Official: Not Treating Rejection!

It is now official: we are not going to treat for rejection.

Diuretics have made a world of difference and now his echo looks great and his BNP has reached baseline. His tricuspid and mitral valve are no longer "leaking".

We now have to figure out if we want Job euvolemic (to have a normal fluid balance) or if he needs to be dry or wet. His electrolytes are all out of whack now, so they are also determining which supplements to give.

And all of that will probably take a few days.

Tomorrow's Decision Day

I was trying to explain to my friend how very odd this hospitalization is.

Job feels fine. He's irritated by the pokes and confinement, but he's getting close to his pre-transplant attitude plus with more energy. He actually probably feels better than fine. His *smile for the camera* still looks like a grimace though.

He had a grand ole time today, with his brothers and toys and room service food delivery. He even started insisting on sitting on the toilet rather than wearing his diaper, but we'll see if that lasts.

Tomorrow is the big day: have the diuretics made enough of a difference to definitively push aside the suspicion of rejection?

His BNP has gone down and he's been peeing well and now his Tacro is in range - all good things. He'll get labs at 8:30 and an echo around 9am or so and then the team will convene and hopefully be able to make a decision.

If it's clearly that his too-small, stiff-from-being-on-ice heart couldn't handle being fluid overloaded and the diuretics did indeed make a big difference then I'm not sure what we'll get to do. Go home, but when?

If they can't clearly see the diuretics made a difference then we treat for rejection, even if there aren't any other clear signs of rejection. And that will mean at least 4 days of further treatment here.

Thanksgiving Day

We spent today playing and eating, just the five of us.

Despite intensive diuretics, nothing changed on Job's echo... which is very disappointing.

He gets two more days to keep trying diuretics and if nothing else changes then we have to start treating rejection. Even though the biopsy came up clean, if fluid retention isn't the answer for his issues then rejection must be. Although it's not ideal to give Job such high doses of steroids with confirmed rejection, giving unnecessary steroids is a small price to pay compared to not treating rejection at all. Something is wrong with his heart and they have to treat the worst thing.

His Tacro dose is climbing up, closer to range, but his dose also went up since it's not in range yet.

He's pretty happy and active and enjoyed spending the day off monitors, wandering around his room playing with his big brothers.

The big boys and I ran over to the Ronald MacDonald house and picked up a turkey dinner and brought it back to our room. I'm so thankful for the legions of volunteers at RMAC who daily donate their time and resources to feed and house families at SCH. I wish, mostly for the big boy's sake, that we could get a room there and they could spend the night but I understand and fully support giving rooms to families from much further away. It's just a big blessing that they now give out day passes to families like us.

I've been thinking all week about the timing of this hospital stay with the Thanksgiving holiday and really, I've been thinking about this since we listed for transplant. I'm thankful for Job's life... but I don't know if I'll ever be able to understand how grateful I am, let alone ever express it. I've almost felt numb to the emotions of all of this (this week and this past month) and I think that's largely because I can't even comprehend the magnitude of it all.

Biopsy Day Updates

9:00pm update:

The biopsy results came back.

Showing no sign of rejection.

We're all surprised - the symptoms Job is experiencing seemed to match rejection so well. His Tacro dose was so low that the bias was immediately towards rejection and necessarily so, because treatment had to begin.

They stopped his intensive course of steroids (methylpred) but can of course restart it if it is determined later that this is rejection that just didn't show up on biopsy.

It's possible this is a false negative and so further monitoring is needed in order to determine what is going on in Job's body.

He'll get an echo in the morning and more labs and the focus will probably be on finding the right course of diuretics for him. We'll need to stay here for several days, probably not to treat rejection  but to figure out what we need to treat and how. I guess Job just likes to keep his team busy researching.

Job is back to himself after this morning's activities, engaged and interactive. 

He's not quite back to his pre-transplant self activity-wise, but this little hospital stint doesn't seem to be negatively affecting his attitude much (at least not yet). He spent most of his time out of his crib and had our full attention and a steady stream of new toys from Child Life, so he was pretty happy.

5:00pm update:

Job drank 38oz of thickened juice from 12-5pm today. And he's eating a lot of food as well.

He's drawn and played with blocks and animals and done puzzles and watched his favorite video multiple times. He's been mostly happy - some pain at the incision site for his biopsy at his right jugular vein.

12:00pm update:
Job is back to his room and recovering well from the procedure!

All signs point to rejection (increased pressures and decreased function and fluid overload) but pathology is on a rush order to get his samples processed as soon as possible to make the official declaration.

We're starting treatment as though he's in rejection though because he probably is. He'll be on high doses of IV meds and we'll get lots of labs. A discharge this weekend could be possible, I suppose, but we'll see how it all goes.

Theoretically he'll be happier this admit because he can eat and isn't on viral precautions but he is starting mega doses of steroids so maybe we'll just be back to raging.

8:30am update:
Job is already off in the cath lab!

Their first case was cancelled and Job fell asleep and stopped eating/drinking early so he was eligible to go! (So glad I didn't wake up to my alarm in the middle of the night to force feed him in the middle of the night as I had planned to do!)

