Tuesday, September 16, 2008

some Evelyn news :)

Hello!  I just wanted to share a few things we've enjoyed with Evelyn lately:

She is such a happy girl - she's been smiling again for a few months now and it's so nice to see what she enjoys.

She's bearing weight!  We just started using her stander, as well as just standing with mommy or daddy (we hold her under her arms and will use our knees to support her hips if needed.  I'm so excited about this - Evelyn doesn't even have full head control, cannot sit unsupported, but seems to really enjoy standing up.  She'll bear all of her own weight - I can even put her arms on my shoulders and she'll just stand there.  It's so cute, because she's knows what a big girl she is and is SO proud of herself.   She's wearing her AFOs so her ankles and knees are safe.. it's just so neat to me how much she enjoys it.

She's eating Cheetos!  Okay, they're baby snacks, and it's approved by her dietitian for the Keto diet, but she really loves to crunch them.  Her oral movements and chewing skills are really improving with this.  We're a long ways away from eating hamburgers, but she's really enjoying food.  She's also eating yogurt, tofu, pudding, and we've been spicing up her foods - which has made a huge difference.

Like any child, she has her ups and downs - including a new affinity for crying at bedtime (I thought we got through this!) and refusing to eat/swallow things that aren't yummy to her (like meat - which is a big part of her diet).  I also can't figure out how to get her to hold her own head up... it's kind of hard to walk around without head control... she'll figure that out eventually I suppose.  :P

Anyways, we've had a good few months, and are really enjoying year two with our little lady.

Saturday, August 16, 2008

doctor week

Well, the past week or two somehow managed to be "doctor week" - Evelyn had a variety of appointments, all of a "routine nature" - all were monitoring-type appointments we've been working on for some time - they just managed to coincide in the same chunk of time. ;-)

24-hour EEG - Neurology
On 4-Aug, we went into CHOA-Scottish Rite for a 24-hour EEG. Thought it sounds "pretty scary", it's a pretty "normal" check - essentially exactly what it sounds like - wiring Evs up on an EEG and video for continuous monitoring over a full day. It'd been a while since we really had any good captures of seizures on EEG, so that was the main impetus for the test.

Though we got there around 8am, Evelyn wasn't really wired up until at least noon. Other than having a bundle of wires glued to her head and being confined to a hospital room, it was really a pretty normal/unobtrusive day for her (as normal as any day spent in a hospital can be). Evelyn flirted with the nurses & doctors; she & Kim played, read stories; etc.

My folks graciously came by after their work-days to watch Evelyn while Kim & I ran to get some dinner. I stayed with Evelyn that night (where she let me know that as fun as the hospital had been that day, staying in it overnight was not, in fact, acceptable ;-) ...), and Kim came early the next morning, remaining there until noon the next day.

The overall results were largely "as expected" - we got some confirmation that a lot of Evelyn's little "jerks" during the day are, in fact, small or partial seizures (over and above her "obvious" seizures). While that was something of a downer, we finally resolved that we thought she was doing really well before the EEG, and a simple EEG certainly didn't change how well she was doing - so on we go. :-)

Coming out of this, we did decide with Evelyn's neurologist to try a new medication; specifically, one to cover a broader "spectrum" of seizure types, as were seen on the EEG. We will start Evelyn on Lamictal in the near-future, as soon as a Ketogenic Diet-friendly and small enough dose can be determined and agreed-upon between her neurologist & dietitian. See, Lamictal's smallest tablet is 25mg, which is too high of a dose for Evelyn to begin on (Lamictal, as with many anticonvulsants, have to be ramped up very slowly). There is a 5mg "chewable" version, but it contains some level of sugar (contraindicated with the Ketogenic Diet). A complicated lady... ;-)

Consult - Endocrinology
Because a big part of Aicardi Syndrome is a "mid-line" issue in the brain - that is, the agenesis of the corpus callosum - endocrine-system issues are not uncommon. So, we had a first appointment set up with one Tuesday 12-Aug. Kim was very happy with Evelyn's new -ologist, and he ordered a few labs, but was not overly concerned with anything at present.

