Lots of appointments to come!

Soooo, it's been a few days a while a month forever.  We've been okay, hanging in and trying to take things one day at a time.  Things are just so overwhelming right now, and writing about them is sometimes just a little more than I can handle.  But really, we are doing okay, we just have so much on our plates.

We've done some fun things in the past month - the Strides for Safe Kids walk was a good time and Aidan did surprisingly well in the crowd.  After that event, Aidan got to go to a Shopkins trading event - which was *awesome* - he did such a great job talking to the other children and asking if they wanted to trade.  No tears - I was so proud!  We also attended the Autism Awareness event at the zoo a few weekends ago, which has grown into a really nice event with great attendance and helpful networking opportunities.  I love that it's at our local zoo, which Aidan knows so well that it doesn't overwhelm him.

He's also been working hard on feeding skills, and has started feeding therapy.  It's only twice a week, and due to scheduling stupidity, it works out to 3 times every two weeks, which is only slightly better than nothing.  Luckily, he has a great team of people in his life, who continue to work with him and encourage him every day at school, and recently I happened to walk in on him actually nibbling at a rice flour pancake.  Proud mama over here!  He doesn't have much of a bite with his molars, especially on his left side, and getting anything bigger than a crumb in his mouth makes him gag - but he's getting there!  Unfortunately, his swallow is questionable and needs to be more formally evaluated for airway safety later this month, so until then, we're not working on liquids.

October brought us the tiniest bit of drama - an urgent care visit, and eventually an ER visit for a yucky stoma.  I really hate stoma problems, and we're always so lucky that his stoma is so beautiful and infection-free.  But really kind of out of nowhere, it sprouted granulation tissue that got infected and painful, and things were just no fun for a while there.  Right, as you would imagine, when I had no time to spend in the Urgent Care/ER all night long, because of course.  But I'm thanking my lucky stars that's all it was, because as I sit here writing this, I'm watching some of the babies I love from afar go through ten kinds of hell inpatient, and I know how much worse everything could be.  Aidan's been doing pretty well lately, and I don't take that for granted even for a minute.

We've had to stop Karate for now.  Aidan's little body is just too tired.  He just can't do it.  And I can't keep forcing him to do something that's just too much. 

Heis participating in soccer, which is super adorable.  Although I might be using the word "participating" loosely, since he really likes to just sit on his ball and watch once he's tired, and without 1:1 coaching, he forgets what he's doing and wanders off.  Don't care.  Adorable.  After soccer wraps up, he'll be taking a break from sports until we get his energy levels figured out, because I really think there's something awry here.  I'm hopeful that either CHOP or CHP has some ideas for us.

He's the dainty one, seated while everyone else plays.

The rest of the month is coming at us fast and furious - this week we'll see Genetics (this one's kind of a big deal, since we're looking for big answers here, or at least big ideas and a path forward) and the dentist (not a scary one for us, but a rough one for Aidan).  Next week, the Complex Care/Diagnostic Center which is another big deal for us.  I don't even really know what to expect from that visit, I just know that we need help, and we're hoping they can give it.  The following week, Aidan's swallow study, and then he's inpatient for a tube change.  Hopeful that he stays healthy and is discharged and feeling well enough for Halloween fun.

We have forwarded his records along to Pittsburgh and are awaiting word onwhen we'll be traveling.  I'm nervous - not knowing exactly when we'll go, and how I'll handle it with work, and whether it will even give us any answers - all if it makes me sick to my stomach, if I'm being honest.  But these are all the right things to do, and the right steps forward, and I just have to trust that Aidan will find answers down one of these avenues.

We'll keep you posted as appointments happen and travel plans come together.  Wish us luck!

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

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.

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.

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. 

Patch Results 2013

Last week, we Patch Tested Aidan again, in an attempt to find some foods that are safe for trial.  It's been a year since we tried pork, and although that trial was pretty awful, we know we have to get back on the horse eventually.

Night 2 - I'm a little nervous that the tape won't last another day.  I'm sending a roll of tape to daycare with instructions to tape him like crazy if need be.

Night 3 - It's Removal Time!  Reinforcements not required - the tape held up!

The two days spent with patches on were pretty uneventful - not much to say, other than that we were anxious about the results.  Finally, Thursday arrived, and it was time to remove the patches.  It proved to be more difficult than last year due to the aforementioned hairy toddler back.  Sorry, Little Bear.

One thing was immediately clear when we got the patches off: He was itchy!

Sorry for the blurry picture.  His teeny little toddler fingers are just too cute for words.

  

I'll do you the distinct favor of cutting to the chase.  After we cleaned off the crusted-on food, it was pretty easy to see the reactions vs. non-reactions.  I wish I'd gotten out my camera instead of relying on my phone, but it was a hectic night.

The right side grid shows some possibilities!  I see some blank spaces!

The allergist confirmed positive reactions to Milk, Egg, Soy, Broccoli, Wheat, Beef, Chicken, Turkey, Ham, Sweet, Potato, White Potato, Corn, Oat, Rice, Peach, and Carrot.  There was a mild reaction to Pear and no reaction to Apple, Banana, or Strawberry.