Going early means less time NPO which means less time grumpy! We basically woke him up to give him his pre-op "bath" and then whisked him away.

He should be back in his room by 10 or 11am and, because this is probably just a biopsy (measuring right side pressures and taking tissue from the right ventricle) and not a cath (assessing his vasculature and measuring venous and arterial pressures) they will only go in through the neck which means he doesn't have to lay flat for 4 hours like he would if they needed groin access. That's so great! It has been so hard to lay still as needed the last few caths.

Possible Rejection

Well, we brought Job to the Emergency Department at SCH this afternoon to assess for possible rejection.

He is scheduled for a biopsy tomorrow as second case (so around 11am).

In the biopsy they'll go in with a catheter through his neck vein and snip off pieces of tissue from his right ventricle, which should go to pathology by afternoon. We find out whether or not he is in rejection (and what kind of rejection) tomorrow evening or Thursday morning.

Rejection is treatable but certainly this is hard news. Frankly, we don't even know enough about transplant yet to have perspective on all of this.

He's not terribly upset when he's eating/drinking and not being poked.

More details:

Tacro is Job's primary immunosuppressant medication and his "level" of it is supposed to be between 10-12. It was a 6 last week so we upped his dose and drew labs again yesterday morning. Those came back today at a 4. So we upped his med but his level still dropped.

Also yesterday we noticed that his face was pretty swollen and puffy and his stomach was huge and firm. The team had us give extra meds last night and he did look less puffy this morning, but we were already on high alert when his Tacro level came back so low today.

So we packed our bags and drove north. He's had his echo and EKG and x-ray and a whole team of people assessing him.

He has some fluid in his left lung but the main issue of concern (other than his inadequate Tacro levels) is that his mitral valve and tricuspid valve reguritation is moderate after two echos with no regurge (one each week).

He could just have excess fluid in which case we'll up his diruetics and go home. But the head of the transplant team told us he'd give that about a 20% chance.

Or we'll stay 3-7+ days and treat for rejection. And reset the clock on his intensive course of immunosuppressants and steroids (when we were already on week 5 of 12).

Good bye, Home Care Oxygen Supply!

I'm thrilled to be sending our tanks back and yet also packed them up with tears in my eyes.

I hate our old CHD. Hate it! But I miss it too.

Hypoplastic Left Heart Syndrome was familiar. After studying it for almost three years, it finally felt comfortable. Normal.

I could do single ventricle care. I knew the procedures and medications.

The projected outcomes were still terrible but I understood them. I had sorta kinda made peace with them.

I was really invested in the current research focus on the Fontan.

I knew the SV doctors and nurses and medical assistants.

I hardly needed our pulse oximeter to accurately assess Job's oxygen saturation at that particular moment because his color and work of breathing and energy levels were so telling to me.

I wanted to keep his native heart, as broken as it was. As scary as the projected outcomes were for Fontan physiology.

So even though I now look at Job and marvel at how pink he is and weep with gratitude that some other devastated family chose to donate life, I'm also grieving the loss of HLHS deeply.

And giving up Job's oxygen supply is a very tangible reminder that we have a totally new disease now.

Grief is weird. Emotions are weird.

These last five weeks have been so very full I haven't been able to do much beyond just the next step. Processing anything emotional or spiritual has seemed a superfluous luxury, but very much needs to happen.

The big boys are probably most helpful to this process with their constant questions. And can you even imagine explaining all of this to Job someday?

VFSS Results

JOB PASSED! HE CAN EAT AGAIN!!!!

Oh, I couldn't be more excited but I think that pales in comparison to Job's excitement.

Job got to sit in this special little chair and I gave him food/drink with barium while they took x-rays.

He swallowed food and purees just fine but aspirated on thin liquids (regular water/juice). So then we tried nectar thick liquids (juice with a thickening agent in it) and he aspirated, so then we tried honey thick liquids (juice with extra thick thickener in it, to the consistency of a thick smoothie) and he could handle that. YAYAYAYAYAYAYAY!

Our first stop after the appointment was the cafeteria and I bought him whatever he pointed at.

He even asked me to stop midway home for more food! And then once home we scrambled eggs and he devoured four eggs before I made him take a break.

This means that the intervention (gel injection) Oto did last week worked! We assume it's his right vocal fold working to overcompensate and reach to the plumped up left vocal cord to give him voice but also to protect his airway as he swallows.

It's just not all the way back to pre-transplant baseline to close in time before the fast moving thin liquids approach his airway.

We'll reassess in about 90 days with another VFSS but we'll see Oto in 33 days to talk about whether or not we need to do a more permanent intervention.

First Week Home

Short version: Job has been home four days now and transplant-wise is doing great! Attitude-wise it's been pretty rough: he's grumpy because of steroids.

Long version: We had our first outpatient transplant (TX) clinic today and his cardiologist was very very pleased. His Tacro (main immunosuppressant) level was really low, so we're going up on his dose, and Job was vascularly dry so we're taking away one of his diuretics and adding more fluids.