However... Evelyn didn't really feel like donating any blood to the cause. We did attempt to get some blood during the sedation for the MRI (below); however, she still just didn't really want to share. ;-) So, we'll be going in for another stick another day.

MRI - Otolaryngology & Neurosurgery
This past Wednesday, 13-Aug, we had an MRI scheduled for Evelyn. It took some late-game finagling, but we finally managed to get two different doctor's practices to get their results from a single test(!!!).

Evelyn's Otolaryngologist (a.k.a. ENT :-) ...) wanted a face & throat MRI to get a look at a small piece of tissue in the back of her throat. It's just a "watch item"; nothing of really any big concern, but he did want to get a "proper" look at it.

Evelyn's Neurosurgeon wanted a CAT scan of her brain, just for her semi-annual check-up on her fluid & cysts. He decided to order the CAT scan, as though an MRI produces a better image of soft-tissues, takes longer than a CAT scan, and thus requires sedation, especially for a longer MRI. Moreover, since this is just "fluid monitoring" - the rougher image of a CAT scan would be fine.

This, however, this left us in the situation of having Evs to be both sedated for an MRI and then blasted with radiation for the CAT scan. In the same day. Through a bit of fancy phone-work and sweet-talking (hint: talk to the nurses, not the office staff), we finally managed to get a single MRI to cover both doctors. So, we went in early Wednesday morning to begin that. Regrettably, since it requires sedation, it easily triples or quadruples the amount of time otherwise required for the MRI.

At any rate, the MRI (and sedation) went off largely without a hitch, and results from both the Otolaryngologist and Neurosurgeon were positive - no cause for concern for the piece of tissue in her throat, and no change in her ventricle size & cysts. Thus, we'll now move to annual checks on her noggin (yay!) - via CAT scan. ;-) As noted above, we tried a blood-draw for Endocrinology, since an IV had to be placed anyway for the sedation, but Evelyn lived up to her reputation as a "hard stick" and being stingy with her blood. ;-)

In general
Anyway.... other than "lotsa doctors", we've all been doing pretty well. Evelyn has, as usual, trooped through all of this, probably with more of a "spring in her step" than Kim & I. :-)

Monday, July 21, 2008

a year already!?!?

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

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

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

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

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

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

Friday, May 9, 2008

evs at therapy!

Well, Evelyn had a bit of a rough start to her birthday - less than an hour before her birthday-day (11:20pm on May 8), she had a "Diastat seizure" (a seizure that didn't stop within pre-defined bounds of time / postures; thus a special "stop-the-seizure-NOW" medication was needed). These aren't a huge deal in the scheme of things, but they also aren't generally too common for her, so it's always a little bit of a downer when we have one. :-(

But, as we always find the silver lining around here - Due largely to the Diastat (it's a form of Valium), Evelyn slept like a champ last night (which means we all slept well!) - no seizures overnight, etc. So, she woke up presumably feeling refreshed for her birthday (as did we all!). And maybe that's why she did so well at therapy...

Yes, just because it was her birthday today didn't mean she was off the hook for Physical Therapy. Happily, I took the day off work, to help get ready for her party tomorrow, so I was here for this therapy session, as was Evelyn's Grandpa Mike (Kim's dad), in-town for the big weekend.

Evelyn was a little reticent to participate, at first (as is typical). But after some of her usual exercises, her therapist (who we adore) decided to try something new today - putting Evelyn in a seated position. Now, Evelyn's head control and trunk control aren't so great - she's never "sat" on her own - let alone pulled herself into a seated position - and when she is in such a position, it's always with us holding/supporting a big part of her weight, and being ready to catch her head when it falls.

So as you'll see below - this is big stuff for her!! HUGE!! Her therapist started her off in a "seated-crunched-over" position (with her trunk brace on) - Evelyn was then weight-bearing on her arms, having to support her head "bent over", and even pushing herself up with her arms, trunk & neck muscles!! Needless to say, we are SO HAPPY and SO PROUD, especially on her birthday! :-)

(1-3 min each. Apologies on the sloppy camera work; the best I could do on short notice when it was happening was get the web-cam up, and it's consequently a bit wobbly)
Part 1:

Part 2:

Part 3:

Part 4:

Happy Birthday, Evs!

