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

All Of The Unfair - and why it's okay for me to whine about it sometimes

Sometimes, life is unfair.

It's the kind of thing you tell your kid when he so desperately wants that new toy, or so desperately wants not to eat that broccoli.  You're the adult, and you know that he can't have exactly what he wants, exactly when he wants it.  Life gets in the way, you need to buy groceries instead of toys, and you need to eat broccoli to grow big and strong.  Sometimes, life is unfair.


Picture, if you will, a little boy who loves Chuggington Trains.  He loves them more than almost anything.  I'd never ask him to choose between Mommy and Chuggingon Trains, because frankly, I don't think I'm emotionally prepared for his response.  Chuggington is so immediately loveable for him, because the songs are easily memorized (ah, familiarity), the characters are clear and concise as they describe their thoughts and feelings (nothing ruins a good show faster than having no clue what's going on!), and the toys look just exactly like the trains on TV (there's our good friend familiarity again).  Other little boys seem to like playing with them too, whether they're Chuggington fans or not, so they're a pretty universal social uniter.  Aaaaand, the show doesn't make me want to insert ice pick A into brain stem B, like Thomas the Train does.  Really, Chuggington for President.  I digress.

So there's a little boy who loves Chuggington, more than almost anything.  As a special Christmas gift surprise, he got tickets to see Chuggington Live on stage with his Mommy.  It was going to be a really special big boy Mommy/Aidan day, and since Mommy's not insane, Aidan wasn't told of the surprise until the day of.  But boy, was he excited!  Big, real Chuggers!  No way!  Let's go!!!

Now picture, if you will, that little boy starting to feel sick.  The drive to Chuggington is long, and as time goes by, the little boy feels sicker.  By the time he arrives at the show, the sad truth is obvious.  There isn't going to be any Chuggington today.  There's just going to be a 75 minute drive to the Emergency Room, where he'll be poked and prodded while nervously asking if he can go see those Big Real Chuggers yet.

Then, he'll go home, and get sicker.  The next day, he'll go to the doctor, who will send him back to the hospital in a big, scary Ambulance.  This isn't Big Real Chuggers, Mommy.  I know, Baby.  I'm so sorry. More poking, more prodding.  X-Rays, IV's, no sleep (we didn't even get admitted up into a room until 8am!), and certainly no Big Real Chuggers.


And I get it.  Really, I understand.  Life isn't fair.  I know how to say all of the right things to Aidan, to make him understand, and to help keep him from being an angry and entitled little urchin.  But here's the thing - who's going to come explain it to me?

Because It isn't fair that catching some plain old run-of-the-mill virus sends him to the hospital, and other kids wipe their noses on their sleeves and get on with their lives.

It isn't fair that everyone is signing up to bring candy, juice, and cupcakes to this week's school party, but all my kid wants is to hug a banana.  "I won't eat it, Mommy.  I promise.  I just want to hug it.  Please?  I love that yellow guy so much."

It isn't fair that everything that happens to him forces me to doubt whether I can care for my own child at home.  While other parents wonder if if their kid is too sick for school or healthy enough to throw at the wall/hope it sticks, my brain is overflowing with phrases like feed intolerance, elevated ketones, hypoglycemia, dysmotility, and direct admit.


It isn't fair that my kid *just knows* that he wants the IV supply cart removed from his ER room before he'll set foot in there.  Or that he chastises his nurses for using sanitizer instead of soap and water.  Or that he requests specific toys from the floor's playroom, because he knows they're there.  It isn't fair that all of this is so normal to him.

It isn't fair that he gets a cup of water and a spoon at the ice cream shop, while everyone else gets ice cream.  "Don't worry mommy, I can just pretend it's ice cream, because I love it!"

None of this is fair.  He deserves so much better than he gets.  He doesn't really know how unfair any of it is - not yet, anyway.  He knows that his life is pretty great, and that he's loved, and that he's safe.  He knows that he has everything he could ever need, and most things things he's ever wanted.  Unfair, to him, is bedtime when he'd rather it was TV time, going home instead of going to Target, and mom meaning No when she says No.