We're also working to increase his calories and get some real food blends into him. Thursday is his swallow study so it's possible he'll get to start eating some foods by mouth after that assessment, but in the meantime we'll start giving fruit and vegetable purees by tube 6x a day. It's a lot of work to get his feeds ready and to make sure we're on time with them. Much more work than I remembered from two years ago when we were on tube feeds, actually.

It's hard to avoid eating or drinking in front of him - we have to be very calculated about who will go eat where and when and who will entertain Job while everyone else is eating. But it's hardest to avoid talking about food and drink in front of him! Wow - food is a common topic!! And we're all a bit dehydrated because we can't carry out water bottles around and drink from them all day.

Every day Job gets stronger and more active and can stay awake longer. He's actually sleeping pretty well at night - better than we were expecting!! We're starting up PT and SLP therapies next week.

His meds aren't very difficult. It's actually an easier schedule than we were on pre-transplant. Two of his meds are very time sensitive and have very precise blood draw times, so that is a bit of extra work, but it's not so bad. I almost have all his meds memorized by name and dose and bottle size/color and med color, which happened more quickly than I was hoping and makes life much easier. I could have drawn up his old meds with my eyes closed and was apprehensive about how much extra time it would take to learn his new meds, but for naught!

'Roid rage is the hardest part of post-TX life right now. Job gets so very angry so many times throughout the day. He's most grumpy with me but will play for moments of time with his brothers or dad or grandma and grandpa. He's really sad to not eat by mouth and keeps asking for food/drink politely... and then angrily. And he's deconditioned from surgery and all that bed rest, so he's irritated about that. But he just bursts into rages all of the sudden. Sometimes as (over)reactions to some provocation but sometimes completely randomly. We'll be off steroids on November 3pth and that day couldn't come soon enough.

He likes walks in the stroller outside and baths always but is rather fickle about other activities and people that he did like pre-TX.

It is completely understandable that he's this emotional. I can't even imagine how scary and sad and frustrating and confusing and uncomfortable his life is right now (and I am usually the one forcing him to do xyz)! But it's definitely hard to figure out how to encourage him and entertain him and take care of him right now.

First Weekend Home Post-op

We're so happy to be home! Even though it's really hard right now (mostly because of attitude and exhaustion).

Day 22 Post-op Updates

7:00pm update:
We're home! We're finally home!



The discharge process was excruciating and I'm about to go to bed and leave Job to play with Grandma and Grandpa or Daddy or anyone else.

But how amazingly adorable and sweet was our welcome home signage? We have the best friends.



3:30pm update:
I remember when I was pregnant with Job and talking to another mom at our homeschool co-op whose daughter had been at SCH multiple times. She said one of the things she wished she had known about hospitalizations was how long discharges take and therefore was warning me so that I would set my expectations accordingly.

We've had some long discharge days before but today takes the cake. I don't understand why the paperwork is taking so long. Every other nurse has been able to get it ready before rounds so that we just have to get the attending to check off... but not today.

Job is melting down worse than I could have expected.

12:40pm update:
We get to go home!!!! The attending needs to come assess Job and then the nurse needs to get our discharge paperwork together and we have to grab meds. Yay! Yay! Yay!

8:00am update:
Job had a pretty good night. He hates sleeping in his crib and tossed and turned but didn't have any increased work of breathing or desaturation episodes. Now we wait til rounds (~12pm) to hear the official word on discharge.

Day 21 Post-op Updates

10:00pm update:
Job and I were cuddling, trying to get a nap in and I felt all this wet stuff all over us. I thought it was his feeding tube leaking and started to clean it up, but once I turned on the light I saw it was blood. He yanked out his IV that was placed during the surgery today. They were leaving it in on the chance they needed access tonight but he decided he didn't need it.

So he got a bath, which he really liked, at least in part because it allowed him to sneak as many drops of water into his mouth as he could manage. He kept lifting this toy up above his head so it would drip water all down his face, into his mouth, since we wouldn't let him just take actual sips. Sneaky sneaky.

Annnddddd I packed up a bunch of our things and put them in the car.

6:00pm update:
I don't think I've never fully explained what the procedure today did.

So to back up, in April 2016, at five days old, Job had the Norwood surgery to reconfigure his circulation and basically keep him alive for future interventions. The nerve to his left vocal cord wraps around the aorta so it's very common (75%!!) for kids undergoing the Norwood to have some impact to the laryngeal nerves. Everything is just too small and too intertwined to always avoid traumatizing that nerve, try as they might.

If the nerve doesn't regain function within a year then it likely won't, and we're all pretty positive that Job's left vocal cord never regained function (although the formal studies we did to establish this were only done in his first year of life - not recently enough to definitively determine that it was still absolutely paralyzed).

