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"!