Every Patient Tells a Story (6 page)

There are only a few diseases that would cause this kind of injury. Viral encephalitis—an infection of the brain that is often caused by herpes simplex—was certainly the most common. Autoimmune diseases like lupus could also cause these kinds of abnormalities. In lupus, the body’s natural defenses mistakenly attack its own cells as if they were foreign invaders. Finally, certain cancers can do this too—it’s usually lung cancer, usually in older smokers.

The young man’s symptoms had been coming on gradually over two months. Abend thought that made an infection like herpes less likely. The patient had already been started on acyclovir—the drug usually used to treat herpes encephalitis—since the disease can be deadly when it infects the brain. Although Abend thought it unlikely, they would need to do additional tests of the spinal fluid to make sure there was no evidence of this dangerous viral infection.

Lupus seemed even more unlikely to Abend. It is a chronic disease that can attack virtually any organ in the body and is generally characterized by joint pains and rashes. The patient had none of these symptoms. Still, perhaps this was the first sign of this complex disease. It would be unusual, but so was the young man’s extensive memory loss.

Although cancer was an uncommon cause of this kind of injury, it seemed to Abend the most credible in this patient. Even nonsmokers can get lung cancer. And other cancers can cause the same type of brain injury. Moreover, if these symptoms were caused by a cancer, there was a good chance that they would resolve once the cancer was treated. He ordered a CT of the chest, abdomen, and pelvis. Ordering all of these scans communicates uncertainty about what you are looking for and where it might be located, but Abend felt strongly that they didn’t have time to be wrong.

Results from the tests trickled in over the next few days. He wasn’t having seizures. It wasn’t a virus. He didn’t have lupus. But by the time those test results arrived they already had an answer. The CT of Randy’s chest had shown a large mass—not in his lungs, but in the space between them, the area
called the mediastinum. A biopsy revealed the final diagnosis—Hodgkin’s lymphoma, a cancer that attacks the immune system. He had what is called a paraneoplastic syndrome, a rare complication in which antibodies to his cancer attacked the healthy cells in his brain.

Randy had surgery to reduce the size of the mass and then started chemotherapy. And slowly, remarkably, his memory began to improve. But the trip to New York remains vague, and his only memory of his weeklong hospital stay is his nurse telling him he was going home.

His fiancée remembers the day she realized he was getting better. It was several weeks after leaving the hospital. She reminded him that he wanted to get a haircut. He told her that he tried to go the day before but the line at the barbershop was too long.

She almost cried. “At that moment,” she told me, “I finally knew that the man I loved was still in there and that he was coming back.”

When I called Randy after receiving his e-mail, he still couldn’t remember much of his ordeal, but he understood the illness and the prognosis. One doctor stood out from the crowd of physicians caring for him. Marc Wein was a medical student at Brigham, and he had become fascinated by Randy and his illness. He read voraciously about the disease, tracked down case reports of other patients with a similar manifestation of cancer, and came back again and again to explain it all to Randy and Leslie. Together Marc and Randy created the story of this remarkable diagnosis that made sense to both of them. And that made all the difference.

Randy tells me he was never in pain but he hated the way he became a clean slate every five minutes. He hated the worried looks he saw on the faces of those he loved. He hated the loss of a sense of who he even was.

He embraced the story that Wein put together for him. Leslie had to remind him frequently of the particulars of that story, but he remembered that he had a cancer and that curing that cancer would restore him to himself. He welcomed the surgery and never minded the pain from the incision down his chest. He even looked forward to chemotherapy. Watching the intravenous needle pierce his skin, he remembered it meant he was one step closer to getting better. I spoke with Randy several times as he faced his
ordeal. His optimism never flagged. He is now disease free and his life has moved on. He returned to work five months after that strange weekend and got married the next year.

Randy’s body may have been cured by the chemotherapy, but his mind was healed by a story.

CHAPTER TWO
The Stories They Tell

A
t a recent conference of the American College of Physicians in Philadelphia, a friend who knew of my interest in diagnosis encouraged me to attend one lecture in particular. The title stood out from all the Updates on Cardiology and Innovations in Nephrology, Hematology, or Urology. This talk was called simply “Stump the Professor.”