So I know, these are all my thoughts, and not his.  But you know what?  That's okay.  It's okay for me to hate the cards he was dealt sometimes.  It's okay to say "How am I today?  Not so good...  This day is not so good at all..."  There's no special trophy for pretending everything's just wonderful all the time.  My little boy is brave, and and he's tough, but he shouldn't have to be - and it's A-OK for me to feel that way today..

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. 

CHOP Inpatient - Musings on Feeds

After Aidan's scary-yet-fun (?) ambulance ride to CHOP on Tuesday night, we settled into our room on 5 South and awaited a plan.  I swear, that's all we ever do here.  Wait for someone to figure out how to help him.  And in the end, it's always a patch up/send home job.

Aidan had no problem immediately falling asleep.  I, on the other hand, stayed up to panic about work and ponder what the appropriate hour was to text my boss.  Finally, I had to reach out and admit that I was here, with no access to my work laptop or phone, and would be missing several important meetings.

I hate this part.  Obviously my child is the most important thing in the world to me - but I pride myself on the work that I do, and not giving 100% does not sit well either.

Listless boy :(

Anyway - what Aidan really needed were IV fluids for hydration and gut rest.  Late in the day, we started up Pedialyte, and he finally produced wet diapers.  He really seemed to perk up quite a bit.

Toddler Problems: he's a righty.  no coloring :(

When we started up the 1/2 Pedialyte/1/2 Elecare mixture, he starting showing signs of a flushed face - but no fever.  Odd, and I actually asked several times that his temperature be rechecked because he felt warm to the touch - but no fever was recorded overnight. 

He was clingy and moaned a lot in his sleep.  But no fever, and no vomiting, so we advanced the feed to full Elecare.

We did get some sleep overnight, despite his discomfort, and when I woke up, I did my usual check of the room.  I always double check his feeds and rates and vitals - just to see how he's doing.  What I noticed made me instantly sick to my stomach.

Elecare Jr. Vanilla.  VANILLA.

Yup.  The doctor ordered the wrong formula.  The nurse hung the wrong formula.  They fed my baby the wrong formula.  Formula he's reacted to in the past.  This is not okay.

I feel guilty for being asleep at 6am when this was hung.  But I can't feel guilty for sleeping.  I can't feel guilty for their error, and their lack of quality review.

Aidan is okay.  He was uncomfortable and flushed in the face - but he is okay.  What if the mistake had been a dairy-based formula?  Or a soy-based formula?  What if he'd suffered anaphylaxis due to this mistake?  It's not okay, and CHOP needs to get it together.

The rest of the day was frustrating and disappointing.  The usual CHOP fare.  He's not pooping (surprise, surprise) so we're giving Miralax (no results).  He's feeling much better now that he's hydrated and is really ready to come home.

The main thing slowing things down is that I spoke to a GI, explained alllllll of our sordid formula history (the neocate drama - the G feed intolerance - the GJ tube change - the ensuing frequent hospitalizations).

It is my genuine belief at this point that he DOES NOT tolerate Elecare Jr.  When we give it by mouth or G-Tube, he gets rashes, vomiting, and GI pain (Screaming.  So much screaming.  Especially at night).  When we give it by J-Tube, because we have bypassed the stomach, the symptoms are different - but we get decreased motility (leading to constipation so severe, he ends up hospitalized for cleanout) and pain/discomfort.  He's not getting the rashes and vomiting - something about the chemistry of the jejunum vs. the chemistry of the stomach seems to produce a different set of symptoms.  But it seems clear to me.  Before we started Elecare Jr, do you know how many times he had these lower gut issues?  ZERO.  It's related.  It has to be related.  And I think it's the same, whether it's flavored or unflavored.  We never had these issues on the well-tolerated unflavored Neocate Jr or Neocate infant (but, interestingly, we did have the same set of symptoms on flavored Neocate Jr and Splash).

The GI I spoke to this morning seemed on board.  He said that clearly there was an allergic process in play, and that we needed to investigate the micronutrients in the formula to see what he was reacting to.  He even said he knew of a GI in the hospital today that he thought could help.  But he never came back.

