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"!

Botox!

Yep; you read correctly.

Last week, we had Evs in for a Botox treatment. No, not to treat any premature wrinkles - this was to treat her spasticity. While injecting a neurotoxin into your 2-year-old isn't really high on anyone's list of fun stuff to do around here - it has helped many in this regard, and is otherwise proven safe enough (if not by those that do it cosmetically).

The basic idea is this: The Botox is injected into the "problem" muscles; that is to say, the ones that Evs often has "locked" due to a spastic episode. For her, this includes her pectorals (leading to pulling forward/hunching over), her thighs (leading to locked knees), her calfs (leading to tight ankles), her elbows... etc. Over a week or two, the Botox starts blocking the release of certain neurotransmitters that aid in muscle contraction - rendering her muscles able to contract less; thus hopefully helping with the spasticity.

We'll have to "play it by ear" and see how it goes ('cuz that's just how we have to roll around here) - but ideally, it should help her spasticity, for a period of 3-4 months.

At any rate, we had that done last week, and have definitely seen some improvements to her tone. Evs has been working on some teeth between then and now, which typically causes her tone to increase (as well as increased seizures, but so far, we've avoided any ER episodes) - but we're hopeful that this is an overall "good thing" for her - so far, it seems to be.

In other, "more fun" news - Evelyn has also been making great use of a communication device and communication switches. I'm going to save more on that for a separate post - but we're really excited about the possibilities with being able to help her tell us about her copious - and strong - opinions. ;-)

At any rate - that's the latest!

Spasticity & Seizures

Hi all -

I thought I'd drop a brief update on Evs, since we're about 3 months overdue. Evs' last round of hospitalizations ended up - finally - with a diagnosis of spasticity, which is essentially uncontrolled tightening of her muscles. It's not uncommon with neurological disorders, but it really took us a long time to get to the diagnosis - we worked through her entire GI system; checked the her intracranial pressure; did a full-body scan for broken bones... you name it. We even, actually, asked about dystonia or spasticity, but were assured it was not - after seeing a doctor specializing in it, he diagnosed & started treating for it - and Evs started feeling a lot better. SO - frustrating that it took us so long to get to that answer - but, happily, we finally got one.

After that episode, Evs had a reasonably-good run of it, until mid-June or so - including a week at the beach, which of course she enjoyed. ;-) Partly owing to higher-tone from the spasticity, her ankle tone has been very high, so we got some new AFO's made. But, all in all, she had a good May - mid-June; doing therapies, and generally being a happy kid. :-)

Around mid-June, we believe that she started working on more teeth, and - as is her habit with teeth - had some prolonged seizures, that none of her emergency meds would touch. So, she landed back in the hospital for just shy of a week in late June. She was discharged on a short-term regimen of Dilantin, which we'll keep for a week or two to "bridge the gap" while ramping up her existing Lamictal a bit more. That's kept her a little drowsier, lately, but we're hoping that will prove short-lived - shorter-so than the Dilantin regimen itself.

Anyway - that's about the latest, here! Happy 4th of July!

Update on Evs (@ CHOA-Scottish Rite)

We've been remiss in updates the past month or two, so thought we'd send one out, since there's been "some action!"

The reader's digest version is that we're currently in Scottish Rite Hospital with Evs - where we've been for 6 days & counting - getting some tests run to try & explain some otherwise "unexplained pain" that's been plaguing her for the past few weeks. We've got the near-term steps defined - believed mostly abdominal/GI issues - and pending the outcome, hope to be out of here by early to mid-week next week (perhaps 17-Mar or 18-Mar). Evs, Kim & I are all doing "okay", but obviously would prefer not to be here! :-)

Now the largely unabridged version for those interested (or the patience ;-) ...)...

For the past 3 weeks, Evelyn has had what we have only been able to describe as "unexplained pain". This would present to us as her being extremely "tight" and "stiff" (on a child who's laundry-list of particulars includes "low tone"), arching her back, agitated, and often fussy if not outright howling. While perhaps not outside the realm of possibility of "typical" behavior for a "typical" 22-month-old, after a few days, it started to get concerning.

Our initial suspect was teeth - Evs has always been a delayed teether, and most who have seen her gums will testify to the fact they look "painfully full and ready to pop". ;-) She has had a number of teeth (both molars & incisors) cut in the recent days and weeks, so it seemed an obvious conclusion. However, after visiting 2 dentists who could find "nothing remarkable", and several more days of the continued behavior, we started to get more worried.

