Brain Storm

By Tony Dajer|Friday, October 01, 1993
Grandpa missed the toilet bowl.

Missed the toilet bowl? I felt instantly muddled. Now, what the heck does that mean? I asked Jean, the only nurse in the emergency room who could speak fluent Mandarin.

She pivoted to face my patient’s family--all six of whom were bobbing in a worried huddle. She repeated my question. More rapid-fire Mandarin.

It means that when Grandpa got up to go to the bathroom this morning, his aim was wrong, Jean said. He peed all over the bathroom floor. And then he started acting funny.

How funny? I asked.

More consultation.

Like he didn’t know what day it was. But he still wanted to go outside. In his pajamas. In October. They had to hold him down, and he started shouting. Since then he’s only gotten worse.

Throughout the conversation, Mr. Chu--its subject--had been sitting up on his stretcher eyeing us suspiciously, like a prisoner freshly behind bars. Then, just as Jean finished translating, he suddenly swung an arm over the side rail and thrust a knee up.

He’s going to fall! I yelled, lunging for Mr. Chu and grabbing his well-muscled shoulders. Jean, get security. We need restraints.

Heaving and straining, I tried to wrestle the 56-year-old flat with the help of two of his grandsons, who grabbed a leg each. The rest of the family swarmed around us, cajoling and pleading. Mr. Chu fought with animal desperation. Then, as suddenly as it had begun, it ended. Mr. Chu simply fell back and started dozing. Seconds later the security guard rushed up with a restraint vest. He stopped when he saw the peaceful, snoring form, and his eyes questioned me.

Why don’t you leave it here, I panted. We might need it if he acts up again.

Sure, Doc.

I started questioning the family again--through Jean--after we’d all caught our breath.

Did Mr. Chu take any medicines? I asked.

No, just some antibiotics and pain pills the dentist gave him three days ago for a tooth infection, came their answer.

Pain pills! I exclaimed. Immediately a list of mind-bending substances--some legal, some not--popped into my head.

What kind? I asked heatedly, hoping my sense of urgency would bounce off Jean and jog the family’s collective memory.

They don’t know. White pills, maybe. He took them for three days and had no problems, Jean said, shrugging.

It didn’t sound like anything, but just to be sure, I sent one of the grandkids home to find the pills. Then I continued pumping the family for information.

Until today he’s always been healthy? No alcohol, no strokes, no psychiatric problems?

The family, as one, vigorously wagged their heads. There was no language barrier here: their expressions clearly said no way.

I took Jean aside.

I have no idea what’s going on with this man, I confided. His temperature’s only 100.9 and his neck doesn’t seem stiff, so I don’t think it’s meningitis. There’s no paralysis, so a stroke’s unlikely. And it’s come on too fast for a brain tumor.

Jean peered patiently at me. Evidently she had faith that something decisive would eventually come out of my mouth.

What I do know is that his brain isn’t working right, I said, trying to be worthy of her faith, and we need to figure out why. Fast.

Mr. Chu, however, wasn’t cooperating. As if he’d heard me, our patient bolted upright, started moaning, and then resumed his escape attempt--this time by standing up and trying to jump off the stretcher. Again the family, Jean, and I wrestled with him, but like a panicked swimmer, he pushed us off. During the struggle his shirt slipped off, and I noticed streaks of black and blue on his flanks. Had we done that? I wondered. It was obvious that we needed to tie him down before we did him-- or he did himself--any more harm. The security guard handed over the restraint jacket and, as gently as we could, we strapped Mr. Chu into it.

Though the immediate struggle was over, I still had no idea what I was dealing with. All I knew was that a neon sign was flashing in my head--the same neon sign every emergency room physician carries. It reads prevent disaster.

In Mr. Chu’s case the most likely disaster was meningitis, an inflammation of the membranes surrounding the brain and spinal cord that can be deadly if left untreated. I didn’t think that was Mr. Chu’s problem, but to rule it out officially I’d need to do a lumbar tap so I could check his spinal fluid for bacterial infection. The problem was that before I could do the tap, I had to see a CT scan of Mr. Chu’s head, to be sure there was no pressure buildup: removing spinal fluid from a person with increased cranial pressure can actually herniate the brain--draw it down into the spinal column. To further complicate things, the CT technician had already gone home, and it would take a while for him to get back to the hospital. If Mr. Chu did have meningitis, he couldn’t wait that long. So I asked Jean to hook him to an IV immediately and begin giving him two grams of ceftriaxone--an antibiotic that kills almost all meningitis-causing bacteria--just in case.

