Solo con un click

"Only with one click"

Along the lines of Aug Comm - I thought I'd share this video, of a Spanish(?) girl that appears to have some type of movement disorder (perhaps some type of Cerebral Palsy - however, I do not know her specific challenges) - but still manages to make incredible use of augmentive & assistive communication devices.  Evelyn uses some of the same tools (notably the hand-activated switches seen in the video), and some we haven't used yet.  According to the blog where I came across this video, some of the devices are older - with the very key point that "it is the communication that matters not the tool being used to communicate".

Evelyn's "talker"!

(As originally published via Facebook Note by Kim, 6-Apr-2010)

As requested, here is the story of how Evelyn found her voice.

Evelyn's diagnosis comes with all kinds of negatives: seizures, health challenges, developmental delays, medications, blah blah blah. But Evelyn herself comes with all kinds of positives. We always felt that she "had a lot going on in that little head of hers", but were hesitant to say or do much about it in a public manner. Our reticence was largely due to the fact that Aicardi Syndrome is associated with significant cognitive delays (aka mental retardation). So it seemed presumptuous to think that our little toddler who can't walk or talk (or sit or move very much at all independently) could think and understand.

Well, if you know Daniel and I, we didn't waste much time wringing our hands and wondering - we started testing her. Every month we go to Lekotek, a special needs toy lending library, and have been since Evelyn was about 7 months old. This was her first introduction to switches and computers. We would hold a big button in front of Evelyn, support her arm, and show her how to press the button. A wire would connect the button (switch) to a toy, and when she pushed the button the toy would dance, or sing, or make loud annoying noises. She figured that out pretty quickly, and would bang away - she became really good at this around her first birthday. Lekotek also had very simplistic computer games - push the switch and a song plays, push it again and it skips to the next song and plays it. She also became really good at that - and would even skip through four or five songs to play the same one over and over again.

We continued to use games and toys like that for a while, until we felt more of an urge for specific communication. She would cry, and I would blame it on teething, but not know for sure. Or, just to see what she wanted to do, instead of always choosing for her. That led us to the Augmented Communication Department at Children's Healthcare of Atlanta at Scottish Rite. In July of 2009 with met with a communication specialist who spent about three hours evaluating Evelyn (along with an occupational therapist) and talking with us about motivates her and what strategies we could start to use at home to elicit more communication. We came away from that meeting with a loaner 'device' and some great tips to use.

From July 2009 - October 2009 we practiced 'scanning' with Evelyn. We gave her three choices (three foods, or three toys, or three activities) and she picked. We would record "yes, that's what I want" on a switch, say each choice and she would hit the switch when she heard the one she wanted. Often we would rearrange the choices and ask her two or three times to make sure she was really understanding the concept. She understood. for real. and it was awesome. :) She loved to choose strawberry sauce or yogurt to eat, to play in her little room, and to read stories. We still weren't totally convinced, but it seemed to work, and several times temper tantrums were avoided by using the button to discover she wanted a bath (and to go to bed, not that she'd admit it) or needed orajel for a cutting tooth.

So, we went back to the communication specialist, and we ordered her device. She has a Dynavox VMax - google it to see more details (hers is pink). Basically it is a fully functioning computer, with a touch screen, and some super fancy software (though Daniel says he could totally make it himself). We plug her switch into the device, much like you might plug a mouse into your computer. We start the software, and it begins scanning through her menus: Want, Play, Go, Talking with People, Feelings, Time to Learn, About Me, My Words. It announces each category out loud, and when she hears the one she wants, she pushes her switch. Then it opens up the menu for that category and starts scanning all over again. When she hits her button, it basically talks for her. So, if she hits the switch when she hears "play" the talked will say: "I want to play".

