overdue pictures!

Well... I've caught flak from at least 2 folks now (you know who you are!! ;-) ...) asking for pictures, so I thought I'd finally acquiesce to the request. Five albums, linked to as below! :-)

Grandma Clark (11-Nov-2007) Visiting Evelyn's Great-Grandmother Clark!	Random (Nov-2007) Random pics from November 2007 - Doug and Almost-Aunt Megan and playing with maracas!
CVI Christmas Party Evelyn's Christmas Party at the Center for the Visually Impaired. Sadly, she never really woke up. Yes, the Santa is blind. :-)	Swinging (7-Dec-2007) On an early Saturday in December, it was a very fair & pleasant weekend - so we went swinging with Evelyn at a park. :-)
Random (Dec-2007) Carolyn & Susan accompanied Kim & Evie down to the CVI for the "end-of-semester event", and a few random shots, with some GREAT ones of Evie's eyes (sometimes hard to get)! :-)

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 teeny halloweeny

Okay, Kim made me title it that way. I swear. ;-) But - we wanted to post the Halloween pictures of Evie as a fairy. :-)

Halloween 2007

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. ;-)

Evelyn's Story: Ongoing (Nov 2007)

Since Evelyn's initial discharge from the hospital post-delivery, she's had an up, down, but overall "okay" time of it.

Her seizures started as of on cue with Aicardi; right about 2.5 months of age.  It was literally as if somebody flipped a switch.  We spent about 12 days in the hospital, trying to get the medications dialed in.  We were discharged after we and her doctors were "comfortable with things".  Over the next few months, we played with a number of medications (7 in all),  and as of this writing, are seeing reasonable reductions in seizures since having started the Ketogenic Diet.

In October of 2007 we moved from Phoenix, AZ to Atlanta, GA, to be closer to family for support and such.

Overall, we feel like Evelyn's doing really well.  Her seizures are definitely a challenge for the best of neurologists, but we've got the best ones we can find "on the case", and between seizures, Evie has been much more alert and able to play, which has been great.  :-)  She still doesn't have head control, but now that we're able to do a bit more with her in between seizures, we're working hard on that.  It could be a while; it could be soon - you never know, but we're just working on it as hard as we can.

So far - so good!  :-)

moved!

Well, it's been a good while - over a month! - since I've posted. It's with good reason - we've been busy!

Way back in July, Kim & I decided to begin trying to make our way from Phoenix, AZ back to Atlanta, GA, to be nearer to family & such. In late September, after a ~2.5 month remote job-hunt, I received an offer for a very good opportunity back in Atlanta.

So, on October 9th, my dad flew out from Atlanta to Phoenix. An hour & a half later, Kim & Evelyn boarded the same plane (literally) and headed the other direction. Dad & I worked on the final packing & loading of things, and then headed out on October 11th to begin our 4-day cross-country extravaganza, with The Boys (Rock & Rufus) in tow.

We made stops in Santa Fe, NM; Lawton, OK; Memphis, TN, before arriving in Atlanta on Octoer 14th. We did about 500-550 miles/day over 11-12 hours (two dogs and a 26' moving truck carrying all your worldly possessions and towing a car behind it will slow you down some), but made it without any trouble - not even any rain!

After arriving in Atlanta, Kim & I were able to catch up with my brother, home for some R&R from Afghanistan. We had a week of "relaxing" (which mostly consisted of initial doctor visits, house-shopping, and all that) before I started work on October 22nd.

While we're looking for a house, we're staying with Daniel's parents, who graciously opened their home up to us - which considering that we come with a baby and two large dogs is not insignificant! ;-)

Anyway, that roughly brings us up to date. Sorry for the lack of updates, but hopefully this explains it some. We'll try & return to the previously-scheduled posting frequency hereafter. :-)

thoughts & prayers

Lots of folks in "the army" are of the praying persuasion, and others are not.

We would not be true to ourselves if we denied that our (Christian) faith plays a large role - perhaps even the biggest role - in helping us out with all this. At the same time, neither of us is really very inclined to make pointed sermons out of anything. But, for those wondering "how we deal" - that's a large part of how.

In either case, a number of people have asked what to pray for, as far as Evelyn goes. Others want to know what to hope for, what to wish for, or just what to generally "send positive vibes for".

