Updates - Complex Care, Rare Care, and Besties

It's been a better week.  I feel like I've accomplished more, anyway.  I've made strides toward getting Aidan where he needs to be.

The Diagnostic and Complex Care Team reviewed Aidan's chart and called to set up his consult with Dr. Magnusson.  I don't know whether this means it's a visit to decide whether they'll take Aidan on, or it's the first step in the process of having Dr. Magnusson on our side.  Either way, it's a step in the direction we so desperately need, and it isn't even that far off (October 23rd).  I've heard truly amazing things about this doctor - that he's amazing to have on your side, that he's smart and he'll fight for you, get you what you need, and put you in your place when need be.  All of that and more - and we need it all so badly.  I'm looking forward to the appointment, but I also know that I need to seriously organize my thoughts before then.  I need to be armed with a clear and concise history and how and where we need his help.

I've also been continuing to talk with Children's Hospital of Pittsburgh's Center for Rare Disease Therapy (Rare Care).  I am working with CHOP to gather Aidan's medical records, and once I have them and can get them to Rare Care, we'll be able to figure out exactly which doctors Aidan needs to see there.  I've faxed the requests over to CHOP's records department, so now I wait.  I have no idea how long it will take for CHOP to gather and mail his records to me.  I'm oddly curious to see them, actually.  But mostly, I'm anxious to get them to Rare Care so we can get the ball rolling.  They have appointments available in November, but think that if we can get his records over quickly, they may be able to squeeze him in sooner.  November would be great.  Sooner would be amazing.

All of this is so much more direction than we had even two weeks ago.

On the home front, sleep has been hard to come by this week. I think Aidan's been sleeping more restlessly than usual - we've had two accidental unhookings overnight, and when we check on him overnight, he seems especially entangled lately.  A few nights ago, we were all awoken at 4:30am to a low blood sugar, high ketone boy.  He was cold and clammy and had been vomiting repeatedly in bed - all because his extension popped out and we fed the bed.  Totally commonplace in the feeding tube world, but very dangerous for a hypoglycemic kiddo.

This has to be a strangulation hazard - right?

On a fun note - Aidan got to spend time with his bestie over the weekend.  It will never stop warming my heart to see him just get to be a little boy.  These boys have so much to teach each other.  I love that T pushes Aidan to try bigger, braver, better things. I love that Aidan shows T that it's okay for everyone to do things in their own time, at their own speed.  I love that they can both learn how to meet a friend where he is, even if that's not where you are.  T is a sweet boy with a sensitive heart who has loved Aidan like a brother since birth.  We are so lucky to know him.

Best Friends go with it when you rename their clubhouse "The Charmed House"

He also (creative boy!) had a great time making a robot that could do all of his smiling for him.  Maybe I've been irritating him too much, asking him to smile for pictures?  He was so proud of his robot's big smile, and said that now HE doesn't have to smile.  Great problem-solving, little Vulcan.  So proud :)

All in all, it's been a better week.  Over the next week, I'm hoping for more communication with Rare Care, a special Mommy/Aidan date on Labor Day, and my first time at a special needs parent support group - something that's been FAR TOO LONG in coming.

New Ideas and Next Steps

I feel like things have been heading slowly but surely downhill.  Not for Aidan, really.  Just for me. 

As a mother, I feel like I owe him so much more than a childhood full of fear and pain and “my body isn’t working anymore.”  We are spending so much time managing today that I’m almost afraid to even think about the future – but if I don’t, who will?  And it’s a scary thought.  If at age four, we’re already managing his anxieties pharmaceutically, what will it look like at fourteen?  If at age four, his body is too tired to spend a day on his feet at the zoo or the amusement park, what will it look like at forty?  Why is it that he has the appropriate skills to run, jump, and play – but not the energy required to actually do it?

I look into his future and see nothing but the best and brightest for him. He’s amazing.  He’s brilliant and quirky and funny.  He’s sensitive and sweet, and a charming mix of silly and puzzlingly serious.  He will go to college and learn everything he ever wanted to know.  He will get a job and do fantastic things for this world.  He will meet someone who wants to spend their life figuring him out, because with him, the destination will always be worth the ride.  I know all of these things and I never, ever doubt them.

