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. ;-)