Of all the patients I cared for during my internship, one group holds a special place in my memory. I call them the sleepers – patients who lay motionless for days or weeks at a time, their eyes closed and their arms resting peacefully at their sides. These were my greatest burdens, yet caring for them gave me a sense of transcendent power. Day after day, I attended to their every need, with no clue from the souls who dwelled inside those still and silent bodies.
Maria Gonzales, the most beautiful of my sleepers, was six years old when I admitted her to the neurosurgery service. She had dark eyes and magnificent dark hair, but fate is seldom swayed by beauty. Vomiting and terrible headaches had brought her to the hospital. My examination revealed choked discs, swelling of the optic nerves caused by high intracranial pressure. Adrianna, the unmarried aunt who brought Maria to the hospital, sat alone at the bedside for hours every day, her face glowing with tenderness as she stroked the girl’s hair, straightened her pillow, held a cup of water to her lips. I learned from a social worker that Maria’s parents had deserted her soon after her birth.
The neurosurgery team had only an intern, a resident named Ron Goldstein, and a tall, gaunt attending who had retired from the navy some years before. Ron and I called him the Admiral. He carried a pipe, often chewed on the stem, though the pipe was seldom lit. When Maria’s cerebral angiogram was completed, Ron laid out her films on a view box. After a long, morbid silence, the Admiral spoke.
“Christ – look at that tumor blush in the left parietal lobe. It has to be a glioblastoma.”
“Yup,” Ron said, “no doubt about it.”
“We’d better start the Solu-Medrol,” the Admiral said.
“How much?”
“A hundred milligrams a day.”
We had nothing more to say. The child was doomed. Solu-Medrol, a form of cortisone us for intravenous infusion, would lower her intracranial pressure and relieve her headaches for a while, but no treatment known to medicine could stop the tumor. It would soon devour the poor girl’s brain.
Maria’s headaches vanished. For a month, she ran up and down the pediatric ward, spent hours watching TV and trading soft toys with the other kids in the playroom. Every evening, Adrianna set the child on her lap to read a bedtime story. When the headaches returned, Ron and I worked out the dosage of IV morphine needed to control her pain, but this made her drowsy, and soon she took to her bed. Adrianna now kept a round-the-clock vigil.
The nightmare seemed to go on and on. One morning while examining Maria, I discovered lumps in her neck. The Admiral gently felt the lumps and shook his head in amazement.
“I’ve never seen this before – the tumor has metastasized outside her skull.”
Two days later, Adrianna gave us more devastating news.
“Maria doesn’t wake up very often, but when she does, she says the headache is terrible, and she claims she can’t see anything.”
The Admiral, his face grim as death, opened Maria’s lids with his fingers and shined a flashlight into each eye. The light did not arouse the girl. Her enormous pupils remained fixed and dilated.
“Yes,” he said, “I’m afraid the child is blind.”
Adrianna stood motionless, staring ahead as though blind herself. Silent tears flowed down her cheeks. The Admiral gave her a hug. He seemed ready to speak again, then shook his head and removed his arm from her shoulder. Ron and I followed him down the hall to a conference room. He waited for us to enter, then closed the door.
“Well,” the Admiral said, settling into a chair, “the Solu-Medrol doesn’t seem to be working. We might as well stop it.”
“Yup,” Ron said. “That sounds like a good plan. I’ll write the order.”
Something about the idea bothered me. I probed my memory, seeking a fragment retained from a textbook or a lecture.
“Ah, but wait,” I said. “Surely you don’t mean we should stop it all at once. A hundred milligrams a day is enough to shut down her adrenals. If we just cut her off cold, her blood pressure will drop like a rock. Shouldn’t we taper off her dosage and give her adrenals time to recover?”
It was a proud moment. I had dredged up a gem, an erudite fact to save my mentors from a dangerous lapse in their reasoning, but Ron and the Admiral didn’t thank me. They gazed at me in silence. Perhaps they were embarrassed by their lapse, or surprised by my precocious knowledge. The Admiral lit his pipe, took a few puffs, grunted to himself and shook his head. Ron turned red in the face.
“Jesus Christ, Gamel,” he said. “How dumb can a man get? Did your mother have any children that lived?”
The Admiral’s plan worked. Over the next thirty-six hours, Maria’s blood pressure fell lower and lower. She died without regaining consciousness.
