Every Patient Tells a Story Read online

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  CONTENTS

  Author’s Note

  Introduction: Every Patient’s Nightmare

  PART ONE: Every Patient Tells a Story

  1. The Facts, and What Lies Beyond

  2. The Stories They Tell

  PART TWO: High Touch

  3. A Vanishing Art

  4. What Only the Exam Can Show

  5. Seeing Is Believing

  6. The Healing Touch

  7. The Heart of the Matter

  PART THREE: High Tech

  8. Testing Troubles

  PART FOUR: Limits of the Medical Mind

  9. Sick Thinking

  10. Digital Diagnosis

  Afterword: The Final Diagnosis

  Acknowledgments

  Notes

  AUTHOR’S NOTE

  The stories I tell here are real. In order to respect the confidentiality of these patients who were kind enough to share their stories with me, I have changed their names. In some instances I have altered certain identifying details as well. The doctors featured in these pages described in detail some of their most difficult diagnoses—mistakes and all. They are distinguished not by these errors but by their willingness to discuss them. No one should be punished for simply being honest, and so I have changed the names of these brave doctors.

  The use of pronouns when you are speaking of an individual remains problematic in writing now that we can no longer just use the generic “he.” There is no rule on this at this point, so in this book I will refer to the generic doctor as she and the generic singular patient as he.

  INTRODUCTION

  Every Patient’s Nightmare

  Barbara Lessing stared out the window at the snowy field behind the hospital. The afternoon sky was dark with yet more snow to come. She looked at the slender figure in the bed. Her daughter, Crystal, barely twenty-two years old and healthy her entire life, was now—somehow—dying. The young woman had been in the Nassau University Medical Center ICU for two days; she’d been seen by a dozen doctors and had scores of tests, yet no one seemed to have the slightest idea of just what was killing her.

  It all started at the dentist’s office. Crystal had had a couple of impacted wisdom teeth taken out the month before. But even after the teeth were gone, the pain persisted. She’d called her mother halfway across the state just about every day to complain. “Call your dentist,” she’d urged her daughter. And she had. Finally.

  The dentist gave her a week’s worth of antibiotics and then another. After that her mouth felt better—but she didn’t. She was tired. Achy. For the next week she’d felt like she was coming down with something. Then the bloody diarrhea started. And then the fevers. Why didn’t you go to the doctor sooner? the trim middle-aged woman scolded her daughter silently.

  Barbara had gotten a call from a doctor in the emergency room of this suburban hospital the night before. Her daughter was ill, he told her. Deathly ill. She drove to Syracuse, caught the next flight to New York City, and drove to the sprawling academic medical center on Long Island. In the ICU, Dr. Daniel Wagoner, a resident in his second year of training, ushered her in to see her daughter. Crystal was asleep, her dark curly hair a tangled mat on the pillow. And she looked very thin. But most terrifying of all—she was yellow. Highlighter yellow.

  Wagoner could feel his heart racing as he stood looking at this jaundiced wisp of a girl lying motionless on the bed. The bright unnatural yellow of her skin was shiny with sweat. She had a fever of nearly 103°. Her pulse was rapid but barely palpable and she was breathing much faster than normal despite the oxygen piped into her nose. She slept most of the time now and when awake she was often confused about where she was and how she had gotten there.

  To a doctor, nothing is more terrifying than a patient who is dying before your eyes. Death is part of the regular routine of the ICU. It can be a welcome relief to the patient, or to his family. Even a doctor may accept it for a patient whose life can be prolonged no longer. But not for a young girl who was healthy just weeks ago. These doctors had done everything they could think of but still there was a fear—a reasonable fear—that they’d missed some clue that could mean the difference between life and death for this young woman. She shouldn’t die, but the young resident and all the doctors caring for her knew that she might.