But I also know that it’s my job to get him there.  It’s my job to keep him safe.  To make sure he has a childhood as free of hurt and fear as I possibly can.  To make sure that we’ve done absolutely everything to keep that silly “not working today” body in check.  Come on, little body!  Aidan needs you!  

Amazing kid... Tired body

Over the past few months, we’ve seen new specialists who have given us new (half) answers – a genetic mutation that may or may not be causing some (but not all) of his problems.  “It is likely there is another mutation not identified.” (Read: We know, this doesn’t explain everything.  We wish we could help more.  We’re sorry.)  New meds to treat anxiety and attention deficits.  And they’re helpful, because on any given day, we are doing more or less okay - but the overall picture isn’t really improving.  We have a child who can’t eat anything.  Can’t sleep.  Requires hospitalizations far too often.  Can’t stay hydrated despite our best efforts.  Can’t regulate his blood sugar.  Can’t count on his own body to keep him going all day long.  Isn’t growing enough, despite the best nutrition we can give him.  It’s too much.  Too much all happening at once in a “healthy, normal child.”

So it’s strict food avoidance.  All feeds via J tube.  Melatonin for sleep.  More melatonin for wakefulness.  Water flushes.  More water flushes.  More water flushes.  Lots of wardrobe changes when he sweats out all of the water we’ve flushed in.  Blood sugar checks every 3 hours.  Inpatient fasting before procedures.  A special needs stroller when his legs just won’t carry him any further.  And therapy – so much therapy.  OT. PT. Speech.  Feeding.  Behavior.

We’re told that this is what well-managed looks like.  When the next problem arises, we’ll find something to address that too.  That’s how you manage a child with “lots going on.”  But, really, though?

So where do we go from here?  What do we do? 

Today, I reached out to CHOP’s Complex Care team, in hopes that they can help us.  I don’t know if they’ll take him on as a patient – I hope they do.  We need more help.  We need more answers.  We need more avenues to explore.  What is happening to this little body?  How do we make it better?  How do we make our lives better?  If he’ll never eat normally, do we keep pushing foods?  For everything we do, every decision and intervention we take, I want to know – will this make his life better?

I also reached out to another hospital (Children’s Hospital of Pittsburgh).  I have been thinking about this for a long time, but actually doing it felt like staring up at Everest while lacing up pink bunny slippers.  Is this really doable?  Am I crazy?  Maybe.  But I’m not going to stop until I’ve gotten him the best care I can.  I hadn’t considered them before because they don’t have a program for Eosinophilic Disorders, but with this new genetic finding, I am hopeful that they may be able to help us.  Glycogen Storage Diseases are among the conditions they treat in their Center for Rare Disease Therapy.

Very shortly after emailing the department, I connected with the coordinator at the Rare Disease Center today, and talked through some of Aidan’s history.  I heard five of the most encouraging words in the English language: I think we can help.  So now, I work through gathering Aidan’s medical records and hopefully planning a trip to Pittsburgh in the near(ish?) future.  They will help coordinate a place for us to stay while we’re there – there is a RMH attached to the hospital, and some hotels nearby in case the RMH is full.  The hope is that we can coordinate our visit so that we see everyone we need to see while we’re there.  Whether this would be “gathering ideas to bring back to our CHOP team” or “starting with a new team altogether” – I don’t honestly know.  But the prospect of new eyes on Aidan is encouraging.

I don’t know where this is all heading.  I do promise, though, to keep you all better updated as we figure things out and move forward.

This is the face of insatiable curiosity.  I think he gets it from Dad <3

Checking In...

Sometimes, so much time passes since I last wrote, I feel like I should have something monumental to say.  But I don't, so I don't say anything at all, and then you don't hear anything from us until something awful happens.

Nothing awful has happened.

We're still here, taking things one day at a time and trying to keep it all in perspective when I think it's all too much.

We finally completed a sleep study in January (dreadful) and determined that, although he wakes up at least ten thousand times every night, it's not a respiratory problem.  Obviously great news from a pulmonary perspective, though not the most helpful thing to hear from a "let's get this kid snoozin'" point of view.  But we've started using Melatonin with some success, so we're at least moving in the right direction.

Hi, I'm checking in for my Awake Study?

