Oral Aversions and Therapy

Job seems to continue to improve. Yay! He slept for an entire four hour stretch last night!?! Amazing!

He had a good therapy session today (something that we haven't been able to say for the last three weeks). I'm so thankful for the amazing therapists we have! Job lost quite a bit of ground this last month as he's been so sick, but for every two backward steps he takes we're going to keep pushing for a forward step now and then. A new concern that, likely because of tasting so much vomit for so long, Job has developed some oral aversions. This is yet another completely new issue for us to now navigate.

Possible Improvement?

We're surviving and maybe, just maybe, seeing improvement!

I think we're realizing that Frightful just isn't strong enough to make it through a cold or tooth coming in without great trial. It's sad and discouraging to realize this, as well as just plain hard to deal with practically speaking as he needs constant, intense care when so sick.

Sunday, Monday and today have each improved upon the day before so we're hopeful this trend continues and we could have a good weekend and early next week before his cath and hospitalization on the 8th.

It's nice to be back at a place where he can cope for 30ish minutes without being held so we can do things like quickly set up a Christmas tree and such. I'm scared something else will come up between now and his cath but I'm trying to choose hopefulness and trust instead of pessimism and anxiousness.

HLHS Explanation Video

https://youtu.be/gyMq0xhnVMU

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Miserable Again

I think this photo sums up the last two days well. Job is miserable again.

We had five wonderful days and now we're back to almost where we were 3 weeks ago in terms of his fussiness (though vomiting seems to be under control?).

Job is definitely teething but we can't think of anything else that has changed. Maybe he's operating on such slight reserve that even something like teething sends him over the edge of misery?

GI Clinic in Everett!

We got to go on our first family-of-five "adventure" (since The Vomitting began) on Thursday to Gastroenterology Clinic. It wasn't the most thrilling destination but it was really nice to be together and out of the house.

We're looking at a likely diagnosis of gastroparesis, which is where the stomach empties slowly. Too slowly. This, with his severe acid reflux, seems to be the primary reason for the vomitting, but teething and a cold haven't helped.

The two medications Job is on do seem to help (omeprazole as an acid neutralizer and reglan as a motility agent).



We have and will continue to investigate diet, but allergies don't seem to be the issue. Our next step would be an endoscopy if things don't improve in a month. We then also need to consider an NJ tube. I didn't realizing a feeding tube was even on the table and the very idea of moving so far backwards is crushing. (But, you know, so would be continued weight loss.)

We have learned how to manage his care in ways that seem to help, like giving 2oz of milk every 2 hours instead of 5oz every 3+ hours. Since he's both getting less milk and still vomiting we'll likely begin fortifying again for greater caloric density. His weight loss seems to have stopped but we have yet to see significant weight gain again.

We haven't even tried to wean off oxygen nor will we for a while. Likewise many of his therapy goals are on pause.

Job's cath is scheduled for December 8th. It will be at least one night inpatient and I think we're all expecting interventions, which might mean a longer hospital stay.

This isn't where we expected to be two months post-Glenn and it's been hard to realize that emotionally but it's been especially hard to just deal with practically. Harder even than when we had bigger cardiac issues, weirdly enough. But it seems like we're starting to emerge from the worst of it? We're at least in a brief respite and very grateful for it, especially because Job isn't so constantly miserable. He's even happy sometimes! Each day this week has been an improvement on the day before.

GI Issues?

Loonngg appointment today, including a full cardiology consult, an echo, a belly x-ray, full blood panel, and new meds.

We won't hear about the blood draw until tomorrow but everything else checked out ok.

It was gratifying to hear Job's team acknowledge that this isn't isn't right and to have them order such a full work up of tests, even though I would have preferred for them to tell me that I was being paranoid and impatient.

We're mostly sure that it's not heart related, and while we're hoping GI has answers (beginning in our Thursday appointment), we're now exploring liver and pancreas issues as possible culprits.

Job was surprisingly accommodating but of course sad.

