it's been a bit of a week!

Tuesday (17-Jul-2007), Evelyn started having much stronger & more pronounced seizures. Not "grand mal"-type seizures, but clenching a fist by her head, turning her head to the opposite side, and jerking a bit, typically with her eyes kind of "bouncing" from side to side.

As these were much more pronounced than anything we'd seen before, we called her neurologist who upped her phenobarb doseage. As one might expect, it was a bit unsettling to see, but after a day or two, we were starting to get the stride of things. The neurologist said to call if things weren't getting better within about 3 days.

Friday (20-Jul-2007), Evelyn was still having seizures, and perhaps slightly more often & stronger. Typically, she would have them most often while waking up, and occasionally going to sleep or other random times. At any rate, after advising the neurologist things weren't improving, he prescribed the addition of Topamax, another anti-convulsant. The plan was to ramp Evie up on Topamax, then (assuming improvement) back down the phenobarb.

Sunday (today), the seizures continued at their "normal" pace (as it were), until Evie woke up at noon. She then had back-to-back seizures for a good 45 mins or so, with only 2-5 minutes between them. So, we called the neurologist who said he'd get her admitted at PCH, but to proceed to the emergency room in the interrim, just in case.

Things were pretty uneventful at the ER, until Evie woke up again, and had another string of stronger seizures (though not quite as back-to-back as before). In any event, they opted to put Evie on a faster-acting anti-convulsant for the night, with an EEG and visit with her neurologist on tap for the morning.

Aaaand that about brings us up-to-date! I will now settle down for some "sleep" in my hospital-room chair, try to keep an eye on my 2 ladies, and keep you posted.

Thursday, July 12, 2007

Evelyn's Slideshow

Well, here is a link to Evie's slideshow, courtesy of Kate Martin - friend and fabulous photographer. These pictures were taken when Evelyn was about 3 weeks old - she's grown so much!

(more pics, including black and whites and family pictures will be added in a few days, so check back)

Monday, July 2, 2007

Smiley Face!

Well folks, Evelyn has smiled her first 'real' smiles!

This evening she decided to have a little conversation with Daniel and I that included some squeaks and snorts and several very beautiful smiles. As opposed to her 'gas smiles' these were full-faced bright little lovelies as she looked into our eyes and smiled in response to us smiling and talking to her.

She is recovering from her first cold, and slept for most of the day - I think she learned quite a few new tricks while she was sleeping. We've 'thought' we saw some small smiles before - as well as some tracking objects with her eyes, reaching on the rare occasion for a toy, and putting her hands in her mouth. Well, tonight she's done them all in a very definite manner!

This is great news on many levels, as of course it makes us even more enamored with our daughter - but it is also a big indication that her development is right on track at this point.

:) As soon as we can capture one, we'll post a pic of Evie's new toothless grin.