Here's an example:

• Evelyn hits play, so it says "I want to play"
• then she hits "Computer" so it says "I want to play on the computer"
• then she hits "Read a book" so it says "I want to read a book on the computer"
• so then we load one of her books on cd (living books - google them, they're awesome)

From those main categories there are layers and layers of menus, so under "I want something" she can tell us she wants her hair done, or lip gloss, or a massage. Under feelings she can tell us if something hurts, and which body part. This device has the capacity to function at the highest level (so someone with quadriplegia could run their business with it, by using a switch or eye gaze, etc) - so basically we can program it to enable Evelyn to do just about anything. Currently she can skype people, load websites to play games, and talk to us about lots of different things.

The response time is very different than having a "normal" conversation with someone, but since getting the device in December she's done remarkably well. We love our daughter, and seeing her gain some level of independence is just amazing. It was so encouraging to set up her talker in the hospital and give her the chance to tell us how she felt. She wanted to get in her stroller and go home. Her head hurt and she was tired. She wanted to watch Tinkerbell. It's hard to hear that your child doesn't feel good, but it feels SO good to know she's able to tell us.

This has given her wings, and it is probably the greatest blessing she has received thus far.

VNS

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

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

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

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

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

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

Fundo done

Well - Evelyn is now the proud owner(?) of a Nissen Fundoplication.  In short, the impetus for the surgery was to mitigate any reflux as a likely partial source for aspiration pneumonia.

We went in to the hospital on Sunday 18-Apr, just to be "checked out" by the pulmonology crew, and generally get ready for the procedure.  At 10AM on Monday, Evs had the procedure.  Happily, it was able to be performed laparoscopically, so Evs came back with 5 little heart-shaped bandages, in a sort of a rainbow across her tummy (see pic).

The first day was a little rough, as due to Evs' very recent respiratory issues - the anesthesiologist wanted her to remain on the vent for the entire first day following the surgery (instead of being extubated shortly after surgery "as usual").  That's actually the first time Kim or I have seen Evs on a vent for any significant period of time (if honestly ever actually seeing it) - so that was a "tough first" for us.  True to form, though, Evs said "enough was enough" - in part due to some seizures being out-of-whack (anesthesia'll do it every time...), coupled with some... ahem... "general agitation" - Evelyn extubated herself at around 5AM the following morning.  ;-)  She wasn't quite ready to go it totally on her own, though, and had to go back on bi-pap for the next day - but other than that - it was an uneventful surgery, which is the kind we like.

The rest of the week was essentially "recovery" - ramping down the bi-pap & oxygen usage; managing post-op pain; downgrading from the PICU to the TICU - "etc".  We also managed to squeak in a visit with the rehab doctors for another Botox treatment (seriously, Botox.  It helps with her muscle tone; see Botox!).  By late week, she was starting to look much more chipper, and was on "room air" during the day - pretty much back to baseline.  After taking her on a few tours around the TICU Friday, and the general hospital grounds Saturday - We were discharged early Sunday (for once, not during rush-hour!).

So - all's well that ends well, I guess.  Kim counted, and to-date, we're at 39 days of 2010 in the hospital.  Here's to hoping this surgery does the trick & we're much less frequent flyers at the Scottish Ritz for the balance of 2010!

Here we go again...

Well... "Here we go again... It's the same old song..."

Since the blog's a few weeks behind, let me rewind it, briefly.  After the last PICU/TICU hospitalization in mid-January, we were discharged, and home for about 4 days.  We then had to bring Evs back in for another ~4 days or so, just as she was starting to work a little hard again at breathing, but it was a relatively uneventful stay on "the floor" (non-ICU).  This made for more days of January spent in the hospital than out - but, nonetheless - we got out!

Coming out of all that, we ended up with nursing care at home during the week, to help keep up with Evs' daily routine (breathing treatments throughout the day, etc).  And from then until now - things were going pretty well!  Evs was returning to "herself"; she wasn't needing significant oxygen at night; she was getting back in to therapies - "etc".

But, then - we get to "now".

Right at a week ago, when the weather was equally nice, we took Evs out for a few hours, and opened the windows in the house, to get the "stale winter air" out, and get some of that fresh spring air in.