* * *
Granny Simpson, a seventy-year-old widow, distinguished herself as my most bizarre sleeper. During the course of a week, she worked her way through a cascade of life-threatening disasters, all the while urging us to stop the treatments and let her die. Even her family opted for death, arguing that Granny had suffered enough.
It started one night when ambulance attendants pulled Granny from her wrecked Cadillac and brought her to our ER. She had ruptured her spleen on the steering wheel. Lancy Allen, my senior resident that night, scheduled her for emergency surgery, then typed and cross-matched six units of blood, but getting Granny to the OR proved an uphill battle. She refused to sign the consent form, insisting that she was a worn-out old woman with nothing left to live for. Soon her two grown children showed up and gave Lancy the same story: Granny had been depressed for years, insisting time and again she would be better off dead. Why not let nature take its course?
Lancy was at his wits end. Since Granny smelled of liquor and had just suffered a major trauma, she was in no condition to make a rational decision. The family kept telling us the old lady was worn out and ready to die, yet before the wreck, she had been in perfect health except for her depression. When Lancy spoke to the hospital’s lawyer, he said we should go ahead with surgery. To do otherwise, he insisted, would be nothing less than a mercy killing.
So we hauled Granny off to the OR kicking and screaming in protest. Her surgery proved a close shave. The ruptured spleen had dumped pints of blood into her abdomen, dropping her blood pressure to a critical level, where it stayed until the anesthesiologist had given her several transfusions. The attending surgeon thought her liver was enlarged, suggesting alcohol abuse. Lancy wouldn’t buy it. True, she had liquor on her breath, but she had been well-dressed when brought into the ER, and her children seemed solid members of the upper class.
Indeed, Granny didn’t look like a typical alcoholic, but on the third post-op day, she went into florid delirium tremens. By the time I got her tremors and hallucinations under control with a generous dose of intravenous valium, she turned blue and began gasping for breath. A stat pulmonary consult diagnosed shock lung caused by the prolonged drop in her blood pressure.
For any hope of survival, I had to put her on a respirator, but she would have nothing of it. After she pulled out her endotracheal tube three times, an anesthesia resident helped me set up a curare drip to keep her paralyzed twenty-four hours a day. This turned Granny into a special type of sleeper. She was awake yet couldn’t move a muscle, couldn’t even open her eyes, lying still as a corpse except for the rise and fall of her chest in rhythm with the hiss . . . clunk . . . hiss . . . clunk . . . of the respirator. Her children visited the ICU every day. Now their protests reached a fever pitch.
“Poor Granny, why do you keep tormenting her like this? Why don’t you let the old lady have her way? She’s miserable and wants to die.”
Every other day, Lancy was on the phone to the hospital’s lawyer, who still insisted that so long as there was any chance the patient might recover and lead a normal life, we should do all we could to save her. It mattered not that she and her family fought our every move.
A week after these disasters began, Granny’s lungs cleared up. Rather than the corpse she had hoped for, she was a healthy woman, sober and clear-eyed, with no tremors and a recovering liver. Best of all, we could stop the curare drip. The moment we took her off the respirator, allowing her to speak, Lancy and I were astonished to find ourselves heroes. She grabbed my arm every time I came near her bed.
“Oh, doctor,” Granny said, “thank you so much. I just can’t tell you how grateful I am. You saved me, and it’s wonderful to be alive. I’ve never felt better in my life.”
Her family was even more effusive. They brought a huge basket of fruit for the ICU staff and sent a card to Lancy and me, a flowery message thanking us for saving their beloved mother. Most memorable of all was the gratitude shown by one of the granddaughters, a young woman with long blonde hair who came to visit one night wearing a halter top and skin-tight denim shorts. She grabbed me in the hall outside the ICU, stunned me with a hug that cracked my ribs, then rose on tiptoes to whisper a message. Our cheeks touched. I felt her warm breath on my ear. She would do anything, anything in the world, to show her gratitude.
I’ve forgotten my mumbled reply, but I remember well Lancy’s outrage when he heard of the encounter from one of the ICU nurses.
“You selfish jerk,” he said. “I’m the surgeon, the one who saved the old girl’s life. If you had a decent bone in your body, you’d tell that babe she ought to be hugging up on me. Besides, I’m single, and you’re married.”
In the end, the story fell short of the fairy tale one might hope for. I kept in touch with Granny’s daughter, the mother of the affectionate granddaughter, and she told me that for two months after Granny’s discharge, she seemed to have a new lease on life. The old lady took up golf, joined AA, began keeping company with a widower who lived next door in an upscale apartment near San Mateo. Then she fell off the wagon. A few weeks later, the apartment’s manager evicted her for drunk and disorderly conduct.
