Day 13 Post-op Updates

3:00pm update:
Job is now completely off of milrinone, which is the last med he needed to wean off of in order to move down to the floor. No more IV meds!! Woo hoo!

SLP came by and officially cleared Job to now eat purees. Just 1oz at a time, three times a day. Job's work of breathing definitely increases when he eats, so we have to go slowly, giving little bites. But he couldn't be more thrilled.

PT and OT came by and I gave them Job's medical history and therapy history and some of my concerns and goals for him. They will alternate days from now on until we discharge. Even though I cognitively understand that Job just came through a major trauma, the fact that my previously active toddler can't even sit up on his own yet two whole weeks later is rather hard emotionally. Actually, everything is still hard emotionally despite how well Job is doing.

He's starting a new med because his blood pressures are too high. He's stable on his anticoagulants but not on his immunosuppression meds.

His lungs are clearing up and he's coughing less and he's down to only 2 LPM of 100% O2, so no positive pressure from hi-flo, just the equivilent of wall oxygen (though through the hi-flo machine in case we need to go back on hi-flo). He only pulled out his NG tube once today, so we only had to endure one traumatic reinsertion.

It seems like every bit of his body is marked up in some way, from some needle or scalpel or pressure wound or adhesive or infiltration. The back of his head now has two bald spots, one of which looks red and sore, so we added those on to his list of skin areas to monitor.

He got two good naps today, which were sorely needed (in addition to the good night of sleep). Job has been so very sleep deprived because he just can't get comfortable.

7:00am update:
Last night Job slept better than he has all week (since extubation and sedation wean and coughing began)! So he was pretty interactive this morning, though he still gets really tired quickly.

We're seeing stamina very slowly increase, however. On Monday he couldn't hold his head up at all, but today he held his head up for about 20 seconds on his own when I was holding him up to sit up. He also has let me hold him up to sit for longer and longer periods of time.

Day 12 Post-op Updates

5:00pm update:
Highlights from today: Job got to play with extra sensors and leads and put them on his monkey. He watched Winnie the Pooh. And he proudly triumphed after pulling out his NG tube and hi-flo cannula on multiple occasions.

Lowlights from yesterday and today: he has a cold virus. Very frustrating and disappointing. We'll be on viral precaution for several days (which means his providers have to gown up before they enter the room and his brothers can't come visit), and that is definitely going to slow his post-op recovery.

He is not very symptomatic, just a wet cough. But he has de-satted to the mid-80s, probably both because of the virus and fluid in his lungs that is still there from surgery (aka pulmonary adema). They put him back on hi-flo because of the de-satting, and his saturations are back in the mid to high 90s on 4 LPM of hi-flo.  He probably won't leave the CICU till he is off oxygen support again.

He continues to wean down milronone, which is the last medication they must discontinue before he can leave the CICU.


Overall, things are still on track and he is doing well. This virus is just a bump in the road, Lord willing.

12:54pm update:
Job's doing really well on 4 LPM of hi-flo now. And it's taped in a way that is bothering him less.

9:09am update:
Wet cough, satting in low 90s during breaks from hi-flo. X-ray was fine. Dr. Kemna said echo was very good. Still off insulin. Last day on his milrinone wean. Hates his cannula.

2:13am update:
They tried to lower his hi-flo to 4LPM (from 8) and he de-satted to the mid-80s. I guess he really does have a virus. 😞

Day 11 Post-op Updates

3:17pm update:

Job is back on hi-flow. 8 liters per minute, which is a lot of support.

I'm crushed.

The ICU is the best place for him. He's ok. But this is going to get worse before it gets better and we're going to be here a lot longer.

Why is every step of this journey extra hard for Job? Complications are constant.

1:15pm update:

SLP came by and I gave the very long history of Job's vocal cord paralysis and diaphragm plication and oxygen requirements and thickened feeds and crying leading to breath holding leading to coughing leading to vomiting... and then she assessed him and will start coming by every day to work on feeding and work on his expressive language while we're here inpatient.

I'm so excited to have intervention in this area because it's been a lingering concern that has been pushed to the side this summer especially while we focused on his failing heart.

11:21am update:

Job is desatting occasionally to the low 90s. He's still coughing. He's off insulin and no longer getting his blood checked every hour (just once a day!!). He's down on milrinone.

He's much much more himself today, though very low energy. He's not sleeping well because he can't get comfortable with his lines and because of his cough and because the steroids he's on for his transplant (TX) make it hard to sleep too. It's so so fun to see him interact with us again, though they're "just" little smiles. He whispered/mouthed "Dad" and "Mom" and has signed "please" a few times, but it probably hurts him to talk much.

Whhhyyyy is he coughing, though? He swabbed positive for rhino but the thought is that that might be a remnant of the cold he had the week before transplant. He's definitely not managing his secretions well. SLP will come by to assess later today.