Squeaky Wheel Gets an Appointment

Well, I guess I've been enough of a squeaky wheel that we're getting some grease.

Or, put more clearly, Job has a cardiology appointment in Seattle tomorrow morning.

I think (hope?) it's more to placate us than because his cardiologists are so worried, but I appreciate that they listen so well and are so happy to consider our concerns.

Over the last 3.5 weeks Job hasn't gone more than 30 hours without projectile vomiting. He is more consolable but still quite uncomfortable. We have a diagnosis of severe acid reflux, which has been compounded by a cold and by teething the past two weeks. We have a gastroenterology appointment on Thursday in Everett.

Job has had some ok days with some contentment and manageable fussiness and only one or two vomits, but never two in a row. He's eating less and I guess I'm worried about dehydration and just need reassurance that we're not missing something.

Tired and Uncomfortable Still

This photo probably accurately captures where Job is at right now, poor guy. He's slightly more calm and interested in the world again, but still tired and uncomfortable.

He is doing better, but he has a long way to go before he's back to himself. He's sleeping for slightly longer stretches (2ish hours at a time) and he occasionally lets us put him down for a few minutes. He'll even attempt to play with his toys sometimes. He's more consolable; his body is less rigid and tense and he'll snuggle up to us. He's only vomiting twice a day now, on average. These are all improvements.

Wednesday's upper GI scope gave us the diagnosis of "severe acid reflux". Thankfully, it seems as if reglan, which we started Monday morning, is doing it's trick, but we have a full gastrointerology work up next week. This is a totally new area to us and so I have all sorts of questions.

I assumed he had a cold since the big boys and I got sick last week/through the weekend, but he didn't have obvious symptoms like we had... but we weren't sure. But by Tuesday evening it seemed as if the balance was shifting from (what we know know was) agonizing reflux pain to common cold discomfort with more teething pain for good measure. That's still where we are now. His pediatrician checked his ears today, but there's only a bit of redness, no obvious infection. Job's down to 79% oxygen saturation on 1 lpm of flow (which is a decrease of 6-7 points from where he was last week, after we turned him up to a full 1 lpm), so this cold is certainly affecting his respiratory status. (But YAY for having oxygen and not being in the hospital!)

Sadly, his cath is pushed out 4 weeks from today because of this cold. We knew that was likely and of course we understand and agree that we don't want to mess with anesthesia and ventilation when he's sick/recovering from a cold. But it is disappointing that he got sick at all (why can't we keep germs at bay?) and that we have to wait to know the full extent of his cardiac situation. I'm also very sad that Job can't travel and needs me to stay with him through the weekend, so I'll have to miss my grandpa's funeral in CA this Saturday.

But we're grateful for small improvements! Job isn't constantly writhing in pain any more and we have some goals to work towards now. We've been able to get more sleep too. It's still hard to find contentment and joy this week, but we're working on it. God has been very gracious to us this week and we want to be grateful. Certainly we can (and have!) acknowledge(d) the hard things, but we don't want to wallow there.

A Bit of Sleep!

Guess who slept the best they have all week last night??? Well, what three persons actually!?!!

Now Job's off to spend the day at the hospital with Daddy for all sorts of fun assessments to see if we can start getting some answers about this vomit issue.

Awful Week

Quite frankly, this has been an awful week.

Job has been ridiculously inconsolable. He vomits 2+ times a day and has a cold, but it's his irritability that is probably most challenging and exhausting (physically and emotionally).

I was so discouraged, but by so many means and people God sustained us for yet another day. I'm trusting that He will through the night as well.

I've posted this song before, but it has been on repeat this past year and certainly was today:

"Your plans are still to prosper
You have not forgotten us
You're with us in the fire and the flood
You're faithful forever
Perfect in love
You are sovereign over us
Even what the enemy means for evil
You turn it for our good
You turn it for our good and for Your glory
Even in the valley, You are faithful
You're working for our good
You're working for our good and for Your glory"

https://youtu.be/B66DHRY1ITs

Hard Stuff

When I look at this picture I realize how far Job has come, thanks to his physical therapist and special ed teacher. He has gained so much core strength and spatial awareness! I think we have the two best therapists we could have asked for! They teach us so much every week and Job is making huge strides developmentally.

