Inpatient - Endocrinology - Day 1

A planned admission is such a different animal, and yet really, it's all the same zoo.

We arrived early enough to have the luxury of taking our time as we schlepped all of our gear through the hospital.  I felt 50% absurd for actually bringing luggage, but also 50% justified, because I know that we're going to be here for a few days, and I'm a human being that needs food, clothing, and the occasional shower.  It makes perfect sense that I might pack those things in a bag and bring them with me when I arrive.  It's just that it adds this air of "vacation" to the whole experience and it feels so inappropriate.

Anyway, I let Aidan pick out a treat from the gift shop - he went with a Doc McStuffins magic ink book - and we went to the Asplundh Center in the Main Atrium for Admissions Registration.  We answered a few questions and I signed some papers - I assume this is the stuff I normally do bleary-eyed and half asleep in the ER at 3am while awaiting a bed on 5 South.  It was downright luxurious to kick this party off 12 hours earlier in the comfort of a quiet room that didn't smell like the previous occupant's vomit.

We had a short wait while they got his room ready - namely, swiped a crib from some other room, because I know, he's a million and should be in a bed by now, but get off my back, okay? - and then we headed up to 5 West, where we settled into Room 9.  We'd been warned that the rooms up on the Endocrine floor were small and not private - so we were prepared for the worst - but actually, no complaints here.  Ours is small by 5 South standards, sure, but rooms up in Gastro are pretty huge (I've always wondered why.  Were they meant to be doubles?) and it's definitely private.

Nobody else is fitting in here.

Aidan had no problem getting right down to business, by which I mean getting his flashcards sorted and his DVD player going.

He quickly decided that one measly show was insufficient.  Any kid worth his salt needs at least two forms of electronic entertainment at all times.

I had to give the backstory to the docs here - which I realize now that I never gave on this blog - so here's the very short version.  A couple of weeks ago, Aidan had a routine scope at CHOP Exton.  The prep included being on Pedialyte for the entire day prior.  He seemed fine at the time, but for some reason, we didn't check his blood sugar that day.  I really wish we had, in retrospect.

The next morning, we woke up early to go down to Exton for the scope.  He seemed lethargic, but it was also 5am, and who isn't lethargic at 5am?  I didn't think much of it.  He fell asleep in the car on the way there, and I had trouble waking him when we got there.  I checked his blood sugar, and it was critically low - 34.  I rechecked twice, and got 31 and 36.  He was rushed back to the PACU and started up on IV dextrose, which brought his blood sugar up to 146.  He was taken off the dextrose for his scope, but within 15 minutes, his blood sugar dropped to 111.  In another 30 minutes, it dropped to 80.  He was given formula boluses, but it fell further to 71.  They put him back on IV dextrose, which got him up to 186, and then he was released to go home.  30 minutes later, he was at 118.  30 more minutes later, he was at 67.

Since then, he's been on 24/7 feeds with no breaks at all, because he's not holding his blood sugar up on his own.  The thought was to admit him today to figure all of this out with a plan in place for what tests would be done to help get to the bottom of things.

I was thinking that it was the smoothest admission we'd ever had, which was obviously the stupidest thing one can ever think in a hospital setting, because you're begging for trouble.  Literally as I was thinking these hazardous thoughts, our nurse brought news that despite the multiple conversations I had with the admissions and endocrine people prior to our arrival, they were unable to find any formula for Aidan.  And we only had this much left of the feed he came in on:

Long story short, I had someone drive some in from home, and like last time, by the time it got here, CHOP was able to rustle some up.  I really wish they could get their act together.  I know it's not a common formula, but it's prescribed by our CHOP doctor and he's gotten it inpatient before - I know it's here.  Why do we have to go through this every time?  Is it too much to ask where they found it, so that next time this happens, I can tell them where to look?  Sigh.

Anyway, the plan for the rest of the evening is to keep him on his continuous feeds and check his blood sugar every 3 hours to establish his baseline (90something at 6pm, 80something at 9pm).  Tomorrow, when the team rounds, we'll discuss the plan for his fast.  That's when the real fun starts.  I'll keep you posted!

CHOP in June - Part 2

EEEEEK!  It's AUGUST and I didn't finish telling you about our trip to CHOP in June.  Life really got away from us.

When you left us, Aidan had been painfully vomiting green bile.  After a weekend of this, we were sent to the ER for some tests, and ultimately allowed to go home.  A week passed without improvement and we followed up with GI, who sent us directly back to the ER, where Aidan was admitted on a Friday night.