Day 10 Post-op Updates

3:29pm update:
I spoke to Dr. Law for a while about Job's cough and went over Job's long history of left vocal chord paralysis and his various swallow studies and his propensity to vomit when agitated. The thought continues to be that this is not a cold but something physiological. Is his NG tube irritating the back of his throat? Is it soreness from the ventilator? Is it difficulty passing saliva through his oral larynx? It sounds as if Job is clearing his throat when he coughs rather than something from his lungs. His x-ray is clear and he continues to be asymptomatic, so hopefully Speech will have some answers for us tomorrow.

In addition to starting Speech Therapy, I also asked that we start Physical Therapy and Occupational Therapy tomorrow. Obviously we'll go very slow, but I don't want to wait until Job is ready and then wait a few days more for the consults to go through.

Dr. Law also answered my questions about Job's left lung and left pulmonary artery (LPA). Even 10 days post-op, they haven't gotten a good picture of the flow through the LPA and the assumption is that it is indeed stenotic (narrowed). Job's LPA was stented two years ago and during the transplant itself the LPA replacement and new connection took a great deal of time, so we've all known there could be additional issues. If there is stenosis, then a stent will likely need placed again.

If this LPA stenosis was Job's only concern, it wouldn't be an issue. Apparently, it's not uncommon to transplant a heart with only one functioning lung! But because Job has had many other complications in his life, this LPA stenosis will probably mean a slower recovery for him.

10:00am update:
Rounds were pretty boring today! The main question is what to do about Job's blood sugars and whether or not his cough is because of a virus. Both questions can only be answered with further observation.

I've received many questions about our ability to connect with the donor family. As I understand it, we cannot receive any information about the family from the hospital in order to protect their privacy. We were told that the heart Job received was small and that it was on ice for 3.5 hours, but they can't even tell us some of the medical details about the heart (like what markers for disease or infection it has). We get to trust the team here at SCH that it was the best fit for Job.

One year post transplant (October 18, 2019) we can send a letter to the donor family through LifeCenter Northwest, our regional organ donation procurement organization. The family is then notified they have a letter and they can choose to accept it or wait to accept it. LifeCenterNW facilitates these first rounds of communication, which is kept mostly anonymous, until both families consent to sharing contact information. We'll be allowed to share that we're in Washington state, but not which city, and our first names, but not last names, and so on.

Some families choose to meet and stay in touch and other families choose not to - it will really be up to the donor family.

8:00am update:
Job rolls around in his bed, on top of all his tubes and wires. He likes laying sideways and holding on to the crib "slats".

He was really happy to see me when I woke up and smiled at me and interacted with me a little bit as I showed him some of his dog toys. Then he got tired out and covered his face with his hands and turned his head away from me.

I was expecting him to be tired... but probably after 10 minutes of activity, not 2 minutes. I'm trying so hard to not have expectations but it seems impossible to let go of them completely. So then I try to hold my expectations loosely... but that's hard too.

When he was in surgery and during those first days post-op it was easier to just be grateful he was even alive. Now, perhaps, I'm taking that for granted and focusing too much on the next step and how soon we can get there. I have a lot of quiet time sitting in this hospital room, by Job, so I've had lots of time to think about how deeply discontent I am and how deeply I want control.

I've always known these things about myself, but Job's medical issues give me ample opportunity to grapple with the reality of how pervasive my desire to orchestrate everything really is and how very impatient I can be.

Day 9 Post-op Updates

9:00pm update:

Job had a really, really good day today.

We took off his hi-flow cannula right after rounds and he never even skipped a beat today, breathing like a champ. No problem whatsoever.

He had a number of moments throughout the day where he was alert. I even got him interested in a book enough to turn two pages himself. We watched some movies and talked a lot and pointed to our various body parts, but every activity is exhausting and needed a nap afterwards.

Pooping is still a cause of agitation for him but he's almost regular again and after making a dirty diaper he was like a whole new kid for a few hours. He was even pretty happy sometimes. I got a few half smiles.

He went back on insulin for a bit but it's off again. Every time he gets one of his steroids he spikes and then we chase it but I think it's happened enough times that they're comfortable saying it's the steroids. Most if not all of his immunosuppression meds can affect his blood sugar but they don't anticipate us needing to check his sugars at home.

His feeds are going well and they allowed him to begin drinking clear liquids. He nicely, if lethargically, signs "please" for his water.

He threw up twice but why is hard to say. I think it's likely related to his constipation and/or a dilaudid wean side effect.

His cough got better through the day. When he was calm and sitting up he could go a few hours without coughing. It's still very dry and he has no other symptoms, so we're all hoping it's merely an effect of hi-flow.

He got a bath and new clothes and smells so fresh and clean and looks so much better.

11:00am update:
Cuddles!

Job is off of even more support today. It's amazing! No epi, no insulin, no milrinone, NO RESPIRATORY SUPPORT. We'll see if he can stay off of all of it, but it's exciting to even try.

He started coughing last night. We're hoping that coming off of hi-flow helps, because it was drying him out (it's essentially like a constant gust of wind in his nose). If he doesn't stop coughing by this afternoon we'll have to do a viral panel and figure out if this is a cold.