The last week has been rough as we've all struggled through a cold. The kids are slowly getting better! I'm grateful that we have oxygen so that Job could stay home instead of go up to the hospital.

His vomiting continues, despite new medications. I was so hoping they would work! I'll find out Monday morning if we can continue with the GI scope this week or if it will have to be postponed like the cath because of this cold.

Job hasn't slept through the night well since our last discharge, but his cold has exacerbated his sleep issues. Seth especially has taken on the brunt of Job's irritability at night, but between the two of us we're up 6-8 times a night with Job. We're both so nervous about his vomiting, so every cough and choking noise sends one of us (usually Seth) jumping out of bed.

As with everything, we're supposed to divide Job's actual age in half because of his condition and the invasive nature of his surgeries, so when I was asking about Job's (lack of) sleep habits this week I was told to think of him like a 3.5-4 month old baby. I suppose that helps a bit, at least to manage our expectations, but Job was such a fantastic sleeper until the Glenn that it's hard to adjust. We're tired!

To end on a positive note, we had our best week of school ever! The big boys are finally settling into the routine we've been trying to establish since coming home and I'm absolutely thrilled. I would love for us to have good health and good sleep and good school, but since we apparently only get one of those things at a time, I'll take school!

Cardiology Clinic (6 weeks Glenn post-op)

Job had his big cardiology clinic appointment yesterday. I had been counting down the days until this appointment with a great deal of trepidation, along with a bit of desperation (largely because of his frequent emesis).

I think the bottom line report is mostly good, but there is just so much involved in Job's care that I need to write out my report of what all we talked about in order to process it. Want to read along?

Oxygen
Let's just start with the most mortifying piece of information: for the last 2.5 weeks, while using the concentrator at home, I have only given Job 1/10 of the oxygen he was prescribed (but while out and about on his portable tanks he's received his full amount of 1 lpm). I am horrified and embarrassed and feel utterly irresponsible. I guess we must have played around with the settings during the training and the concentrator was set on .1 lpm for some example... But came home that way too. I did notice that it said .1 instead of 1, but assumed that the flow meter used a different unit measurement and .1 of whatever this unit was translated to 1 liter of flow. Since we now use the (otherwise unfamiliar) metric system for all of Job's medical measurements, I often have to clarify his meds with various providers as they often use different units. One doctor will talk in mgs while another will talk in mcgs and so on. When clarifying these med doses, I always feel silly (I am still insecure about my lack of mathematical confidence, after all) but have done it anyway to make sure. And I guess this time I just gave up clarifying. Perhaps the lesson is that unit conversions in Saxon Math homework really is helpful later on in life.

So Job's sats have slowly climbed from 76% to ~81% while on "1" (really .1) liter of oxygen, but this very slow rate of improvement to a mere 81% had us all worried. But last night as we decided to put Job down to .5 lpm on his oxygen, Seth - not me - went to change the flow meter. And he couldn't figure out how to go down lower that where I had it set... As the realization of what I had done set in, we put Job up to .5 lpm from .1 and immediately his sats rose to 86%. Today in clinic he was reading at 89% on .5 lpm.

Job's cardiology team just laughed at (with? maybe someday...) me for my mistake, since the whole time at home Job hadn't ever dipped below 75% O2 sats (his cut off). They probably laughed in great relief too, now that we had an answer for his "lack" of *acceptable* improvement.

It's now up to us to play with his oxygen wean as we see fit. For these next few days we'll probably keep him at .5 lpm most of the time and if he continues to thrive we'll probably keep him on .5 lpm during the night for a bit after that.

We're going to keep our tanks and the concentrator for the foreseeable future in case of a cold or emergency. It is nice knowing we have oxygen available so that if his sats start declining we can out him on oxygen at home instead of take him to the hospital (if that was the only reason to admit).