Sleep is for losers...

Aidan spent some time inpatient in February with a GI virus that he picked up (probably at school) and I went to a very dark place.  Parents of healthy children aren't living this life, and I hope they never have to.  (Just like *we* aren't living the lives of NICU and PICU families, or Cancer families, and hope beyond hope we never know their "normal.")  But the GI virus that sends a classmate home early one afternoon for some extra rest and fluids sends Aidan to CHOP by ambulance (inpatient for 4 days, home for 4, inpatient again for 5), leaves him on anti-emetic medication for weeks to combat constant vomiting because the virus disrupted his motility, and steals away all of the weight he'd gained since October.  And I'm so angry because it's so unfair.  But that's another topic for another day.

I'm sick, but ambulances are awesome!

A boy and his bucket

His wife will thank me for this later...

FINALLY time to go home!

We checked in again with Endocrinology when the madness that was our inpatient stay(s) wrapped up, and were pretty soundly brushed off.  Aidan has good weight gain and poor linear growth, but our endocrinologist is generally unconcerned and feels there's really nothing to be done.  At this point, her only concern for him is his Ketotic Hypoglycemia, so she's referring him over to her colleagues on the Hypoglycemia team.

March brought new focus to Aidan's Developmental concerns.  I think sometimes, it takes a really forceful nudge - just the right person saying just the right thing - to get ready to change your approach.  In our case, it was Aidan's Psychologist and his Behaviorist.  Tom and I both really respect them, and when we met for our tri-annual review, we all talked through some new things that we hadn't really discussed before.  I think this is going to lead to some different approaches to Aidan's behavioral interventions.  Stay tuned on that.

The next few months should be interesting, as always - in April, we will be seeing orthopedics to check in on Aidan's legs and feet, Developmental Pediatrics to review our new plans and services for Aidan, and he'll be heading inpatient on the 20th for his GJ change on the 21st.  In May, we head to Metabolism for the first time, and we meet with an Endocrinologist from the Hypoglycemia team.  In June, back to Developmental Peds.  And somewhere in here will be a first visit to CHOP's Genetics department, since we've only ever seen genetics once at another hospital, and everyone agrees this is something that needs to be revisited.  His case is currently under review, and we should hear back within the next few days regarding which doctor will see him and when.

In the meantime, we're doing all of the usual stuff.  Aidan's going to a new school and is doing well there, especially now that we've added full time private duty nursing (Bayada has been a huge positive addition to Aidan's team).  He's still getting lots of therapy and making amazing strides with his speech and motor delays.  He started Karate in January, and holy smack, does he ever look adorable!

More to come, soon I hope, but for now, know that we do appreciate every single time you reach out to check in.  We're still alive, and sometimes I need a little (big) nudge to dig out from under 10,000 pounds of life to return your call (text/email/attempt at actual verbal communication).

Inpatient - Endocrinology - Day 4

This post will be blessedly short.  On day 3, we felt like we had his Endocrinology stuff figured out as best we could, but true to form, Aidan's motility tanked, because why wouldn't it?  We spent one last night tucked into an impossibly tiny hospital crib up on 5 West.  Did we sleep?  Nah.  But we were together, so who needs sleep?!

Love this guy

Saturday morning found Aidan feeling much better.  The docs had given him Senna, which is what GI recommended the last time we were in.  It worked great.  After two doses, things were moving again, and we felt good enough to get a move on.

See this forlorn face?  He was sad to leave because he likes it there.

All in all, it was such a productive trip.  We learned how to better manage Aidan at home (no more breaks from feeds, no more fasting at home before procedures!).  We learned what to look out for and how to better recognize when he's in trouble with respect to his blood sugar.  Every hospital stay finds him less anxious and fearful, and more trusting of doctors and what they're doing to help him.  And, sadly, we learned not to just trust that CHOP will have the nutrition he needs.  I wish that weren't the case, but the fact is, we don't need the added stress every time we go there.  We need to start bringing our home supply.

Thank you - all of you - for all of your well-wishes and thoughts while we were at CHOP this time.  While he's not old enough to understand, I certainly am.  It means the world to me.  We are happy to be home, and even happier that we made it home in time for this year's Strides for Safe Kids Food Allergy Walk and Expo!