Your vocal cords vibrate when you turn them on and essentially meet in the middle of your throat to produce sound when they touch and also when you swallow, to prevent food from slipping into your airway. A cough is one of your body's mechanisms to protect your airway, so when Job was trying to swallow but his vocal cords weren't able to move to meet each other because of the trauma of intubation, he coughed and choked on purees and thickened juices. If food/drink does go down the airway into the lungs this is called aspiration and can be terribly dangerous. Food and drink particles do not belong in the lungs!!

Even if Job's left vocal cord remained paralyzed, it didn't entirely matter because Job had compensated to gain enough function to learn to swallow. We're assuming that his right vocal cord was just over compensating and going past midline to reach/almost reach the left vocal cord and protect his airway enough when he swallowed. His voice was raspy and weak but present enough for some use and we were hopeful intensive speech therapy would help him learn to compensate further.

He wasn't aspirating, though I had some concerns about the quality of his swallow, and his quickly declining heart took precedence this summer over an Oto follow up.

Fast forward to extubation following transplant and it was immediately apparent that Job's vocal cords weren't quite working the way that they had before transplant. Speech came and did a lot of inpatient work to assess his swallow, and for a few days while still on hi-flow Job was cleared for purees. Once off the support of the positive pressure of hi-flow, forcing good air into his lungs, Job couldn't handle purees and lost that privilege to eat them (because he was just aspirating them!).

While we were hopeful that his swallow would improve each day following extubation, it actually got worse and thus Oto got involved and did their scope Wednesday and then this procedure today. (Which is just amazing timing and I am so so so grateful they made room in their schedule.)

So, Job's vocal cords weren't meeting to produce sound or protect his swallow, which necessitated an injection laryngoplasty and bronchoscopy. Essentially they went in and injected a gel into Job's left vocal cord to plump it up so that the slowly improving right vocal cord wouldn't have as far to travel to meet it. Even if the left side was truly still paralyzed (which we think it was), it now is closer to midline. The pictures taken in the procedure show a stunning improvement - I'm shocked! But his voice is now working better than it has since before transplant (which again really means before intubation) and that's a good indication he can again be able to swallow safely.

Here's the mark they made for where to make the injection:

The concern was that, if they injected too much gel, the airway could be obstructed because the left vocal cord would be too plumped up. They then err on the side of caution and sometimes don't inject enough gel to make a difference.

The gel used today only has a lifetime of about 2 to 3 months effectiveness. This could buy the right vocal cord enough time to improve and then continue to overcompensate for the left side as we think it was before transplant. Or, the right side might need more time or never recover, so if in 2 or 3 months Job's voice and swallow suddenly worsen and then we'll come in for another procedure but this time (since they know the correct amount of gel to insert) they'll use a longer acting gel that lasts 6 to 9 months. And if, after that extra amount of time, the right vocal cord doesn't regain pre-transplant function, we'll do a more invasive surgery to permanently fix the left vocal cord to a more midline position. We might also eventually do a nerve innervation.

We really want Job to have voice and swallow. He was so sad already after a mere two weeks of not having either, and I just couldn't wait indefinitely before intervening when his quality of life was so affected. Speech couldn't help therapeutically and regaining function without intervention could have taken up to a year.

He is still NPO until he gets a videofluoroscopy (VFSS) which is scheduled for next Thursday. That means, we go home on the feed pump and stay on it probably though January until he is able to safely swallow enough food and drink for full nutrition, if he can indeed regain that ability. He may be able to eat or drink some things as early as Thursday if his VFSS goes well and shows no aspiration, but he'll be very limited in quantity until he regains strength, which is a very gradual process.

The surgery done today is usually performed outpatient but because Job is at such danger if he gets an infection because of his immunosuppression meds, we'll spend the night for observation.

Which puts our hopeful discharge at TOMORROW.

3:00pm update:
This morning was really hard. Job didn't sleep well last night, to say the least, and he was grumpy grumpy grumpy this morning. It was really hard to wait for 9:30, when we expected a team from the OR to come get Job... and even harder to wait until 10:30 when they did show up... and then the ride through the halls to the OR was fun... but the hour wait in the PACU pre-op was really really not fun. The iPad was distracting for a few minutes, but not nearly long enough. I've tried so hard these past months to get Job interested in electronics so he could rest, when he had HLHS, or so that he could just relax and recover while he's been in the hospital, but so such luck.

It's so tempting to lose my patience with Job when he's so irritated and inconsolable, but the poor kid doesn't fully understand what's going on, just that he's not comfortable and I won't give him what he asks for. I've developed quite the arsenal of "distract kid!" tricks over the years, but Job is super bored of all of my efforts these past two weeks in particular. He was not having it this morning.

He finally went back to the OR at 11:45 and I rushed to the cafeteria for food and the laundry room to start a load and then they suddenly paged me to talk to the surgeon, so I rushed down to the consult room... and then while finishing talking to Dr. Parikh, I got the call that the CPR Training Team was a bit early and already waiting in Job's room for me, so I rushed back up there to do my mandatory training. And as I was finishing that, they paged me to please come console Job in the PACU, so I rushed back down there.