Saturday on 5 South was exceptionally uneventful - no feeds for Aidan and no vomiting.  Scattered episodes of stomach pain, but nothing severe.  Around lunchtime, we started Pedialyte at 35ml/h (half of his normal rate) and it was tolerated well.  Around dinnertime, we increased to 70ml/h and after some initial discomfort, he settled down and tolerated that well too.  I don't know - maybe he just needed some gut rest?

A gut at rest :)

On Sunday, the doctors rounded around 10am and came up with a plan.  We'd be starting feeds slowly and increasing every four hours as tolerated.  If he had an episode, we'd call radiology for emergency imaging.  If not, we'd do imaging on Monday morning.  I figured we'd have a quiet day.  But it wasn't without bumps in the road.

For instance - did you know that if your child is on PurAmino, you should come prepared for no one - ever - to have heard of it?  For the formula room to insist they don't have it?  Nor do they know how to mix it.  Tom had to make an emergency trip downtown with a supply of formula to save the day.  When it arrived, it took hours - literally, hours - for the formula room to figure out what to do with it.  The kicker?  They eventually realized that they did have it (still didn't know how to mix it, so thankfully I recently blogged about how we mixed it - otherwise there's no way I'd have remembered).  Long story short - the 10am decision to start feeds actually took effect at 3:30 due to all of that hoopla. And advancing feeds every 4 hours?  HA!  We got up to 45ml/h before having to go NPO.

Sunday also brought some stoma pain - It's looking a little bit infected, which is a shame since it's normally such a beautiful stoma.  They ordered up some mupirocin (easy enough) and some tylenol, which caused a(nother) pharmacy debacle.  See, we don't use their tylenol, we use ours.  They don't have dye-free tylenol because dye allergies aren't real, evidently.  So we have to bring our own and send it to their pharmacy, where they verify it (whatever that entails) and eventually it arrives back in our room to be administered.  Unfortunately, the "eventually" is sometimes a while. Like an hour or two.  Which is really unfortunate for a kid who's in pain.  I later learned that the order was written incorrectly, which confused the pharmacy.  Then the pharmacy wrote a confusing note back to the doctor, who was (you guessed it) confused.  Then our nurse inexplicably left the floor without her phone, and the nurse filling in knew nothing about it.  Sigh.  We did work it out eventually. 

So as of Sunday evening, the plan to advance feeds was pretty well compromised, because he would have to be NPO (nothing by mouth.  Is that common knowledge?  I don't know if pre-all-of-this-me knew those particular letters, but we know them well now) at midnight in preparation for tomorrow's Upper GI.  We'd started at 35ml/h at 3:30pm, and around 8:30 we moved up to 45ml/h, but no further advancement because of the looming Upper GI.

An Upper GI is a special kind of X-Ray that helps doctors see the gastrointestinal tract.  Preparation includes drinking barium contrast (so some people call this a "barium swallow study") and then taking lots of pictures - usually about 2 hours' worth, and sometimes even more!

On Monday, we woke up just in time for the Upper GI.  Aidan took one itsy bitsy sip of Barium and then couldn't be convinced to take more.  Luckily, we aren't too concerned about the "upper" part, and really just needed to see the barium move through the stomach and small and large intestines.

The first thing we did with the Upper GI was take a "before" picture - his tummy without any Barium.

I spy a tummy!

 Then, Aidan took his tiny sip of Barium, and they took X-Rays.  And then Barium was injected into his G-Tube, and more X-Rays.  Then we got to cheat a bit, since he has a J-Tube too.  We got to inject Barium into the J-port, and the tech said that he thought we'd get out of there early!  After that, we had to wait in a little room for 20 minutes while the Barium moved through his intestines, and then back to the X-Ray machine for more pictures.  I'll speed this story up and tell you that we did not get out of there early, even with the cheating.  The tech was thinking we might only need one or two cycles of waiting/pictures - so we'd be done in less than 90 minutes total.  Actually, it took over four hours.

Ho hum.  Waiting is the worst!

Aidan's motility is not the best, even on erythromycin (his motility med), so the Barium really just wasn't moving through like we hoped it would.  It took forever.  But eventually, we did get a belly full of Barium.

We'll file this away under "things only a mother would love"

When we finally got back up to our room, Aidan took a nap and I got some work done while waiting for the doctor to come talk about the results and our plan for him.  Turns out it would be a preposterously long wait, since GI never actually came back and eventually left for the day.  Thanks, jerks.