Well, it's been one year since Evelyn LeeAnn Forester entered the world - and what a year it has been! In so many ways the time has flown by; we've made it through one big diagnosis, two surgeries, a move across the country, two-point-five teeth, countless seizures, countless diapers, countless sleepless nights, and countless moments of pure joy.

Evelyn has navigated her first year of life with such strength and grace that we cannot wait to see what the next year brings. She is such a happy little chubby baby. She cracks us up on a regular basis, is a very curious and dramatic girl, and such a hard worker! There were times in the first few weeks and months, before we really got to know her, that we wondered what Evelyn would achieve, and even if she would be happy. Well, she's certainly shown us to be foolish in worrying -- even before she could smile she showed us how happy she was every day. She learns or does something new so often that it's getting hard to keep track, and she has opinions about EVERYTHING, and isn't afraid to let us know how she really feels. :)

Parents always say that having children changes your life, and it doesn't really sink in until you have your own - but they're right. While it has been hard and even seemingly impossible at times, it has been wonderful to experience this first year of parenthood. Having Evelyn join our family has been an amazing blessing; we are better people as a result of the love, happiness and perspective she has brought into our lives. Happy Birthday, Evelyn!! :-)

Thursday, April 10, 2008

orthopedist: good news!

A few days back, Evelyn had her first meeting with the orthopedist. This is something we'd had on back-burner, but wanting to do for some time. For a variety of reasons - both due to some genetic predisposition (including malformed vertebrae) and muscle tone issues - many children wtih Aicardi suffer from scoliosis.

This can be something seen roughly at the outset (e.g. from birth), or can develop over time. "First priority" with Aicardi is largely seizure management, followed perhaps by feeding issues, therapies.... so, having seen no immediate outward symptoms of scoliosis, we hadn't been to see an orthopedist yet. It has been one of those things that "we'd get to", but still kind of resting in the back of our mind, wondering - "Could Evs have any scoliosis?"

Happily, we are able to report a resounding "No". ;-) Of course, this something that we'll still need to watch over time - but as of now, Ev's spine is straight as an arrow. ;-)

Monday, March 10, 2008

Orlando Sentinel Covers Aicardi Family

I thought I'd point out a story in the Orlando Sentinel that has an Aicardi Family from Florida spotlighted. This isn't to focus on the politics of it or anything else, as much as just a pointer to a family in the news, that tells "their story" some.

Disability assistance could ease juggling act for family

I do guess that it can highlight the different viewpoint that I/we now have in looking at "regular pictures" - the IV pole in the corner of the main picture for the story jumped right out at me (likely holds a feeding pump, etc); I'm guessing most would've missed that. ;-)

Sunday, February 10, 2008

evs eats peaches

Well, it's been about 2 weeks since Evelyn's surgery, and she's really been back to her old self for a good while, now. It's all so cliché; "they bounce back so fast when they're young!", but boy, is it true.

Evelyn actually had an MRI this past Wednesday (6-Feb), and had to be sedated for it, to keep her still enough for a good image. While we really weren't thrilled about her being put under anesthesia twice inside of 10 days, we do take it as a positive that she was fired-up enough (and showing it) for it to be required. ;-) It's actually standard operating procedure at CHOA for them to sedate for MRI's; we were just hoping to avoid it. The MRI was just a check-up to see how her brain's lookin'; to make sure the cysts are still not growing or (hopefully) still reducing in size. We'll be getting those results a bit later.

Anyhow, without further adieu - especially since we've been so remiss with pictures, I thought I'd post some brief video of Evelyn (kind of) eating peaches - she really wasn't cooperating too much (naturally, since I was trying to film it). However, she's really been doing pretty well with eating, lately - we normally try to make her "work" to slurp it off the spoon (which she occasionally does a little bit in this video). Also - because we figure either many folks might be curious or not know "what's a feeding tube look like" - both of which applied to Kim & I prior to all this - I include that on there, as well.