In answer to that, some "specific yet general" requests are below. "Us" here means Daniel, Kim, and Evelyn. :-)

• For us to be strong
• For us to be happy
• For us to focus on the positive
• For us to be prepared for the worst, but hoping for and working hard towards the best
• For us to get enough sleep and to generally "remember to take care of ourselves"!
• For Evelyn's doctors to be "on top of their game"
• In particular, her neurosurgeon (Dr. Moss) and neurologist (Dr. Bernes).

Of course, anything late-breaking will be on the blog.

Everyone thinking of & praying for Evelyn really, really means a lot - and it helps. If we can return the favor - please let us know.

aicardi

As discussed in evelyn's story, Evelyn was diagnosed with Aicardi Syndrome when she was 5 days old.

what is it?

In general, more detailed information may be found on, or linked to from, the Wikipedia page on Aicardi Syndrome (of which Daniel has been a recent active editor, and plans to continue to expand it), or see the Aicardi Syndrome Foundation.

In brief, though...

Aicardi syndrome is a rare genetic disorder, with about 500 reported cases worldwide. It's Daniel's own personal theory that it may be a bit more prevalent, going un- or mis-diagnosed in more developing countries. In any event, in almost all cases, it affects only girls, except for a very few known cases of boys with an additional genetic disorder that gives them an extra "X" chromosome.

At this time, a specific gene or chromosome has not been isolated. This means both that it cannot be tested for, and that it is a "clinical" diagnosis; a recognition of outward signs or symptoms. Aicardi Syndrome's "classic" markers are:

• Agenesis of the corpus callosum (ACC), partial or complete - the failure of the thin band of tissue connecting left brain and right to form, or form only partially
• Lacunae (lesions) of the retina
• "Infantile spasms", or basically, seizures

It also commonly includes symptoms such as cysts in the brain, other defects of the eye, and similar related issues.

what does it mean for evelyn?

Or in medical terms - "What's the prognosis?"

At this point, it's very hard to say - as the old saying goes, "time will tell". Symptoms don't usually become pronounced until several months of age, and as of this writing, Evelyn is still quite young. Generally-speaking, though, the range out outcomes isn't terribly great, but it is also highly variable.

• Moderate to severe developmental delays
• Eyesight ranging from simply "less acute" to near or even complete blindness
• Seizures on a regular basis (multiple daily are common)
• Shortened lifespan (median ~18 yrs; average of &10)

Additional complications can sometimes include:

• Scoliosis
• Gastrointestinal & feeding issues
• Respiratory problems

what does it mean for daniel & kim?

As you might figure, getting a diagnosis with any one of the above symptoms or outcomes as a new parent is enough to send you off the ledge; let alone all of them together! We certainly had our time of that, and we're also sure that we're not through - we will continue to have our "up" days and "down".

Largely, though, it's just a fine reminder to take things a day at a time. Evelyn could come and surprise us all, or things might go exactly according to "average prognosis". The doctors might end up dead wrong on their diagnosis, or they might end up being exactly right.

So in the end, for us, we need the same basic things pretty much any parent does:

• Take things one day at a time.
• Be ready and prepared to provide for your child's needs...
• ... While at the same time not placing limits on them.
• Stay positive.
• Be strong.

And of course, being able to boil it down and oversimplify it doesn't make it any easier to do. But, hey - it's a start. :-)

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. ;-)

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. :-)

the brain post

Yep, you read it right. I started this way back in the hospital, since a) we had received Evie's MRI records, and b) we finally figured out the hospital had wi-fi.

Primarily, it's to maybe explain "why Evie had brain surgery when she was 2 days old."

The picture on the left is from just before she went into surgery (11-May-2007); the picture on the right was from about a month ago, when we were in the hospital (24-Jul-2007). Looking at the left picture, it doesn't take a brain surgeon to see (pun intended) - "somethin' ain't right". The white part is all water, either enclosed in cysts (choroid plexus cysts on the sides, and an arachnoid cyst in the middle) or built up in her ventricles (causing hydrocephalus), because the cysts were "blocking the drain". It's hard to see the "dividing line" of some of the cysts, but if you look close, they can be seen.


The grey matter is .... grey matter. ;-) Seriously, that's "good brain tissue". Thus the impetus for surgery can be seen - if you look close around the rim of the cysts - especially on the left side (actually the right side of her head - MRI images are flipped) - you can see a very thin, grey strip. That's compressed brain tissue. Ouch.