Dainty

When GI came back the next day, we did learn that Aidan's Upper GI was not normal.  We already know that he has delayed gastric emptying - when food (or formula, whatever) is introduced into his stomach, it doesn't move through at the normal rate.  This is why it took so long for the Barium to move through.  The test also showed some not-too-specific inflammation of the colon - which could be from normal illness, or could be from an EGE flare.  Not really any more information than we previously had.

Ultimately, we decided to bring Aidan home, with orders to return immediately if the bilious vomiting began again.

It was more or less the same as ever.  Aidan in crisis, needs gut rest and IV fluids for several days, and a slow reintroduction of feeds.  I wish I knew why this keeps happening to him :-(

Side Note...

The whole time we were inpatient, Aidan was on contact precautions due to a suspected c-diff infection.  The hospital was unable to confirm or rule out the infection because - true to form - Aidan's digestive system shut down and no test sample could be obtained.  This particular monster would come back to bite us later on.

Final Thoughts...

I understand that CHOP is ranked #1 by US News & World Report, but clearly, they've never actually stayed here.  Or they don't have children.  Or they don't need fancy luxuries like a pillow to sleep on.  I do wonder how different our experience would be with a planned admission for a surgery.  Emergency Room visits that end in admissions are just always so nebulous and vague.  Who knows what we're doing or when we're going home?  My experience here would be at least 80% better if doctors could be bothered to clearly communicate. 

PurAmino - Why aren't you easier to use?

Puramino.  Oh, Puramino.

There's a special place in my heart for this product.  I'd like very much for it to work out.  When we tried it a year ago, we only had two cans, and so could only trial for three days.  But unlike the other formulas we've tried, so far, nothing terrifying has happened while on PurAmino.  And this time, we were able to get much more in the way of samples, thanks to the tireless efforts of our R.D.  I'm excited, of course, but nervous - I really don't have the time for a hospital visit right now.

Actually, what I am mostly is annoyed.  PurAmino is a 20 calorie infant formula, with deceptively simple mixing instructions:

To Make	Water	Powder	Weight
1 fl oz	1 fl oz	1 unpacked level scoop	4.5 g
2 fl oz	2 fl oz	2 unpacked level scoops	9 g
4 fl oz	4 fl oz	4 unpacked level scoops	18 g
8 fl oz	8 fl oz	8 unpacked level scoops	36 g

I'm guessing you see the pattern.  An ounce plus a scoop equals an ounce of finished product.  Easy peasy, right?  Right.  But let's say your kiddo needs a few more calories than your average bear.  You could just increase the volume that you feed - though in Aidan's case, we'd need to feed him 28.5 hours per day (you heard me) to get enough calories in with a 20 calorie formula.  Not humanly possible, next option please!

You'd probably check the can for 30 calorie mixing instructions.  After all, Neocate and Elecare has them.  No such luck.  You might also head to the product page on manufacturer's website, hoping for more detailed 30 calorie mixing instructions.  After all, 30 calorie dilution is standard for toddlers.

It took me a couple of hours of googling obscure combinations of phrases (and stumbling back across my own blog entry detailing my identical dilution quandary one year ago!) before I found this - a slightly helpful, more than slightly infuriating, piece of documentation.  WHO WROTE THIS THING?!

Here's my (hopefully not too terribly flawed) math:

We need 30 calories per ounce.  So we're only interested in the very bottom row.  We already know that 1 unpacked level scoop is 4.5g, and I use a gram scale, so that's how I prefer my measurements.

4.5g powder + .7oz water = .8oz formula
divided that all by .8 to determine that
5.625g of powder + .875oz water = 1 ounce of formula
at 30 calories per ounce, and knowing he needs 1350 calories, I multiply by 45 to get (roughly)
253g powder + 39.4oz water = 45oz formula


Redoing it in mL, since the manufacturer recommends this measurement for accuracy:

4.5g of powder + 19mL water = 23mL formula
divided by 23 to determine that
.196g powder + .826mL water = 1mL formula
at 1.01 calories per mL, and knowing that he needs 1350 calories, I multiply by 1337 to get (roughly)
262g powder + 1104mL water = 1337mL formula
262g powder + 37.3 oz water = 45oz formula


Um, say what?   Those are not the same.  After no small amount of crying and yelling at the can for not giving up more helpful information, I let the R.D. do her job.  I do not know exactly how she did this math.  I think she allowed for displacement.  And also, magic.  What I do know, is that I'm blogging this for future me to find.  In fact, I'll cross out my crappy flawed math.  Hey, future me.  Use this one!

