Courage Under Fire

The patient was a teenager, but he looked like he was 12-years-old.
In the 45-minutes I was with or near him, he passed out four or five times.
His normally-beating heart was betraying him, his atria quivering when they should have been contracting in synchrony.
It's not the sort of thing a young man's heart typically does.
We started two IVs on him; the ED added a third.
His heart rate never dropped below 130 beats per minute, and twice -- once when we moved him over to the ED bed and another time for no good reason -- accelerated to 280 plus.
Eventually, the ED threw the drug box at him -- Amio, Mag, Versed.
Before the cardioversions began they even gave him some Etomidate.
Anesthesia came down with their bag of tricks as the docs considered intubating him, but this kid was tough.
He maintained his own airway and defied our system's plans to take away his ability to breath unaided by machines or people, probably the most basic proof we have that we're alive.
The docs shocked him 5 times. A helicopter was called, and by the time they took him to the city he was doing much better.
As they wheeled him out the door I tapped him on the shoulder and told him to hang in there.
He nodded, but it seemed like he was already dealing with his condition better than any of us.
Today was a bad day, but not the worst for this young man in his brief life.
Diagnosed with a form of bone marrow failure and chronic anemia when he was just a few months old, the patient's disease has left him with an alcoholic's liver and a 90-year-old's heart, and he bears a 12-inch half-moon scar down his abdomen from where they'd removed his spleen at one point.
And despite all this, despite having a million and one reasons to complain, to feel sorry for himself, to do any one of the legion of things I might do if faced with a similar path, our patient was calm, almost stoic.
He answered questions and described his condition with the maturity of someone three times his age.
I don't know that I've been more impressed with a patient.
We hear a lot about courage and heroism.
Most of it is crap.
This kid was the real deal. He knew how sick he was, better than us -- better than ANY of us there that day, and he was the calmest one in the room.

The patient has what's known as Diamond-Blackfan anemia.
If you haven't heard of it, don't sweat it.
There are only 500 cases in America.
The condition is a member of the family of inherited bone marrow failure syndromes (IBMFS), and according to imedicine.com it's incidence in the U.S. is somewhere between 2 and 6 cases per million people.
It usually appears first in early infancy and is marked by a pure red blood cell aplasia, which means that the patient's bone marrow lacks any red blood cell precursors.
About a quarter of those cases result from gene mutation; the other 75 percent, apparently, appear mysteriously.
The treatment generally includes lots and lots of transfusions, which can lead to cardiac arrythmias, congestive heart failure, iron overload, stunted growth and infections.

For my partner and I, the acute dilemma was twofold.
The patient presented with a rapid AF in the 150s, but also threw pretty frequent runs of Vtach.
It was a unique ECG strip, to say the least.
His pressure was Ok, 124/70 bilaterally, but he was able to tell us that he has had episodes of acute hypotension with Dilt.
So we worried about dropping his pressure.
The Vtach was problematic, too, because we worried about giving Amio due to the hepatic issues, what with Amio being excreted mainly by the liver.
We ended up contacting med control.
The online doc, having already spoken to the city specialist who treats this young man, told us to give a little fluid but, as long as he continued with a stable hemo status, otherwise just observe.
Given how complex his treatment course in the ED became, I'm glad this is the course we took.
Sometimes less is definitely more.

Even as I write this, I wonder how this patient is doing.
Unless we return to his house, I doubt I'll find out.
And if we have to return to his house, it can only mean that things aren't going well.