Jobbers!

Job isn't quite himself but we're not sure why. No obvious signs of sickness, like fever or snot or other such symptoms. 😷 He's just extra needy and doesn't really want to play like he usually does. I suppose we're hoping he's teething? It's always bad news when he gets sick, but to be sick this close to surgery would mean we would have to postpone. 😨 He certainly has been ramping up his separation anxiety the last two weeks so perhaps that's part of it too.

Ezra took this photo and I processed it through an app.

Only TWO Med Times?

What is this magic? Down to a mere TWO med times! And only 7 meds!

-Enalapril for heart function
-Lasix and spironolactone for diuretics
-Omeprazole and erythromycin and miralax for digestion
-Lovenox for anticoagulation

We finally got off reglan (and switched to erythromycin) because of the potential awful neurological side effects. We're really really grateful that reglan worked (no more writhing reflux pain! no more weight loss!) but it is such a relief to be off of it! The downside of erythromycin is that the prescription is only good for ten days. Lots more pharmacy phone calls for me! They know us well. 😜 All joking aside, Lincoln Pharmacy has been absolutely wonderful! I'm so grateful Tacoma has a compounding pharmacy and we don't have to go up to Seattle for Job's meds.

It was so overwhelming to give Job his meds when he first came home. I believe he was on 14 different medications with 8 different med times? But we've slowly weaned off/down. 🙌 There are probably a few things he'll take his whole life but that doesn't seem like such a big deal any more.

I also have forgotten to post about the "final" GI diagnosis we received back in January. Well, I think we had a good idea that it was secere acid reflux and gastroparesis (delayed stomach emptying), but what I didn't post was the conclusion that these are ischemic issues, stemming from his cardiac issues.

Ischemia is the inadequate blood supply to tissues, or inadequate oxygen and glucose supply for cell growth.

We have always known that Job's lifetime of restricted oxygenation will lead to ischemic issues with his organs. It sounds like this is a bigger problem for most HLHS patients rather than heart failure (once past all three surgeries). But I guess I didn't expect it to have such severe repercussions so soon.

Job's digestive system is suffering from his low oxygenation and thus his stomach doesn't empty quickly leading to acid build up. We're still learning about the digestive system and trying to treat this with diet too, but the fact that so much of this is cardiac related is limiting. But that's true of all of his long term care and that's what so many researchers are focused on now that they have had growing success with the cardiac surgical side of HLHS care.

Fistula Pre-Op

Yesterday Seth, Job and I spent the entire day together up at Seattle Children's for Job's many pre-op appointments. (Usually the pre-op appointments are scheduled closer to the surgery date but our surgeon will be out if town next week.)

Our days in Seattle are always long as they try to schedule back to back appointments in order to decrease our number of trips up north, but yesterday was especially long and so Job and I were really glad to have extra company. Sometimes too, when I'm only relaying information to Seth, I miss important details or nuances, so it was good for him to hear from the surgeon and doctors first hand.



I feel like my head is still spinning from all that we talked about and did, so this will be an incomplete update as I need to process and research some of what we talked about. Nothing bad! Maybe even something good.

We met with the surgical NP and went over the various details leading up to surgery, like the meds Job will take beforehand, and went through his entire health history to make sure all the relevant details were noted. We did a chest x-ray. We did a 12 point EKG. We met with PT to evaluate how Job has been tolerating his thickened feeds. We did a blood draw to check his CBC, INR, electrolytes, BUN, creatinine and prealbumin and give a sample for the blood bank (to reference for his post-op crit transfusion). We met with Dr. Nuri (who has performed Job's three previous surgeries) and discussed the auxiliary fistula Job is scheduled to have in March 7th. We talked to anti-coagulation about Job's elevated INR levels, decreased his lovenox dose, and discussed the likely anti-coagulation plan post-op. We went to the pharmacy. And we did an echo to check Job's heart function and tricupid valve regurgitation. Whew!

A day like yesterday serves to underscore my complete wonder and awe at the amazing complexity of the human body. I'm just so grateful that all of these evaluative tools are available now and for the knowledge and wisdom Job's doctors possess. Even though most of my days now are focused on some aspect or another of Job's health, and we have learned SO MUCH about the body, sometimes I get complacent and take Job's care for granted... And then I'm reminded again at how very complicated and multi-factorial his care is and the realization just overwhelms me and fills me with gratitude.

Standing!

Job has been working so hard the last few weeks to learn to stand independently!

He's now adept at standing if you set him down in a standing position. He cannot stand up on his own, though he can sometimes pull himself up to standing by using nearby furniture.

We're working on greater stability and confidence in his standing, and, slowly, taking steps! In this photo he was so excited about putting these necklaces on the plastic squigs (on the mirror) that he would forget himself and take a few sideways steps.

Mid-February Update

Here's a silly photo I made with a photo editing app of the five of us cuddling on the couch.

People ask me, actually multiple times a day, how Job is doing and I never really know what to say. [I am so appreciative of these inquiries! It means so much to us that so many people care for Job!] But I never know how much information I should share. And ultimately the answer - at least so far - has never been "great!"

But Job is currently stable and still at home, so we are very, very grateful.

