Post-Op Follow Up (With Details!)

Here's a photo from (a week ago) Friday's echo. I took it while we were waiting for the final ok from his cardiologist that the tech had indeed gotten all of the pictures needed.



An echocardiogram (ultrasound of the heart!) usually lasts about 40 minutes in a dark room (which is why they have a store of light up toys to play with!). Job wears EKG leads and I get to lay on the bed playing with him, but between the gel and the pressure of the probe he's not such a big fan of them. Although, he's such a cheerful and content little guy that even his cries of protest aren't too ridiculous. The hardest part of the echo is when they look at his aortic arch because I have to lift him up on the bed, at his neck, so they can get the probe up under his neck rolls. It's awkward and uncomfortable.

The results of the echo were essentially unchanged from his last echo before surgery, which is a good thing. We have been concerned that the increased volume of blood from his fistula would increase the leakiness of his valve, but there doesn't seem to be a change. This is such a relief!

Tricuspid valve regurgitation is Job's biggest cardiac problem at present. The Tricuspid valve runs between the right atrium and the right ventricle and is supposed to prevent blood flowing back, or leaking, into the atrium. Of course, Job only has one ventricle or pumping chamber and one atrium (as the separation between the right and left atrium was removed so his red and blood blood mix together in a "common atrium" now).

Because the less powerful right atrium is doing all the work (think lawnmower engine) of the non-existent left ventricle (think BMW engine) the stress of this extra work and his reconfigured physiology have enlarged the right ventricle and caused it to regurgitate blood back into the atrium each time the ventricle contracts. We have watched his leakiness increase to the point that it is now well in the moderate range and he will need the valve replaced (with a mechanical or pig or cow valve).

The hope is that we can wait to do this valve replacement until his third open heart surgery, the Fontan. Otherwise that's an extra OHS. And the hope is that he can wait until about 2 years old to get his Fontan (though usually it's done at about 3-4 years old). We at least need to get him to 18 months old or 11 kilos before he can get the Fontan. (Which is part of the stronger push to gain weight.)

His liver was a bit distended and his lungs sounded a bit wet so we upped his lasix quite a bit. He gets lasix twice a day and sprionolactone one a day for his diuretics, omeprazole and erythromycin for his digestion, enalapril twice a day for heart function, and aspirin once a day for anticoagulation. That's only 4 meds in the morning and 6 at night! I'm so thankful! And we hope to start working off his digestive meds soon.

Job's oxygen dependency is a problem, but we think the fistula did it's job and we're seeing gradual improvement. His sats are going up while his oxygen liters per minute (lpm) are going down. We don't have another cardiology appointment UNTIL MAY!!!?! and Dr. Files expects Job to be off oxygen by then. (I'm hoping for like July, but it's been established how skeptical I am of Job's ability to follow expectations.)

So we titrate between 1 lpm and .5 lpm currently, but we're also trying to up his caloric intake and slowly introduce fatty foods and his poor, weak digestive system needs the extra oxygen so it's slow going although there is improvement every week. He's also working really hard in PT and needs extra oxygen for the first few days as we're working on _____ new/more advanced skill. He's done multiple breaks off oxygen though, holding steady sat wise at 75-78% for a good 30-45 minutes! That is such an amazing difference from January where he'd desat to 50% within 3 minutes off oxygen!!

So! Too many technical details, but that's where he's at right now. I think I only updated last week that he's doing "decently well" which is too vague for me and I need my more detailed record written down because I can't keep it in my head with dependable accuracy!

Vocal Chord Paralysis Study

I actually thought that paralysis and dysphagia were more common than this study indicates. That's probably the definition of being caught up in your own experience.

This also makes me feel a bit better about our need to thicken Job's milk: "Dysphagia was found in 73.5% of Norwood and 69.2% of arch subjects who had documented VC paralysis."

And it makes me fall in love with SCH all the more because we have had great oto follow up as well as VFSS and PT care.