We don't know yet if that's what did it - maybe triggering some allergies - or if Evs picked up a random virus while out & about, or otherwise - but Monday (29-Mar), she started having problems keeping her oxygen saturation up, and Kim & her nurse took her down to the ER.  She seemed to settle out over the day, so she was sent him.  Tuesday morning (30-Mar), she had a follow-up appointment with her pulmonologist.  She saw the PA, who didn't see anything acute of note, and just had a general follow-up-type-of-visit.  By that afternoon, though - testament to how fast these things can move - she had a mild fever a was on 3L of oxygen, and still wasn't quite keeping her saturation up.  So, back to the ER she went.

After a somewhat-aggravating experience (as Platinum Medallion/Super Elite Frequent Flyers at Scottish Rite, we're not used to having a hard time in getting our way ;-) ...) and a chest X-ray, Evs was admitted, with likely diagnosis of pneumonia.  Again.  At any rate, over the course of the evening, she really just couldn't keep her oxygen saturation up - the pulmonologist on call for the night, who had thus far been working from home, came in about 2AM to check her out & work his magic in-person, but he finally had her sent upstairs to the PICU.  Again.

After trying their luck with a high-flow cannula - which was the most support she ever got on her last stay - we still weren't getting good numbers, so they bumped her to a BiPAP (what Evs is so graciously modeling in the picture).  In very short, it's a mask that seals around her face, and provides both inhale and exhale pressure, to help give her a little more "oomph" to her breathing.  This both helps keep her airway open, and also helps "fill out" her lungs a little more, to help fight or prevent atelectasis (when the alveoli in your lungs - like teeny little balloons that help you breathe - deflate or collapse on themselves).

After about a day and a half of that, she really started to show some good progress - they bumped her back down to a high-flow cannula, and she was really holding her own.  Also encouragingly, her blood cultures didn't show signs of infection, which likely ruled out a proper pneumonia, and likely just the aforementioned atelectasis.  She was clearly starting to feel better, as she was able to use her button to tell us she wanted pigtails, what she wanted to do, etc.

And then she had a seizure.

Now - Evs has seizures.  We don't like it, but that's her baseline; it's part of her life.  Honestly, it was probably prima facie evidence to the fact that she was indeed feeling better - "returning to normal" - which for her, includes seizures.  However, this one didn't stop (also not uncommon for her seizures when "coming out of a painful episode", such is illness, or cutting teeth, etc) - so they had to administer Ativan.  Which still didn't cut it, so they had to go for a little more, and finally some Diastat.  Both of which are related sedatives, and make an already-floppy airway more floppy.... and you can probably see where this leads.  It could have been an aspiration episode during or after the seizure (of refluxed food, or her own secretions), or it could have just been more obstructed breathing - but ultimately, it led right back to BiPAP.  Which is where we are tonight.

It's certainly frustrating to take two steps forward... then one back... then at least another half-step back (if not a whole step).  On the up-side, Evelyn's body doesn't seem as stressed as before - likely because it's not an infection or "proper" pneumonia - last time, her heart-rate was consistently 160+ BPM, and her respiratory rate was equally-consistently 60+ breaths/minute.  Both measures are significantly less this time (though her heart-rate crept up some today...), which is a good thing - she just seems to be having a hard time keeping her oxygen saturation up.  On the down-side, it's certainly unpleasant that she's having these issues at all.  It's also a little more bothersome that she's spending more time on the BiPAP - which for all the trouble she had on her last stay - she never used.

At this point, it's most "watch, wait, and continue respiratory treatments".  To try and get more to "root cause", it boils down to two issues (at risk of oversimplifying) - preventing her from catching germs (viral or bacterial), and preventing her from aspirating.  The former should be getting easier by the day, as the weather warms up, and there's obvious measures we can take to at least help (keeping her out of crowded areas, etc).

The latter is a little more complicated.  Outside of gradual speech therapy, there's not tons you can do to quickly & markedly improve swallowing skills such as to prevent aspiration.  So you're left with treating the cause of things getting into the airway to be aspirated in the first place.  In Evs' situation, those things likely come from 2 places: things she may reflux up, and her own secretions (e.g. saliva), particularly during a seizure.