When I arrived at the designated ballroom I was amazed—the place was packed with hundreds of doctors. As I picked past feet and knees to claim a rare open seat, I looked at the casually dressed, mostly middle-aged audience. There was a sense of giddy anticipation in the air, reminiscent of a college-age trek to a distant concert arena.

Finally, a tall and slender woman with a volleyball of gray curls and a broad smile strode onto the stage, nodding and smiling at her devotees. The audience exploded with applause.

This was Dr. Faith Fitzgerald, a flesh-and-blood version of TV’s Dr. House. She is the doyenne of the diagnostic dilemma. This auditorium of hundreds of doctors had come to see her take on a series of challenging cases—patients whose stories had been submitted by medical students from around the country and handpicked for this presentation because of their difficulty and complexity. The patient’s story and medical course would be presented to Fitzgerald, a bit at a time, and her job would be to figure out
the diagnosis by the end. Throughout the presentation she would take the audience through her thought process, acting the modern Sherlock Holmes to her own crowd of Dr. Watsons. It was another mark of our time: diagnosis was now a form of entertainment.

After what appeared to be a completely unnecessary introduction to this crowd, Fitzgerald set her glasses halfway down her long, aquiline nose, and greeted the adoring fans. Like all good speakers, she started off with a joke—a doctor joke: “Before we get started, and just for the record,” Fitzgerald growled in her tobacco-raspy voice, “I’d like to mention—endocarditis, tuberculosis, Wegener’s granulomatosis, Kawasaki’s aortitis, Jakob-Creutzfeldt dementia, and eosinophilic gastritis.” She rushed through this list of arcane diseases and ended with a laugh. “I don’t know any of the cases I’m about to hear but there’s a darn good chance I’ve mentioned at least one case diagnosis in that list. Just so you know that I did say them.”

The crowd laughed appreciatively. In this forum, even if you don’t ultimately figure out the case, you get credit for having the final diagnosis among the diseases you considered on the way. Fitzgerald was acknowledging that the cases she would be likely to confront that day would not be the same as those doctors routinely see in daily practice. Instead they would be the “fascinomas,” the intriguing cases physicians share at the watercooler, the nurses’ station, or in hospital stairwells.

Javed Nasir, a twenty-something graduating medical student from the Uniformed Services University Medical School, walked onto the stage. He would present the first case—a patient he cared for in his third year. “Good morning, Dr. Fitzgerald.” His voice wavered slightly. He began, with what is called (by tradition) the chief complaint. “‘My wife is not acting right.’” The young man looked out at the large crowd uncertainly and then continued. “This is a seventy-three-year-old woman with a three-month history of progressive confusion, brought to the hospital by her husband.” He then detailed the patient’s symptoms in the conventional medical format.

Over the next ninety minutes these doctors watched and occasionally helped Fitzgerald work her way through Nasir’s and two other patient stories, revealing through each the internal machinery of making a diagnosis.
She had never met any of these patients, had never examined them. Instead Fitzgerald made her diagnosis using a doctored-up version (quite literally) of the patient’s story. That story contained only the barest bones of the original patient’s story, stripped of all that is unique, personal, and specific, reshaped by the doctor and augmented by the physical exam findings and test results from the investigation. All this was presented in a highly structured and familiar format.

Although this is done as a kind of entertainment, a kind of brain-teaser for the audience full of doctors, it’s a simulacrum of what doctors do at the bedside. The kind of stripped-down and highly structured story on which this exercise depends is one of the most important tools doctors have for translating the abstract knowledge of the body—gleaned from cadavers, test tubes, and books—into a diagnosis of the patient before them. It is a familiar exercise to doctors because we are the authors of these stories for our own patients and audience for other doctors seeking help with theirs.