A mere 90 minutes later and there was an immediate difference in Job's voice! He was so much clearer and SO MUCH louder with his sounds! He was fussy as could be but glad to see me. And then as we were waiting for sign off, a nurse on break within eyeshot started drinking his Diet Coke and Job just lost it. He wanted the drink so badly and was calling for it and signing for it and crying for it and pointing at it. Ahhhhhh! I finally asked our nurse to please ask that nurse to go drink his contraband elsewhere, where Job couldn't see him.

We got back to the room relatively quickly, all things considered, and I tried to get Job to nap without luck... until just before a team of photographers came by to take pictures of Job and whichever other kids on the floor to use for the annual SCH shareholders report. He's on his own timeline that exactly never matches up with my timeline.

Day 20 Post-op Updates

9:00pm update:
Job got another shower and we replaced his replicare and tagaderm on his cheek for his NG tube, which is at least a two person job. I've given all of his meds and feeds on time. I set up Home Care services (delivery of his medical supplies). I met the woman who set up the SCH transplant program. Dr. Nuri came by to check on us. I signed consent for tomorrow's surgery. I tried to keep Job somewhat entertained.

I started putting clothes on Job because he was getting cold (and I couldn't handle the heat being so high in our room) a day or two ago. The problem is that he thinks that means we should be going outside. He found his hat today and put it on and spent the next hour trying to get out the door or window.

1:00pm update:
Hallelujah! Oto made room for us on their schedule. Get this: for TOMORROW!! I thought we'd have to wait for mid to late week next week! Yay yay yay!

10:30 start time, so probably rolling to the OR around 9:00.

Child Life spent probably at least 90 minutes playing with Job while I talked to providers. It was so good for Job to get to engage with someone besides me and it was wonderful to not juggle kid and consent forms. Although, I have to say, in all the appointments we have had here, I have never once felt chastised for needing to take a moment away from my conversation with ___ provider to help/discipline/protect/guide ___ child. They do a lovely Job of including even fussy Job in our conversations about anesthesia risks, for example.

12:00pm update:
I started my 24 hour care just now, after rounds. So I have to ring for the nurse to bring me meds at all of our med times (1pm, 5pm, 9pm, 9am - easiest med schedule ever!) and then ring for her to tell her I started a feed (3pm, 6pm, 9pm, 12am, 3am, 6am, 9am, 12pm). When she brings the meds, I have to tell which ones are due and how much and, depending on the nurse, what the med is for. I change all diapers and bedding and clothes and all that regular ole kid stuff. I think I also have to change his dressings, but we'll see about that. Basically, they want proof I can keep him alive.

His transplant meds are very time critical so I have to be very prompt with my call button.

10:00am update:
'Roid rage. 😳😳😳

We'll be playing and having fun and then Job will get intensely and irrationally angry with me. Really really really angry.

He feels better in so many other ways - he's pink and breathing easily! It's amazing to see such a drastic change so quickly.

He's quickly regaining his baseline gross/fine motor skills and all around getting back to himself (besides the abrupt mood changes) and just looks really healthy.

PT came by and was really happy with his functional abilities. He'll build up stamina with time, but he's doing great. He walked to the fridge several times and tried to open it to get food, which was heartbreaking but impressive.

Day 19 Post-op

6:00pm update:

My cousin and aunt came and visited with us for a bit this evening. They got Job laughing while I signed consent for his surgery tomorrow morning.

Job got a shower today, since his PICC line is now out. Woo hoo! He smells so fresh and clean but he also loved it. He just sat there, spraying warm water over himself for about 45 minutes.

He's pretty grumpy again, if not just outright mad, so any and every distraction is a balm to both Job and I. I think it's just going to be really hard until he gets to go home. And until he's off steroids.

3:00pm update:
Today has been exhausting! (And it's not even finished yet!) Every day down on the floor has been, actually, which amuses me because to even be on the floor Job has to be doing pretty well. When he was the sickest was actually the most relaxing, at least physically speaking, because I just sat next to him and held his hand. But here I'm engaging with him or talking to providers with hardly a spare moment. Job crashed on the couch this afternoon and my plan was to nap with him but I ended up doing an extra training instead. I'm getting so many things checked off! It's a good distraction so I don't focus on my disappointment about his swallow.

12:00pm update:

Oto came by and scoped Job's vocal chords.

The left doesn't move at all and the right hardly moves.

I was holding Job in my lap, holding his arms down, and watching the scope and it was astounding how very wide the gap between his chords are. It's no wonder he's aspirating so badly and his voice is so so very faint.

It's such a dramatic problem that Oto had no problem quickly recommending we do a procedure as soon as they can fit Job into the OR - provided Transplant and Speech sign off on it.

It's devastating that this is such a problem. But it's probably also a bit vindicating because I do feel like I had to really insist Oto get involved and we not write this problem off as a cold virus.

I'm just so so sad today. I want to be grateful we caught it and most of all grateful that Job got a transplant and not begrudge intubation for "ruining" Job's swallow and voice but right now I'm struggling to let go of my bitterness.