Forty-five minutes later, the CT technician arrived, and we were ready to load Mr. Chu onto the mechanical gurney that slides into the hole in the doughnut-shaped machine. He was still asleep, so we took off the restraint jacket. But in the middle of the transfer he began to stir.

Jean, ten milligrams of Valium, quick! I said.

Jean immediately injected the medication into the IV port, but it was too late. Mr. Chu lurched into a sitting position. This time we pinned his arms to his sides and then secured him to the gurney with a wide Velcro strap. But the mad elephant stomping around inside his skull wouldn’t quit: he struggled and babbled until we’d pumped him with a total of 35 milligrams of IV Valium--enough to snow the pachyderm.

The CT scan, after our knockdown brawl, turned out normal. So did the lumbar puncture I performed right after we saw those results. The workup of Mr. Chu’s blood--which I’d ordered when he first came into the emergency room--showed a mild anemia but nothing else. Even the pills, which his granddaughter had finally retrieved, turned out to be nothing to worry about. The only odd result came when we tested his urine: it was tinted red, which meant that blood was present. But that could just mean he’d been hit in the kidneys during one of his struggles, or maybe he had a slight urinary tract infection. In any case, it seemed trivial next to the drama at hand.

Summarizing the results for the family, I had to admit that from a diagnostic standpoint all the sound and fury they’d just witnessed--the blood test, urine test, CT scan, and lumbar puncture--had revealed basically nothing. Still perplexed, I handed Mr. Chu over to the intensive care unit. But I couldn’t stop thinking about him. At home after my shift that day, I pored over Harrison’s Principles of Internal Medicine. Twice I called the resident in the ICU who was now handling the case.

Carbon monoxide poisoning! I announced the first time.

Um, sorry, Dr. Dajer. Blood oxygen content is normal.

Twenty minutes later I had phone in hand again. Mercury poisoning! I blurted.

The resident sighed. We asked the family about possible heavy- metal exposure. No dice. We’ve already called in the neurology and infectious disease consults. Neither has a clue. Maybe we should all just get some sleep.

He was right, of course, but as I tried to follow his advice, one maddening thought kept going round and round in my head: What the hell are all of us missing?

The next morning Mr. Chu’s case was presented to the assembled experts. Their overwhelming response was, Beats the heck out of me.

When the meeting was over, I detoured past Mr. Chu’s glassed-in cubicle. His smooth, rounded features swelled gently with the soft rhythm of his breathing, betraying nothing of the previous night’s fracas. But I would have preferred to see the raging elephant. He was now barely arousable: his brain, battered by its mysterious malady, was sinking relentlessly into deeper levels of coma.

My brain, however, kept stumbling along, generating lists of tantalizing clues and exotic diseases only to reject them. But it came to an abrupt halt when the resident I’d been pestering the night before appeared at my side.

We know what’s wrong, he said, waving a flimsy lab slip. I wheeled around.

Huh? How?

Thrombotic thrombocytopenic purpura, he enunciated carefully. TTP.

Thrombotic thrombocytopenic purpura. A mouthful that makes much more sense when broken down. A thrombus is a blood clot; thrombosis is clot formation. Thrombocytopenia is a decrease in the number of platelets (also called thrombocytes), which are tiny cell fragments that float in the bloodstream and are one component of blood clots. And purpura means bleeding into the skin and mucous membranes that leaves purplish marks like bruises on the skin. Together the words mean a condition of abnormal clotting that uses up platelets and leads to black-and-blue marks--just like the streaks I’d seen on Mr. Chu’s flanks.

Since last night his hematocrit and platelet count have dropped like rocks, the resident went on, sounding smug and exhilarated at the same time. Now it all fits. It’s just that the first count in the ER was taken too early to show it.

A hematocrit is a test to determine the proportion of red blood cells. The same machine that does the hematocrit also routinely counts platelets. Even under normal conditions, platelets make up only a minute proportion of the blood; nature’s trick is to turn them sticky so they’ll clump together and form a clot when there’s a break in a blood vessel, and then stop clumping once the hole is plugged. In Mr. Chu’s case, something had triggered a biochemical avalanche, so that his platelets were glomming together for no good reason. Since the platelets were being used up so quickly, the machine counted even fewer than usual, giving us our diagnosis.

But the effects of the unnecessary clots could be seen in more than just the hematocrit. The clots were essentially acting as microboulders in the bloodstream, against which the fast-flowing red cells were dashing themselves to bits. My mind jumped back to the mild anemia and red-tinted urinalysis from the night before. Now it made sense: the hue had been due to hemoglobin--the oxygen-carrying component of red blood cells, which gives them their color--released by the chewed-up cells and filtered out by the kidneys. There’s always a clue or two, I told myself ruefully, but there isn’t always a Sherlock Holmes around to make sense of it.