Inpatient - Endocrinology - Day 3 (Come for the Endo, Stay for the Gastro!)

Last night was a long one.  Since we didn't begin the IV fluids until 10pm, we couldn't start weaning the formula until then either.  So, starting at 10pm, we began weaning his formula by 14ml and checking his BG and ketones every 30 minutes.  I don't know how our poor nurse (Annie) got anything else done all night, honestly.  The feed wean went from 10pm to midnight, and his BG went from 113 to 86, ketones holding steady at 0.2.  All good.  In the next half hour, he dropped to a BG of 74 (while still on IV dextrose) and his ketones climbed a bit, reaching 0.5 (still fine).  I vaguely remember a BG of 70 at 1:30am, while we were weaning down the dextrose, and then I drifted off to sleep sometime shortly thereafter.  Kind of.  I was in and out every time Annie, bless her heart, continued to check his BG and ketones all night long.  She later told me that his ketones were slowly climbing during that time.

Annie's party ended uneventfully at shift change (7am) and Aidan was holding steady around 70.  Our morning nurse got him at critical on her very first check - 46.  When she rechecked him, he was 51, and when she sent off bloodwork for confirmation, it came back at 51.  Just a hair over "critical."  So the fast continued.  His checks at 8:30 were in the low 50's.  Then, at 9am, a new wrinkle.  BG 51, but ketones were suddenly at 3.3.  WHAT?  Where did that come from?  The 9:30 check showed BG 41, ketones 3.8 - so the fast officially ended, both for low BG and high ketones.  Things happened pretty quickly after that - the lab confirmed his BG at 41, and the first round of bloodwork went in.  He got a shot of Glucagon (a hormone that's supposed to bring up his BG quickly) at 9:45, which immediately made him nauseous, and didn't even help.  He fell down to 33 by 10:00, and they decided to halt the test and put him back on IV dextrose. 15 minutes later, on IV dextrose, he was up to 50, which is still too low, but heading in the right direction.  He spent the rest of the morning slowly working his way back up to 70 on IV dextrose.

Not a happy camper

Dr. Langdon decided to go ahead with some growth hormone labs, so those were drawn an hour after the Glucagon and every 30 minutes after that until lunchtime, when the final labs were drawn and feeds were restarted.  The results that could come back immediately came back around 4pm - it doesn't look like he has a growth hormone deficiency - or at least not one severe enough to be the cause of his hypoglycemia.  So, this means a couple of things.

1. This tells us that Aidan's form of hypoglycemia is called ketotic hypoglycemia.  This basically just means that his body doesn't tolerate fasting, and while his blood sugar falls, he produces ketones, which are no bueno. 

2. We still don't have any answers about his poor growth.  I guess we'll have to take that up with his Endocrinologist when we follow up with her in a few weeks for the rest of the bloodwork results.

The plan is to remain on 24/7 feeds, since fasting proves dangerous to him, but it's thought that with a careful wean, Aidan could probably tolerate up to 4 hours off feeds safely.  He absolutely cannot just halt feeds (you know, exactly the thing we were doing).  We've been instructed that if he's sick and not tolerating feeds for more than 2 hours, we are to take him to an Emergency Room for IV dextrose.  We have a letter explaining his condition as "severe" and giving treatment protocols for him.  It uses intentionally terrifying phrases like "seizures" and "brain damage" when explaining the consequences of untreated hypoglycemia.  Honestly, it's kind of sobering and gives a new air of fragility to an already nerve-wracking kid.

The way he acted with critically low BG and high ketones makes me concerned that his sugars are low and ketones are high from time to time at home while he's on feeds, and we're not aware, because how on earth would we even know?  His face was flushed but clammy, he was acting really tired, and he was insistent that he's "really thirsty" - which is something that he very rarely says. Except for the times that he acts exactly like that, late at night.  Or maybe he's just 3 and manipulative.  Ugh.  I hate this.


We'll be checking his BG a few times a day, and probably also overnight, with additional checks anytime he's symptomatic. I know that we are okay, and we can do this.  There's just suddenly so much more to think about.  How will we prep him for scopes?  What about tube changes?  He needs to go NPO so he can be sedated.  But now we can't safely go NPO.  What happens when his pump breaks (a-g-a-i-n) and Homecare says they'll be there in "a few hours"?  Do we wait for him to crash?  Proactively bring him in for dextrose, knowing that he'll definitely crash? 