* * *
Greg Caraway was a forty-five-year-old airline pilot who lived in Cupertino, a few miles from the San Jose Airport. When he developed progressive nausea, fatigue and depression, his private doctor admitted him to a local hospital. According to the records, the doctor found no abnormalities on physical exam, but he described the patient as “lethargic and confused.” He ordered a battery of tests, then went home for a good nights sleep. The next morning, all the lab tests were normal, but he was disturbed to find his patient obtunded, almost comatose. The doctor considered all the relevant details, including the fact that Memorial Day weekend was coming soon, then dispatched Greg to Santa Clara Valley Medical Center, where I admitted him to the medical ICU.
The first time I saw Greg, he inspired a strange thought: how could anyone give up such a beautiful patient? Not that he was terribly handsome, but lying there in coma, his arms resting at his sides, he seemed beatific, his face an embodiment of peace and repose. The ICU nurses often stood by his bed, gazing in wonder at his seraphic features. I examined Greg from head to toe and found no abnormality except for his coma. Walter O’Conner, my resident on the medicine service, had no better luck. Together we looked through the records sent over from the private hospital.
“Damn,” Walter said, “here’s an EKG tracing, but there’s no report.”
“Ah, too bad,” I said, but my own heart fluttered with excitement. During a cardiology elective, I had learned to read EKGs, and on the tracing I saw marked shortening of the QT interval and a prolongation of the T wave. These were signs of hypercalcemia, an elevation of the serum calcium, which can lead to depression and coma. What a treat – Walter had missed the clue!
Rather than tell him of my discovery, I decided to wait for a measurement of Greg’s serum calcium to confirm my diagnosis. This assay was not included in the routine lab tests performed during that era, but I asked the pathologist on call for special permission. Two hours later, report in hand, I set out to find Walter.
“Hey, take a look at this,” I said, thrusting my ace under his nose. “Mister Caraway’s calcium is off the chart. That’s what put him in a coma.”
Walter glanced at the slip, then glowered at me. I was delighted to see that my brilliant coup and obnoxious grin had pissed him off.
“Well, smartass,” he said, “you got any idea what’s causing this?”
“Uh . . . no, I don’t.”
“And treatment – any thoughts on that?”
“Uh . . . no.”
“Then you’d better get busy.”
Walter and I were pleased by what we found in The Merck Manual of Diagnosis and Therapy. The section on hypercalcemia included a long list of possible causes, and most were benign or amenable to treatment. Perhaps Greg had overactive parathyroid glands. Perhaps he suffered from an inherited disease, or had taken too much vitamin D or too many antacid tablets. The list also included disseminated cancer, which could dissolve the calcium in a patient’s bones, but the tests done so far had revealed no hint of malignancy. A few hours later, our optimism collapsed when a radiologist paged Walter with devastating news. Greg’s X-rays showed hundreds of dark spots scattered through his skull, his ribs, his spine. The poor man was doomed.
This left us with only one question: where had these tumors come from? We requested consults from oncology, gastroenterology, and urology. In the end, the urology resident found the answer. After his first exam, he pronounced Greg’s prostate unremarkable, but the next morning, I found him back at Greg’s bedside.
“I lay awake half the night thinking about this guy,” he said. “Since the textbooks say those mets most likely came from his prostate, I decided to take another look, and this time I convinced myself things don’t feel quite right. There’s no obvious lump, but I don’t like the texture of the left lobe. I’ll be back this afternoon for a needle biopsy.”
Bingo. The next day, I was at the ICU charting desk when a clerk delivered the report from the biopsy. Greg had cancer of the prostate. It was a terrible moment. Twenty feet away, his wife and two teenage children stood beside his gurney, gazing down at his peaceful face. The wife was a frail, anxious woman with dark circles under her eyes. I dreaded the speech I had to give: Ma’am, I’m afraid we’ve got some news, and it’s not good . . . No, I had a better idea. I paged Walter and told him about the biopsy.
“Look,” I said, “I had to tell Greg’s wife about the mets in his bones. Now it’s your turn.” A few minutes later, he arrived in the ICU, gave me a look that said I was a rotten coward and would owe him big time, then led Greg’s wife down the hall to the hospital chapel.
Would that this had been the end of Greg’s story. What we needed to do was obvious – let the poor man die in peace – but an enemy leapt upon us.
