Identifying the Assassin
Naming and blaming a syndrome twenty years after it tried to kill me
Back in 2004, a week before I was supposed to move out of a dude’s basement and back into a real apartment, my aorta dissected. I should have died on the spot. I definitely should not have survived the two days of misdiagnosis I got at the hospital, including an overnight in the psych ward when they thought I was making it all up (after they thought I was just there for free drugs).
And I absolutely had no right to randomly be driven to the hospital, by said dude (who took the last of my cash while I was in an oxygen-starved stupor, and who wouldn’t accompany me in because he was too high to be seen in public), only to find myself at the home base of an internationally-renowned cardiothoracic surgeon.
Funny old world.
My memory of everything in the vicinity of that event was blasted to atomic beach glass by the anesthetic I was on during my nine days in the ICU, but I remember a couple bits and pieces very well. One of the clearest shards is of Dr. Moront telling me they were going to sedate me, to get my apocalyptic blood pressure under control, and then he was going to assemble his best team overnight, some hauling ass back from Chicago at that moment.
He'd do the aneurysm repair, and the valve replacement now necessary due to damage from the prolonged dissection, in the morning, “if you survive that long.”
I seem to recall him telling me, either then or during my recovery, that my chance of survival was about three percent. My wife remembers it as two. At that point, it probably doesn’t matter.
In the immediate aftermath and recovery, I wasn’t overly concerned with chasing down an explanation. I went through the same hamster wheel of questions with every doctor, nurse, and mildly-educated bystander in a ten mile radius. Yes, it was a dissection. There’s a pig valve in there now. We don’t have my mom’s family medical history, so I don’t know what might have gotten passed down. No, it’s not Marfan Syndrome. Yes, Abraham Lincoln had that. No, I’m not insured.
The closest thing anyone could come up with was that it was an undiagnosed birth defect, aggravated by hereditary hypertension on my dad’s side, and that with my history of caffeine and alcohol consumption, poor sleep hygiene, and rock and roll drumming to that point, it’s even dumber dumb luck that I didn’t just fall down and die mid-show in a dive bar in the middle of Indiana at some $50 gig.
A year after all that, unbeknownst to me, the first instance of Loeys-Dietz Syndrome was documented. Nineteen years after that, an immediate family member with joint hypermobility issues got genetic testing and found themselves with markers of a type of that syndrome. One of the symptoms of that syndrome is a preponderance of aneurysms around the aortic “tree.”
I stress that I have not yet begun genetic testing. It could still be a real wacky coincidence. But it’s kinda hard to think so at this point, right?
Right now, the concern is focused on my family member. I’m not having joint issues, and any problems related to this syndrome on my radar are being monitored. I’m three frightening surgeries in now, so I’ll be getting annual CT scans for the rest of my life, and my blood pressure is managed. Despite being overweight, my cholesterol is low, and my labs come back good every year.
But it’s weird, what a tangle of emotions come from just putting a name – even a potential name – to the thing that tried to take me out. I’ve stepped on more than my share of rakes since 2004, but almost all the good things in my life happened after that, not before, and I was given a mathematically-preposterous second chance to grow into a somewhat better person than the one who stumbled into the ER that night.
It really brought a couple things out of the shadows, including the ever-present but unspoken bugaboo of waiting for the other shoe to drop. This happened in 2020 when I had my abdominal aortic aneurysm repair, and at the risk of sounding melodramatic, I’m not sure I’ve got the gumption to survive another thing like that. I don’t know if knowing more about this syndrome will lead to any additional preventative measures or quality-of-life enhancements down the road a ways, but more information is always better than less.
Tomorrow I talk to the big man and find out what to do next. Nothing’s functionally changed in my life. But boy, do things feel extra goopy and uncertain under my skin tonight.
Jesus... I knew of your heart issue several years later, and far more recently you posted that at the time a doc told you that if you'd ever taken one bump of coke you'd have died, but I didn't know about this episode in 2004. I'm grateful to have you here now, and not just reminiscing with Mark about you on Facebook.