A1 - Milk, Egg, Soy, Broccoli, Wheat.  A2 - Beef, Chicken, Turkey, Ham, Sweet Potato.  B1 - Potato, Corn, Oat, Rice, Apple.  B2 - Banana, Peach, Pear, Strawberry, Carrot

The plan at that point was to run blood IgE levels on Banana, Strawberry, Apple, and Pear (since the reaction was mild).  Assuming those showed no IgE allergy, we would be cleared to trial any of those.  My thought was to start with Banana, then Strawberry, then Apple.  I'd probably save Pear for last, just because there WAS a reaction, even though it was mild.


But then it got a little tricky, because by Sunday night, things looked worse.  Some reactions calmed down - but others flared up.  Pear looked more like a legit reaction, and Apple looked less "safe".

A1 - Milk, Egg, Soy, Broccoli, Wheat.  A2 - Beef, Chicken, Turkey, Ham, Sweet Potato.  B1 - Potato, Corn, Oat, Rice, Apple.  B2 - Banana, Peach, Pear, Strawberry, Carrot

A1 - Milk, Egg, Soy, Broccoli, Wheat.  A2 - Beef, Chicken, Turkey, Ham, Sweet Potato.  B1 - Potato, Corn, Oat, Rice, Apple.  B2 - Banana, Peach, Pear, Strawberry, Carrot

So this leaves me to wonder - trick of the light?  Trick of my eyes?  Too late for it to even matter?  The Pear doesn't bother me, since I'd pretty much ruled out trialing it anytime soon - but Apple is another story.  It was patch positive last year, and I'd really like to know whether or not we're ok to trial it this year.  I tried taking a few different pictures under different lighting - at this point I don't even know what I'm looking at anymore.  My eyes are tired, my brain is tired, and I hate food :(

Update: We spoke to Allergy and confirmed that even 3 days later, the results do matter.  We won't be trialing Pears or Apples.  But we are still left with Bananas and Strawberries.

We will wait to hear that the bloodwork is ok (we don't expect any problems there, but we would rather be cautious than not) and then move forward with a Banana Trial.  Wish us luck! 

2013 Patch Testing

Just a little over a year ago, we patch tested for the first time.  I don't think we really had much purpose at the time - CHOP suggested it, so we did it.  This time around, we're really specifically looking for something to trial.  Anything that is negative here will be followed up with an IgE blood test - and if that's negative too - we'll proceed to oral challenge, and then trial.

Back then, we tested for milk, eggs, wheat, rice, corn, oat, barley, potato, beef, chicken, ham, turkey, green beans, carrots, peaches, and apple.  The results weren't awesome.  He reacted positively to milk, egg, wheat, corn, barley, potato, beef, green bean, carrot, peach, apple, rice, and oat.  That left just chicken, ham, and turkey for trial.  We chose ham, and trialed it as pork.  We never talked much about it, but it didn't work out.  It really didn't work out.  He pooped blood for days.  It was so awful. 

This year, we tested for milk, eggs, soy, broccoli, wheat, potato, corn, oat, rice, apple, beef, chicken, turkey, ham, sweet potato, banana, peach, pear, strawberry, and carrot.  So we're taking away barley (I don't know why) and green beans (too close to his IgE allergy to peas).  And we're adding soy (I have no idea why - we would not trial this since he's IgE allergic, I believe it was added in error), broccoli, sweet potato, banana, pear, and strawberry.  Broccoli because it's uber healthy, and sweet potato, banana, pear, and strawberry because they are sweet and he'd probably like them.

A1 - Milk, Egg, Soy, Broccoli, Wheat.  A2 - Beef, Chicken, Turkey, Ham, Sweet Potato.  B1 - Potato, Corn, Oat, Rice, Apple.  B2 - Banana, Peach, Pear, Strawberry, Carrot

After the ultra-fun flare behavior we got last week from his skin prick testing, I can only imagine what's in store for us this week.  Here's to hoping we make it through the next 48 hours!

Night 1.  Love how the samples are oozing out.  This will smell great by Thursday night.

Brand New Allergist & Skin Testing Fun

We've been without an Allergist for some time now, since Allergy & Asthma stopped taking our insurance and CPED is just so far and so inconvenient and sloooooooooow.

When we were at APFED this summer, we spoke to several families that use one particular allergist right in King of Prussia.  Looking back at old notes from our fabulous Nutritionist, it seems she has recommended her before, we just never pulled the trigger on making the switch.

As of this week - consider the trigger pulled.  Aidan has officially screamed it up in Dr. Beausoleil's office.

So far, I like her - she understands my fears and my hesitation - and at the same time, she recognizes that we do want and need to trial something.  So our goal is to do everything we can to find the safest options for trial.

Step 1: New round of Skin Testing

Step 2: Blood testing

Step 3: New round of Patch Testing


We kicked off Step 1 this week, and Aidan was none too pleased. But we got some results, so I consider it a success.

Skin testing tells us the IgE part of the picture.  The IgE allergies are the ones that keep mothers awake at night hoping they'll be able to administer that Epi Pen and praying they'll never have to.

At this point, Aidan's IgE allergies are Peanut, Tree Nut, Soy, Peas, Melons, Squash, and Zucchini.  We're told that all gourds should be avoided - so I guess we aren't going to be a pumpkin kind of family.  Who needs them, anyway?

Steps 2 and 3 are soon to follow.  Stay tuned :)

PS - Do my eyes deceive me?  When did my toddler get a hairy back!?