The biggest positive is that we're told most kids grow out of this.  We get to repeat this little slumber party next year, to see if he can pass the 36 hour fast.  That seems so absurdly impossible right now.  But eventually, most kids do.

So Endocrinology is ready to release us, and we'll be going home tomorrow morning sometime.  We're spending one more night for a GI concern - his motility seems to have slowed way down and we're worried about heading home without being sure he's going to continue tolerating feeds, especially now that not tolerating feeds is not an option.

It's a good thing he's cute...

Inpatient - Endocrinology - Day 2

The doctors rounded our way around 9:30, and came up with the day's plan.  It's always interesting to hear someone else give your kid's story - what's important and relevant, and what's not.  This part, at least, is familiar enough.  Grumpy old doctors, residents trying to impress them, nurses, and whoever else joins the cluster all gather around the door and discuss your child.

The plan was to start an IV at some point today so that later tonight, we could wean down his feeds and start the fast.  Silly me, I thought we'd do the fast today and not tonight.  But it sounds like actually we'll be doing more of the same today as we did yesterday - continuing on normal feeds while checking his blood sugar every three hours.  His blood sugar was stable overnight, though it fluctuated between 79 and 110.  I still wonder why it goes down to the 70s when he's on feeds.

The Endocrine doctor, Dr. Langdon, came by around 11:30 to discuss Aidan and the plan.  He has a low IGF1 level (40) - "impressively low for a three year old" - but they don't know if it's low because he's got a growth hormone deficiency, or because he's malnourished (well isn't that just a knife in the gut?).  He is short for his parental curve, but he does seem to have good growth velocity.  He was born near the 50th percentile, fell to the 10th percentile by 2 months old, hovered between the 3-5th percentile until he was 1 1/2, and has been in the .5-1st percentile for the past 2 years.  So yes, he's fallen, but he's remained on his curve for the past two years.  There are some clinical signs of growth hormone deficiency that he doesn't display, but others that he does - so there are mixed indications.  We really don't know what to think at the moment.

I have trouble believing he's malnourished, honestly.  The formula is supposed to be nutritionally complete. He's finally putting on weight.  Can he really be malnourished?  Now I just have this image in my head of this guy, Dr. Langdon, shaking his head and saying "what a terrible disease" (the Eosinophilic Gastroenteritis).  I immediately felt defensive, because we feel like we have it so well controlled.  I just hope it hasn't been at the expense of his growth and development :-/

Sarah from Nutrition came in to talk, and helped me feel better about the malnutrition thing.  I don't know if he's been getting what he needs or not - I suspect he has been, because he's been gaining weight - but if he hasn't, it's probably because of a malabsorption-type issue.  Not because we're not giving him what he needs.  We've worked so hard to find a formula that works, and when we found it and fought for it and use it and still hear that may be malnourished - it's hard not to feel defeated.  I hope the growth hormone stim test gives us more answers.

You know what I forgot to mention?  The whole hospital transitioned to Infinity pumps!  I love this!  I know exactly what the alarms means and how to shut them up!  Yes, I know we aren't supposed to touch the pumps, but talk to me again at 3am when it's been alarming for 15 minutes and nobody's answered the nurse call button.  Pausing the alarm is a perfectly reasonable course of action.

The other reason this is awesome is that it gives us the opportunity to finish out the feed we brought from home while CHOP inevitably makes a debacle out of finding his formula.

Infinity Pump!

By awesome coincidence, CHOP is having a radiothon this week.  There are activities all day today and tomorrow.  We ventured out of the room at lunchtime to meet Elsa, who is evidently some uber-popular chick from Frozen.  Aidan was unimpressed, to say the least.  He did his best Joe Friday impression (am I dating myself?):

Who are you?  What is this dress?  Is that your hair?  What are you doing here?  Are you sick?  Where's your tubie?  Are you coming to see Dr. Liacouras?  Do you have allergies?