He is really restless and grumpy still.

He is super shakey and just threw up, probably narcotic withdrawals.

Day 8 Post-op Updates

9:06pm update:
Job had a good nap while Grandma sang and read to him and Seth and I took the boys swimming and for pizza at the Ronald McDonald House (those on the waiting list for housing, like we are, can now go in with a day pass and eat meals and utilize the facilities).

But now that his brothers are gone he's gotten really agitated again. I think because he's gassy again? So we're watching the World Series and trying to cuddle him to sleep.

3:00pm update:
Seth brought Ezra and Isaac up to the hospital and I was apprehensive about them visiting while Job was so fussy... but he actually calmed down quite a bit when they walked into the room.

Then Daddy held him while I took the boys to go play and the suppositories and Miralax finally started working. I'm shocked how much more comfortable Job is now that his bowels are moving! He's practically a different kid.

12:00pm update:
Grumpy boy got his chest tube out. And is gassy and mad he's gassy.

He's been thrashing around *on* me!

10:00am updates:
Rounds were exciting this morning! We DCed a bunch of orders and meds and are switching several meds from IV to oral. He gets his chest tube out soon. I will get to hold him. His brothers and Daddy called and told him all about the history of weapons. His restraints came off.

7:00am update:
Job is the saddest little boy there ever was this morning. He can't get comfortable and keeps looking at me and mouthing protests. His voice is horse from being intubated so he can't make sounds and his hands are tied up in restraints so he doesn't yank out all of his IVs or cannula. So he's unable to communicate, still waking up from 7 days of sedation, uncomfortable from his chest tube, constipated and gassy, and overall confused and irritated and tired.

He looks really really good, though. He's so much less puffy and his skin tone is better-than-his-old-normal and his incision and (4!) line infiltrates look really really good.

We just have to get over this hunp.

He had a hard night, discomfort wise (and starting off the night with a 3 poke traumatic IV insertion didn't help).

Day 7 Post-op Updates

5:57pm update:
Job is finally asleep!

The head of the transplant team came by and told me he "couldn't be more pleased" with how Job is responding to his immunosuppressants - he's already starting to show stable levels - and that his heart looked really really good on echo this morning.

Next is getting Job's bowels moving because he's so gassy and constipated that he's really uncomfortable.

And the next thing that needs to come out is his chest tube because that will make him feel so much more comfortable and allow me to hold him.

3:00pm update:
Job is consolable but can't settle down long term and take a good nap.

He got his pacing wires out!! He's starting to look naked!

1:00pm update:
Tube is out! Satting 98 with no increased work of breathing.

Calming down but still upset and breath holding sometimes.

Today will be all about making sure he can stay extubated.

10:50am update:
Extubation is set for noon!

His Extubation Readiness Trial (ERT)s yesterday and this morning went well but Job has a plicated diaphragm, compromised lung vessels (because of 2.5 years of oxygen depravation), and pulmonary AVMs working against him as well as the fact that extubation necessitates cutting his narcotics in half so he wakes up to cough and breathe on his own. At two years old this is going to be confusing and scary and infuriating.

Hi-flow is the machine to the right and his vent settings are to the right:

8:30am update:
Boring night = great night!

 I sleep so well here at the hospital. I think I'm just really tired when I lay down?

And then I sit up and see these glorious fall colors out my window:

Day 6 Post-op Updates

We're approaching one week, tomorrow, post-op. I can't believe it's been a week today since we got the call that an offer was accepted!

9:09pm update:
Job recovered nicely from his PICC placement. He got a "bath" and total line change. I had to take a picture because it's so amazing:

3:00pm update:
Job is in the OR with Interventional Radiology (IR) getting his PICC line in. It's so wonderful that they were able to fit him in to a cancellation spot today while he is still on the ventilator because if they didn't have that cancellation he would have had to wait to get his PICC tomorrow or the next day and then would have had to be reintubated for the procedure and then exubated again.

His room looks so empty but I'm going to try to get a nap in before he comes back.

1:00pm update:
Fantastic news! There is no fistula!

There is only a clot in the vessels in his left calf. Where did it come from and when? They're not sure (yet?), but he's already on heparin (an anticoagulant) because that's standard post-op care so he's ok for now. They will continue to watch closely but he is getting blood to his foot, just more slowly than his right foot is being profused.

We'll eventually transition to lovenox injections twice a day at home. I hated giving him shots before but I know I can do it again.

9:50am update:
Rounds were hopeful! "Job is hemodynamically stable and we are working towards extubation." Hooray!

We're working on decreasing meds to be able to extubate. The thing that might hold us up is when the IR team can place a PICC line, because usually that does necessitate being on the ventilator. If they're really busy today and can't place the line then Job may need to stay on the ventilator until they are able to place the line.

And, of course, he has to be able to pass his ERT (Extubation Readiness Trial), showing us that he can breathe (some pressure support allowed) well enough without the ventilator.

