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!