TTP has been known about since 1924, when Dr. Eli Moschcowitz of New York City described the case of a 16-year-old girl who was brought to his hospital with a high fever, extreme pallor, and pain in her arms. Within a week, before Moschcowitz’s disbelieving eyes, she lapsed into a coma and died as if smitten by an angry god. At autopsy, almost all Moschcowitz could find were innumerable microscopic clots plugging up her smaller arteries and capillaries. These tiny corks had completely cut off the blood flow to her vital organs.

Moschcowitz was never able to pin down the agent of disaster in his patient’s case--indeed, that agent has yet to be found. Nor does anyone yet know for sure where the problem originates, though most researchers think it lies in the pancake-flat endothelial cells lining the blood vessels, which ordinarily are responsible for activating the platelets in the first place.

What we do know is what happens when platelet clumping begins. Although any or all of the organs may be affected, the plugs reveal their most dramatic and terrifying effects when they hit the brain. By altering blood flow in random, shifting ways as they block first one and then another vessel in the brain, they spark an oscillating delirium that can progress to seizures, coma, and death.

Though ttp is mercifully rare, just two decades ago its diagnosis was tantamount to a death sentence--barely 20 percent of patients survived the platelet onslaught. Then, in 1977, some doctors tried infusing normal plasma--the fluid part of the blood, without the cells--into victims’ bloodstreams, on the theory that TTP came about because patients with the disease lacked some sort of anti-clumping factor. A number of their patients recovered in dramatic fashion. More recently other researchers have had great success with a more aggressive approach: drawing off and centrifuging the patient’s blood using a technique called plasmapheresis. The blood cells are returned to the patient and the plasma is replaced with normal stuff, to make sure the body is rid of any plasma-borne platelet- clumping signal. Today, with this technique, 80 percent of TTP patients survive.

Unfortunately, our hospital didn’t have the facilities or the staff to perform plasmapheresis on Mr. Chu. So the plan was to pump him full of normal plasma and send him posthaste to a hospital that did. But there was an unforeseen complication: Mr. Chu had no insurance. There are two questions a hospital asks about a patient in need of specialized care. The first is, What’s the diagnosis? The second is, Does he or she have insurance? If the answer to the second question is no, there’s no need to ask anything else. An uninsured patient’s only option is a public referral hospital, and these are notoriously overburdened and often don’t have any beds available. Mr. Chu’s case was no exception. No bed meant no transfer, and no transfer meant no plasmapheresis.

So for two days we watched helplessly while his coma deepened. On the third day he started to have seizures. Full-blown convulsions lifted him off the bed. His arm and leg spasms were so severe that they could be controlled only by putting him under general anesthesia. Frantically, his doctors gave him clean plasma, steroids, and every antiplatelet agent they could think of, then spent hours on the phone trying to convince someone-- anyone--to take him for plasmapheresis.

After five harrowing days the transfer went through, but Mr. Chu’s family was inconsolable. His brain, they were told, had undoubtedly sustained severe damage. The plasma and the drugs had staved off death, but his seizures were so severe and his coma so profound that no one could predict how much brain function could be salvaged--assuming he survived at all. En masse, the family followed him uptown. They diligently attended the hours-long plasmapheresis sessions; they quietly stood watch through the stormy nights of delirium and seizures.

In time, their attention and devotion began to pay off. Slowly, as the plasmapheresis cleared his system, the swirling storm clouds inside Mr. Chu’s skull gave way to the first thin rays of returning lucidity.

He’s sitting up! the resident announced to me one morning. A few days later, he stopped by to report, He’s speaking. Real words, too. Despite the good news, I couldn’t really celebrate. If Mr. Chu had received the proper treatment right away, he might never have been in such danger.

Six months later I called Mr. Chu’s home, just to see how he was getting on. A young granddaughter--the family’s only English-speaker-- answered. I explained who I was, then asked, How is your grandfather? Can he walk? Can he talk?

There was a pause.

Oh, he’s okay, she answered carefully, somewhat confused. He can even do both at the same time.

Hippocrates said, Life is short, the art [of healing] long, opportunity fleeting, experience treacherous, judgment difficult. Mr. Chu’s battle with TTP has since set me to pondering, once again, the art of diagnosis and the myriad disguises each disease can adopt. But it has also made me think about our American health-care system and how, with its tangle of insurers, red tape, and restrictions, it’s managed to do what even Hippocrates couldn’t--make medicine the easy part.
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