Vinh Pham, BS, Diana Connelly, RRT-NPS, CCRC, Julie L. Wei, MD, Kevin J Sykes, MPH, CCRC, and Jim O’Brien, MD2, "Vocal Cord Paralysis and Dysphagia after Aortic Arch Reconstruction and Norwood Procedure" ( 2014 Feb 10)

An Article on Transplants

Until we started the beginning conversations about a possible eventual transplant for Job and met a few transplant families, I definitely underestimated transplant complications. I thought this article was a good one to share: "A Transplant Is NOT a Cure"

Pain and Brokeness

One of my childhood friends' baby is currently in the Seattle Children's OR for her first open heart surgery.

Also, my best friend lost her fourth baby this morning.

Sometimes the pain and brokenness of this world feels overwhelming.

https://www.biblegateway.com/passage/…

Blog Link: Caths in the 80s and 90s

This is fascinating! And good perspective for me because we had one pretty destructive cath and one sort of destructive cath.

"Wildflowers With Warrior Blood"

Post-Op Eval (Brief Update)

Job had a long day at the hospital for a big post-op evaluation. I will write more about it later, but things are decently good.

We also got to visit with our friends who are currently inpatient and and marvel in person at how well Shirley is doing... though Job fell asleep on the couch while we were chatting.

The tape is on his cannula because he ripped off his stickers so many times this morning and I neglected to pack extra stickers in his bag.

Mid March Update

Job really enjoyed the sunshine and a visit to the park on Sunday! We're excited for spring and the ability to be outside a bit more!

We continue to titrate between .5 lpm and 1 lpm on his oxygen and have a big cardiology appointment this Friday.

Swimming and Hem Labs

Job got to go swimming for the first time in a pool today! And by "swim" I mean I held him and dipped his toes while his brothers swam.

We came up to Seattle Children's for our third attempt at a blood draw for his hematology panel regarding his hypercoagulation issue... But again it didn't happen. Actually the first two times he did get draws but the first time they didn't get enough volume for someone who has been on Lovenox as long as he has and the second time the sample thawed before it was transported to the lab at UW.

This has been so frustrating! But, in our eleven and counting months at SCH, they haven't even made one mistake, so to mess this up three times is very notable but perhaps tolerable. I really have been so impressed with the incredible quality of care and attention to detail at SCH - far, far beyond what I have ever experienced at any other organization. Maybe this is the exception that proves that rule?

ETA: I forgot to say why it's a big deal for Job to be in the water! Until about December Job HATED water!! He didn't touch any water until he was about three months old and then, when we tried to get him wet he screamed and screamed and screamed. I'm sure it didn't help that I just plopped him in the sink for his first time without any thought to it, like I would have one of my other kids, traumatizing him. But it's been long process of gradually reintroducing him to water in less threatening ways. OT gave us lots of helpful suggestions, but it's also just taken months. So, this felt like a triumph of hard work.

NICU Follow Up: "8" month appointment

We're hanging out in the neonatal follow up clinic today. It's through Mary Bridge as Job was in their NICU for 36 hours before he transferred.

It's been a good thing to see them every 4-6 months instead of Seattle Children's because they are so unaware of his cardiac issues that they are really just looking at his gross and fine motor development. Of course we give them updates on his cardiac stuff, but I realized today that there is a benefit of being in a separate system for this reason.

I am so deeply thankful for the various services Job receives. Today's OT consultation was phenomenally helpful! I guess I think of Job as my little research project sometimes and these consultations as my oral history interviews and find some measure of research satisfaction in studying his cardiac condition as well as all of this new-to-me childhood development information.

OT gave some great suggestions about ways to increase Job's intentional toy manipulation, about table foods that would be appropriate for him to begin, and dancing/large muscle movement ideas to begin to incorporate. Even though childhood development was never one of my interests, I am really enjoying learning more about the science behind this discipline. Certainly seeing the necessity and the effectiveness of it play out right in front of my eyes increases my appreciation of it. I want everyone to be in therapy! It's becoming my soapbox to other CHD parents because it has completely changed Job's life in such positive ways.