So, we are beginning to talk about a Nissen fundoplication to prevent or rule out any reflux as part-cause of aspiration.  We've also been considering a Vagus Nerve Stimulator for a few months, as a next-step in trying to get her seizures under a little more control, and will likely take at least the next steps towards that during this hospital stay (a 48-hour EEG).  The timetable on either of those is still a little uncertain - but will likely be in the "near-ish future".  Of course - first thing's first - she needs to get to breathing better!

So that's where we're at.  Not a lot of "great" news, I'm afraid - but, at least as of this writing - she's holding her own, and not in a rapid or marked decline.  The more these things happen - and the more "help" she needs - the more it bothers Kim & I, to be sure.  But, as usual, Evs is fighting it all with superior grace, style, and just a little bit of 'tude.  ;-)  So I will sign-off focusing on that, and thank you all again for the thoughts & prayers - and wish a Happy Easter to all!

Out of the PICU; in to the TICU!

Well, so far - so good!

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

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

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

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

No news is good news

Apologies for the lack of update yesterday (hence the subject, aptly quipped by a friend) - Daniel's mom spelled us at the hospital overnight - so we both got home about 10PM, hit the bed, and didn't move for a solid 8 hours.  Thanks, mom!  ;-)

At any rate - I'll keep this brief as it's late again (I'm back on night-duty) - but the last day or two has been reasonably quiet, and filled with (as expected or hoped for) "slow but steady positive progress".  The doctors even decided to forgo a lung X-ray this morning, as Evelyn's previous day X-ray looked so much better, and when they listen to her breathe - she sounds much better.  Evs spent all day yesterday awake - which though by the end, she was "ready for bed" - that is a first since Saturday.  So that is all very exciting!

Oh, and as per the picture - we did take a brief moment yesterday to "be with the Jackets" in spirit, in their ill-fated Orange Bowl venture.  No, Evs didn't actually watch the game, and that's probably what the issue was for the Jackets - sorry boys.  ;-)

We have had a few "minor" issues in the last day or two - there was some concern over kidney stones, but that (as of this writing) has been nullified - However, they're still keeping close watch over things in that department.  Evs also had a little agitation late yesterday - but I think I'd be agitated, too, by this point.  ;-)  Not at all to "downplay" or "mock" her feelings - but frankly we're glad that she's feeling well enough to express them.  :-)  We're also working through some more of her "regular" stuff that we've had to put on hold over the past month due to the hospitalizations - just getting in consults with some of her other doctors, and figuring out how to adjust various ourses of treatment given the added complication of being hospitalized (Rehab, Orthopedics, Ophthalmology, etc).

At any rate, based on our most recent consult with Evs' pulmonologist - assuming Evs continues the uptrend, of course! - we expect to remain in the PICU through late this week, and then be downgraded to a "regular" room, where we'll likely remain through late next week.  As painful as a solid 2-week tour might be - I think her pulmonologist really just wants to see her "completely and 100% well" before giving us our walkin' papers.  Which is hard to argue with.  ;-)

With things on the up-tick - assuming they stay that way - I'll probably slow down the flow of "push" updates by e-mail a bit, just to give everybody's mailbox a break, and let the subject of this post be the prevailing sentiment. However, I'll try to continue to get several updates over the course of the week posted here, and drop a less-frequent mail or two to let you all know how she's doing.