His feeds were stopped because he's constipated, so they're starting a constipation bowel regimen of glycerin suppositories and Miralax.

His left leg is noticeably cooler than his right leg so there's concern he has a fistula (resulting from so many pokes from his caths) and ultrasound is on their way up to assess. If he does indeed have a fistula (as shown in last night's ultrasound) then he would need that fistula connection repaired in the OR next week. We don't want his blood mixing in his leg (though last year we needed that in his arm).

Day 5 Post-op Updates

7:52pm update:
Grandma brought Ezra and Isaac up for a visit and hung out with Job while we hung out with Ezra and Isaac.

Job went back on his epi and back on insulin, but feeds are going well and overall he's doing well.

1:17pm update:
Feeds started!

12:00pm update:
Everyone who comes in or walks by comments on Job's crossed legs:

10:30am update:

Yay yay yay! I'm so happy!

He just looks so much better. His swelling is way down, he's pink. He got new tapes and new dressings and new bedding, so that always helps him look better too even though it's purely superficial.

Job had a great night, after two pretty good days of calm recovery. I think the exact phrase was "Job is doing well and we can now move forward." Yay!

He is getting his epi turned off, his insulin turned off, his replogle (suction from his stomach) tube out, his vent settings turned down and his Notric Oxide turned down. Yay! And he's getting a NG tube placed to begin feeds, his lasix gets turned on again, and he starts one of his primary immunosuppression meds that he'll be on at home. Yay!

So much progress!

If he acclimates to all of these changes well then it's possible he could extubate tomorrow or the next day, which will make him so comfortable and allow them to lift his sedation so he can be awake! The other thing that will make him very comfortable is getting his chest tube drainage tube out in a couple days.

Day 4 Post-op Updates

Here are the updates for Day 4 Post-op! (10/22/18)

5:53pm update:
Magical words! "Job is doing quite well today." Yay yay yay!

Very few changes were made; the focus was on keeping him comfortable and stable.

The Benadryl he gets before Thymoglobulin helped him get a good nap this afternoon.

He's uncomfortable when he wakes up but is consolable.

10:28am update:
Job is waking up a bit and it a lot of pain and is scared, so today is going to be more of the same: trying to comfort him as best we can.

Yesterday wasn't very "bad" in that he was pretty calm all day, so today is probably actually the day I've been dreading as far as writhing goes. It's such a delicate balance to have him comfortable but not too comfortable.

They won't make any changes to day and just observe to make sure he's stable. If he is, then tomorrow we could try to make progress getting off various supports.

Seth entertained and distracted Job this morning with reports on the various NFL and collegiate games played this weekend:

Day 3 Post-op Updates

Here are the updates for Day 3 Post-op! (10/21/18)

8:16pm update:
I just got to listen to Job's new heart beat. I can't believe he has a new heart. I can't believe he's still alive.

7:40pm update:
Job had a pretty quiet day. He's woken up a little bit here and there and thrashed a bit but calmed down pretty quickly when we talk to him and rubbed his arms and legs and head. He got a PRN of dilaudid (an extra dose of narcotics) which helped during the worst of his pain. He got a dose of Benadryl before his dose of immunosuppressants so that helped him take a good afternoon nap. He's still too hot unless the cooling blanket is running. He was on huge doses of two different kinds of steroids which made him hypoglycemic, so they stopped one of the steroids and put him on insulin. It's a typical problem with the steroids, so he should be off the insulin soon.

1:37pm update:
A slight position change to the right side send Job's HR and BP down again, necessitating pacing for a bit. He recovered quickly but it's frustrating to the whole team that they don't know why this is happening.

10:56am update:
Vec is off, so Job's wrist restraints are on. His narcotics are upped to try to keep him comfortable, but he hates his mouth tube from the ventilator so he'll probably be uncomfortable and keep trying to get the tube out (thus restraints). I'm going to cuddle him as best I can and not be brokenhearted as he writhes. Maybe.

8:44am update:

I am guessing today is going to be a hard day as we try to wean Job's muscle relaxant, which has been keeping him paralyzed so that his body can recover from surgery. But he can't initiate his own breaths or cough while on this drug, so we can't make any progress towards extubation until we get off of it.

I am anticipating him being very uncomfortable and needing a lot of comforting.

When Job has been turned from his back on his side, especially his right side, (necessary to prevent bed sores) his HR and BP have dropped. He needed paced briefly twice overnight. So that instability could be the roadblock preventing other progress today.

He's peeing really well so they will probably yet again take off his diuretic.

Day 2 Post-op Updates

Here are the updates for Day 2 Post-op! (10/20/18)

9:36pm update:
We're keeping Job on his muscle relaxant tonight to keep him calm so that he can settle into all of the changes that happened today.

3:50pm update:
Today has been a good day. Job has made lots of very small gains. His BP is good, even being on much less support, his HR is good, but they want to keep pacing him to maintain good function and profusion. He's peeing so we're starting to see progress in his fluid balance. Yay!