Job is certainly still working with a significant development delays, but he IS making progress. I have to remind myself that it's good for me to see his progress through his therapist's eyes sometimes, because I do get discouraged (like last night, filling out the surgery for today's appointment, being reminded how Job can't do many ~8 month old skills). It's slow progress, but it's definite and appropriate. It's progress.

Everywhere we go people stop us to smile at Job and talk about/with him. His sweet, cheerful spirit just shines through and people take notice. Why a little baby is on oxygen is also *quite* the conversational topic.

While we were waiting for our valet parking today I had a pretty lengthy conversation with 8-10 year old boy about how crazy it is a for someone to live with half of a heart. Again, it's good for me to have these conversations over and over and over again, lest I forget the wonder of Job surviving on such a compromised physiology.

Early March Update

This cutie is experiencing a lot more discomfort than was obvious originally. Actually, right after I posted triumphantly that he was off Tylenol I had to give him another dose. We are pushing him to go longer and longer without it, but he definitely still needs it.

It's nothing particularly concerning: of course he's sore! They sliced through a muscle he uses constantly! But it is sad. Sometimes I forget how sad because despite his pain, Job still smiles. And smiles brightly.

We are still really, really excited that Job is home already, but the exhaustion is setting in. All of the real life stuff that we paused for a few days is tumbling back in.

Last night after switching out Job's cannula for a new one, giving him all his meds, plus doing the regular baby stuff like a diaper change and food, I just collapsed into bed (and left Seth to put the big boys to bed by himself). Job is so much work, all of the time.

One thing that is extra exciting is that Job is off of his anticoagulant, Lovenox!!! He was on it last summer and then got switched to aspirin but after his multiple clots in September (including his atrial clot!!), we went to a very high dose of Lovenox. Giving him an injection twice a day has probably been our least favorite aspect of his care, so to be done with it is so wonderful. Perhaps even more (emotionally) painful than giving the shots was seeing his thighs and arms covered in dime shaped bruises. I will be so glad when they all fade! Job is now on a half-tablet of baby aspirin (which he'll be on for the rest of his life, though the dose will increase with his size).

The surgeons and anesthesiologist were determined not to place a central line for his surgery on Tuesday (as every clot as been line provoked) and they were successful! That did mean some extra work and extra pokes in the ICU to monitor Job in recovery without those lines, so I'm very grateful everyone was on board and willing to put up with a mere PIV.

Hematology is proceeding with a full work up, however, to determine why Job is hypercoaguable. I'm very curious! We have seemingly ruled out most genetic causes but will wait for the final results in coming weeks.

We're still keeping him pretty secluded for fear of germs and because he's pretty touched out. As we were writing about last week, he's going through a big developmental stage change and doesn't want people too close to him. I think today will be a come late and leave early kind of day at church. He is warming up to his brothers again and now that we're being more aware of what he needs, he is much happier.

We took the boys out on a fun excursion to the Naval Museum and to the Bug/Reptile Museum in Bremerton to pet snakes (!!?!!) yesterday. They can't stay cooped up as much as Job needs them to be. They had a blast! And they really have done so well adjusting their desires for what's best for Job. I wish it didn't need to be such an either/or choice, but such is life.

Job is also eating sooo much! I need to hurry up and make another big batch of sweet potatoes+carrots puree for him as that's his favorite meal 3-4x a day! As disappointing it is that we need to do so, thickening his bottles has really helped as well. He must be going through a growing spurt with how much he's eating but we have yet to see a big weight gain.