As discussed back in Evelyn's Story (The Longest Week), the surgery was an endoscopic fenestration of the cysts - in short, going in with a small cable, and popping them like water balloons. This would allow the cysts to drain, and when they drained, they would also begin to stop "blocking the drain" for the rest of the ventricular system. A reservoir was placed to collect any fluid if it did continue to build, in place of a shunt (a more permanent device that can have complications).

As can can be seen on the right - things have progressed very nicely since the surgery! The cysts have shrunk in size, "unclogging the drain", and brain tissue is growing & expanding. Yay!

don't forget the big syringe

We had a check-up with the neurologist today down at PCH. Which meant the inevitable task we'd been dreading had finally come: having to feed Evelyn "out in public" with her feeding tube.

Now, it wasn't really the "public stigma" or "embarrassment" factor causing this fear - I'm not entirely sure that'd bother us much anyway, but even if it were to - c'mon; this was the hospital we were at. No, it was the fear of the logistics involved with such a task. The pump, and the feeding bag, and the formula, and the syringe to flush the tube, and the medicine for her 2:00 Depakene dose, .... daunting, to do it "outside the house" for the first time, to say the least.

So, Kim dutifully packed everything up, picked me up from the office, and we headed on down to the hospital. We had our meeting with the neurologist at 1:00pm (more on that in a later post), and then it was time for Evelyn's 2:00 feeding. So, we went down to the lobby of the outpatient building, and set up. Kim pours the formula in the newly-unsealed bag, and..... Yep; Mr. Murphy visited, and the bag had a leak.

At this point, we were a bit stressed - what do we do now!?!? We left The Big Syringe - to pump food in manually - at home! We had no other way to get the food in, except via the pump that needed a bag that had a leak! While I held a growing-fussier-by-the-minute Evelyn and schemed on patching the leak up with chewing gum, duct-tape, and/or bailing wire, Kim went around to see if she could find another feeding bag - we were at a hospital, after all!

Luckily, she found another mom with a child being fed via feeding tube as well, who had a spare big syringe, just in case. I then hand-pumped in 2.5mL every 30 seconds for 120mL (that's 24 minutes). ;-) While an onerous task, it wasn't so bad, and probably beat the chewing gum, duct-tape and bailing wire solution I had drawn up.

Lesson learned: Don't forget the big syringe. :-)

outta there!

Man, has it been a long week 12 days! So long, room 141-B! Sorry for the lack-of-updates - I can't believe it's been since Saturday that I last posted - but things have simultaneously sped up and slowed down, if that makes any possible sense. But, as the subject would indicate - finally, we are home! Evelyn is still experiencing seizures, but we (and the -ologists) feel reasonably comfortable, at this point, with the frequency, intensity & duration of them, in addition to Evelyn's general health, such as to be handled on an outpatient basis. The caveat is that her eating has been off, so right now, she's on an NG tube for feedings. That's the short of things. The gory details, as usual, follow....

the gory details

This was really just the week of wait-and-see. The hard thing with most anticonvulsants - or most other neurological medicines - is that they typically take a while to work. Levels must be built up over time (and often similarly ramped down, when a medication is stopped). Accordingly, "it takes a while", to see the actual result of a given medication. While this is clearly understood rationally, when you're pushing 11 days in a teeny-tiny half-hospital room with an alphabet soup of drugs being pumped into your baby, at times with little apparent effect - your capability for rational thought begins to diminish markedly. ;-)

In any case, over the course of this week, Evelyn was brought off of fosphenytoin and phenobarbital, had her Topamax doseage increased, and had Depakene added. Klonopin was also added once daily, and as needed for acute seizing activity.

Last Sunday (29-Jul-2007), things seemed to be a little better, seizure-wise. However, it was likely due to a big "bump" of phenobarb, and thus Evie slept much more than normal (her seizures typically manifest when she's waking up).