Nasir continued with his patient’s story, explaining that she had been in her usual state of health until a few months earlier, when she became increasingly forgetful. First, she began to have trouble finding the right words when she spoke. Her husband got really scared when she started to get lost driving even in her own neighborhood. At the time of her admission she was having difficulty with the most basic daily activities; she could no longer cook or even dress herself without his help. She was unwilling even to leave the house without him.

Fitzgerald is an internist, and a dean of medicine and humanities at the University of California at Davis. As the medical student told this patient’s story of rapidly worsening confusion she paced up and down the stage. Her long black coat flapped behind her, revealing slim black pants and black turtleneck—her usual attire.

An old hand at this format, she was clearly enjoying the challenge and the crowd—a mixture of old hands and novice trainees. Fitzgerald has been a regular feature at conferences like this one for more than a decade.

“On physical exam, the patient is a thin, frail woman who appeared timid and fearful,” Nasir continued.

“Timid and fearful?” Fitzgerald asked. (In the movie version, she might puff on her calabash about now.) “Hmmm. That could be part of her confusion or could be her personality. Did you get a sense of what she was like before all this?” The student shook his head. “Well, it would certainly be hard to feel confident in a world that you suddenly don’t understand.”

The rest of the physical exam was unremarkable, the medical student told her.

Fitzgerald stopped pacing. “By that I guess you mean that it was normal?” she asked.

Nasir nodded. “Even the neurological exam—completely normal?” Again he nodded. Fitzgerald was silent as she considered the story so far.

“Would you like to order some tests?” the student prompted. In this structured performance the doctor can ask for any test and if the patient had the test that data will be shared.

“Sure.” She quickly called out tests she’d like to order and the results were provided. A spinal tap was normal, there was no elevated white blood cell count, her liver and kidneys were working fine.

“So basically what you’re telling me is that we have here a woman with a rapidly progressive dementia but a completely normal physical exam otherwise and no sign of infection or laboratory abnormalities?” Fitzgerald asked. She then turned to the audience. “I am not at all offended if people shout out the answer at any time,” she called out to the audience. “Anyone? Well, at least it’s not obvious to anyone else out there either.”

It certainly wasn’t obvious to me. As Fitzgerald considered the data available on the patient, she started to describe how she was thinking about what she’d heard. “At this point I like to develop some kind of structure on which to hang my ideas. To help me put together a thorough differential diagnosis, I often just start with the different areas of medicine. So, could this be some kind of congenital disease that causes dementia—like early Alzheimer’s? Maybe. Or could this be infectious? Did she have a life of adventure that would put her at risk for some colorful, sexually transmitted diseases like syphilis or HIV?”

As she reviewed her thinking, she developed a list of possible causes of
these symptoms. Voices called out from the audience offering additional diseases to add to the differential. “Parkinson’s dementia” a man called from the end of my row. “Jakob-Creutzfeldt” (mad cow disease) offered a woman in front.

“Get a head CT,” called out still another voice.

“Hmmm—a head CT.” Fitzgerald considered the suggestion. “This lady has no neurological findings—right?” She turns to Nasir, who again nods his confirmation. “No weakness, no seizures, no tremor—nothing except confusion. Given that, I don’t think a CT scan will show me much. In my hospital it’s almost impossible for a patient with mental status changes to come through the ER without getting a head CT. And yet the odds are that hers will be normal, so …” She paused thoughtfully. “I say we skip it.”

Once the case had been presented completely, it was time for Fitzgerald to make her diagnosis. She went through her differential. “Well, common things being common, this would most likely be multi-infarct dementia or maybe Alzheimer’s. But this is stump-the-professor time and so it’s never the common thing. Hmmmm.” She turned to the audience. “Can I talk to a really old doctor?” Chuckles from the audience were followed by a few more suggestions.

“Any other ideas?” Fitzgerald conceded. “Okay, I give up. Let’s hear it.”

“Maybe you should have gotten the head CT after all,” quipped the medical student, pleased that he actually stumped the professor. He projected the final slide onto the large screens at the front of the room. An image from a CT scan of the head revealed a huge, white, irregularly shaped circle bulging into and distorting the familiar spaghetti swirls of the brain. It was a brain tumor.