Sometimes it helps me to write out these big long posts so that I can see that not everything is dreary. We have much to be thankful for. It's hard to acknowledge that sometimes, as it's easier to focus on all the hard things, to wallow in our exhaustion and pain and fear. It definitely is a discipline to give thanks amidst hardship and It's one I need to exercise more often. It's just honest. Things are hard but God is good. That's not a trite Pollyanna-ism. It's true. And it's hopeful. Sustaining.

"There is strength within the sorrow.
There is beauty in our tears.
You meet us in our mourning,
With a love that casts out fear.
You are working in our waiting.
You are sanctifying us.
When beyong our understanding,
You're teaching us to trust."

https://youtu.be/B66DHRY1ITs

Surgery Week

March 6th

Drink up, Job! Only 9 hours to go before Job loses his eating/drinking privileges in preparation for surgery. Being NPO is always really hard, so we would appreciate specific prayers for Job's patience and stamina in the morning.



We came up to Seattle tonight to alleviate some driving-while-NPO time for Job as being stuck in the carseat while hungry is particularly difficult. But he'll have both Mommy's and Daddy's attention to distract him allll morning.

Iiiifffffff he doesn't start showing cold symptoms tonight or tomorrow morning. We had an exposure to a cold a few days ago and it seemed as if Job was coming down with something this morning - but was that just my paranoia?

All of today we called and emailed with Job's nurse and doctor and NP about whether or not to cancel and decided to keep him on the schedule as he had yet to show any symptoms. We'll cancel if we need to but aren't expecting to do so currently.

March 7th
Here's the mark for Job's incision! We're about head to the PACU!


Job is out of surgery. He's tired and hungry but doing well. His cardiologist says that as long as everything continues going well, he'll go home tomorrow! How strange for a kid who has made a habit of spending weeks in the hospital.

Thank you all for prayers. We praise our faithful God for all things as we continue to pray for Job's health and development.

These two were pretty glad to hear surgery went well! This was the photo Dad took in response to the photo we sent them of Job recovering. They're having a grand old time with Grandma and Grandpa!


March 8th
Hanging out in the hospital: https://youtu.be/1wrP4kVLj4g

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Job's welcome home committee:

March 10th

Guys, he's so happy!

We haven't had to use any Tylenol today and he's more active and more cheerful each day post-op.

He's even satting a bit higher! That's probably just because of the increased blood volume due to the fistula and not because his AVMs are dying off. But I'll take a number in the 80s any day.

WE'RE HOME!

I feel as if I'm in some fantastical dream and keep expecting to wake up and find out that, no, we're still in the hospital.

But we're not. Job's home. Happy. Eating so! much. Taking a bath. Playing with his brothers.

This feels like the very first time in his life that Job has "cooperated" and met expectations (or actually even exceeded them). I keep asking Seth if I'm forgetting about some other event in his life where he responded typically (or not catastrophically?), but we can't think of anything.



We found out that this was actually only the second auxiliary fistula done at Seattle Children's. We knew it was rare, but maybe not that rare! It's just that if kids develop pulmonary AVMs ("bad veins"), they usually do so two or three years after their Glenn (OHS #3) and are thus old enough to get the Fontan (OHS #3).

It was both amusing and frustrating to be in the ICU and field so many questions about why Job needed this surgery! The fellows and even some of the ICU attendings weren't familiar with this procedure and were very cautious both because of their unfamiliarity and because of Job's history, wanting to keep us in the hospital for another day or two.

But Job's surgeon and his team of cardiologists most familiar with him were thrilled with his post-op recovery and convinced everyone to let us go home. We feel very comfortable, now, assessing Job and are, unfortunately, quite familiar with his distress signs. Plus we have so many numbers to call if we are concerned, with such immediate responses, that it sometimes feels as if we essentially have a cardiologist living with us.

Fistula Coming Up!

Happy piano playing boy!

We are still on the schedule for Job's auxillary fistula on Tuesday, March 7th, at 12:15 (10:45 check in). It is so weird to go through the process of going to a scheduled, outpatient surgery! We've always been inpatient for previous surgeries, which is much different. We have pre-surgery appointments and phone calls and meds to give - all things that were just taken care of for us while inpatient.