We next turned to her GI tract as the suspect. Many Aicardi kids have a number of issues in that department, and being on the Ketogenic diet can certainly exacerbate things. After several visits to her pediatrician - who also finally managed to observe or instigate some abdominal pain - Kim took her to the ER at his recommendation. The net result of that visit was "not much".

We took her back to her Pediatrician the following Monday (2-March), with Evs seeming her normal, cheerful, bubbly self. Of course, the next day, things picked right back up where we'd left off. By late in that week, we'd managed to raise the issue with enough of the Ologists to get them to order a complete GI series - pretty much following some barium from top to bottom (in every sense of the word). Results, as with everything so far - were "not remarkable".

The next Monday (8-March), Evs was again seeming "pretty okay" in the presence of her pediatrician (who I guess by now was thinking he could heal through laying of hands ;-) ...).

After a great night, Evelyn woke up Tuesday (9-March) with a prolonged seizure. It wasn't overly-dangerous - she still has several seizures every day - but it went beyond the pre-defined time-bound by our neurologist, and Kim exhausted our "at-home" emergency protocol options. The seizure seemed to move from her "standard" seizure into the exacerbated "tight" and arching position - precluding putting her in a car-seat - So, Kim & Evelyn took a ride down to Scottish Rite via ambulance.

The seizure was dealt with shortly after arrival at the ER, but the exacerbated tight arching & agitation still continued, and given the past 2 weeks, the ER doctor opted to have her admitted.

The focus then shifted to "what's up with the apparent pain episodes". While not thrilled to be in the hospital - or what was required to get there - we were not necessarily unhappy to have her admitted, to get some stronger attention on the "pain episodes" (which, heretofore, had taken some stamping of feet to get the Ologists' attentions).

Thus was kicked off 6 days (and counting) of admission to Scottish Rite to figure out "what's going on".

After the neurologist-on-call - along with Evelyn's usual neurologist - witnessed one of Evelyn's "pain episodes", they concurred that it was "pain" - as opposed to a "neurological event", which is an easy thing for the Ologists' to chalk hard-to-explain things up to in kids with neurological disorders. This, of course, was a relief to us, but the question remained "what was it?"

Focus then shifted back to GI. We started off with a ultrasound of pretty much every organ in Evs' little torso that can be seen by ultrasound, mostly looking for kidney stones, gallstones, etc. Thursday, still seeing continued pain episodes, our attentions turned to reflux as the possible cause. Evs has had some reflux, but our thinking perhaps that for whatever reason, something had changed & it was now worse. Thusly, we started a 24-hour pH probe on Friday (13-Mar), to measure acid levels in her esophagus over time.

The analyzed output seemed to indicate that Evs was indeed having some reflux events, but at least in the doctor's analysis, more as a result of her arching & crying, than as the cause of it. To put that theory to bed one way or the other, Evs then went NPO (nothing to eat or drink; IV fluids only) for 24-hours - theory being that if there was nothing in her stomach, there'd be nothing to reflux - so if the "pain episodes" continued, reflux couldn't be the root cause, but if they diminished, perhaps it was the root cause.

Other than being grumpy - markedly different from the "pain episodes", and certainly understandable given being cooped up in a hospital bed and hungry(!) - Evs seemed quite a bit like her old self on Saturday (14-Mar)! Not 100% by any stretch - but "more like herself". Accordingly, we were feeling optimistic that we were finally - at long last - on to something!

In consult with the GI doctor, we opted to continue another 24 hours, just to see how things shook out. After sleeping all night Friday night - a welcome relief to all involved - she awoke Sunday (15-Mar) okay, but then started up an atypical seizure (atypical for her, anyway) - one that is kind of a "crunching" motion, with her abdomen literally shaking. It's one we've seen before, just quite rarely - it is possibly a more "classic" infantile spasm. The nurse came in for & administered Evelyn's morning meds, and we sent her back for some Ativan (Evelyn's 1st line emergency drug), as her seizure wasn't winding itself down with the prescribed time.

Shortly afterward the nurse arrived back with the Ativan, Evelyn began stiffening, arching, and crying - a complete return to the "pain episodes" we hadn't seen since early Friday. The Ativan administered, Evs drifted to sleep, where she's been since.

This brings us to the present.

While this could have blown a hole in our theory that reflux was the root of the problem, our hope is that the meds on Evs' empty stomach - along with increased abdominal pressure due to the seizure - caused another reflux episode, which kicked off her pain response.