Instead, he sent someone else - a resident, I think - to tell me that he was in the OR for the rest of the day, but thinks we should follow back up with our own GI in a month or so, and consider seeing an allergist.  Sound familiar?  It should.  It's what we're always told.  It's nobody's problem.  It's nobody's responsibility.  GI thinks Allergy should help.  Allergy refers us back to GI.  Everyone feels that things are going "generally well" for Aidan.  Everyone but Aidan, who's living in pain every day.  Aidan, who's dying to eat, but starving to live.

Someone has to help us.  Someone has to care.  I was hoping we would find someone today.  We didn't.

Aidan's First Ambulance Ride

Laundry.  Dishes.  Catching up on work.  Watching an episode of True Detective.  These are the things I thought I might be doing on a Tuesday night.

Aidan had other plans.

Aidan usually has other plans.

That looks suspiciously like an ambulance...

It started on Monday.  Aidan's teacher had called to let me know he seemed "off" and "half of his head had a fever" (???)  When we got him home, he seemed happy and playful, so we weren't too concerned.  I was in the kitchen washing some dishes when he ran over and asked, rather frantically, that I take his sweatshirt off.  I guess his fever was spiking pretty rapidly at that point.  I thought he was just being Aidan and/or Three Years Old, so I told him to be patient until I was finished.  No more than five minutes later, I joined him in the living room as he projectile vomited all over the living room floor.  His temperature (on both sides of his head, thank you very much) was 102.

I gave him tylenol and put him to bed - he seemed okay, but would be up several more times needing tylenol for his 102+ fever and vomiting in his bed.  It was a long night.

The next day, I left him with Tom and went to work.  Tom let him sleep in, and when he woke up, he seemed fine.  Not feverish and not vomiting.  We decided to give him some gut rest, so put him on pedialyte feeds for the day.  Tom dropped him off at daycare (in retrospect, probably an error in judgment), but daycare called in the early afternoon to report that he had a 102+ fever and was "shaking uncontrollably."  Tom picked him up right away.

At that point, I was thinking that this seemed very similar to Aidan's flu symptoms, and I wanted him checked for flu quickly - if it was the flu, we could still get him tamiflu and hopefully avoid the weeks of misery and weight loss that accompanied the illness last month.

Aidan's pediatrician was booked solid until the next day, so I decided to take him to Urgent Care - thinking that surely they'd be able to administer a flu swab.  When I got home from work, Aidan was doing pretty well - likely thanks to the tylenol Tom had given him that afternoon.  I held off on taking him to Urgent Care- just put him to bed and hoped for the best.

Around 9:30, Aidan woke up feverish (102.4) and vomiting.  We immediately sprang into action - packing him into the car and heading for CHOP's new Urgent Care facility in King of Prussia.  We arrived around 10:30 (whew!  they close at 11.) and Aidan was still vomiting in the waiting room while I was checking him in.

Urgent Care got some tylenol into him, drew some blood for labs, and started an IV, but because of his high heart rate and dehydration symptoms, they decided to transfer him (via ambulance, no less) to CHOP.

Aidan was none too pleased with this plan.  He hated the restraints, he hated the "big truck" (he specifically asked for a "tiny truck"), and they didn't take him to "Aidan's House" as requested.  Oh well.  That's disappointment for you. 

It's like a really big rear-facing carseat, because safety first!

Watching them strap him onto the stretcher was actually kind of scary.  I guess I didn't realize how sick he was.  I was looking for a flu test and an Rx.  But don't get me wrong - I'm thankful that they recognized he needed more support.

When we arrived at CHOP (around 1:30am), I learned that he had orders for a direct admit.  I was confused and exhausted - and of course didn't have my perfectly planned and packed hospital bag or ANY of my work things.

The Emergency Department was full, so I was glad that Urgent Care had secured him a spot - because the GI floor was also full, and it would be four more hours before a bed opened up on 5 South.