A last update on the other character in the Forester household, as several have asked - Mr. Owen has been such a big boy, with all of this going on.  He has endured numerous car-rides, waiting rooms, caretaker hand-offs, bottle-feedings, late-nights, seeing lots of strangers, absence of "mommy AND daddy", not seeing Big Sister at all - ad infinitum - with exceptionally good humor and disposition. We continue to thank God every day that we've been blessed with a baby who sleeps well, has a good disposition, and tolerates all the circumstance.  We know very well that not all babies are "so easy", and we know that even Owen could "turn on a dime" in that department - but for now, we figure it goes to show "you get what you need, when you need it".  Anyway, all-in-all, for the positive - he's gotten lots of extra grandparent time, as well as enough praise and adulation from every passer-by that comes within 10 yards to give the kid a head the size of a  bowling-ball - so he's making lemonade out of his lemons.  ;-)

Anyway, thanks again, so so much, for all the thoughts, prayers, concerns, notes of encouragement, "Likes" on the Facebook posts, and offers & instances of help.  It's really hard to imagine grinding through all this in absence of it, and it is very much appreciated.

Vest therapy & a good day

Well, I think we can say that all circumstances considered - Evs has had a pretty good day today.  She got a bath early this afternoon, which she wasn't altogether happy with - but seemed to be feeling "herself" as much as she has since being here, which was evidenced by her stomping her feet through the "bath" (hospital bath-on-a-bed), since she didn't necessarily enjoy it.  ;-)  Afterwards, though, she was pretty "awake" and "on" for a while, and so we put her in some pajamas from home (her Tinkerbell PJ's; a fun Christmas gift from a friend), we got her glasses on, and just got a little "Evs time", which was really nice.

I took the opportunity of her being "awake" and doing well, and having an opportune moment with the web-cam, to grab a quick clip of her in the Vest therapy, since I don't know if my earlier descriptions of the subject sufficed (or ever could!).  Per earlier description, in very brief, it's essentially an air-filled vest that is filled via an air compressor.  This does two main things, both designed to help "bring stuff up".  Firstly, it just constricts & tightens some around her abdomen - think of your tightening diaphragm & core muscles when you cough - Evs has a hard time with that, so this "does it for her".  Secondly, it applies chest physiotherapy, which can be thought of roughly akin to "slapping somebody on the back" to help them cough:

It uses a compressor to inflate and deflate the vest rhythmically at timed intervals and thus imposes high frequency chest wall oscillations that are transferred to the lungs.

So, all that said - here it is in action, for the curious.  ;-)  I'll apologize that the video is not the best; the audio has moments where it gets a little scratchy, and the web-cam had a hard time re-focusing on "all the stuff" - but here-goes:



All in all, with today at a close - we can say it's been a good day, I think.  Definitely a good day, when coming on the heels of yesterday.  In brief consult with her pulmonologist, the name of the game is pretty much also as earlier-described - wait, watch, and wean - where the latter is weaning down or ramping down treatments as she tolerates, or otherwise hopefully improves - e.g. dial down the oxygen; put more time between - and less medicine into - the nebulizer treatments; etc.  We're definitely not out of the woods yet (I guess if we were, we'd be out of the PICU), but "today was a good day", and we'll take that!  Thanks again for all the thoughts & prayers - they are so very much appreciated.

morning update - hopeful bottom-out & up from here!

Well, just a short update - Evs had a pretty good night last night - the first half of it was better than the second half, as she seemed to be a little agitated for a while in the wee hours, which also got her heart rate back up - but for now, that's abated, so I'm hoping it was a "I'm-feeling-well-enough-to-complain" for a little bit.  ;-)

At any rate, the big test of her lung x-ray was done this morning, and I'm please to report that it looks better.  Not "clear and resolved", by any stretch, but "progress has been made", and that's the answer we needed.  Evs also managed to dodge any intubation last night, so that's also very positive.

The name of the game today, for the most part, will be "watch and wait" - she'll move from continuous to intermittent breathing treatments (nebulizers), as well as titrating down her Dopamine drip, so long as her blood-pressure holds (which so far, it has).  With any luck, she'll hold steady or maybe have some small improvement, and that will be a good day.

So I find myself using a phrase that I use at work more - we are "cautiously optimistic" that we've bottomed-out and are headed up.  As we've seen, things can swing in either direction quite quickly - but hopefully they're more "upswings" than "downswings" from here on out!