(link to the video directly on YouTube if you have issues viewing it below)

Friday, January 25, 2008


Well, after many long months of feeding Evelyn via NG-tube ("nasogastric feeding tube"; a tube in her nose), in consult with her doctors & therapists, we have decided to have a G-tube ("gastric feeding tube"; a tummy-tube) placed for Evelyn. Evelyn is still continuing to make very good progress with eating by mouth - she is spoon-fed a reasonable amount of oiled-up baby food with most meals (the oil for compliance with the keto diet), and has done very well with swallowing. Sucking still seems to be an issue, but we're working on it. So, Evelyn still depends on a tube for her primary nutrition.

While neither Kim nor I really relishes the thought of another surgery - or a (semi-)permanent hole in our baby(!) - all the -ologists and therapists are in agreement with their independent recommendation & counsel. Other than an NG-tube typically being a short-term measure - it's been 6 months now - it can actually be something an inhibitor to re-learning to eat, as your throat is always a little bit open with the tube hanging down it. It also is a general irritant, if you can imagine something always being in your nose & down your throat. So, all opinions & advice are that having the G-tube will actually help Evelyn in the re-learning-to-eat department. In the Aicardi world, about 40% end up with a G-tube, so it's not a majority, but not exactly a trivial minority (see the Aicardi Syndrome 2003 Medical & Developmental Survey for more; Growth & Nutrition; Gastrointestinal).

Long-term, the G-tube is an easily reversible procedure - if you take it out for too long, it actually starts to close up on it's own - so if we do make good progress with Evelyn's eating, it's not really much to have the tube removed. But in the nearer- and longer-term, it lets us continue to make sure Evelyn's getting all the calories she needs, and also provides assurance that if we need to get some medicine in Evelyn quickly - it can be done. Not to mention that your average anticonvulsant tastes particularly awful when crushed (and compounded/flavored liquid versions aren't an option because they typically include sugar, which is off Evs' diet). An additional tangential benefit is that we (okay, Kim) won't have to shove tubes up Evelyn's nose each week any more, something all three of us are looking forward to. And - as much as it will kill us for Evs' cute little tummy to have a hole in it - it will be so nice for her beautiful face to shine freely, unencumbered by tape or tubes. :-)

We opted for a Mic-Key ("mickey") tube, primarily as it's the one most of the other Aicardi families seem to prefer. It is essentially held in by a little water-filled balloon on the inside, with a flat input on the outer tummy surface (see picture at the top). The other type has a more rigid internal mechanism to hold it in place. Though the other type is harder to pull out, we figured if Evelyn got hold of it and was really tugging (from intent, a seizure, or otherwise), we'd rather it "break away" more easily. Also, the Mic-Key is easier to change than the other type, which could require annual trips to the hospital - potentially under anesthesia - to change it.

Of course, at this point, Kim & I have constantly questioned ourselves - did we do the right thing having Evelyn put on an NG-tube way back in August? Are we doing the right thing now? There will always be a little bit of nagging wonder - "Would she have picked eating back up in a few more days if we'd held off with the NG-tube or the Topamax [an anticonvulsant Evelyn was on then that is a known appetite suppressant]?" However, one thing is for sure: We have one of the pudgiest, cutest babies around. We actually refer to her as "monster baby", occasionally, as most people's reaction is, "...She's huge!" - and yes, she is both big, and tall. ;-)

While that's all very cute - which we totally appreciate - it is also something more important: Healthy. Lots of kids with these types of issues end up struggling to gain and maintain weight, making them more susceptible to other illnesses, or making otherwise-benign illnesses more dangerous. If you don't have much weight to lose, your average bout with the stomach flu becomes much more serious. We feel very thankful that Evelyn - like her father - has a healthy buffer, "just in case". ;-)

At any rate, Evelyn's surgery will be Monday, 28-Jan-2008, at Egleston, from around 11am-1pm. It will be at least a one-night stay in the hospital, if not two. While by all accounts, it seems to be a pretty simple & routine procedure, it's still surgery and anesthesia - so all positive thoughts & prayers are much appreciated; in particular for the skill & good work of Evelyn's surgeon and gastroenterologist, and Evelyn's speedy recovery. :-)