He then decided he was done with her highness, leaned in close, and said "I would like to go find a canyon today."  And that was it.  Little girls were dying to meet her, and he was so bored he couldn't even stand it a moment longer.  Not long after that, someone started banging cymbals together - in the huge echo-y atrium - and Aidan was done-zo.  He gave Spider-Man a once-over, nonchalantly informed him that "food is not safe for my mouth, I have allergies.", and hightailed it back up to his room.  I'm not sure I'll be able to coax him out of bed again.

There is no such thing as too many of these pictures.

So that was today.  Aidan's blood sugars were monitored all day while he was on feeds, to continue establishing his baseline.  Around 7:00pm, the IV team came in and attempted to place two IV's - one to administer fluids, and the other for blood draws.  He needs two, because they don't want the blood they draw to be potentially contaminated by anything they push in through his IV - so they can't draw blood from the same site that they're using to administer something like Glucagon or Dextrose.

Hotpacks - we hate them!

I said attempted because, as we all know, Aidan is the worst stick in the history of bad sticks.  They blew three veins before finally getting their fourth (and final) one placed.  That one will be used for blood draws, but we still needed something for IV fluids.  So they gave him an hour off, then hotpacked his left arm for an hour, and tried again.  This was when things got sad - Aidan realized he had no hands left to play with toys, iPads, or crayons.  It was a long hour.

Sad boy

When IV team came back, they meant business and they got that IV placed like it was their job.  Which, you know, it is.  But still, it was pretty great.  It really isn't their fault that his veins are so awful.  So now he's pretty incapacitated and looks fairly pathetic.

He's not sleeping, he's just hamming it up, and refusing to look at me is sadder than anything.

We'll start up his fluids and hopefully get this fast process started soon.  It's supposed to include weaning his feeds down over 2 hours, and then weaning his fluids down.  During that time, we'll be checking his blood sugars hourly (potentially half-hourly) and waiting to see how long he lasts before going low (70) and then critical (50).  When he goes low and/or critical, there is bloodwork that needs to be drawn immediately (hence the second IV).

My worry with this approach is whether we'll be able to recreate what we saw a few weeks ago.  We don't normally wean him down over 2 hours, while keeping him on IV dextrose, which we then wean down.  Which they know, so I'm sure they have their reasons.  I just hope we get some information that helps us in our daily life, you know?

One more picture for the night...  It's been a really rough night with way too many needles, and it's all been really unfair to him, but you know what?  Nothing gets him down for long.

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!

A foray into blood glucometers

After a whirlwind visit to Endocrinology, we found ourselves in possession of a slew of diabetic testing supplies, with instructions to test Aidan's sugars for the next few days as he comes off feeds to monitor for uncontrolled low blood sugar episodes.  I'm writing this post for me - because today, on day 1, I remember everything I've been told.  I may need to read this later.

Supplies - A grocery bag full of vampiric goodies.

We were given a Contour Next EZ Meter.  And no training whatsoever.  My wonderful colleague, Bob, took the time to show me what to do, and I felt at least seven hundred times better once he did. There is also a really helpful instructional video on Bayer's website.  A note - the meters should be operated in temperatures between 41 and 113 degrees (you'll get error code E1 if you're out of temperature range).

I also received a bottle of control solution - which contains a premeasured amount of glucose.  It's useful for practicing with the meter, on the off chance you don't want to stick yourself a few dozen times.  It's also used for testing the meter to ensure that the meter, test strips, and your technique are all working correctly.  It comes with instructions, but basically, you put a drop of control solution on a nonabsorbent surface (they recommend wax paper), and touch and hold the tip of a test strip to the control solution until the meter beeps.  You compare your result to the normal control range on the test strip bottle (and different bottles may vary - mine do).  If the results are questionable, the meter user guide should offer some suggestions regarding next steps.

I am nearly positive this is the wrong stuff.

Alcohol Prep pads - for cleaning skin before testing.  Self-explanatory.

Contour Next Test Strips - Obviously, for use with the meter.  They test blood glucose levels between 20 and 600 mg/dL - numbers I expect NEVER to see.  This should go without saying, but they're single use only.  Never reuse a test strip.  It's also important to keep the bottle closed tightly to protect the strips from humidity and contamination.  A nice feature that I think most test strips have now is the "sipping" technology - you just have to put the test strip up next to the blood and it draws it up into the meter - you don't actually have to soak the whole strip in blood.  Older meters/strips didn't work this way, and needed more blood to test.