"She's a very sick little girl"

Well, I wish I had better news to report, but "the news is the news". Evs did fair through the night last night (outwardly, anyway), but overall today was up & down, and the doctor's prognostications of "it'll get worse before it gets better" seem to have held true. Tonight left us teetering on the brink of intubation, but so far, holding off.

The quick run-down of the day:

• As of this morning (chest X-ray), Evs' right lung is ~75% "crudded-up" - this is a "marked" degradation overnight from the previous night
• Her left lung was "hazy", but "okay" (for now)
• Labs this morning showed a little more conclusively that it is bacterial (rather than viral) pneumonia
• This means it was a likely result of aspiration, which we'll have to re-evaluate the cause & future prevention of - e.g. it could be anything from her coughing & aspirating what she coughs up, to aspirating some of what she eats, to have some level of reflux & aspirating that.
• Her antibiotics were upgraded to stronger, broader-spectrum antibiotics - Vancomycin andMeropenem.
• We added a Vest to her treatment regimen, which is essentially an inflatable ... well... vest, that can have air-pressure and percussions applied to it, to help "squeeze & shake out" the mucus in her lungs.
• Evs really just struggled up-and-down over the course of the day - sleeping, for the most part, but not getting much rest, due to constant "huffing and puffing". She stayed reasonably oxygenated (over 90%), but her heart rate was consistently 160-170 bpm (peaking at 180), respiratory rate 60/min (that being a breath a second, sustained!), and by later in the afternoon, her blood pressure started to dip (which is also likely contributing to her increased heart rate, etc).
• The low blood pressure was likely due to her own body not quite reproducing enough new blood cells due to the infection, and her just working so hard all day to breathe.
• Evs' supplemental oxygen flow (well, heliox & other associated breathing treatments included) topped out at 15L - this, if nothing else, is indicative of the day she had - 15L is huge. We're still pretty new to the respiratory world, but at home, we considered 2L "bad".
• Dopamine was added (IV drip) to help with the blood pressure, but it didn't seem to completely do the trick, so she risked slipping into shock. Thereby, late in the day, a blood transfusion was started, to combat the falling blood pressure. New IV sites for that wouldn't hold (means more worthless sticks... :-( ...), so they started a central line (femoral IV).
• If her blood pressure, respiration rate & heart rate don't stabilize - she will likely be intubated & ventilated.
• We will likely start a 7-10 day course of IV antibiotics via PICC line tomorrow.

As you can see - quite a day!!

On the positive side, while having an X-ray taken to verify the placement of the central line, they could pick up some of her lower lungs on the film - while not the entire lung (as it wasn't the subject of the X-ray), what they saw did seem to show that the infection was clearing some - so that's heartening. Also, as of this writing - the blood transfusion is about half or more complete, and her heart-rate has dropped a good 20-30 bpm's or more, and her blood pressure is looking better. As long as she can maintain where she's at or better for the night, we should be able to avoid intubation - so here's to hoping for that!

In short, I think my dad summarized it best earlier today - "she's a very sick little girl". It's also just been a good long while since we got a proper "waiting room debrief" from a gowned doctor, which coupled with their sincerity in relaying the seriousness and relative precariousness of Evs' situation - is a little unnerving.

Anyway, other than monitoring her various rates overnight, the next "big check" will be early tomorrow morning, when another chest X-ray is taken, to see how her lungs are looking. More then, or as news breaks!

Respiration & Pneumonia

Well, I had intended to use this weekend to finally update the blog on "December 2009", as Evs had a bit of a rough-patch there - but this now finds me writing yet again from her bedside in a hospital room, so the rough-patch has not quite left us behind with the rest of 2009!

December 2009

In very brief, in December, Evs had 2 stays at The Scottish Ritz for respiratory difficulty. We think it all started with a rough seizure, that lead to several administrations (in consult with Neurology, of course) of Ativan & Diastat (her PRN seizure medications). Those are both strong sedatives, and it's not uncommon for her to have a "floppy" airway afterwards - but the multiple administrations stacked-up to really give her a challenge with moving air, and so we ended up in the ER and then were admitted for the night. We were discharged late the next day, as she seemed to be moving air quite a bit better, but with some new equipment to add to our arsenal - a nebulizer and associated medications (Albuterol & Pulmicort), a suction machine & a pulse-ox monitor.