Day 18 Post-op Updates

4:30pm update:
So many people came by today! Here's the list (so far):
-the resident, for his daily assessment
-the nurse, every four hours for vitals and two blood draws and one dressing change and probably several other check ins
-the team, for rounds
-SLP, for a swallow study
-a sonographer, for an echo
-Child Life, to check in and then again to bring new toys I requested
-OT, for a session
-Nutrition, to make a plan for Job's tube feeds
-one of our two primary transplant nurse coordinators, for a training
-one of our four primary transplant pharmacists, for a training
-the IV team, to remove Job's PICC line
-someone on the patient experience team, to take a survey on our experience in the CICU
-the attending, for his daily assessment and to answer any questions I have
-a fellow from Otolaryngology who was quick to order a scope after listening to Job

3:16pm update:
Over the weekend I started giving Job's meds and starting and stopping his feeds and taking his vitals. These are all things I will get to do daily at home.

I had one training on Friday and two more today and have two scheduled for tomorrow with the transplant nurse coordinators and with pharmacy. Can I recognize signs of rejection? Do I know the side effects of his various meds? And so on. I have a binder and flashcards and all the notes I've been taking on transplant since June, so I've been studying away when I'm not playing with Job. I read and reread the binder so many times this summer that I did feel pretty comfortable about these transplant specific things, but I'm really appreciating all this hands on training too.

Well, except for the things I've already done before. Do I really need to do a lovenox injection training again? I did it last time we needed anticoagulation and I've been giving all his injections this weekend. Do I really have to take out his NG tube and reinsert it? And so on. I understand why hospital policy exists buuutttt I also really don't want to abide by it every time when *I* think it's excessive. We'll see what flexibility they will allow me - it will probably depend on how the nurses think I'm doing with Job's care.

Eventually this week I'll have to do a 24 hour care, where I do all of Job's cares for that time period to show I can really take care of him at home.

12:06pm update:
Job had an even more disastrous SLP assessment today. His swallow is getting worse and worse as each day goes on. But now we're going to get Otolaryngology involved, which I wanted to do last year and have really wanted to do through this hospitalization. So I'm disappointed but trying to stay hopeful. Oto can do all sorts of things to optimize Job's swallow if needed and can give the msot clear answer of what is going on.

I've wanted them to take a look at Job's throat all year because he's never quite been right following his vocal cord paralysis during the Norwood surgery in April 2016. He did learn to eat and drink well, but he's had weird swallow/vomiting issues and he's quite speech delayed as well. But when I started asking for Oto involvement this summer, Job's heart started failing even more quickly so that obviously took precedent.

When he started coughing and especially when he started aspirating, I started asking again. I'd love to do whatever we can while inpatient because outpatient care is harder to arrange and happens more slowly... but if they couldn't see us inpatient for a week then I'd rather go home and do that outpatient.

So the thought it that his left vocal fold was paralyzed but his right vocal fold was overcompensating and worked to close off his airway from aspiration while eating these past two years. He learned how to make do.

But during intubation, the vent tube was down his throat pushing both vocal cords to the sides. So even after he was extubated, his right vocal fold still has to recover from that trauma.

Child Life brought by new toys, so we're enjoying those too. The same toys in the same room for the same two days was getting a little stale. I didn't realize how much I take environment for granted. When we're at home our environment is constantly changing so even though our toys aren't, we don't get as bored as quickly with the same toys.

And I was just thinking about the nature of contentment because I think this week will be a little hard for me as I long to discharge but still need to jump through the hoops of all the training we need to do.

9:00am update:
Another area where I've been receiving frequent questions is regarding Job's cold.

It almost seems that Job didn't really have a cold. His only symptom was coughing and those couple days of needing extra oxygen support.

His cold hardly affected him other than to make his providers wear gowns and masks and to not allow the big boys to visit.

His doctors said that his response (non-response?) to this rhino virus is really really good news because he should have been super set back because he was so immunosuppressed.
Since he was strong enough to handle it while so immunosuppressed, he should be pretty strong and safe this winter (relatively speaking) because he'll be on less immunosuppression (probably).

We all saw him getting sicker and needing a transplant this summer, but so many kids gets so much sicker as they wait for a donor match and are then weaker going into the transplant and struggle to recover from surgery quickly and then also struggle to recover from a cold. This is one of the reasons SCH works so hard to list kids "early" so that they perhaps get their match "early," before they're really really sick and weak.

Day 17 Post-op Updates

9:00pm update:
Job hasn't been sleeping well because of vitals every four hours, his steroid prednisone, some explosive night diapers, general post-surgical discomfort and disorientation... They started melatonin a few nights ago and that has been a great help but we really need to get his steroid dose moved to the morning. Moving med times from whenever they were ordered in the CICU to times that are reasonable to give at home is a big reason we stay in the step down ward for a while post-op, and it's a process because the med time can only move by 20%/day. So tomorrow Job will finally get his steroid in the morning which will then really help his sleeping at night.

All of that is to say neither of us have been getting great sleep this past week at night, so we took a great afternoon nap, all cuddled up on my bed. We were woken up a few times for lab draws and then we'd snuggle together again and Job would smile at me while we fell asleep again. It was wonderful.