* * *
During my years at Stanford, the oncology department was both famous and notorious, a collection of brilliant lunatics. They won millions of dollars in research grants. They published dozens of papers in prestigious journals. But at their worst, they tormented cancer patients with a consuming and irrational passion, a passion that often served only to prolong hopeless misery. Dr. X, the department’s chairman, was so aggressive in his treatments, a dark jest haunted him throughout his tenure: Dr. X. rushes to a graveside just as the pallbearers lower the coffin.
“Wait! Wait!” he shouts. “There’s still time for one more dose of 5-FU.” Five-fluorouracyl is a toxic drug used to treat advanced malignancies.
When we first discovered the cancer in Greg’s bones, an oncology fellow from Stanford had answered our request for a consult. Trained by Doctor X. and his mignons, he did a thorough job, listing all the sites from which the tumor might have spread and all the tests we needed to order. He also gave us a treatment protocol for bringing George out of his coma by lowering serum calcium, but with no hope for a cure, we decided to let nature take its course. A few days after the consult, Walter paged me to the ICU. I found him fuming and red in the face.
“What happened?” I said.
“It’s that asshole from Stanford – the oncology fellow. He came back for a follow-up exam and chewed me out. Called me a terrible doctor, threatened to sue me for malpractice.”
“My god!” I said. “What the hell is he talking about?”
“He insists we have to treat Greg’s hypercalcemia, pull out all the stops so we can wake him up.”
“Wow!” I said, buoyed by a surge of optimism. “You mean they’ve found something new, a drug that might wipe out all of Greg’s mets?”
“Hell no. That moron made it clear – there’s nothing new for prostate cancer, nothing at all. They’re still stuck with 5-FU, but this guy wants us to start pouring it in right now. Maybe it’ll shrink the tumors by fifty percent, but only if we give whopping doses. The best we can hope for is a few weeks of survival, a month or two at best.”
I stared at Walter. My brain struggled to take in this lunacy.
“Wait a minute,” I said. “They want us to wake the man up so we can tell him he’s a goner, tell him he’ll spend the last few weeks of his life writhing in pain from his tumors and puking up his guts from the 5-FU?”
“Yea, that’s what he wants us to do.”
Despite the oncologist’s rant, all went well in the end. Walter sat down with the ICU director and worked out a protocol. We gave Greg intravenous diuretics and calcitonin, the standard treatment for hypercalcemia, plus a few milligrams of 5-FU, but the doses weren’t enough to do the job. I wrote a standby order for IV morphine in case he woke up screaming with pain, but it was never needed. Greg remained in a coma for five days, his face calm and peaceful as a sleeping baby’s, then died without stirring a limb.
* * *
My most prestigious sleeper was Michael B., a ten-year-old whose father had served as the mayor of San Jose and the head of the county school board. The trouble began when a pediatrician treated Michael with aspirin for what seemed a typical case of influenza. He complained of a headache, developed severe nausea and vomiting, then was admitted to a private hospital for intravenous rehydration. At midnight, a nurse called the pediatrician to report that Michael’s urine output had dropped to almost nothing. He had also become confused and disoriented. These symptoms gave the final clue: Michael had Reye’s syndrome, a mysterious affliction that devastates the brain and other vital organs. There was no proven treatment. The pediatrician, reluctant to spend fruitless hours at the bedside of a doomed child, shipped Michael to Valley Medical Center.
At three a. m., as ambulance attendants transferred him to a bed in the ICU, I introduced myself to the parents. All three of us had been up for thirty or forty hours. We sagged from exhaustion, but they had managed to dress for the occasion in a suit and a stylish dress, as though on their way to church, while I wore rumpled OR scrubs. Michael lay a few feet away, a beloved golden-haired child, but he already had the jaundiced skin and urine-like stench brought on by liver failure. The father was so distracted, I had to repeat my questions two or three times before he could muster an answer. The mother remained silent. Again and again, they turned to watch the ICU nurses as they tucked the boy into bed and attended to his IV. He was delirious, screaming at the nurses, flailing his arms and legs in a wild struggle. I had attended a lecture on Reye’s syndrome during medical school and knew the dreadful truth: soon, the boy would be in deep coma.