Over the next 4 days, she "wobbled" a little, seeming to be getting a little better - we suctioned, nebulized, and monitored tirelessly (or rather - in spite of being tired), but eventually we had to bring her back in, as she was really struggling with moving air again - just "junky", congested, and wheezing; in spite of all our treatments. This time she was diagnosed with bronchiolitis; essentially, she seems to've just picked up a "wintertime bug" (perhaps at her first stay in the hospital), but since she is less mobile & has lower muscle tone - it's all just a little more difficult for her, to cough things up & the like. We spent about 6 nights on that stay, and came home with her on a slow-but-sure uptrend. We added oxygen and a cough-assist to our equipment inventory at home, and started Evs in consult with a pulmonologist (coincidentally, the father of a classmate of my brother's from West Point).

Evs weathered Christmas & New Year's pretty well - she had a few up days & down days, but all-in-all seemed to be continuing the rough upward trend - she was looking perkier, acting happier, not needing oxygen, and just generally seeming "better".

Current Situation

However, once New Year's was over (I mean, literally - an hour-and-a-half past the end of 1-Jan), she started to have some more difficulty. We put her to bed Friday 1-Jan with a little bit of oxygen (0.5-1L), since she seemed to be breathing a little fast, but not of tremendous concern. Then at 1:30AM on 2-Jan, her heart-rate really started staying above the "normal" limits (~150bpm) - whenever she's seemed to be anything but "breathing easy" over the past weeks (when we've been at home!), we've kept her on the pulse-ox monitor - which also measures heart-rate, in addition to oxygenation - so it alarmed & woke us up. Over the next 1.5 hours, we took her temperature, which was feverish (101-103), and her heart-rate continued to rise (160-170bpm). We continued to add oxygen (up to 2L), mainly to see if that would help her relax - her oxygenation was "okay" given the amount of O2 she was on - she was generally hovering ~94% or better (ideally, a "healthy" person would be pretty close to 100% when "awake", on "room air"). Given all that, though, we suited-up and brought her in to the hospital (ER) at 3AM (on the up-side, the ride down GA-400 was as smooth as it gets!).

While in the ER, on the same amount of oxygen - her oxygenation really started to deteriorate (less than 90%), and continued to do so in spite of increased oxygen flow & breathing treatments (including heliox and magnesium sulfate, in addition to the usual suspects of Albuterol & Xopenex). A chest X-ray showed that she had some pneumonia, primarily in her right lung. So, those two things taken together got us promoted from the "regular" floor to the PICU, which is where I'm writing from now.

After a long day there, Evs seems to be (crossing my fingers!) stabilizing some. This is our first bout with a pneumonia, and as we saw, they can move quickly, and also get worse before they get better. So far, they've had to keep her oxygen flow up pretty high, but we still have a step or two between where she's at and "rock bottom" in respiratory intervention (that being, intubation & ventilated), so that's hopeful. But - we shall see.

Overall

In general, we knew that respiratory issues were a definite possibility, if not an eventuality for Evs - it's just kind of the expectation for anyone dealing with cerebral palsy-like symptoms (low mobility, low muscle tone, low coordination, etc), and it is certainly not at all uncommon amongst others afflicted with Aicardi Syndrome. To those ends, we're thankful that Evs made it through her first 2.5 years without any appreciable respiratory issue. However, anticipated or not, it's certainly no fun to enter yet another new sub-discipline of medical management, and especially anything dealing with pneumonia. But - here we are, so on we go!

Thanks so much for the thoughts & prayers - they are definitely helpful. Kim & I are both staying the night tonight, thanks to my folks watching Owen at our house overnight. We'll try & post a few updates along the way - but the main name of the game is "keep her oxygenation up and move it higher" - which comes by way of "clear lungs"!