It's hard to believe this was all one day. I woke up at 4:30 AM to sign on to Ticketmaster and try to get tickets to Taylor Swift's Paris concert. Ariel, the verified fan, was on a red-eye, so I logged into her account. We texted and video called while I was stuck with 800K people in the queue ahead of me. By 5:30 AM, it was called. The Swifties took down Ticketmaster yet again, Euro edition, and the sale was paused. I miraculously went back to bed.
I spent the morning "working" or whatever constitutes working for me lately. I tried to do some deep thinking which, in hindsight, is a total joke. My brain was overwhelmed with uncertainty after Jude's last scope, and my house was a loud construction zone. I got a call around 11 AM that Jude's breathing had deteriorated rapidly.
Jude's last scope was on Thursday, July 6th. I have the workings of a post about it kicking around, but things have escalated so quickly that it is now woefully outdated. Here's the abridged recap:
Leading up to Thursday, Jude's breathing was deteriorating. He needed multiple doses of his racemic epi, a steroid, to breathe comfortably. Due to this increased need, the doctors, specialists, and nurses agreed to bring Jude into the OR for another dilation and DLB scope to understand the change in Jude's status. The nose shouldn't respond to the racemic epi, but it appeared to. Most likely, mucosal inflammation in his subglottis was responding to the racemic epi, which is not unexpected for a patient with his airway.
The DLB showed a progression of both his subglottic web and subglottic stenosis. His tracheal stenosis actually looked open but could not compensate for the critical passage above. The plan was to keep Jude's inflammation minimal and hold him steady until his August 9th surgery. The toolkit to do that was:
None of these are permanent solutions, but we'd hoped these temporizing measures would buy us time intermittently to get to August 9th. They didn't.
On Tuesday morning, things escalated very quickly. Jude was having a lot of trouble breathing, and the whole bag of tricks wasn't helping—something needed to be done. Dr. H called and told us the plan to bring him into the OR for a nasal dilation and to place a PICC line that could stay in until and through his surgery so that in an emergency, we could give him what he needed quickly and not be fumbling to place an IV.
By 1 PM, Avery let me know that much bigger conversations were happening about the next steps for Jude's care. I told Avery I'd be in by 3 PM, but she said we needed to speak sooner. I got in an Uber, and at 1:50 PM, we all got on Zoom. The cavalry, including the NICU, other ICUs, ENT, cardiothoracic surgeons, and anesthesia, were all discussing how to treat Jude now that his condition had progressed and max medical intervention wasn't helping. The team agreed there was a strong possibility they wouldn't be able to intubate (put a breathing tube down his airway through his mouth) or trach (put a breathing tube into his airway through his neck). Jude was already showing evidence of tiring, so, likely, Jude wouldn't be able to breathe on his own, or he would go into respiratory or cardiac arrest and stop breathing suddenly. We needed a different intervention.
They agreed and decided to bring him to the OR to cannulate to ECMO. There was massive urgency. Given his deterioration, it would be reckless to continue without current treatment and leave him vulnerable. If he had an episode in the middle of the night, we'd be doing an emergency surgery. We needed to do it with every resource and preparedness possible in a controlled environment.
As we chatted, Dr. H had to excuse herself to care for Jude quickly. I told the room I was 5 minutes away. By the time I got there at 2:25 PM, Jude was already in the OR. I waited in his empty room for Ariel to arrive.
We met with Avery and Ariel's Karen (charge nurse, it turns out we had different Karens) and made our way to the family waiting room on floor three. There we met our new Becky (social worker), named Kayla. She told us his new digs would be in the medical surgical ICU (MSICU) and not to expect him there until 7:30 PM.
We got a call from Dr. B around 4:45 PM that they had successfully gotten him to sleep with anesthesia using a type of breathing intervention called an LMA, something they weren't sure they would be able to do. However, he still had to do the actual cannulation to ECMO, which involved putting different tubes in different veins and arteries in his body. I don't understand the details, but things were going well, but there was still more to do.
Once successfully on ECMO, the next step is to advance his surgical repair as quickly as possible. He cannot be on ECMO for thirty days. ECMO (Extracorporeal membrane oxygenation) is when a heart-lung machine pumps blood outside of your body, removing carbon dioxide and sending oxygen-filled blood back to the body. Essentially this is a bypass. It is very rarely done proactively like this. ECMO is often done during surgery as a life-saving and emergent measure.
To prove that Jude is one in a million, they are approaching his ECMO in a highly unusual way that may have never been done before. A funny thing about ECMO is that even though the machine gives blood oxygen, patients still need air to move in and out of the lungs to ensure the lungs don't collapse or get filled with secretions. This is especially important for Jude because although his airway is a disaster, his lungs work great, and we don't want that to change. Therefore, usually, ECMO patients are either 1) completely sedated and intubated to allow air movement or 2) completely awake and moving air in and out of their lungs on their own. For Jude, neither is an option because he can't be intubated and when he is awake, he starts working hard to breathe, which they want to avoid. Therefore, they chose an in-between, and he is lightly sedated only to allow some air movement. They weren't sure this would go well, but it seems to be okay thus far.
Ariel and I left the hospital and got some shmancy sushi and a few drinks to take the edge off. At dinner, we got a call from Dr. B that the ECMO was successful, and ORL affirmed the incredibly narrow airway and the need for this procedure to keep him stable.
Finally, around 8 PM, we could see Jude and chat with the MSICU attending and Jude's anesthesiologist. He was stable on ECMO and in good hands. I took an Uber home, fought with Trevor over something trivial, purged my enormous boobs, and went to bed. I got a few hours of what constitutes as sleep these days.
In the morning, I caught up with Ariel and the doctors. Overnight, Jude's arm flinched a few times. Neurology reviewed the video and agreed that it was likely a seizure. They hooked him up to an EEG, turned him into a little rainbow medusa, and put a camera on him. From here, we wait and hope there isn't another seizure. If we can make it 24 hours, that's great; 48 is even better.
So far, today has been uneventful. I sit here, trying feverishly to write it all down and prepare for the next few days. We'll need all the strength we can get going into Friday; and any Ambien we can get our hands on. I'm officially going on work leave. My support systems are in place. It's almost time for the big event. Wish us luck.