To have a whole month "off" (waiting for his March 7th surgery) feels amazingly luxurious. There are still many appointments each week and various other assessments/reviews and plenty of phone calls and emails keeping everyone in the loop... but the frantic "why can't he get off oxygen??" questions (and therefore lots of testing) have been answered. We're not waiting in the same way we were from October through January and it really does feel as if a burden has been lifted.

We're using this time to really work on Job's physical therapy goals and feeding goals. And we're trying desperately to avoid germs!

VFSS and Thickened Feeds

We got to do a videofluoroscopic swallowing study (VFSS) today. Essentially, barium was added to Job's bottle and carrot puree and x-rays were taken of him swallowing.

Job wasn't very cooperative and for what seemed to be the first time in his life, he wasn't interested in eating. We didn't feel like we got a fair assessment of his swallow, but he did aspirate while drinking thin liquids (his regular milk). He did fine with his puree and with the thickened milk because he can't swallow these as quickly so it gives him time to protect his airway.

It sounds like it was already the plan to put Job on thickened liquids because of his oxygen dependency but catching an aspiration on video didn't do him any favors. Well, that sounds like being on thickened liquids is a bad thing. It's not, or at least, aspirating is a really, really bad thing so it's not a bad thing comparatively. It's just that thickening his milk is yet another really time consuming and, well, annoying thing to add to Job's care.

I also enrolled Job in another research study and had to sign consents and do a blood draw for that. This study is looking at that elusive "hepatic function" so they'll do another draw two months after the AV fistula and compare results.

And Job had a three hour ultrasound of his upper extremities to assess his veins and arteries in preparation for his next surgery. Both right and left side are patent! (There's good blood flow.) Yay!

And we also had a consult with the PT we have seen inpatient. She has primarily helped with Job's swallow and has directed much of his feeding plan.

It was a really long day and I'm feeling exhausted and a bit discouraged and overwhelmed with where Job's at overall. We just keep adding to our list of complications. That his left vocal cord and epiglottis are still causing him such difficulty with something like swallowing?!? It seems never-ending.

This photo shows the bottle at the left and the black liquid in the bottle is the barium-milk, being swallowed.

Fistula Scheduled!

We finally have a fistula surgery date! Yay! We are scheduled for March 7th with Dr. Nuri, who has performed Job's three previous surgeries (2 OHS and 1 diaphragm plication). We have an incredible respect and appreciation, as well as comfort and familiarity, for Dr. Nuri, so to have him available to do this surgery is particularly wonderful.

It was hard to wait months (since November) for answers for Job's declining sats and to wait weeks to know which surgery would be performed and to wait this week to have it scheduled. I was starting to be a little irritated with what I perceived as SCH's excessive slowness, to be honest.

But I was reminded today, as I spoke to the surgery scheduler who had to put me on hold and call me back several times, that I'm not privy to the hundreds of emails and consultations that are going on behind the scenes with his various providers. So many people have to be consulted to schedule even something as simple as his pre-op! It took a lot of little pieces to get this far because Job is so... Complicated.

I mention this mostly to remind myself, when I come back and reread this, how long some of these processes take. I suppose it's also an opportunity to practice what I preach to my children about patience. I tell them that, for the entirety of their lives they will always be waiting for something and it's how they wait that matters... But I guess I don't always exhibit that myself.

March 7th seems so far away. We were offered February 14th as a potential date but I declined that because it seemed a little to soon to plan for the big kids and because Job is still recovering from his cold. So patience again! And avoiding all germs!

I just love watching Frightful learn to manipulate his body. Look at that little leg! When he's not sick, he makes huge daily strides with his PT! It's really exciting.

And it's the sweetest thing to watch the big boys love on him.

Ten Months Old!!

We still don't have a date for Job's fistula surgery. We expect it to be next week but are waiting for the surgery schedulers to call. I'll probably call our cardiologist directly tomorrow if I still haven't heard. I want to know... But I also need to know, mostly so I can plan for Ezra and Isaac! And yet this is how I'm learning patience.

I did get a bunch of appointments on the calendar today for Job's neurodevelopmental assessments coming up around his first birthday... Which is soon, because:

Job is TEN MONTHS!?!?! today!

We got a whopping 8-9 inches of snow overnight, so this morning we bundled up and went out to play. Job loves being outside and I'm sure he noticed and appreciated the snow.

I guess I make him do silly things like get his snow suit on and pose in the snow with his brothers to pretend as if there's some level of normalcy in his life? I don't want him to be the only brother without a photo of him as a baby in the snow?

Early February Update

I have had a number of people ask me for an update today but I don't know that I have anything new to report, really.

We had an appointment of some sort almost every day this week! And next week we have even more appointments than we did this week! We're trying to check all the boxes on the rest of Job's care - immunizations, hematology follow up, ultrasounds of clots, our monthly Synagis injection (to fight potential RSV infection), our weekly therapies, GI follow up, etc.

Because of Job's weight gain, he has sufficient fat stores in his outer thighs and arms that we can now give his twice-daily lovenox (anticoagulant) injections in those spots too - not just his inner thighs! I'm so excited because his inner thighs are so very bruised and tender from his many injections. We alternate legs ("right at night") and I move around from groin to knee, trying to find a new spot each time, but I was running out of new places!

I keep expecting the surgery schedulers to call me to set up a date for his auxiliary fistula surgery (which we expect within two weeks), but they have yet to call. I called them this morning and might again on Monday.

THEY CHOSE FISTULA!!!