By Tuesday (31-Jul-2007), the seizures were returning to their earlier frequency (about every 3-6 minutes for an hour after waking up), though the intensity & duration weren't quite as bad - though we were seeing occasional "new" types of seizures, involving more jerking. Evelyn also wasn't eating very well. This could have been because of:

• the seizures
• the meds
• the seizures and the meds

Of course, neither us nor the -ologists would feel comfortable sending her home not eating, so it was really not a question at that time. Even so, every day when you hear "Well... Maybe just two more days..." - it doesn't do spectacular things for your morale. Monday or Tuesday was really probably the low point for Kim & I, emotionally: "The meds will take a while to work... okay, fine... Evelyn's still experiencing a number of seizures.... okay, fine... Each time you have to place an IV, you have to stick her 4 times... okay, fine... Another day or two.... okay, fine.... Evie's generally tired & cranky... okay, fine.... Not eating!?!? THAT'S IT - We've HAD IT!!!" Just physical & emotional exhaustion.

Of course, there wasn't much to be done, except press on & work through it. By Wednesday (01-Aug-2007), it was decided to go ahead and place an NG tube (nasogastric - tube through the nose) for feedings, as Evelyn hadn't really eaten at all over a 12-hour period, and had been eating very lightly otherwise. After placing it, she quickly packed on several ounces, but also didn't ... "eliminate"... very much, indicating that it was a well-founded concern, that she might be getting dehydrated.

As she was to come home on the NG tube, we had to learn how to deal with it. This includes inserting the tube (if you've never shoved a tube up your child's nose and down their throats to their stomachs, it's not a process we can recommend as "pleasant", for either party involved), checking the tube for placement (squirting a small amount of air in, and listening for it in the stomach with a stethoscope), and setting up/dealing with the accompanying feeding pump. And of course, Kim has been a champ at mastering it all. We're thinking maybe we'll get her back to school soon for her nursing degree, since it should just be a formality at that point. ;-)

At any rate, Thursday afternoon (02-Aug-2007), because feeding was no longer an health concern because of the tube, and the seizures were under ... "reasonable" control - still having them, but not quite as severely - we finally got our walking papers!

So, we've been at home for right at a day now. We're... settling in. It was very good to just "be here", and everybody sleeping in their own beds - at the same time - for the first time in 11 or 12 nights. For now, it's really been getting used to Evelyn's new apparatus & medication administration. Which hasn't been easy, per se - but is definitely better to do here than at the hospital.

Evelyn's seizures over the past day have been mostly - but not markedly - better. Her most recent "waking cycle" had a reduction in seizure frequency, with 14 or so extending over 2 hours, instead of the usual 1 hour.

So, anyway - that's the latest! Onward we go....

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...

Nurse/admin, 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. ;-)

oh yeah - more pics!

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

• Evelyn's Aunt Calista & Cousin Dominic
  
• Our friend Landon's visit (including some random hiking shots - no, we didn't take Evelyn - it was working towards 115˚ F out...)
  
• Some random pictures, including some personal favorites - some smiles (or almost-caught-with-the-camera smiles) and The Rib Pictures.
  
• Evelyn's Aunt Carolyn's visit, including her first trip to the pool at friend Rob's folks house. Yes, she went skinny-dipping. ;-)
  

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. :-)

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! :-)

Happy birthday Kim!

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

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.

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!

http://www.katemartinphotography.com/evelyn

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

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.

Neurosurgeon Check-up

Read my lips: No new neurosurgery!

For now, anyway - hopefully this will go off better than Bush I's promise! ;-)

The "main event" this pasts week was Evie's check-up with the neurosurgeon (21-Jun-2007), which was primarily a "one-bang" MRI and a subsequent review of the results by Dr. Moss. This was a big check-up for her, as we were really hoping for (obviously) "no new surgery".

As you can tell, we got our wish! Again, "For now" - but we'll take it! :-)

According to Dr. Moss,

• The cysts continue to drain - in particular the cysts on the left that could not be reached during surgery!
  
• Overall, "Great progress" and "Things look really good!"
• The next check-up is now in two months (60 days), another great indicator of "things are looking good"!
  

In any case, after we've got all the MRI results, I'll try and get a "comparison" up, as most people haven't seen the before, after, etc. That'll take a little more time to put together, so I'll save that for another post, to go ahead & get this good news out. :-)

Neurologist & Ophthalmologist

The past week we had appointments with the neurologist and ophthalmologist, in addition to a weigh-in at the pediatrician. After a week of "good" reports last week, we had been kind of dreading the neurologist in particular, figuring any "findings" he had likely wouldn't be as good.