I vaguely referenced a potentially happy development last week and meant to explain it further. It no longer applies, but to gloss over it rankles at my perfectionist record keeping tendencies and I know that, if I don't explain it, I'll be frustrated when, someday, I go back and reread these entries. So this is purely for me and totally unnecessary to read:

Last week's echo confirmed a reason that Job's sats haven't declined as sharply as we were all expecting (because of his pulmonary AVMs): aortopulmonary collateral arteries. These APCAs are small arteries that develop to take blood to the lungs to be reoxygenated.

We first learned about collateral arteries when Job's right leg clotted off back in May (because of his first cath). At the time we marveled that the body could form "side street" routes when the "freeway" artery was blocked off - but we had no idea we would be revisiting this concept again, but this time in his heart!

Job is certainly still very oxygen-dependent, but his sats have crept up rather than dropped dramatically, despite his AVMs. We all got a little excited last week when the possibility that maybe, just maybe, his body could cooperate just enough to at least postpone his auxilary. The idea was that these new collateral arteries could essentially create a natural fistula taking the liver-filtered blood from the IVC (connected to the aorta) though the collaterals to the lungs for both oxygenation and AVM killing.

We then scheduled an extra appointment on March 3rd to check whether or not these ACPAs were significant enough to postpone surgery or even cancel it altogether.

We talked about it leading up to Friday's appointment and then I talked to Dr. Files about it quite a bit at our appointment. We could postpone and maybe his body would surprise us! Though the risks of this fistula are minimal, comparatively, there are certainly risks.

Ultimately, I decided I want to go through with it. Being on oxygen once Job gets mobile but before he has the understanding/obedience to leave his cannula alone sounds awful. Like maybe even the straw that could break the proverbial camel's back of this delicate balancing act. This seems like it could work and I want to try it. Now. Job's not sick, we have a plan for childcare, our emotions are all keyed up. Especially because we would likely have to do this fistula anyway, just later on.

A Spokane Hotel Room

Wednesday and Thursday we took a quick little unexpected trip to go cheer on the Crusaders boys basketball team and meet up with Jim and Yvonne.

We had such a good time together, driving (and alternating song selections to sing along to), playing Isaac's favorite board game in our (heavily sanitized) hotel room, and playing in several parks. It was really sweet to spend some time together before next week.

Here comes the month of March!

Isaac is so proud of this photo he took! (It also shows off Job's trunk rotation!)

As this week has progressed, we have been reassured that Job's clingy, fussiness is at least mostly due to his developmental frustrations. He seems to be experiencing a great deal of separation anxiety and bit earlier and more intensely than our other kids. Job is also extremely irritated with his lack of mobility. We've been working especially hard this week on our various exercises and he seems to be even more motivated than usual.

I often say how grateful I am for the many people on Job's team, but this week in particular I am so thankful for his therapist through the birth to three program (essentially special education, through the school district). Janet comes to us twice a month and works with Job, particularly focusing on helping me help Job with his social/emotional/cognitive developmental needs. She's also a pediatric nurse at Mary Bridge and her dual qualifications have been a special blessing to us (like this week, when I begged her to assure me Job wasn't in fact sick! 😜)

So much of what babies do and communicate to show where they are at mentally and emotionally is expressed through their gross motor skills. So, especially at first, it was hard for me to differentiate between what we did with Janet and what we would do in PT - but the older Job gets, the more stark the difference is! Certainly there is overlap, but I am so glad we are receiving both services.

It was just so good to sit with someone and show her what Job was doing and how he was responding to what I was doing and to talk through strategies to help him cope with his frustrations. It's not even that what Janet suggested was new information, but I struggle to know when Job needs what, and her suggestions were so helpful.

He has been a much happier guy the rest of this week, but I'm getting a bit impatient for him to increase his mobility as I know that will make him even happier!