Friday Update (9/23/16)

From a cardiac and/or respiratory standpoint Job is doing much better. In fact, he's doing so well that perhaps we were a little too aggressive and made too many changes too quickly. This, coupled with restless sleep or little sleep seemingly sent Job into one of the most delirious episodes he's had yet.



When I first walked into the room earlier today Job looked absolutely awful. He was cross-eyed, but his eyes kept rolling back into his head. He couldn't stop flailing his arms and legs, but his movements were so uncoordinated. He was feverish too. It took about two hours to calm him down enough so he could sleep. But thankfully, he finally slept well and eventually woke up in a more lucid state of mind. Making sure he gets good sleep will be a priority this week.

We (I use that term quite loosely) backed off on some of his weans because he showed us he's not ready to be pushed so hard. He did well all day on hi-flow and off of CPAP (yay!!). But this evening right before the boys and I left the hospital (siblings are kicked out at 8pm) he randomly desatted in his sleep. He immediately got deep suctioned which didn't help like it usually does, so he got an x-ray to check his lungs.

We're very concerned about his atelectasis (lung collapse) and even though the x-ray looked good (no re-collapse) the decision was made to put him back on CPAP to be extraordinarily careful. He'll probably stay on it all night just to give his lungs the extra support of positive pressure to make sure they stay open and to relieve some of the work of breathing while he's still weak.

I talked to the NP about it for a while because it seemed like a disappointing step back, but we can't push Job too hard. This is how CICU life goes, after all: one step forward, two steps back. An additional factor in the decision was the high acuity on the floor tonight. Job will absolutely get the care he needs, I have no qualms about that! If his nurse were to call for someone they would be there immediately. But giving him the perhaps unnecessary extra support of CPAP tonight will just lessen a bit of the load on the floor tonight as Job will (at least presumably) need less assessment than he would if on hi-flow.

I want to make note of the special blessing it was this week that Job's primary cardiologist, Dr. Files, was the in-service cardiologist in the CICU this week. Of all the possible weeks for him to be on the floor every day, I am so grateful it was this week. He knows Job best (although really there isn't anyone involved with hearts at SCH who doesn't know Job very, very well) and he is such a very kind and wise man. He took special effort to direct Job's care this week and I will always be so grateful.

Likewise, it was simply wonderful that one of our favorite nurses, Alena, was Job's nurse Tuesday through today. She even picked up an extra shift today, surprising me! to care for Job an extra day. I had teased that I'd call a Code Pink (missing persons) on her if she ever left the hospital and then she showed up again today!

I can't think of another nurse who could have been a better fit for Job's/our needs this week. She advocated for Job and loved on him and answered my million questions. Thanks to her careful and oh so patient teaching, I feel like I might finally be starting to understand where Job is at and how some of his meds work. And she even made him a mobile! She had discovered that Job really likes watching the live cam of the Monterrey Bay Aquarium jelly fish tank and shark tank and so last night she found these sea life pictures and cut them out and hung them for him. Right now he's too weak and tired to play with them or look at them much, but it was such a sweet gesture on her part.

There are many such ways that we were specially cared for this week, but those two people were a particular blessing I don't think I've mentioned yet.

If Job has a good day tomorrow I might just left myself start to feel cautiously hopeful about his prognosis!

Thursday Update (9/22/16)

The page for today's updates in ascending order:

8:30am
Can we just forget everything else about Job's health, long-term and short-term, and celebrate this moment?? I could not be more thrilled right now.

Cath #3 Results (9/21/16)

A cardiac catheterization is the best diagnostic tool available to assess cardiac function and the pressures and flows within the heart. An echocardiogram (an ultrasound of the heart) is of course less invasive but can't always give the best assessment. The "cath" is called such because it entails a thin piece of tubing (the catheter) inserted into an artery or vein (in the groin or neck or, apparently, liver). A cath can also be interventional, allowing the ballooning or stenting of a narrowed artery or vein.

Job recieved a LPA (left pulmonary artery) stent (which was described as a loop of fancy chicken wire). This will keep his LPA from further narrowing and it was placed where the green circle is. It's hard to see the narrowing in this particular picture but it was significantly inhibiting the LPA flow. The tissue around the stent will quickly grow around it, keeping it in place, and it won't grow with Job, so it will eventually need dilated (or replaced in a future open heart surgery, such as his Fontan).