I will probably never stop saying this, but it is SO weird that Job has four chambers and that HIS left ventricle is doing well. But apparently that's pretty common because the left ventricle is such a powerful workhorse and can just get started and pump well.

But the right ventricle, which is now pumping blood JUST to the lungs to be oxygenated, is fighting hard against the high pressures in the lung arteries. These high pressures are because Job's lung vessels have been compromised from his past surgeries, and particularly his Glenn anatomy (which is very stressful to the lungs). This is why sildenafil was always so helpful: it dilated (made bigger) his vessels to allow for greater blood flow to allow for greater oxygenation. There wasn't anything "wrong" with his lungs originally, but over the last 2.5 years they have been so adversely affected that they are providing extra resistance to for his right ventricle right now.

So this new right ventricle is working extra hard to overcome this resistance. It is currently being assisted by nitric oxide, which is a vasodilator that does not lower blood pressure (like sildenafil did). We want Job's lungs to get healthier so his right ventricle can get healthier.

Also, the heart Job received was pretty small and so we're asking it to work extra hard right now to accommodate the needs of a larger body. It will apparently learn to do this, but needs some time to acclimate, which is another factor as to why we're seeing those lower numbers.

1:07pm update:
Job's now being paced at 150 and started a very low dose of diuretics to draw off some of his extra fluid. They'll talk at afternoon rounds about taking off his muscle relaxer and letting him move if all continues to go well.

They did a second echo but it's really hard to see what they need to see regarding heart function, especially on the right ventricle that they're watching most closely.

10:17am update:
I was asking our nurse and the sonographer lots of questions and I have to write down some of what they said, otherwise I won't fully process it.

I knew that we wouldn't be talking about extubation soon but I was wondering about what needed to happen first, before we could talk about it. Job has always needed a lot of respiratory support after other surgeries (and that whole 11 months on oxygen thing), so our other post-op courses have been often focused on trialing off the vent and so on. I think of it as this long, excruciating process in and of itself because it has been for Job.

But actually, this time around he's not on much respiratory support. We're medically suppressing his ability to breathe on his own, of course, because we're trying to take away as much work as we can to let him heal from surgery. But he really doesn't need the vent for respiratory support right now.

What he needs, though, is all of this vasopresser and pacing support, and while working on those issues, it's really important to have a tube as a precaution in case of an emergency.

I think that is SO cool and it's so different than what we've done before. I absolutely just assumed that he was on high amounts of vent support still, like he was Thursday night after coming out of the OR. I hadn't bothered to look at the interface or ask, but they weaned down right away.

So eventually whenever it is time to extubate, there won't be days of weaning down on support. Now, I've already heard from I think 5 different people that extubating a 2 year old is the hardest age to extubate. So it won't be easy. I think #becausetoddler has been my mental hashtag for a lot of the things in Job's life these past six months (for example, is he eating less because he's in cardiac decline or #becausetoddler?) so it's kind of funny to me that even when in the ICU this is still an issue. I mean, why did I think it wouldn't be?

Then also, the sonographer said that, as they use sound for ultrasounds (and the echo is just an ultrasound of the heart), it's especially difficult to echo someone after heart transplant because sound can't travel through air and, post-op, there's a lot of extra air in the chest cavity. So sometimes echos really can't show very much, post-op, but that sometimes even just 12 or 24 hours later, they can get a really clear image. It just depends on the kid.

9:38am update:
Rounds are my favorite part of the day. I, of course, want to know the plan, but I also learn so so much as the fellow and attending interact and all the other departments weigh in.

Basically, we're going to try to peel off support today, as Job allows. The priority is maintaining his blood pressure and making sure his heart rate is in sinus rhythm and gradually decreasing. There's some concern of acute kidney injury but that will be assessed through the day. His neck wound looks pretty good but needs assessed through the day. His temperature needs to stay down. He may get a trial off the muscle relaxant. It's highly unlikely extubation will happen today or tomorrow because he's still on such high hemodynamic support.

8:45am update:

I tried to get a picture of Job's room but it's hard to capture all of the things around his bed. I took this photo standing by the nurse's computer.

I'm spending much of my time sitting beside him, under those two horizontal bars you can see on the wall in the above picture.

The foot of his bed is pointing out to the hallway and to the "office" where the attendings sit (well, when they get to sit). Since Job is so critical, he gets prime real estate right under everyone's nose. When he eventually improves he'll move rooms, closer and closer to the end of the unit, away from the attendings.

The head of his bed is pointing out to the window, which has a beautiful view of fall foliage (that just makes me so happy every time I see it) and Lake Washington and Mt. Rainier (if the fog were to lift). 

Under the window is a couch that turns into a bed for parents to sleep on. There's a TV and a cabinet for us to use as well. We can't eat in the room or use the bathroom, but there's a parent lounge and bathrooms with showers at the end of the unit for our use. So it's a pretty comfortable place to stay, actually. Seth and I have always joked that it's like staying in some fancy Seattle hotel with a gorgeous view.