Neurologist & EEG

So, Wednesday (13-Jun-2007), we went to see Dr. Bernes (neurologist). In short, he thought that she was looking "really good" - better than expected, I guess(?) - but he did want to order an EEG to try and pin-point if and what type of seizure activity might be going on. We were scheduled for one Thursday morning (14-Jun-2007), early (8AM). The picture at left is of the EEG; taken with Kim's camera-phone. It looks quite uncomfortable, but Kim said it didn't bother Evelyn a bit. ;-)

Evelyn did have some small seizures while on the EEG. It was really only outwardly visible in her eyes ("fluttering"), something we had seen & noted at home. They weren't classified as severe, but nonetheless, Evelyn's prescription dosage (phenobarbital) was increased slightly. In any event, we didn't take this as "bad news", per se, but wasn't exactly uplifting, either. Luckily, though, there was a bright spot: Evelyn had a killer hair-do! ;-)

Later Thursday, my folks also arrived back in town, after a brief mini-vacation up to Sedona, Flagstaff, and the Grand Canyon. They stayed until Saturday morning.

Ophthalmologist

Friday morning (15-Jun-2007), Kim and my mom & dad took Evelyn down for her check-up with the ophthalmologist. Dr. Peters seemed to come off her earlier - and fairly depressing - prognosis that Evelyn would "be able to see light and shadows, at best", concurring with the findings from the Foundation for Blind Children. This was a great relief to hear.

Dr. Peters did confirm that Evelyn had a coloboma in (or of?) her left iris. Colobomas of varying types are not uncommon with Aicardi Syndrome. She missed it earlier, as Evelyn's pupils had been dilated. We'd noted it just looking at Evelyn's (undilated) eyes day-to-day - showing itself as an extra "peninsula" of pupil jutting into the surrounding iris, also noticing that her left pupil rarely dilated, and also the more drastic red-eye she got in her left eye in flash pictures (occurring because the retina is more exposed). The dilation and red-eye disparity can be seen clearly above, and you can somewhat make out the misshapenness of the pupil, the "peninsula" pointing inward toward her nose. So, this caused us some concern as to what Evelyn's visual acuity in her left eye might be, but we also noted that she would often still move both eyes in tandem. Dr. Peters thought was that the coloboma itself should cause very little reduced vision, so that was great to hear! Dr. Peters also said that the lacunae were "moderate in density"; e.g. looking at a picture of a retina without lacunae, and a very severe case - Evelyn's is "somewhere in between". All in all - an encouraging visit!

In General

Last week was a little tough on both Kim and Evelyn, with the multiple doctors appointments that are a non-trivial drive away, but we're hopeful that from here on out, the appointments will be spaced out a bit more.

By way of "life in general" - it's really pretty much "new-parent life with a newborn", and all that is entailed therein - little sleep, worrying over the smallest thing (of course exacerbated by knowing there is something out there, but y'know), and all that.

Good news: FBC & Weigh-in

Just a few quick updates....

Last Wednesday (06-Jun-2007), we had a visit from the Arizona Foundation for Blind Children (FBC, but currently a dead link) along with AzEIP, Arizona's Early Intervention Program. While the AzEIP visit was largely paperwork, we had some encouraging news from the FBC check-in. Namely, we received some validation of what we had thought we'd been seeing, but had been a little afraid to believe: That Evelyn does seem to be able to see "some stuff"! Recalling our initial diagnosis ("She will be mostly blind, but will probably be able to see light and shadows."), this was arguably good news!

Kim and I both felt that we kept seeing Evelyn look at things, but you get really cautious about letting yourself believe it -but having a semi-professional (albeit non-medical, but a full-time FBC volunteer) come to the same conclusion and "validate" what you think you've been seeing - really helps. In short - we may not know for some time how much Evelyn can or cannot see, but having that ray of hope validated helps immensely.

Also last week, we had an appointment with the pulmonologist, a.k.a. "sleep apnea doctor". We also received encouraging news on that front, that in reading the monitor, no "apneatic episodes" had been seen, and that it was reasonable to hope that if things went well over the next month, we might be able to rid ourselves of the apnea monitor. Woo-hoo!

In other news, we'd had some troubles or concerns over Evelyn's weight over the past few weeks, with regard to her not gaining enough. However, she had another weigh-in today at the pediatrician, and we've been able to pork her up pretty well - she's now 8 lbs 10 oz. She's also put on another inch for 22". So - also good news!