Because Job is always one for fun extra surprises, he also had a clot where the purple circle is. It was dispersed with heparin and he will shortly begin a lovenox regimen (and we're old pros at lovenox injections!).

And because we're often asked, the orange circle highlights one of his sternotomy wires, keeping his cracked sternum closed.

Miraculously, Job's aortic arch repair did not need repaired as seemed likely. I think we're still in shock, actually. This is such wonderful news!

Job is not satting as high as we would like, even after this intervention, but it bought him a few days to get his sats up. His heart function likewise needs to improve, but again, he can have a few more days to accomplish that as well.

Surprisingly, Job was extubated in the OR and is on the BiPap at present. Reintubation is still a possibility but if he has a good night then that possibility diminishes. We want him to ween to CPAP then hi-flow then nasal cannula then no respiratory support but, because this is Job we're talking about, it will take some time.

Tomorrow Job will get another ultrasound to look at his diaphragm plication which is possibly coming undone. If this is the case he would get to have surgery tomorrow to fix it, but it is extremely rare for this to be necessary (but again, this is Job... He never takes the easy path).

Unfortunately his plication (which was necessary because of his nerve damage sustained in the Norwood) makes his ability to take deep, full and effective breathes more difficult at present. Around the age of two his thoracic/intercostal musculature will be strong enough that the plication will no longer be such a factor, but it very much inhibits him now. If his phrenic nerve was going to heal, it would have healed by now. So the left side of his diaphragm won't ever be able to move (even if it hasn't been plicated, or stapled down).

Job has yet to fully come out of his anesthesia and is pretty well sedated and will be through the night.

I'm not entirely sure what his goals will be for tomorrow. I think it will largely be a day of rest, to hold steady on the small forward steps he he has made and to assess his goals for the rest of the week.

We are so very relieved that Job has seemingly turned a corner. He was so very sick and had us all extremely worried through Monday. His progress has been tentative but to even use the word "progress" is thrilling. He certainly has a long road ahead of him, but we've done long hospital stays before and we can do one again.

Wednesday Morning Update (9/21/26)

This morning Job was awake, looking at us and listening to us!!! For 15ish minutes! But it wore him out and he's back asleep now.

This means his delirium is largely gone and his sedation balance is being better managed (that sounds like an indictment of his team, but it's not! It's just a hard balance to find).

Job got a NG tube on Sunday and has intermittently taken feeds of 5mL/hour as he's been able to digest them. He's still on his D5 (basically sugars) drip but since things didn't go as expected and he's still sedated and ventilated he can't feed by bottle. And his team didn't want his digestive system to go any longer without being reintroduced to the concept of food. He hasn't always been able to digest the food (depending on how sedated he is) so he's gotten several breaks, but he's done pretty well. But he's NPO (no food by mouth) as of 6am because of his cath today.

He just got his daily x-ray and it showed improvement in his lungs, so we're headed in the right direction there. They need to expand and continue to dry out but should as time passes.

We'll likely spend the morning talking to his team and signing consents and then we get to wait (not anxiously?) for the several hours during his cath this afternoon. Just like during his last cath, we're so desperate for the results of the cath that it's easier to send him off to the cath lab than it might usually be.

We wanted his Glenn to go well! We didn't want to be in the situation, waiting and wondering, but it will be so good to get some answers today.

Seth's Update (9/20/16)

Here are a few quick bullet points about Job's biggest issues/prayer needs:

-The squeeze of his heart (aka "heart function") has not been very strong since the surgery. It seems like the doctors don't exactly know why.

-His Aortic Arch, according to recent echo images, appears to be even more narrow than before the surgery, despite the surgeon's attempts to repair/widen it. If (or when) they do a cardiac catheterization, it will be for the purpose of once again widening his arch.

-His sedation needs, due to his intense pain, must be balanced with his need to breathe deeply on his own. According to Dr. Files, the team must do a better job of managing this balance the next time they attempt a successful extubation.

-His pain and discomfort are causing him to have episodes of severe oxygen desaturation, hence the need for so much sedation/pain meds.

-Autumn and I are pretty shaken up at this point, but Job needs us to be focused, hopeful, worshipful and ready for the day.

In Christ's name, Amen

Update (9/20/16)

Job had a pretty good day today. He is less delirious, more consolable, presumably because his pain is becoming a little more manageable. His sedation/pain medications have been weaned a bit (albeit from very high amounts yesterday). He seemed to have a more peaceful day. He even opened his eyes and looked at us for a few seconds (as opposed to his eyes being rolled back whenever open).