266g powder + 38.5 oz water = 45oz formula


Once we decided to go ahead with PurAmino, we had an uphill battle with insurance.  It's an infant formula this, and we don't carry it that.  UGH!  I won't get into the dirty details because if I re-live it, I might actually have to hurt someone, but our wonderful, wonderful R.D. at CHOP worked her - ahhem - bottom off to get us enough samples to keep Aidan fed until our appeals were approved and our delivery finally arrived.  We repaid the Mead Johnson rep with these absolutely adorable pictures of one of their tiny consumers.

PurAmino - bottom line - I'll forgive your user-unfriendliness if you keep my baby out of the hospital.  Deal???

The 2014 Formula Trials - Neocate & PurAmino

After Aidan's last stint inpatient at CHOP, and my musings on feedings, I was left feeling like it wasn't Aidan who was failing formulas - it was us failing him.  We were feeding him a formula that was wreaking havoc on his little body.

Let's recap:

On Neocate Jr, Aidan did well, right up until January 2013, when we started using the "new label" Neocate Jr.  I recognize that the change of labels was not impactful for the vast majority of children, and that it's possible that Aidan's decline and the label change happening in the same timeframe may have been coincidental.  During February-March 2013, our lives were fairly miserable.  Aidan vomited almost everything he ingested.  Things came to a head in April 2013, when he was admitted to CHOP and a GJ tube was placed.  At the same time, we discontinued Neocate Jr and started Elecare Jr.  (In retrospect, I do not know why we did both of these things at once.  I truly don't.  But I was desperate to help him.)

Between April 2013 and October 2013, I scrounged up "old label" Neocate Jr from anywhere I could find it.  We didn't always have enough to have him exclusively on Neocate Jr, and sometimes had to mix Neocate Jr and Elecare Jr.  The last of the "safe" Neocate expired in October 2013, and we moved him to Elecare Jr. in October.

Exclusively on Elecare Jr fed via J tube, Aidan was frequently hospitalized for severe dysmotility, which led to severe constipation and dehydration.  These hospitalizations occurred in October 2013, November 2013, December 2013, and February 2013.

By February, I'd had enough.  I couldn't keep doing this.  I begged our nutritionist to help us.  She understood that Aidan's quality of life just had to improve.

Our first thought was to retry Neocate.  My hopes weren't super high, but I decided to go into it with an open mind.  There aren't many options, so I need to be open to trying everything.  We hadn't tried "new" Neocate in a year.  It was worth trying again.

Aidan disagreed.  What I like about Aidan is that when he fails something, he well and truly fails.  He "vomit on the floor in the nutritionist's office" fails.  I was cautiously willing to push through, but continued retching and vomiting of bile made it pretty clear that Neocate was going to be a no-go.

So back to PurAmino.  We tried PurAmino a year ago too.  The results last year were underwhelming, but we were far less desperate at the time.  All I really journaled last year was that it made him hyper and extra emotional.  We only tried it for about 3 1/2 days, because it was pretty difficult to get samples of. 

I'll post more thoughts on PurAmino soon - but after a much longer trial period, and an even longer fight with insurance, we've settled on Puramino as our formula of choice.  It does, actually, seem to make him hyper and emotional, but I think that's actually because he's in less pain.  He feels better (yay!) so he's more aware of what's going on in his world (yay!) and more easily overwhelmed and overstimulated (boo!).

He has not been hospitalized since we started with PurAmino.  We've had one very close call, about a six weeks into our trial.  The root of the problem seems to be his poor motility, and when it flares up (somewhere in the neighborhood of every 4-6 weeks), he stops pooping, is in excruciating pain, stops tolerating feeds, and gets dehydrated - all in the span of a day or so.  No real warning that this is coming - he's fine one moment and very not fine the next.  In the case of this close call, he definitely had a motility flare, but we were able to keep feeds going, so he stayed hydrated, which is the key difference between going to the hospital and staying home.

I think we're going to stick with PurAmino for now.  We're essentially out of options otherwise.  Wish us luck!

Neo-Gate 2013 - Are you sick of my updates yet?

I'm sorry that I have nothing more exciting than this to write about.  Maybe I should - but I don't. 