Job cracks me up with his poses. He still gets worn out pretty quickly and then leans against whatever is close by:

And we've enjoyed sitting at the window. No more Lake Washington and Mt. Rainier view - we're now looking out to the garbage collection site, but sometimes we get to see trucks and hear them beeping.

Child Life brought cars and tracks and Job has been really enjoying a new rotation of toys.

1:00pm update:
We had another serial panel today, this time for his valacyclovir. So the nurses were in our room a lot again, as well as the resident and attending, but I'm shocked again at how helpful a slow "lonely" day was for both of us. I've been an introvert all my life but I still somehow forget that people exhaust me sometimes. And it's not at all personal, of course. I'm so so so thankful for the (already!?!) hundreds of providers we have seen this hospitalization and for friends and family pouring into us. It's been wonderful. But it's also been really helpful to shut off my phone today and pull the curtains on our room down and mostly just hide out with Job.

We listened to a lot of beautiful music and did crafts and read books all morning, plus the weekly line change fun for which we wore our matching masks.

Day 16 Post-op Updates

10:00pm update:
I've gotten a lot of questions about his swallow.

He's been choking when he swallows stuff (and then that can go into his lungs which is dangerous) so they don't let him eat or drink. He has a NG (naso gastric) tube going down his nose into his throat into his stomach that gives him this formula stuff instead.

He has a paralyzed left vocal cord from his first surgery in April 2016. He learned to swallow despite it but extubation probably messed up that process. He should be about to regain his ability but it will take a while.

He had a NG tube from April to July 2016 (only June and July at home). He learned to drink a bottle way way faster than anyone expected, though we couldn't ever figure out how to nurse and swallow. Many HLHS kids are on feeding pumps for a long time, so this was the one thing (or it feels that way) that he's done to exceed expectations.

Being on the tube this long post-op and even longer still at home is really really frustrating because it impairs his quality of life so much. And we can't eat or drink around him because he can't eat or drink. Friday I was so over it and thinking it wasn't worth the trade. It is. It really is. But it's hard. And if it's this hard inpatient, how hard will it be at home?

9:00pm update:
I am so very thankful for today. Friday I was just despondent and overwhelmed, but today has been restorative.

Job has had a better day than I could have hoped. He still had several fits about wanting food/drink but we were able to manage them better by moving around the room and/or offering a "water pop" as a small comfort.

We had to do about six blood draws today for his MMF panel, to see how his body is metabolizing one of his immunosuppression drugs throughout the day, so the nurses were in our room a lot but otherwise we only saw the resident and the attending and Child Life.

We're really loving our newfound freedom of movement.

4:00pm update:
I woke up today fully expected today to be miserable. And it started out that way!!

But Job was so very miserable that we were given two very great mercies: a water dipped sponge every hour (no more dry mouth!) and days off cardiac monitors (we can go anywhere in the room! well, when not on a feed... but the pump is on a pole that can be wheeled around with us).

It's only been a few hours but life is sooo much more bearable for Job now.

8:00am update:
Job is almost back to full mobility. He pulled himself up to stand several times and I think he'll be standing independently by the end of today or tomorrow.

He doesn't have his baseline stamina yet but he's getting there quickly.

We're hanging out on the floor a lot today, on the mat they gave us. His cardiac monitors have long wires but he tube feed doesn't. Every three hours he gets a feed for two hours (though we'll continue to work on condensing that further), so he'll get an hour off soon and then can move around more.

But he is very very sad to not be eating and drinking. He knows foods and beverages exist and he keeps signing for them. He just can't figure out swallowing post extubation to not aspirate.

He's had some epic tantrums where it's hard to keep him safe; trashing and climbing so much. Last night he saw my water bottle and screamed and hit and kicked for 38 minutes. I would hold him until he was about to throw himself out of my arms and then I'd lay him on the crib until he was about to concuss himself hitting his head on the crib, then I'd hold him until I couldn't any more and put him back on the crib...

And he's had a couple more tantrums today, too, and just isn't interested in my distractions. He doesn't understand why I won't give him what he's asking for.

Day 15 Post-op Update

9:00pm update:
Job is now NPO. Nothing by mouth. We're so so sad, but that's nothing compared to how he feels about it.

The hope of going home without a NG tube seems nonexistent tonight. And SLP doesn't consult on the weekend, so we won't try to eat/drink again until Monday. I'm trying to think of this as two extra days of healing rather than two days of no progress.

He'll probably be off viral precautions in a day or two which would finally mean his brothers can visit him.

Though he's feeling better and more mobile and more himself, he's withdrawing into himself more and more. He's bored. He's annoyed. He likes to cuddle but has little to no interest in any of my stories or songs or games or silliness or efforts to engage him.

4:00pm update:
SLP came back and Job again aspirated 50% of the time when eating or drinking. She's going to leave the decision to the team, so we'll find out later today.

