What the heck is wrong with this guy?
I kept repeating the question, though I was the only doctor around to answer it. It was 4 A.M. An ambulance had just brought a 50-year- old alcoholic into the emergency room, and he had everyone confused. At first he told the paramedics he was coughing up blood, so they wheeled him to the medical section of the ER, thinking he might have tuberculosis. Then he complained of leg pain, so they hustled him over to the surgical side.
Hey, my chest hurts, he mumbled as they lifted him onto a stretcher.
Heart attack? One medic raised his eyebrows at the other. Back to the medical side.
It was at this point that I jumped in. I had just finished with another patient, and besides, peripatetic stretchers are hard to ignore.
What’s the story, gentlemen? I asked.
The medics looked up. Don’t know, Doc. The shelter called us to say he was acting weird and complaining of pain. Problem is, he can’t tell us where it hurts.
What’s his name?
MR. BOYLE, I half-shouted, WHAT SEEMS TO BE THE PROBLEM? (Someday an enterprising researcher will find out why ER doctors always half-shout.)
His answer was cut off as the medics eased him onto a hospital bed, so I asked again.
Oh, can I take a rest now? he moaned.
Rest? From what?
From the ... from, you know, it hurts. My shoulder ... Mr. Boyle’s voice trailed off. His face had the gaunt, used, stubbly look of a lifetime drinker. All he could tell me was that the pain had started two weeks earlier off and on, like, that he hadn’t had a drop to drink for five days, and that he wasn’t taking any prescription medicines. Every time I asked him to pinpoint the pain, he waved his hand over a different part of his chest or abdomen. His physical exam revealed nothing.
I was stumped. And laboratory tests being the last refuge of a stumped doctor, I ordered everything: a blood count, a check on his electrolytes (the concentration of certain ions in the blood and other body fluids), a test to make sure his cardiac enzymes were all in order, an EKG, a screen for drugs and other poisons, chest X-rays, and a blood gas (to measure the acidity of his blood and the levels of oxygen and carbon dioxide). I drew the gas myself, since it requires a blood sample from an artery rather than a vein and is harder to get. When I grasped his right wrist to feel the pulse, which gives away the artery’s location, I had a bit of trouble finding it. No matter. The left wrist had a fine pulse, so I took my sample there.
Waiting for the test results, I kept drifting back to Mr. Boyle’s bedside. Each time he was lying in a different position, like someone with a kink in his back who just can’t get comfortable. And he kept repeating one question, almost annoyingly: Can I rest now?
From what? I would ask, hoping this time I’d get a more telling answer.
From the pain, he’d reply. And we would go around one more time with the vague-answers-to-half-shouted-questions routine.
I considered a shot for his pain, but Demerol would make his blood pressure go down, and it was already a bit low. Besides, I didn’t know anything about the pain, not even where it was.
What the heck was wrong with this guy?
Just before I got the results of the first lab tests, Mr. Boyle decided he needed to urinate, which was convenient because it also meant we could do a urinalysis. One quick look at the sample gave me my first hard clue: Mr. Boyle’s urine was a muddy brown, the color of kidney trouble.
Now things made some sense. Alcoholics like Mr. Boyle are rarely picky about where they pass out to sleep off a binge: often it’s on a hard surface like a sidewalk. A concrete bed puts extra pressure on the body, crushing muscle tissue and releasing myoglobin (an oxygen-storing molecule, the muscular version of hemoglobin) into the bloodstream. Dutifully, the kidneys’ tiny tubules filter out the myoglobin as they do all the body’s wastes. But it’s a kamikaze mission, since myoglobin is toxic to the cells that line the tubules. If all this occurs in a person who is also dehydrated--as alcoholics usually are--the tubules don’t have enough water to flush out the dead cells, and they sludge up. If you looked at the urine under a microscope, you would see twisted tubule-shaped casts of cells that, like Pompeians fleeing the eruption of Mount Vesuvius, were fossilized in the act of dying.
The blood tests confirmed my hunch: the measures of Mr. Boyle’s kidney function were seriously out of whack. What’s more, his chest X-ray showed that fluid was leaking into his lungs, most likely a result of his kidney failure. That would explain his chest pain.
Satisfied with my diagnosis, I called the admitting resident. Bob, got a simple one for you: acute kidney failure. Just hydrate him carefully and watch his labs, and he should be okay in a few days.
Bob’s sleepy voice mumbled, I’ll be right down.
Without giving the case another thought, I went to sleep. After all, with good management, and as long as the damage hadn’t been too severe, Mr. Boyle’s tubule cells would regenerate in a week or two.
The next day, I ran into Bob. So is Mr. Boyle better? I asked him.
Mr. Boyle? He gave me a puzzled look.
Yes, you know: kidney failure.
Bob’s eyes opened wide, as if anticipating my surprise. He died.
My heart froze. What? Then a quick, hopeful thought: he must mean a different patient. No, Mr. Boyle. The alcoholic I admitted to you at 5 A.M. yesterday.
Yes, that’s the one. He died.
Jesus. My brain sped backward, like a videocassette rewinding, to sift for missed clues. What happened?
Soon after he got to my floor, his blood pressure started dropping, Bob said, shaking his head. No one could figure out why. He explained that in order to better monitor Mr. Boyle’s falling blood pressure, they’d put a catheter into his subclavian, a large vein found under the collarbone. To check the catheter’s position, they took another X-ray and noticed that his mediastinum--the space between the lungs that contains the aorta, among other things--looked wider than normal, and wider than it had in the earlier pictures. Then we lost the pulses in his legs, Bob continued. By the time we got him transferred uptown to the thoracic surgeons, he was gone.