We stretched our two cans of PurAmino out to last 3 1/2 days.  The household is kind of split on how we feel about it.  Tom thinks it's the best formula we've tried.  Megan has no opinion.  I think it's no worse than Elecare, but really no better either.  I think it makes him hyper and extra emotional, which I guess the Elecare does as well.  All other things being equal, I take equality as a reason to choose Elecare, because it's easier to get my hands on if (WHEN) Medco decides to delay our shipment for no apparent reason - as they've done EVERY three months since we've started these formulas.

The plan right now is to get him back on Elecare Jr. (CHOP - I'm coming to raid your samples closet) until we can get in to see Dr. Liacouras (GI) on the 29th.  I need to spend some time between now and then gathering my thoughts and coming up with a game plan (seeing Liacouras without knowing what you want from him is a disaster).  I think the game plan will include having him scoped and biopsied as soon as humanly possible.

In addition to the (IMO) obvious - ruling out any anatomical issues causing this sudden feed intolerance - My rationale behind the scope is this:

Possibility 1 - He scopes clean - Great!  Fantastic!  We keep him on Elecare Jr. and decide that the behavior changes we've seen since things with Nutricia went awry are not related to his EGID.  We can then turn our focus back to weight gain and feeding and speech work.  We can also donate our small stash of "safe" Neocate Jr. to children who aren't able to survive on Elecare Jr.

Possibility 2 - He doesn't scope clean.  Elecare isn't working.  We decide how best to stretch out the "safe" Neocate that we have and what to do as we move forward.

I'm cautiously hopeful for Possibility #1 even while I think Possibility #2 is the more likely scenario.  But it's a plan, and it's as far ahead as I can go right now.


No news yet from Nutricia about the testing done on the product that we shipped back to them.  We are supposed to hear something this week.  I doubt they'll find anything, but God do I hope they do.  It's insane to think of the lives that could be changed (infinitely for the better!) if this could be straightened out.

In other news, and this is really a post all of its own, I've been able to team up with several other moms whose children are affected by this disaster.  They've put me in touch with people willing to get us some safe formula at low cost.  There's not that much formula out there at this point, and it warms my heart that someone ensuring that no one goes without while there's still something we can do about it!

So as soon as we get Aidan to see his doctor, we can start him back on his safe formula  - at least a little bit, at least sometimes, at least it would be a break from what's making him feel so badly.  Some boxes of formula arrived yesterday and I cried (happy, grateful tears!) the entire time I was putting that precious powdered gold away.  I'm so thankful that people like this are out there, willing to help give my little boy a chance. 

This is what I'm fighting for.  This is why I'm doing this.  This is why I can't just "leave it alone".  He's sweet, and he's beautiful, and he deserves to be happy and healthy.

Neo-Gate 2013 - PurAmino

Neocate Infant was a NO - but our wonderful RD pulled another one out of her hat for us, and asked us if we'd like to try another Amino Acid formula called PurAmino.  I'd never heard of it - it's made by Mead Johnson and isn't as common or popular as Neocate or Elecare, but obviously we're willing to try it.

As a product, it's frustrating, because it's a 20 calorie formula.  meaning it comes with an itsy-bitsy scoop and no 30 calorie mixing instructions.  And where Neocate gives a page-long grid of 30 cal instructions (and Elecare at least provides "small", "medium" and "large" batch instructions) - PurAmino gives instructions for a quart or .77 oz.  Thanks.  Helpful.

But I got out my handy dandy notebook and did some (hopefully correct) math and Aidan spent Saturday and Sunday on PurAmino.

My impressions:

• It mixes really lumpy.  I'm not sure why.  I mix it the same way that I mix the others.  But this one's clumpier.  It hasn't mattered though, and as long as it's not causing pump alarms, I suppose I don't really care.
• I don't know how it tastes - honestly, we haven't been pushing oral intake with Aidan.  For better or worse, our efforts are focused elsewhere.
• It doesn't smell half as badly as Elecare. 

The really important thing is how he tolerates it.  So far, no throwing up.  Which is great!  But we're only two days into the trial, with about enough powder for another day and a half - so it's going to be very hard to tell if it's better or worse than Elecare Jr. in terms of his behavior and how he feels overall.  I hesitate to shout WIN from the rooftops, but it's not an abysmal failure like the Neocate Infant was, either.  I'd love to say that time will tell, but we just don't have the luxury of enough sample product.