We also had our first transplant training! There are six transplant coordinator nurses and they divided the alphabet into thirds, so we have two nurses in particular following us (as we're in that last third of the alphabet) that we have gotten to know through the listing process and now transplant process. All six nurses and five doctors on the Heart Failure and Transplant team are intimately involved in Job's care. These two just especially. They're who I call first. They're the two we're closest to in this new TX family we're trying to get to know.

We sat for an hour and a half and talked about all sorts of aspects of his care. She thinks it's likely we'll discharge mid week next week, depending on how his echo from this morning looked, how his immunosuppression med levels stabilize and how his swallow study stuff all shakes out.

12:30pm update:
Rounds are later here on the floor because the team rounds on the CICU first. It's very different being on the floor and I knew it would be. But the transition TO the floor is so very complicated and I had forgotten that.

I'm thrilled that we're here, absolutely. But policies are different, the entire staff is different, expectations are different. We're on our own much, much more - nurses only take vitals very four hours and, if meds aren't due in between, we only see the nurse at those four hour marks (unless of course something happened/we called for them).

I've spent all of today in near constant communication with various providers, making sure all the nuances of Job's care are properly shared with the correct people. Little tiny emphases here and there actually make a big difference. Small miacommunications add up to big ones quickly.

And I'm advocating hard for help with his swallow and trying to explain years of concerns and interventions and pushing for formal studies to both help him get back to his baseline feeding and drinking and his speech delays.

I'd like to crawl in a little bubble and hide away from people for a few hours but that just hasn't been possible today and it definitely won't be possible next week. But the weekends here on the floor are very very low key. Almost nothing changes and only the most essential providers are on shift, so Job and I ought to have lots of alone time to recharge our introverted selves.

10:00am update:
Job is really struggling to take his two oral meds. SLP came by and I addressed that concern with her and we tried to give Job yogurt and honey thick liquids and he aspirated on both. I asked that she please please come back this afternoon when he's better rested and that's the plan. But I'm seeing the writing on the wall that he'll get his purees taken away.

He was really active all morning, playing and sitting up independently and interacting with people coming in the room.

Day 14 Post-op Updates

11:00pm update:

Here's Seth's summary update for the day:

Job is out of the CICU! This is our first night in the stepdown ward, aka surgical floor, aka River 6, aka River C. It has a lot of names. No matter what you call it, it's a huge step!

He is still on viral precautions, but his sats and respiratory rate are well enough that he is no longer on any oxygen support.

Job has been pretty withdrawn and lethargic the last several days as he slowly regains strength and recovers from his cold virus. We were able to get him to sit up for small segments of time today, but he tires quickly.

Speech and OT are going to visit Job tomorrow, so hopefully we will make headway in those areas while we are here in the ward. As of now, he is still only cleared to eat applesause and other pureed foods.

One major thing that must be accomplished before we go home is stabilizing the immunosuppressant levels in his blood. This is something we are going to learn more as we go, and we will update more as we become more educated on managing and protecting his body with a now compromised immune system.

6:07pm update:

Job has had two big fits today and cried audibly and pulled himself up to sit and even to stand, using his crib. What prompted such an energetic burst of activity and emotion? The sight of food and drink.

It's really exciting to see him be so interested in something and to make sounds and to move his body... but it's really sad because he does all of that work but we can't reward him. We just try to tell him we understand what he wants but we can't give it to him even though he's asking really nicely.

4:00pm update:
Job went down to .5 LPM of oxygen when we arrived on the floor and then after an hour or two of that he pulled off his cannula and hasn't desatted since. Well, he has but then he coughs and his sats pop right back up. He hasn't desatted and stayed down there in the 80s.

We're getting all moved in to our new room. They brought over a play mat and we've spent a lot of time rolling around on it and cuddling on it. Eventually I'm sure we'll spend more time in an upright position but right now we can only sit up for a few seconds here and there.

As a a transplant kid, Job will from now on always get his own, single room. The ICU, is, of course, all single rooms, but the floor has a majority of double rooms. It's really nice to have our own bathroom (ICU bathrooms and showers are for patients only) and our own fridge and such a huge room with lots of floor space (and not very many machines!!), and the fold out couch can be pulled up right next to Job's bed (unlike the bed in the ICU which was pretty tucked away from Job). We can eat and drink in here too, although we can't let Job see us do so or he gets really upset because he wants to eat and drink too. It's just really nice to get to do whatever we want without all of the ICU precautions (that are totally reasonable for the level of care required there).

1:00pm update:
Here we gooooo!

12:00pm update:
 All packed up and ready to go. He just sits and watches everything skeptically, with his legs crossed.

10:30am update:

WE'RE MOVING! Eeee!

His Art line gets to come out, his PICC line can be hep locked, he can wean off oxygen, more meds switched to entral... fun stuff! But no more ICU!

We went down to 1 LPM of oxygen at 100% FIO2,

8am update:
Cuddles make everything better. He's bored of stories and my attempts to get him to watch TV and my silly faces and games, but he doesn't have the stamina to do much else yet. Even just a few hours into this day I'm so excited to see a huge difference in his responsiveness compared to yesterday.