I love that it's so close to Job's bed. You can see it's just 10 or so feet from his head:

So there you go! Second only to the question "how's Job doing?" has been the question of what his room is like/our accommodations, so these photos are to answer that question.

6:30am update:

Job had a relatively boring night. He started to wake up from his sedation around 3am but was uncomfortable so they put back under. He made a lot of progress weaning down off his vasopressers but then when they tried to pace him lower his blood pressure dropped. So he's back up on his pressers (though not as high as yesterday) and back up on pacing. That's the game though.

Day 1 Post-op Updates

Here are the updates for Day 1 Post-op! (10/19/18)

9:27pm update:
Job is in the 90s/high 60s and weaning down on his epi steadily. Yay! Yay! Yay! They also came down on his pacing to a heart rate of 170. They'll continue to go down on both all night and once his epi is under control they'll wean down on his other two vasopressers.

7:19pm update:

Job's blood pressures are the best they have been alllllllll day. I'm so excited! For the last hour they've been in the 80s/60s, so we just went down on his vasopressers.

5:40pm update:
Pacing.
Paralyzing
Sedating

That's been the goal for the last little while and it sounds like it will be the goal through the night. Bascially, they're trying to taking everything out of his hands so he can recover from the trauma of transplant surgery and from his extravastion this morning and his ongoing rhythm issues.

The electrophysiologis (EP) is concerned that Job could get into something called "jet rhythm," which would be a hard rhythm to get out of, so they started pacing him again so as to prevent Job from even getting there.

Tomorrow they will look for opportunities to check on his intrinsic HR and find out what it is, because right now the pacer is hiding his intrinsic HR.

When they paralyze him, he's fully on the ventilator and he can't initiate his own breaths. They want to make sure he's fully sedated when paralyzed so that he doesn't wake up unable to move and get scared.

3:40pm update:
Job's echo looks pretty good in terms of heart function. The right ventricle is slowly waking up. It's so odd that he now has a left ventricle working hard, doing what it's supposed to do! And that it's his right ventricle struggling!?! (Not that his native left ventricle could ever work at all because it was just a tiny little flap of skin.) I'm just still in awe that he has a four chambered heart.

And I guess I'm clinging to that awe because it's been a rough few hours again. This time because his rhythm issues are back. We've never had rhythm issues before, so this is a whole new field. The electrophysiology team has been in his room a lot today but they've been monitoring closely even when not in the room.

There really hasn't been any sort of break in the action because the few minutes in between the stream of specialists checking in, or in between evens, are dedicated to regular cares like turning him in the bed so he doesn't get bed sores or checking his lines and wounds or putting chapstick on him and swabbing out his mouth.

1:25pm update:
Job is ok now but that was a rough couple hours.

The intravenous line (IJ) going through his jugular pulled out of his heart and the calcium and blood pressure meds he was receiving through that line poured into the skin tissues on his neck. They're toxic for skin and so his neck swelled and his blood pressure dropped dangerously low.

Within a few minutes there were about 20 people in his room and they quickly began surgery to remove his bad IJ line on the right side of his neck and place a new IJ line on the left side of his neck.

It was critical to stabilize his blood pressure and to get quick access because without access Job can't receive the meds he needs.

That the line pulled out and leaked these medications is called an extravasion. Essentially, his neck got poisoned and the concern is that this will cause cell death at the site, which would mean he will have a great deal of discomfort and disfigurement there on his neck.

I took this photo probably two hours after the worst of the swelling (caused by the saline + meds leaking into his skin), so it doesn't show how puffy he got, but it will give you an idea:



Three new teams (Venous, Wound Care and Plastic Surgery) are now following him to monitor this wound and between the three of them they're hopeful that, in fact, the medications were diluted enough because of the saline fluid he was also getting. Whether the calcium will build up and create hard deposits that will need to be broken up later is perhaps the most serious issue? And it could mean he would need plastic surgery on his neck in a few months and/or have a big black spot on his neck.

Besides the poisoning, he wasn't getting necessary meds to his heart and so that could have been a big factor in why he was so uncomfortable and why he was all over the place. The thought is that normal post-op swelling began to pull the line out and then when Job was jerking and thrashing about this morning, he yanked it out the rest of the way.

It probably also set us back a day in terms of moving forward. He wasn't actually stable "just" recovering from yesterday anyway, but because of this issue they needed to slow way down and just paralyze him again so he could begin to recover.

Next echo comes to assess the function of his heart.

10:06am update:
It's been a rough morning emotionally because they lifted Job's paralysis meds and so he's been able to move... but that means we've been physically restraining Job as he flails and writhes and screams. Except his scream is soundless because he's intubated.

We just got kicked out of the room so they can do this little surgery to place new lines (which he needs for access and meds). Once he has stable line access then they are going to work to getting him off the ventilator. The tube he has in his mouth for that is really uncomfortable and he hates it and that's probably the biggest source of his irritability.