Sleepy Bear

Sweaty Bear

Not much happened down in the ED - we really just hung out, kept him on IV fluids and nausea meds, and waited for his bed upstairs.

Finally, in the early morning hours, we made it up to 5 South - to the same room he stayed in last March, when all hell broke loose with his stomach and we had his GJ placed.  Small world.  Small GI floor.

Feed Frustrations - Part 2

The plan at the moment is to try giving 1-2 ounces by G tube once or twice per day.  I'm never very optimistic when we try anything, but I know that if we don't try this - if we don't prove out the problem, there will be no help.

Day 1 - Saturday - 1oz by G-Tube in the morning, 2oz by G-Tube in the evening.  Seems well-tolerated.  No vomiting.  Is this what hope feels like?

Overnight - Awake many times, crying and coughing.  Diarrhea overnight with immediate diaper rash.

Day 2 - Sunday - I did not give anything by G-Tube in the morning.  Since he'd had diarrhea overnight, I wanted to give him a little recovery time.  I gave 2oz in the evening.  Noted bright red flushed cheeks after feeding that lasted for several hours.

Overnight - Again, awake many times, crying.  Lots of coughing and retching, but no vomiting.  No poop overnight (abnormal for Mr. A - he's an overnight kind of guy).

Day 3 - Monday - Nothing in the morning.  Daycare reports that he's not napping.  Added Prilosec in case the night crying is due to reflux.  Fed 2oz in the evening, noted bright red rash on cheeks and red blotchy skin on neck.  Several hours after 2oz bolus, noted distended belly, but venting gave no relief.

I finally thought to take pictures.

Overnight - Awake lots.  So much crying.  No poop overnight.  No bueno.

At this point, I reached out to Nutrition, GI, and Allergy.  It's difficult to get anyone to call me back, but Allergy bites first - they feel that the rashes are likely unrelated to what we're feeding, but they'd rather defer to GI.  If I'm very nervous, I can bring him in.  It's really GI that I need to speak with.  I never got in touch with Nutrition.  But GI agrees with Allergy that the rashes are unrelated, and that this "very small amount" of formula is unlikely to be causing any issues.  He probably picked up a virus.  We should take a week off and retry again later.

So on what would have been Day 4 (Tuesday), we fed nothing by G-Tube.  Miracle of Miracles - no rash on his face.  No 9 overnight wakeups.  And he pooped completely normally. 

Nice rashless cheeks...  Kid not crying.  Good stuff!

So now I guess we wait and retry this business in another week or so.  Pardon me while I try to scrape together some optimism :-/

Feed Frustrations

It's been about a year since we lost the use of Aidan's stomach.  We don't know exactly why it happened. 

In December of 2012, we fed Aidan exclusively via G-Tube (into his stomach).  By January, he was vomiting everything we fed him.  Through February and March, we tried different Formulas and different feed schedules and different rates.  At the end of March, Aidan was admitted to the hospital for IV fluids and tests.  A dye study confirms that the tube is in place.  A Gastric Emptying Study was fairly traumatic, and gave us no additional information - probably because Aidan couldn't keep the tracer down.  No doctors gave us any answers, but a GJ-Tube was placed in hopes that if we couldn't fix Aidan's stomach, we could at least avoid it.

The stomach problem was never solved - just brushed aside.  It has always nagged at me.  Why does a stomach work every day of a child's life, until the day it stops working, and never works again?  What caused this?  How can we expect a food trial to be successful - ever - without a functional stomach?  Did this have something to do with the formula change?  Nutricia will argue that nothing happened until they're blue in the face, but Aidan's stomach worked before the formula changed, and never worked again afterward.

So here we are.  Over the past six months, Aidan's been hospitalized for some awful things - severe constipation, feed intolerance (even through the jejunum), intussusception - things just aren't going well for him.  GI agreed that something seemed amiss and felt that it was (finally) time to revisit the stomach problem.

The orders were to give 1-2 ounces by G tube once or twice per day.  And what???, I asked.  "And we'll see what happens."

Back @ CHOP - Feeding Woes, What Else Is New?

It's only been a month since I've written.

It's already been a month since I've written.