On tap this week are the neurologist, ophthalmologist, and a visit/follow-up from the AZ NICP. So, a busy week, to be sure, but hopefully we will get some more good news.

introduction

Welcome to the Evelyn's Army blog. This will just be to have a place to post updates for people that are interested in "how Evelyn's doing" on a more regular basis. Comments, of course, are welcome.

The main site is http://www.evelynsarmy.org/. From time to time, we'll consolidate some of the "news" into a proper summary or update under "Evelyn's Story".

For now, though... Thanks for reading & caring!

pictures

Most of Evelyn's pictures are in Daniel's Picasa gallery - here. :-)

Evelyn's Story: The Longest Week

We were scheduled for induction at 39 weeks, mainly for delivery to occur under a controlled setting, with all medical personnel "all present and accounted for". So, on Sunday, we went out for "the last supper", and on Monday, 07-May-2007, we went in to Banner Good Samaritan Hospital for the induction. And thus began The Longest Week.

Monday 07-May-2007

We were scheduled for induction to begin around 1pm Monday, so we arrived at 11am. After the typical check-in hijinks, we proceeded to the room and "began". This included an initial 12-hour treatment as a pre-cursor to inducing contractions. After 12 hours (1am Tuesday), it was determined things had not progressed quite as much as desired, so another treatment was initiated.

Tuesday 08-May-2007

Around 10am Tuesday, it was decided to press on with inducing labor. By early afternoon, Kim was having increasingly-strong contractions. Around 6pm, though, the game quickly changed: All of a sudden, our nurse - who was otherwise very polite - briskly walked into our room and curtly requested Kim to "roll over", saying something about "the baby's heart rate was too low". The fetal heart rate monitor (cardiotocography) was registering about 83 beats-per-minute (bpm), where her normal range was 140-150 bpm.

As the nurse was requesting Kim to roll to her other side (this "switching sides" an apparent initial remedy for a low heart rate), around 4 more nurses appeared, seemingly out of thin air. One of them was jabbing Kim in the arm with a needle (some manner of tocolytic), plunging it in before warning "Big stick!", and the others were bustling about, when all of a sudden, they were off - running Kim out of the room and down the hall in the bed. The continued at a break-neck clip, careening into a temporary wall, and arriving at the OR. At this point, we were told Kim would be put under general anesthesia, and an emergency C-section would be performed - "Now."

Luckily, when the heart rate monitor was put back on in the OR, Evelyn's heart rate was back up to the normal range - likely owing to Kim's adrenaline! In any case, they called off the emergency C-section, but transferred us to a room nearer the OR, and also placed an epidural for Kim, so that in the event an emergency procedure was required, general anesthesia would not be necessary. The rest of the night was (mercifully) uneventful, with all other treatments stopped until the next day.

Wednesday 09-May-2007

Mid-morning, labor induction began again. Things seemed to be progressing reasonably, though Kim was arguably more comfortable with the epidural. However, over the course of the day, there were five more instances of heart rate decelerations. None were quite as drastic as the Tuesday episode - though one or two was close. By late Wednesday, the doctors felt that continuing through labor would simply not be advisable, and to continue with a planned C-section, rather than be forced into an emergency C-section.

Thereby, at 9:34pm Wednesday night - Evelyn was born! She had Apgar scores of 8 and 9, but after a few minutes, was still taken to the NICU as a precaution. Daniel spent the next few hours with Evelyn in the NICU as Kim was taken to the recovery room, and at some time in the wee hours of the morning, Kim was deemed "recovered enough" to be wheeled down to see Evelyn.

Thursday 10-May-2007

Thursday was an overall quiet day, with one minor exception: the neurosurgeon we had consulted with pre-delivery wanted to have another MRI that afternoon to get a better and more current look at the cysts in Evelyn's brain, to assure there wasn't too much pressure building up, or similar issues. We - and a nurse - did note some possible "seizure-like activity" on an occasion or two.

Beyond that, it was a day full of resting (as best that can be done in a hospital), picture-taking, and getting to know Evelyn. We were advised that an EEG was planned for first thing in the morning Friday, as a partil off-shoot of the "seizure-like activity", and that the neurosurgeon would be reviewing the MRI results - but nothing else "big" planned.

Friday 11-May-2007

After night of little and intermittent sleep - we just had to keep going to the NICU to see and feed Evelyn all night :-) - we went back down to see her around 7:45am, right before her 8:00am EEG appointment. We then found out that the neurosurgeon had reviewed Evelyn's MRI earlier that morning, and wanted to do surgery - at 1pm that afternoon!

