Bomb Cyclone Jack Update

Disclaimer: Long post, plausibly informative, hopefully painless, possibly boring. Don’t read in traffic. Maybe make some popcorn beforehand.

Let me preface this Bomb Cyclone Jack update by noting that it’s intimidating to satisfy expectations to be evocative and interesting after trekking to the hospital in a snow storm and spending 8 hours shuttling a 4-year old (excuse me, almost 5-year old) from one awkward, overly-fluorescent holding area to another, cavernously dark one.

Poor me.

Anyway, Jack had his transcatheter (or “cath”) procedure today (yesterday). Because he was going under for the procedure, his mom put a premium on being at the hospital. Like the rest of the Northeast, metro Philly was under a snow emergency, so I put a premium on mom not dying en route to being at the hospital.

School was cancelled, so Ryan put a premium on doing whatever mom was doing — and seeing his kid brother, of course, in order to dispense some fraternal love and lordly advice. Plus, Ryan loves to collect packets of condiments, which Children’s Hospital of Philadelphia has in even greater abundance than hand sanitizer. We live in a strange and mysterious world.

Preparations the night before were painstaking (and tedious to recount, so we’ll skip it). Think 21st Century Donner Party, minus the cannibalism. The morning of, after some deft 12-mph driving by mom along the City of Philadelphia’s snow emergency routes, we made it to the hospital unscathed if a little white in the knuckles.

The cath procedure Jack underwent has a frustrating backstory, the gist of which will be recognizable to anyone who’s played Whack-a-Mole at Chuck E. Cheese. If my Humanities-degree explanation is too facepalm for the science people reading this, or too eye-glazing for anyone else, you can skip to the end and smile at the cute kid photos. I won’t be offended.

Onward.

So, in Jack’s initial operation performed when he was five days old, called a Yasui procedure, his surgeon did two things: he patched a hole (called a VSD) in Jack’s septum, which separates the right and left ventricles, and he attached a conduit to connect Jack’s right ventricle to his distal pulmonary artery. This path is necessary to carry de-oxygenated blood from the body through the heart to the lungs. Kind of important.

Fast forward: the conduit, which is basically a piece of cadaverous tissue, was discovered to have shrunk pretty much uniformly across its length. They have no idea why this happens (and by “they,” I mean no doctor or medical researcher in the world), but it occasionally does.

This narrowing of the conduit caused the blood pressure in Jack’s right ventricle to climb, as the chamber worked harder to pump blood through a narrower and narrower space. Not good. So he had his second open heart surgery on Dec. 27, at 3 months, 21 days old.

Following some arcane post-op complications, it was discovered that Jack’s liver was enlarged. More detective work uncovered the cause: the VSD patch from his initial operation was now leaking — significantly.

Blood flow moves through the heart from right to left, more or less: de-oxygenated blood comes through the right atrium into the right ventricle, which pumps it to the lungs through the pulmonary arteries. (The geography of this setup was one of Jack’s two congenital heart defects).

The lungs then oxygenate the blood and send it back to the heart through the left atrium into the left ventricle, which delivers creamy delicious oxygen-rich blood to the body.

So it goes.

The upshot is that the pressure in a normal left ventricle is higher than a normal right ventricle, because the right ventricle has a comparatively soft job: pump blood to an organ that’s right next door versus pump blood all the way down to your pinky toes. The right ventricle is kind of a slacker, in other words, albeit a life-sustaining slacker.

Doctors hypothesize that, with Jack’s replacement conduit narrowed and his right ventricle pumping harder, the increased pressure was keeping the VSD patch (which is really just a flap, or baffle) smooshed tight against the septum wall. With his new conduit now functioning properly, and the blood pressure in Jack’s right ventricle abruptly dropping back to normal levels, the patch sort of relaxed and permitted blood to leak around it.

So we are potentially looking at a third open heart surgery for Jack, this time to repair the VSD patch.

However, this cath procedure might provide a surgery-free fix.

In brief, a transcatheter procedure involves running a tiny snake through a large blood vessel into the circulatory system. Caths can be exploratory or they can do things, like place stents, patches, valves, or maybe even pick up pennies from the bottom of a fountain. In any event, not a job for the overly-caffeinated.

As Jack was being prepped, we were briefed in the world’s most migraine-inducing consultation room by Dr. Jack Rome, director of CHOP’s cath lab.

Dr. Rome, who with absurd modesty once described himself in local media as “a glorified plumber,” told us what he was going to do and what the goals were.

First goal was exploratory, to get a crystal-clear picture of what was actually going on with the VSD patch leak.

Second goal, if feasible, would be to place some number of “devices” along the boundaries of the leak. The devices, it turns out, are pieces of metal mesh. They work by coagulation, i.e., as the blood clots at the insertion point they grip the tissue and hold in place. Sort of like repairing a rip in your screen window, except not at all like that.

Dr. Rome’s demeanor can best be described as unalloyed. Maybe he’s actually a fabulous guy to have over at your Super Bowl party, but that wasn’t my impression.

Doesn’t bother me a bit. I’m not looking for new friends. I don’t even need to like any of these people (though in most cases, happily, I do). I want them to save my kid’s life. And Dr. Rome, who I imagined several times was mentally checking his watch while talking to us, has a jacket which is impressive indeed.

We would get a call, Dr. Rome informed us, if he decided to place the devices. And he would only do that if he determined that doing so would have some (whatever) chance of working. In response to Jack’s mom attempting to nail him down as far as probabilities, Dr. Rome said, “There are no tables.”

Tell me about it.

Well, we got the call. Dr. Snuggles apparently thought it was worth the effort.

Jack’s status when we left (besides being hungrier than a wolverine with his head stuck in a jar — that’s why he’s whine-crying in the top photo) is that the devices were placed successfully but that there’s still a lot of residual leakage. Doctors are hopeful that once he starts clotting it will make the devices work better. But we won’t know for a couple of days.