Microlet 2 Lancing device & lancets - This is what you prick the skin with to obtain the blood for sampling.  The Microlet is easy to use - you just pull off the cap, insert a lancet, twist off the top of the lancet, replace the cap of the Microlet, and choose your endcap setting (tiny droplet for shallow puncture, big droplet for deep puncture) based on the patient and test area (fingertip vs. forearm, for example).  You press the endcap firmly against the test area and press the blue button, and it's done.  Like the test strips, you should never reuse a lancet.  Once used, they're no longer sterile and should be replaced.  To remove a lancet, you open the endcap, press the needle into the lancet cap you removed earlier, eject using the blue button and blue reset slider, and dispose as medical waste.

Not scary.

Even less scary.  Itsy bitsy needle.

Basic Use - We do not use any advanced features of this meter.  Aidan is not diabetic - he is experiencing hypoglycemic episodes that we need to monitor more closely.  The advanced features (reminders, meal markers, etc) look really helpful for diabetic patients, but that's not us.

- Load a test strip into the meter, gray square end facing up.  This turns the meter on.  You'll see a blinking picture of a test strip with a blood drop - this means it's ready to test.  If you've loaded it incorrectly, you'll get error code E4.  If you use the wrong test strip, you'll get error code E7.
- Draw a blood sample using Microlet and touch the tip of the test strip to the blood drop.  Hold it in place until the meter beeps.
- If more blood is needed, the meter may beep twice and show a picture of an underfilled strip.  You'll have 20 seconds to add more blood to the same strip - if you don't, you'll get an E2 error code and you'll have to start over.
- Once the meter has what it needs, it will evaluate the sample for 5 seconds and display your reading and store it to memory.
- Record your reading and remove the strip to turn off your meter.  And you're done!


My own testing efforts -

- Attempt 1 with control solution resulted in error code E3 - either the meter is sensing a used test strip, or the wrong control solution was used.  Ummm...  Everything is brand new, and this is definitely the control solution they gave us.  Wha??
- Attempt 2 - E3.
- Attempt 3 - After gently rolling the bottle around to make sure the control solution is all mixed up - still E3.  ARGH.
- Attempt 4 - After being ultra careful that no part of my fingers touched any part of the test strip sipping area - E3.
- Attempt 5 - The control solution is clearly broken.  I tried with my own blood.  I had chicken for dinner about 4 hours ago, and my blood sugar is currently 114.  Humm.  We'll call that "high normal" and assume my technique is okay.  And also, the control solution is broken.

Important points - these are what's important for us.  There are a million and one things that are also important for families managing diabetes.  Thankfully, we're not walking that road.

Normal non-diabetic values are between 70-110 when fasting.If Aidan's fasting blood sugar measures below 70, we are to report that to his doctor within the week so we can change his feeding strategy.  If Aidan's blood sugar measures below 50, we are to seek immediate medical attention.

Some symptoms of low blood sugar (hypoglycemia) are shakiness, sweating, fast heartbeat, blurry vision, confusion, lethargy, irritability, dizziness, and seizure.  Some symptoms of high blood sugar (hyperglycemia) are frequent urination, excessive thirst, blurry vision, fatigue, and hunger.  Some symptoms of ketoacidosis - a serious and dangerous medical emergency - are shortness of breath, nausea/vomiting, and very dry mouth.  All of these symptoms are a good prompt to check blood sugar for a patient with a known issue - and someone with a reading of less than 50 or more than 250 should call their doctor right away.  Again - not anything we ever expect to see with Aidan, but good to know and I figured there should be something informative in here somewhere.

After much reading and self-testing and worrying, I mustered up the courage to test Aidan for the first time.  I let him pick which finger he'd like to test, and he was awesome about it.  Didn't cry at all.  He just asked - are you done yet? The hardest part was getting him to hold still while I touched the test strip to the blood drop.  His reading was 74, while still on a feed, and it officially occurs to me that I never asked what a normal nonfasting blood sugar should be for him.Whoops.

More to come as we test and learn more.