We'll notice a need for more oxygen if his work of breathing increases or he gets particularly cyanotic (blue) but mostly through regular pule ox checks.

Vocal Fold
Job passed his PT swallow check and no longer needs to be on thickened feeds! Yay!

We will almost certainly have to do another scope with Otolaryngology but their wait list is so long it will probably be a few months out.

His vocalizations and cry are still very quiet and raspy so there was almost certainly additional injury to his vocal cord during his arch repair. We originally thought his noticeable inflammation and raspy voice was just because of intubation and reintubation but if that were all it would have healed more significantly than it has. So we will look for continued but gradual improvement over the next six to eight months. It's entirely possible Job will always have a raspy voice because of this, but eventually his vocal cord should close fully (and then reduce his risk for aspiration).

Vomiting
This is the area where we had/have the most concern. Job is vomiting a moderate to large amount every day without a clear reason every time. Sometimes it is because he chokes himself with his hands. Sometimes it is because he coughs up his meds. Sometimes it is because he's still struggling to regulate his digestion and when he strains to poop he vomits because of the effort. Sometimes it is because he got really, really upset (like during today's echo).

Job is drinking so much (~900 mLs/day) and gaining great weight (~20 grams/day) that we aren't yet worried because of dehydration or nutrition concerns.

But it is a very new problem and is currently inexplicable.

So the plan is that we're going to start with a stool softener because his emesis does seem most tied to his bowel regularity, but after a few days we'll also go on reglan. And next week we'll add Gastroenterology to our every growing list of clinic followings and do an upper GI tract scope.

Echo Findings
I pushed for an echo because we're so concerned about Job's inexplicable vomiting. His liver was a bit swollen when palpatated, plus he's Job and has quite the reputation, so I think that makes it much easier to get oil on my squeaky wheel questions.

Good news first: Job's heart function looks good. Yay! His tricuspid valve regurgitation looks the same.

Not so good news: the flow out of his aortic arch seems too fast and there appears to be narrowing in his left pulmonary artery where the stent was placed. Echos cannot accurately show the severity or significance of these issues so it is possible that both of these concerns aren't really concerns at all. But they were alarming enough that we will be getting a cath in about two weeks.

Dr. Chan's best guess, based off of today's echo, was that we will likely need to balloon the stent. As with every cath, Job will be intubated and sedated, but if it's purely diagnostic and no interventions need to be made then he could go home the same day. If interventions are made then he would spend at least one night inpatient for observation. To need to intervene so soon after the Glenn would be very concerning and, of course, a cath carries some risk (perhaps most especially for clotting), but of course we need to know what precisely is happening with Job's heart and lungs, so a cath it is!

Hematology
Our hematology consult was mostly uneventful and unexciting. We were basically just establishing care with them as yet another clinic following Job. They agreed with the cardiology recommendation that we follow a six month regimen of lovenox for Job because he is so hypercoaguable (a standard course is eight weeks). They also recommended that we plan on starting a heparin drip before he has any additional central lines placed in the future. Most of his clots are due to these lines so we want to be proactive about them from here on out. (And they also recommended that he never smoke as smoking will drastically increase his clot risk! I mention this just because it's bith funny and fascinating to me. It's hard to imagine my baby growing up and smoking of all things and it's amazing to realize how invasive the health risks from smoking are.)

We will draw for his Antithrombin III levels at our next lovenox level draw because we need to see what his normal Antithrombin III levels are. I don't think I ever mentioned it but Job needed a dose of thrombate while in the CICU in one of his multiple transfusions. If his levels are still low this could be one of the pieces for why he clots so easily but probably wouldn't be the only reason.


Whew! It's a lot to take in, as always. Sometimes I leave Seattle Children's with my head just spinning with information. I'm so grateful for the incredible level of care we receive there! And as overwhelming as it feels to have November suddenly fill with ten more medical appointments, I'm so grateful Job's team is so vigilant.