A wide mediastinal shadow on a chest X-ray, terrific pain, and asymmetric pulses. My brain’s video playback slammed to a stop at the scene where I was feeling Mr. Boyle’s right wrist for its pulse. Subtle as it was, I now realized it was the giveaway.
Remember how his pain kept moving around? asked Bob, bringing his story to an end. It’s because his aorta was dissecting.
The aorta, which Aristotle called the great artery, receives the body’s entire volume of blood from the heart’s left ventricle once each minute. It is around an inch in diameter and shaped like a question mark, rising from the heart toward the neck, arching back to the vertebral column, then dropping alongside it into the abdomen. With each jet of blood, the aorta must first swell, then smoothly contract to feed the body’s network of arterial tributaries. And it takes this sort of pounding more than 2 billion times over the average lifetime, which is why nature built the aorta with an extra-thick layer called the media, made up of muscle and tough, interwoven collagen fibers. The media is sandwiched between an inner layer of epithelial cells and elastic fibers and an outer layer of collagen and elastic fibers; without its strength, the wall of the aorta would balloon out like the inner tube on an old tire.
But strong as it is, the media is vulnerable to congenital diseases like Marfan’s syndrome, which produces faulty collagen. In fact, a ruptured aorta is the most likely cause of death in Marfan’s. The media can also fall prey to inflammation or, especially dangerous, to high blood pressure. With high blood pressure, the blood surges through the aorta with a stronger-than-normal force that can tear a hole in the aorta’s smooth inner layer, exposing the media to the shearing force of onrushing blood. Once the blood gets access, it just rips through the collagen fibers like a run in a stocking, rapidly traveling down through the media. But this run--called an aortic dissection--affects more than the aorta: the swollen, ballooning vessel walls can block the blood flow in any or all of the narrower arteries that branch off from the great vessel.
Fortunately, this catastrophic scenario is relatively uncommon: only about 2,000 cases are reported each year. And controlling blood pressure greatly reduces the risk. But dissection is a devilishly difficult diagnosis because it can start up anywhere along the aorta’s length. If, for instance, it begins near the carotid arteries, which supply blood to the head and neck, a stroke can result; if the spinal cord’s arteries are hit, the patient may wind up a paraplegic. The kidneys’ arteries are affected in up to half the cases--as were Mr. Boyle’s, which was partly the cause of his kidney failure.
Untreated, a dissecting aorta is almost always fatal. But there is a procedure--devised in the early 1950s by the famous Texas heart surgeons Michael DeBakey and Denton Cooley--that gives patients a fighting chance. DeBakey and Cooley put the patient on a heart-lung machine, used two clamps to shut off the flow of blood in the damaged section of the aorta, and then quickly replaced it with a graft. Thanks to their boldness and to modern refinements, the mortality rate for aortic dissection has dropped to one in four.
Sometimes the body even repairs itself. The blood surging through the media may occasionally tear a second hole through the aorta’s inner lining and reenter the normal flow. Over time, the false channel left in the aorta may grow a normal lining, leaving the patient with a double- barreled aorta.
Mr. Boyle, however, had not been one of these rare cases. His aorta had been dissecting for days, and although it was very likely beyond repair by the time he came to the emergency room and he had few of the condition’s other signs, I still spent much of the next few days kicking myself. I’d missed it. I replayed the whole episode over and over in my head, always stopping at the moment I tried to take that pulse. What I wouldn’t have given to turn the clock back and redo the scene--getting it right this time, of course.
Three days after Mr. Boyle died, I dropped in at a teaching conference at the hospital. As I walked in, a second-year resident looked up and chirped, Dr. Dajer, did you hear about the dissection?
Bad news sure travels fast, I thought, but I simply answered, Of course I heard about it. I was there.
Oh, really? He smiled politely but looked dubious.
The resident sitting next to him joined in. Yes, Bob picked up a dissection.
Well, it’s not exactly as if we ‘picked it up,’ you know, I answered somewhat shortly, thinking, do we have to discuss this so soon? And in public? Besides, I lamented, it helps to make the diagnosis before the patient is in extremis.
Now there were two puzzled looks. It was the first resident who finally spoke up. Oh, he’s not that bad off. Actually, he’s being transferred--he glanced at his watch--as we speak.
I was lost. Just then Bob walked in, holding an X-ray jacket and looking very pleased with himself.
Bob, what’s going on? These guys tell me Mr. Boyle’s being transferred. Didn’t you tell me he’d died?
His smile vanished. Yes, he did. But look at this.
Bob pulled the films out of the folder. A CT scan showed an aorta with the unmistakable signs of a dissection.
Did you take this before he died? I asked.
Dr. Dajer--Bob’s grin came back--this is another patient.
Yes. Yesterday I was making rounds with one of the medicine teams. They had a 70-year-old Chinese gentleman who’d had a heart attack but who kept having chest pain. They’d readmitted him to the coronary care unit, but his second workup was normal. They couldn’t for the life of them explain his pain, he didn’t speak any English, and they didn’t always have a translator. So they figured it might be his ribs and tried to snow him with codeine. But the pain just got worse.
As soon as I heard about it, I thought about Mr. Boyle. And click! The little light went on in my head. ‘Let’s get a CT scan,’ I told them. They thought I was nuts--after all, he’d had the pain for a week. But then they figured, what have we got to lose?
Bob couldn’t stop beaming. Which was fine because by then neither could I.
Two dissections in three days! I exclaimed. Do you know what the odds of that are?
Pretty low, Bob replied.
Let me see that, I said, reaching for the patient’s CT scan.
I stared at the telltale shadow for a long moment, then put it away, satisfied. Though I didn’t get the chance to replay the scene with Mr. Boyle, Bob did. Mr. Boyle had left behind a priceless legacy, a gift the second patient would never even know he had received.