Here we are again.  Like so many other times, the day started out fine.  We were having fun, being silly, taking photos for our Christmas card.  Aidan was in a good mood and no one knew anything was wrong. 

I'd noticed that he hadn't pooped since Friday morning - not something I'm constantly pondering - but when you have a GI kid, it's always in the back of your mind.  I gave some meds to get things going and didn't give it another thought.  We ran some errands, and I actually (insert famous last words here) remarked that he was in a good mood.

As the day wound down, Aidan sat in his high chair with a lollipop, while I cleaned up the kitchen.  I heard his episode of Bubble Guppies end, but no accompanying plea for "Moah Guppies!"

I found this:

My Grandfather on Christmas Day?  Close.

Super cute, right?  Tom and I took pictures and made appropriate amounts of fun of our little old man.  But then he woke up hard.

The all-too-familiar inconsolable screaming.  My mind instantly flashed back six weeks, to the Great Intussusception of 2013, and I immediately knew I had to call the doctor.  Of course we lucked into the practice's worst Pediatrician on call - but it probably didn't matter - we know by now that the Pediatrician can't help us.  He told me what I already knew.  I needed to get him to an ER.  Now.

We made it down to CHOP by 7, and were ushered into a small room called The Wedge - basically a tiny and oddly-shaped triage room that wasn't in use, but served to keep Aidan from freaking out in the crowded ER waiting room.  I appreciated it a lot.  We were triaged and taken back to ER Room 47 - a weird little out-of-the-way corner room with no outside foot traffic (and no working sink.  Yummy.).

By 8:00, we had a plan.  We would X-Ray him to check for belly problems - constipation, gas, the basics - ultrasound him to check for intussusception, and keep an eye on his blood sugar, since he was off feeds and not on an IV.

Absolutely COULD NOT get comfortable :(

The X-Ray happened around 9 and didn't show anything too significant.  Some backup, but nothing earth-shattering.  Two hours later, we finally got the ultrasound, and it looked okay - but we were cautioned that an intussusception is very hard to catch on an ultrasound.  It can telescope - go in and back out - so it can be fine one moment and then very not fine the next.  But as I said - the ultrasound looked okay, and no one really had answers for Aidan - so around midnight, GI was paged and the admission process was initiated.

Around 12:30, at GI's recommendation, they gave a suppository - but there was no effect.  An hour later, an IV was placed, and by 2am, we were brought up to trusty 5 South (5S11).  We settled in and I spent an hour or two working and rescheduling meetings before passing out for a couple of hours.

Zzzzzzzzzzz

After three luxuriously interrupted hours of sleep, I was up for the day to meet with the Attending, and talk next steps.  We gave Aidan an enema in hopes of moving things along for him, but with really unexciting results.  Aidan was given meds by G-Tube, which he promptly vomited.  Over the course of the day, the Attending avoided us like the plague - nothing happened for Aidan at all, other than that he slept and seemed to move past a lot of his discomfort.  He settled into a pale and lethargic state that made me feel absolutely awful for him.

 

Finally, toward early evening, I cornered the Attending and forced the conversation.  What are we doing?  Well, it seems that his GI tract is shut down right now.  What would do that?  Well, you know, we talk about viruses...  

The V word.  It's the dirty doctor word for "I don't know, but I need to say something, so I'll say this."  A virus is a thing that you don't treat - you just wait for it to go away - and it's the most frustrating thing for a parent, because when your child is hospitalized, you want someone to DO SOMETHING!  Fix him!  Make him better!  Help him!

But sometimes you really do just need to wait, and watch, and hold him when he cries.  And then celebrate the successes - like when he's feeling better enough to make a mustache out of his IV tubing.

This kid... I can't even...

I suppose at some point, someone should start trying to feed this guy.  I wish someone would tell me why his GI tract just stops working for no good reason.  While we're making wishes, I wish someone would tell me why his stomach stopped working at all last January.  And why we're now expected to shovel strawberries into it with any measure of success.  But that's another rant for another day, and I'll really, really try to come back and write about it before the next hospitalization.