In 1962, Doctor Douglas Reye, an attending at the Royal Alexandra Hospital for Children in New South Wales, Australia, described twenty-one cases of a mysterious disease. All had occurred in children who suffered from what initially seemed a routine viral illness, such as influenza or chicken pox. Over the course of hours or days, they became delirious, fell into a stupor, developed liver failure and renal shut-down. Seventeen of the victims died. Of those who survived, some had permanent afflictions, including cirrhosis, brain damage, and chronic renal failure. Doctor Reye, noting that all the autopsies showed profound swelling of the brain, liver, and kidneys, named the disease fatty degeneration of the viscera.
This paper was published ten years before Michael’s illness, and in the interim no cure had been found. Thus when the boy entered our ICU, all we knew was that he would soon suffer total organ shutdown, and his odds of survival were slim. The best we could offer were educated guesses.
An hour after Michael’s admission, I saw a gray-haired man approach his bed. The stranger was dressed in street clothes, but when he pulled a stethoscope from his pocket, I realized he must be a doctor. This was Joseph Garvey, a senior attending on the pediatric service. A few minutes later, in an office across the hall from the ICU, I listened while he explained to my resident and me what we should do for Michael. To keep his kidneys working, he needed diuretics. To maintain hydration, he needed intravenous fluids. Most important of all, we should give intravenous cortisone to reduce the swelling in his brain.
From their dour expression and subdued voices, I knew that neither Garvey nor my resident expected the boy to survive. Reye’s syndrome was a cruel, inscrutable beast that devoured organs one by one, transforming a healthy child into a bloated corpse in a matter of days. Depressed and exhausted, I wrote out the orders, then, while the nurses infused the appropriate fluids and medicines into Michael’s veins, I explained our plans to the parents. Garvey had warned me to not to give them false hope, but there was no danger of that. The tears streaming down their cheeks made it clear they expected their son to die.
Perhaps fate was in a good mood that day. Perhaps Michael, unaware of the odds against him, decided not to die. Twelve hours after we began our treatment, he woke from his coma. His jaundice cleared. His kidneys poured pints of urine into the bag hanging at the foot of his bed. A few days later, his mind was intact, his blue eyes bright and clear.
On the day of his departure, the parents ambushed me. They bought a box of candy at the gift shop and had the page operator summon me to the discharge desk. I was weary from a night on call, curious why the discharge clerk had paged me. The mother offered me the box of candy, then, before I could take it, she threw her arms around me. I struggled against her embrace, trying to explain that it was all a mistake – I was just the go-for, a glorified errand boy – but she would have none of it. Doctor Garvey was the brains behind it all, yet I had labored through the night at their son’s bedside. Before I could break from the mother’s grip, the father embraced both of us, pressing his cheek against mine. Tears flowed from their eyes, so many tears I wept myself. Michael sat nearby in a wheelchair. His face turned red, but he kept quiet until the blubbering died down. When everyone had blown their nose and dried their eyes, he stood up from the chair and grasped my hand.
“Thanks, Doctor Gamel,” he said. “I was always glad to see you, especially at night. Don’t you guys ever get to sleep?”
* * *
Of all the advice I have given and received during my medical career, this dictum remains my favorite: if your fairy godmother wakes you in the night to offer a choice between great skill and great luck, take the luck. In defense of this philosophy, I present the case of Michael B. The history of Reye’s syndrome shows just how great a role simple-minded pragmatism can play in the practice of medicine. Over the decades after Doctor Reye published his famous paper, it was discovered that almost all the victims had been given aspirin early in the course of a viral illness. Though theories abound, to this day, no one has discovered the mechanism by which aspirin interacts with a viral infection to destroy every vital organ in the body. Yet despite our ignorance, an international campaign to avoid the use of aspirin for treating febrile illnesses in children has all but eliminated Reye’s syndrome.
As for treatment, pragmatism again saved the day. There is still no specific cure, but now doctors know what to do – use common sense. The victims all suffer from dehydration and renal failure and swelling of the brain, thus we should give them IV fluids and diuretics and cortisone. Studies conducted since Michael’s illness show that these simple measures, if performed early enough in the course of the disease, dramatically improve the odds of survival and reduce the likelihood of permanent damage.
On that desperate night when Doctor Garvey devised Michael’s treatment plan, guided by principles known even then to any third-year medical student, he came up with a regimen that remains the gold standard forty years later. Experience has shown that if we can sustain the victim’s vital functions, the mysterious disease will run its course without destroying the delicate intracellular structures that sustain life. Thus a few simple measures have spared thousands of children from the ravages of Reye’s syndrome. As with many other maladies over the course of human history, when science failed, luck and common sense saved the day.