I didn't get a good picture but there are about 20 people his room right now. His intravenous jugular (IJ) line in his neck was about to blow and it got pretty intense, there were rushing to get the PICC established.

He's pretty out of it but the issue is that they need him to wake up so they can extubate. But when he wakes up he's hurting/scared/mad. So then his numbers plummet and he moves too much. We can't get him comfortable.

Once he gets these new lines then we can work towards extubation. Which will help his comfort. And a big relief is his rhythm is better. They were pretty worried last night because it was so messed up.

9:30am update:
The surgery went well, but we are in a 48-72 hour period that is very turbulent.

Right now Job is having a lot of heart rate/rhythm/pressure issues, which isn't out of the ordinary for someone getting used to a new heart.

And as he slowly shakes off his sedation, he is obviously very uncomfortable. Goes back and forth between sleeping and silently thrashing around when awake. He wants to scream but he can't because of intubation.

Right now they have Job on an external pace maker to control his rhythm.

He came out of surgery with a pretty significant fever, which contributed to his high heart rate (~210). His fever is under control now, and his heart rate is down (they are pacing him at 150).

Also contributing to his high heart rate is that his new heart is smaller than his old one. In particular, his right ventricle is smaller and quite stiff right now. Needs time to adjust to being in a new body.

When Job falls asleep, his blood pressure goes down to a level lower than desired, so they've given him fluids and other medical therapy to manage his blood pressure.

Job's discomfort and fear are things we'll be dealing with all day and probably through the weekend.

Transplant Night Update

Job is out of surgery and enjoying a new look! Pink fingers and toes, and sats in the upper 90s! O how He has blessed us this day!

Transplant Surgery Updates!

6:45pm update:
Job is out of surgery and is going to be settling into his CICU room soon, at which point we will get to see him.

The surgeon was very pleased with how everything went.

Job has a fever, which is likely a reaction to a medication or steroid used, and his heart rate is very high. Fevers always increase heart rate, as does the rough transition the right ventricle always has after a heart transplant. These issues ought to resolve well with medication and time.

The first 24-48 hours post-op tend to be intense, so that's what we're anticipating. Rejection doesn't tend to be quite as much an issue these first days because his immune system is so suppressed right now and because his body is fighting so hard to get used to pumping this new circulation. We're probably primarily looking at "standard" open heart surgery recovery issues through tonight and tomorrow.

2:40pm update:
The old heart is out and the new heart is in. The surgeons are stitching up the connections from the new heart and pulmonary arteries to Job's aorta and the stubs of his original pulmonary arteries now. The new heart, Job's NEW HEART, is profusing well and pumping with assistance of bypass. He's bleeding a great deal, which was expected, but now needs stopped. Once the bleeding is under control then they will begin to wean off bypass and then, if all looks well, close his chest, and then take him to the cardiac ICU (CICU).

We're hopeful we might get to see him around 7pm tonight. He'll be on the ventilator and have the standard wires and tubes that we are familiar with after cardiac surgeries. He'll be very well sedated as well.

So far every thing has gone well, even if it's taken extra time to accomplish.

11:00am update:
The procurement team retrieved the donor heart and is on their way back to Seattle Children's Hospital.

Job is doing well. They started his chest dissection, which will be about a two hour process. They think it's possible the donor heart will be here at the hospital and into Job around 1-2pm and will update us then.

9:45am update:
There has been a delay at the donor's operating room. They can't tell us what the delay is or how long it will be.

Job is anesthetized and waiting peacefully for word on the donor heart viability.

9:00am update:
So the optimistic timeline of today is:
-6:00am met with surgeon, signed consent
-6:15am meet with anesthesiology team, safety check; wheel Job to the OR
-6:30-8:30am put him under anesthesia, get him comfortable, put in PICC line
-8:30-9am the donor organ retrieval team opens the donor chest and lays eyes on the (artificially) beating donor heart and then gives the official "go ahead" *if* the heart is viable
-9:00-11am chest dissection (he has so much scar tissue that it takes a long time!); Job goes on the heart lung machine, which pumps his blood for him
-11-1pm the new heart arrives to the SCH OR and Job's broken heart is removed and then replaced
-1-2pm surgical connection and reconstruction of his pulmonary arteries and aorta; new heart starts beating
-2-4pm watch new heart and stop bleeding from suture sites
-4-5pm recovery, assessment
-6pm taken to cardiac ICU

6:50am update:
Job just went into the OR. We should receive confirmation regarding the viability of the donor heart by 8:30 or 9 am.

Further updates throughout the day will be made on this post!

Donor Offer!

This afternoon we got "the call": a donor offer has been accepted for Job!!

We checked in tonight for labs and vitals and will be wheeling to the OR at 6am tomorrow morning. He will be anesthetized, which is a bit of a process, and then the dissection (chest opening) is scheduled to start at 8am, *if* the retrieval team confirms that the donor heart is viable.

We don't expect to get to see Job until about 5pm tomorrow but may receive updates throughout the day, which we will pass along.