Thus, we're continuing the "no-food" until the morning, at which point we're hoping we'll have seen little (or really - "no") pain episodes. We'll then try a regular (though probably somewhat slower) feeding with Evs, and "see what happens". If the pain returns, then we've got a pretty good line on what to treat. If not... well... it's hard to say.

At any rate - as you might imagine, we're just about "done" with being cooped up in Suite 118 of the Scottish Ritz, but are otherwise doing alright. Kim & I are trading-off nights at home, and Evs - while obviously not in her highest or best of spirits - is doing alright.

At any rate... that's the latest!

Well this is WAY overdue!

Hello friends, family, countrymen,

We have been terribly remiss in updating this blog, and for that we are truly sorry. All is well, and I'll provide a fairly good update below. As a family, things are great. Daniel is traveling to Sweden for business for a couple weeks each month, Kim is enjoying cooking, reading and playing with Evelyn, and Evelyn is enjoying all her various playtimes and therapies. Rocky is enjoying licking the dishes while the dishwasher gets loaded. Here's the Evelyn lowdown:

September:
Evs had a good month; therapies were in full swing, the weather was beautiful, and Evelyn seemed to be moving to a new level - of alertness, learning, progress - altogether just a good, fun month.

October:
More fun and learning. Evelyn absolutely LOVES aquatic therapy, and socializing at CVI during her mom and me music group. She started eating more and more by mouth, and made an absolutely adorable Bumblebee for Halloween. Evelyn also attended the annual Scottish Highland Games at Stone Mountain with mom, dad, and OJ the Great (aka - greatgrandmother Jean Forester). She really enjoyed displaying her Forester pride, watching the log throwers, and listening to all the bagpipe music.

November:
Sweet Evelyn got a bad cold, which went into her eyes, and then her sinuses, and ears. Lather, rinse, repeat. Basically when it hit her eyes she got some clogged oil glands which then became irritated and required surgery (in December) to clear up. Even with antibiotics those darned eyes were harboring the infection, so November was not a happy month for miss Evs. She absolutely had lots of good moments - therapies, Gymboree, Music class, a visit from Grandpa Mike, Thanksgiving... but the sinus infection and eye infection lingered for most of the month.

December:
Hooray for the right antibiotic! (Did I mention we went through two that she was allergic to? It is simultaneously amusing and worrying to watch your child turn red in a matter of hours.) At any rate, Evelyn had surgery to clean up her eyes, and got a good shot of Penicillin for the sinus infection. By Christmas she was back on track.

Just before Christmas Grandpa Mike visited again and he and Daniel built a track and support system for a special suit. This suit (like a super-strong onesie with straps coming up from the shoulders) allows Evelyn to be in an upright position while having the track and supports hold most of her weight for her. "CC" and I designed the suit (based on a very expensive similar piece of equipment seen elsewhere) and CC sewed it together. This has been an awesome addition to our playroom - Evelyn can explore independently in a very natural position. In just a few short sessions in her little suit she has been reaching for toys, turning herself around to get to toys, and moving her feet (again to get the toys!). Our hope is that this suit will encourage not just independent play, but walking as well.

Here are a few skills Evelyn has started to display in the last few months:
1. Evelyn is eating a good 80% of her calories by mouth. Drinking is progressing more slowly, but she does do some drinking at all meals and is doing well using an open cup.
2. Evelyn is learning to approximate the sound 'Ah' - she will easily imitate the mouth shape, and has recently started to 'squeeze out the sound'. It's tough for her to get the actual sound out, but it is so cute to see her twist, open her mouth wide, and vocalize - and then of course smile at her mommy or daddy with pride.
3. Evelyn is starting to make choices using switches and her vision. She will either push a button or look at a preferred item to choose what activity, song, food, etc she wants. This is a very new skill, but she's shown a lot of promise doing the same thing with computer games, so we expect that transferring it to daily life should be successful.
4. Evelyn is bearing weight! She received a new stander in October, and while in or out of it she is willing, able and enjoys supporting her own weight. Coordinating her head, neck, torso AND legs all at the same time is a challenge, so she still gets assistance in staying upright - but she LOVES it.

I have to say, and I think Daniel would agree, that we love Evelyn more every day. She is such an amazing little lady, and we are really enjoying getting to know her. It is so neat to watch her grow up - her opinions, preferences, moods and personality are all emerging so clearly, and it is fascinating to watch her learn something new, or respond to something with obvious comprehension.

Well, that's about it for now. There are lots of new pictures available at http://picasaweb.google.com/dforester

Thank you, as always, for your continued interest and support.
We love you guys!