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.