I wrote this weeks ago but it has answers to many of the questions we have been asked about this process: http://frightfulsheart.blogspot.com/…/08/transplant-faq.html

Nighttime Oxygen

A typical oxygen saturation is about 98-99%. I believe most people are hospitalized if they drop to 90%.

But thanks to Job's surgeries, his body can function, is supposed to function, in the 70s-80s. These low levels of oxygen do take their toll on his body, both in the short term (his digestive issues, for example) and the long term (predicted organ failure).

However, when he started walking back in February we quickly realized his oxygen saturations were dropping really low as he exerted himself. It's been hard to get good reads while he's now running around, but the guess was that he hit 38% on Friday at our appointment.

Job bounces back pretty quickly once he rests, but the downward trend isn't sustainable (thus heart transplant list).

Because Job gets so "blue" when he's active, he should be on oxygen all the time. But he's an opinionated toddler who doesn't want a nasal cannula on his face, thank you very much. Most toddlers don't want oxygen so for this whole summer his teams didn't even want to bother to try.

But in his trials on Friday, we did get him to wear the mask (thanks to Isaac modeling it and Ezra talking about how cool it looked) and his sats bounced up 15 points!

Oxygen wasn't supposed to help him so drastically, but Dr. Files insisted on trying it and sure enough, it worked and well. Well enough to try to force it at night.

The best guess (because Job won't cooperate with expectations) is that his pulmonary AVMs ("bad," narrowed veins) aren't allowing enough oxygenated blood through. This was the reason Job was on oxygen from September 2016-August 2017, but the combo of his axillary fistula (surgery) and sildenafil (medication) worked to get him off.

Since PAVMs are so rare for kids like Job, the fistula is pretty rare too. He was only the second kid at SCH to have it done, and the smallest, so they made their best estimate for the size of the fistula and they ultimately made it too small because the PAVMs are back. They've been back for a few months actually.

So we are now going to start putting him on oxygen at night (or trying to, at least). The thought is that he'll then wake up properly oxygenated and have more stamina throughout the day. I'm skeptical that he'll keep it on but excited to see if it will help!

We kept some of the oxygen supplies from our last oxygen experience but are waiting for a new delivery of masks and larger canulas to officially start this up.

Tricuspid Valve Regurgitation

Short version: Job's heart is steadily failing and he now needs another procedure this month. He's still on the transplant list.

Long version: A friend asked me if yesterday's cardiology appointment was "just routine?" and I responded "yes, routine bad news every month".

That has definitely been the case these past eight months, and really, it's to be expected since Job's in heart failure and listed for heart transplant. I guess I just didn't expect the decline to be so very noticeable, week after week. I thought we'd have a longer trajectory.

I keep reminding myself of how sick he can get still and how many interventions are available still, but I'm just so sad and so weary after this last appointment.

The heart has four chambers and therefore four valves. The tricuspid valve is supposed to allow blood to flow from the right atrium to the right ventricle but not let that blood "leak" back into the atrium.

Job's tricuspid valve does indeed leak blood back to the atrium which causes two significant problems: his atrium is filling up with blood and essentially getting stretched out and he's not sending as much blood along to his body to use as he should.

This first became a problem after Job's first surgery, when his circulation was first reconfigured, because the right ventricle is so stressed from over work. If you remember, Job's left ventricle was the size of a sesame seed and not at all functional, which is why his right ventricle had to take over the job of pumping blood to the body.

The right ventricle is smaller and less powerful than the left ventricle, as it is only supposed to pump blood to the lungs as opposed to the whole body like the left ventricle. Think lawn motor engine vs cadilac engine.

We always expected Job would need a tricuspid valve replacement, until we realized his other issues necessitated a transplant instead of the other HLHS interventions.

We have been carefully monitoring Job's tricuspid valve "leakage" (regurgitation). It was increasing (to "moderate") but then stabilized about 6 months ago and actually seemed to be improving (to "mild").

But since his last echo three months ago, his tricuspid valve regurge is suddenly very severe.

This could be because his heart is failing and failing quickly and this is just one expression of this failure.



But it could be because of narrowing in his aorta which would need surgical intervention. His aorta has been patched and enlarged twice in open heart surgery and has had two cath interventions but the flow is fine at these intervention sites (where we would expect scar tissue to create narrowing).

The team debated about sending Job to the cath lab where they could assess his heart, figure out why the tricuspid valve is suddenly so leaky, figure out if there is narrowing somewhere else, and then potentially intervene. But they decided the risks of an exploratory surgical procedure and anesthesia are too high for him right now. Job is too fragile around with unless it's an emergency.

So we'll be doing a CT angiogram which I know nothing about yet but will be learning about soon.

Job is still on the transplant list and this new finding hasn't "hurt" or "helped" his status. But we need to optimize his health so that he can wait however long for a transplant match to come up.

His huffing and puffing and increased cyanosis (blueness) are likely because of this regurge and they will probably continue to get worse.

We also did an oxygen trial but I'll write about that later because this post is already so long.