Needless to say - that hit us like a ton of bricks! Additionally, it kicked off a very fast flurry of activity... Evelyn would be transferred to Phoenix Children's Hospital (PCH) for the surgery. Thereby, we hustled back up to Kim's hospital room, as with the assistance of the NICU doctors and Kim's excellent recovery, Kim's doctors had agreed to an early discharge so Kim could be at PCH for the surgery.

The surgery would be to perform an endoscopic fenestration of some of the cysts in Evelyn's brain; essentially "popping them" to allow them to drain or deflate. This, it was theorized, would take the pressure off other parts of her brain and hopefully "unclog the drain" of her ventricular system such that it could properly drain and flow. It was hoped this would mitigate any future need for a shunt.

First, though, the EEG was performed, and showed no technical seizures, though the results were somewhat questionable.

From there, Evelyn was taken into surgery. As one might imagine, it was incredibly difficult to see your not-even-2-day-old taken away, put under general anesthesia, and have brain surgery. However, a testament to the fighter that she is, Evelyn came through just fine, coming off the ventilator sooner than expected.

The surgery was targeting popping the cysts on the right, middle, and left. The surgeon went in on the right, as the cysts were largest and the most pressure appeared to be there. In the end, he was able to get to the right side and middle, but did not feel comfortable progressing on through to the left. In any case, he felt this was good progress, and wanted to await in MRI to see how things progressed.

We (Kim & Daniel) went home for the first time since Monday. As one might also imagine - it was also incredibly difficult to "go home without the baby", as it's just not the image you have in your head of returning home from the hospital - without the baby. However, it was likely a bit of a blessing in disguise, as we both got some very-much needed rest, which does wonders for your outlook after 4 days of living at a hospital with just a few hours of "sleep" per night.

Saturday 12-May-2007

Saturday was the start several long days of driving back and forth between PCH and our house (a 45 minute to hour drive one-way). Otherwise, though, it was a fairly (mercifully) slow day, with the exception of an MRI (deja vu?) to see the results of the surgery. Initial results "looked good" - no peripheral damage and the cysts appeared to be draining - but the "full interpretation" wouldn't be until Monday. Daniel's mom arrived around mid-day Saturday to stay for the week. The neurologist did come by to visit, and we discussed the EEG some. He did refrain from officially classifying the tremors we had occasionally seen - a bit more so after surgery - as "seizures", but proceeded to prescribe phenobarbital, as this "seizure-like activity" had been noted by several of the nurses.

Other than that, the day was largely spent quietly with Evelyn in the NICU.

Sunday 13-May-2007

Mother's Day! Sunday was also thankfully quiet, and as it was Mother's Day, the hospital allowed everyone to go outside if it was possible. As Evelyn had some mild jaundice, the nurses actually suggested it, so we took them up on their offer - and the chance to show Evelyn off in her pretty pink sunglasses!

Monday 14-May-2007

Evelyn had an eye-test schedule mid-morning or so. We had been fairly clamoring for this test, knowing that it would confirm or (hopefully) deny Aicardi as a possibility.

The short of it is, the opthalmologist noted lacunae of the retina and coloboma of the optic nerve - both very characteristic of Aicardi Syndrome, particularly when coupled with the agenesis of the corpus callosum, cysts, and (as yet unofficial) seizures. The doctor's further conclusion was the Evelyn would likely be mostly blind, if not completely so.

Needless to say, this was upsetting, to say the least. The neurologist came by again later that day, and confirmed the diagnosis.

In short, it really felt like something of a nightmare coming true - seeing this "remote impossibility" coming to fruition as reality.

The one bright-spot coming out of Monday was that the neurosurgeon did review the results of the MRI and was quite pleased with the progress at that point, ruling out another procedure for at least a week or two.

Tuesday 15-May-2007

Finally, the day we'd been waiting for - Tuesday Evelyn was discharged! Most of the day was filled with preparations for that, including attending an infant CPR class, as Evelyn was put on an apnea monitor as a precautionary measure, from both her general condition, and the medication (a side effect of phenobarbital being drowsiness). Around 2:30pm or so, we finally left PCH, and went home. It was a great feeling, just to get Evelyn home.