Today's echo images confirmed the narrowing in his aortic arch and his need for a cardiac cath. There are some significant "access" issues, as his right leg is totally blocked by a clot and his left leg is mostly blocked (complications from previous caths). At this point, their first choice of access is through the liver. It will be both exploratory and possibly interventional, perhaps resulting in a balloon or a stent in the arch. We are cautiously hopeful about this procedure. It's a major intervention, filled with risks but with significant potential benefits. He is scheduled for second case tomorrow, which means around 1 pm.

His depressed heart function is still a concern, but could be caused, at least in part, by the narrowed arch. We'll see.

We know that many of you have been praying all day, particularly for our peace and strength. Thank you so much. God's kindnesses have been apparent to us too many times to enumerate.

Today was a small but definitive step in the right direction. Praise God.

-Seth

A Sad Day (9/19/16)

I don't even know where to begin this update or how much to say.

Job is not doing very well. There's probably no other way to say it. But there is still so very much support that can be offered to him to help his body as he struggles. He's not in immediate danger.

We need to give him more time, absolutely. But we're getting to the point where we need to start exploring what is going on with his little body because he is not responding as he should be.

Most Glenn patients extubate in the OR or shortly thereafter and extubate to minimal respiratory support. Job is three days post-op and is ventilated and on a great deal of respiratory support. Why?

We're hopeful that he just needs more time to recover from surgery and his heart function will continue to improve and that he will get off the vent soon and his sats will improve. It's possible that time is all he needs. But the longer he takes the more concerned everyone gets and soon we need to explore what else it could be.

Job's Sunday morning echo might have shown problems with his aortic arch repair. He's getting another echo tomorrow morning and the results of the echo will probably tell us a lot about what the rest of this week will look like. A cardiac cath is a very likely possibility in the next few days to further explore Job's heart function if he doesn't improve in the next 24ish hours.

Job did extubate today but after about an hour needed reintubation. We think that his extubation failure was mostly a sedation problem rather than a heart problem or a lung problem. Job is in so much pain from his headaches and so uncomfortable from the breathing tube that he needs a lot of sedation. But he needs to be "awake" in order to extubate and breathe on his own. But he's so delirious from the balancing of his pain meds and sedation that he can't wake up. It's a hard balance to find. So he's off propful again and on dilaudid and we'll try again possibly tomorrow.

Job's left lung collapsed during extubation, likely because of the pressure change from invasive-ventilation (what he's had - the nasal breathing tube) to non-invastive ventilation (no breathing tube but very high support, higher even than hi-flow).

During his brief extubation!

While extubated Job had some secretions and he wasn't awake enough to cough them out, even when they suctioned, because his sedation was so deep (but again, it needs to be deep because he is so incredibly irritated and therefore agitated when he wakes up). If he had been able to cough, he probably could have kept his lung from collapsing and therefore from reintubation.

While reintubating, he got "bagged" and this popped open the top of his left lung. Overnight we'll diurese the bottom of his lung open in addition to his ventilator setting tailored to give him extra pressure support to pop it open.

The boys got to come visit today, which was really, really nice. They did not want to go to co-op this morning but did such an amazing job of being cheerful and obedient and joyfully attentive once there. I thought I couldn't be more proud of them or more grateful, but then they got to the hospital and had to spend three hours sitting in the back of Job's room basically alone, drawing and watching a TV show while I tried to help Job calm down during and after his extubation trial. That wasn't the plan for the day at all! We were going to play together and talk to Job! But they instead sacrificed their desires and served their baby brother and I.

When Job calmed down (well, was heavily sedated and muscle relaxed, following intubation) and Daddy came, they got to talk to him and it just amazes me how comfortable they are looking at his poor disfigured, swollen, scarred body. They love him so very much and are so thrilled to see him, every time. I don't know how, but they just don't even notice his incision or tubes and wires. In fact, they even call him "the cutest baby". It's hard enough for me to look at Job right now and it's such a blessing to me to watch them interact with him here post-op so fearlessly and with such adoration. I have been so concerned about how they would do through this trial over the last seven months but they have been so resilient - it is such an answer to prayer. They help me so much physically and emotionally.

All five of us!

Please keep praying for us. People remark on our strength often but we have nothing in and of ourselves. Your prayers are, through God's strength, sustaining us. We feel very weak right now. This is scary and sad. It's going to be hard waiting for answers these next few days.