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.