CHOP Day 2

Day 2 started a bit rockily - IV checks every hour made for a seriously bleary-eyed mama by 8am when we finally called it a morning and introduced ourselves to our Nurse.  Erica.  Sadly, she recognized us from our last stay.  It's one thing to start recognizing nurses - but when they start recognizing you - it's time to stop having a sick baby!

Erica told us that she didn't know when or even if they would be able to fit Aidan into the schedule today for his Tube Surgery.  Extremely frustrating, but all we could do was wait to see.

A quick check out the window showed a Shields Family first - a gorgeous view of the ER.  This means that we're on the opposite side of the hospital than we normally are - it's so weird to have the room set up "backwards"

The rest of the day was insane:

9am: Another Anesthesia Consult.  We talked through the differences between anesthesia and sedation.  Because of his extreme level of terror, they think it's best to go whole hog (anesthesia).  But they tell me to have hope, and that as he gets older and more cognitively aware, he may start feeling safer and more able to handle what's happening to him without being put to sleep.

10am: Apparently I signed procedure consent?  Honestly, I vaguely remember a doctor asking me to sign something, and I signed it.

11:30am: Erica came in to tell us that we would be moving forward with the procedure, and almost immediately, Transport arrived.  Aidan wouldn't sit on the stretcher, so I carried him down.  While we waited in the PACU (post-anesthesia care unit) for things to get started, Aidan clung to me nervously, and we went through all of his allergies and medical history. 

12:00: Aidan went into Surgery.  I was told that it would be quick - Ten minutes or so for them to change the tube and quickly treat the granulation tissue.

This was the longest wait EVER.  It's always the longest wait ever, but still.  This felt insane.

1:30: They finally came out to speak with me about his surgery.  He did well, but it took (way) longer than expected.  They changed the tube, and downsized to a smaller tube (16Fr 2.0cm instead of 16Fr 2.5cm) - which is great, because we've ALWAYS suspected his GJ pain was due to a poorly sized tube.  They treated the tract with silver nitrate to remove the internal granulation tissue.  But the scary finding, and the reason for the long surgery time, was that they found an intussusception.  Basically, one part of the bowel slipped inside another part, causing an obstruction, which led to lots of pain for Aidan.  Intussusception is a medical emergency, and we're extremely lucky that it was found so early and they were able to fix it before there was any necrosis or tissue damage.  If they hadn't found it and fixed it so quickly, he could have lost part of his intestine, or worse.  Intussusception can be fatal in a matter of days if left untreated.

Looking as sad as I can.

Aidan woke up hard from the anesthesia, but lots of cuddles from mommy made him feel a lot better.

2:00 - Ready for Transport back up to 5 South.  We did not attempt feeds right away - he needed time to recover and rest.  He was snoring like a full grown man.

I felt awful leaving him, but I was starving, so a Nursing Student sat with him while I ran down to the cafeteria for some delicious elderly hot dogs.

Wouldn't be so bad if today weren't October SIXTH.

 2:45 - Aidan's IV became sluggish and the IV team was called in to save it.  We really couldn't afford to lose the IV, since the other hand was already blown.  Success, though - it was saved :)

3:15 - Plan was devised to start feeds at 4:30.  We would start at 30ml/h and increase by 5ml/h every 3h until we reach his goal of 65ml/h.  As long as all went well, we'd be discharged once he was at 65ml/h and tolerating it well.

4:45 - Feed started at 30ml/h

5:15 - Feed halted.  Aidan was in severe pain.  The doctors agree that he's not ready for feeds yet, and we will need to watch him carefully for any signs of a repeat intussusception.

7:00 - The pain has continued even without feeds, so we gave Tylenol and agreed not to restart feeds for a few more hours at least.  The rest of the evening was quiet and uneventful, except that Aidan demanded to be held pretty much the whole time.

If you aren't going to hold me, I'll just lay here and pick my nose.

Midnight - Feeds restarted at 30ml/h without fanfare.  We will increase slowly per the original plan, and if all goes well, we will go home sometime tomorrow afternoon.