  Crystal’s thin chart was filled with numbers that testified to how very ill she was. Wagoner had been through the chart a dozen times. Virtually every test they’d run was abnormal. Her white blood cell count was very high, suggesting an infection. And her red blood cell count was low—she had barely half the amount of blood she should have. She’d gotten a transfusion in the emergency room and another after she was moved to the ICU, but her blood count never budged. Her kidneys weren’t working. Her clotting system wasn’t either. Her yellow skin was covered in bruises and her urine was stained deep red.

  Sometimes, if you just work hard enough to keep a patient alive—to keep the blood circulating, the lungs oxygenating, the blood pressure high enough—the body will be able to survive even a vicious illness. These are the miracles brought by technological advances. Sometimes, but not this time. The ICU team gave Crystal bag after bag of blood; they did their best to shore up her damaged clotting system; she got pressers (medications designed to increase blood pressure) and fluids to help her kidneys. She was on several broad-spectrum antibiotics. And yet none of that was enough. She needed a diagnosis. Indeed, she was dying for a diagnosis.

  This book is about the process of making that diagnosis, making any diagnosis. So often this crucial linchpin of medicine goes unnoticed and undescribed, yet it is often the most difficult and most important component of what physicians do. As pervasive as medicine has become in modern life, this process remains mostly hidden, often misunderstood, and sometimes mistrusted. In movies and novels it’s usually the one-liner that separates the fascinating symptoms from the initiation of the lifesaving therapy. On television it’s the contemporary version of Dr. McCoy’s (Star Trek) magic diagnostic device (his tricorder) that sees all, tells all. But in real life, the story of making a diagnosis is the most complex and exciting story that doctors tell. And these are stories that doctors tell. Just as Sherlock Holmes or Nick Charles (the hero of the Thin Man mysteries) or Gil Grissom (CSI) delights in explaining the crime to victims and colleagues, doctors take pleasure in recounting the completed story of their complex diagnoses, stories where every strange symptom and unexpected finding, every mystifying twist and nearly overlooked clue, finally fit together just right and the diagnosis is revealed. In this book I’ll take you into those conversations and onto the front lines where these modern medical mysteries are solved—or sometimes not.

  Just a hundred years ago, journalist and acerbic social critic Ambrose Bierce defined the word “diagnosis” in his Devil’s Dictionary as “A physician’s forecast of disease by [taking] the patient’s pulse and purse.” And that was true for most of human history. Until very recently, diagnosis was much more art than science.

  But since Ambrose Bierce wielded his rapier pen, there has been a revolution in our ability to identify the cause of symptoms and understand the pathology behind them. In the era in which Bierce wrote, Sir William Osler, considered by many to be the father of American medicine, was able to write a comprehensive summary of all the known diseases in his 1,100-page masterwork, The Principles and Practice of Medicine. These days each tiny sub-branch of medicine could provide as many pages on its super-specialized knowledge alone.

  At the birth of medicine, millennia ago, diagnosis (the identification of the patient’s disease) and prognosis (the understanding of the disease’s likely course and outcome) were the most effective tools a d
octor brought to the patient’s bedside. But beyond that, little could be done to either confirm a diagnosis or alter the course of the disease. Because of this impotence in the face of illness, the consequences of an incorrect diagnosis were minimal. The true cause of the illness was often buried with the patient.

  In more recent history, medicine has developed technologies that have transformed our ability to identify and then treat disease. The physical exam—invented primarily in the nineteenth century—was the starting point. The indirect evidence provided by touching, listening to, and seeing the body hinted at the disease hidden under the skin. Then the X-ray, developed at the start of the twentieth century, gave doctors the power to see what they had previously only imagined. That first look through the skin, into the inner structures of the living body, laid the groundwork for the computerized axial tomography (CT) scan in the 1970s and magnetic resonance imaging (MRI) in the 1990s. Blood tests have exploded in number and accuracy, providing doctors with tools to help make a definitive diagnosis in an entire alphabet of diseases from anemias to zoonoses.

  Better diagnosis led to better therapies. For centuries, physicians had little more than compassion with which to help patients through their illnesses. The development of the randomized controlled trial and other statistical tools made it possible to distinguish between therapies that worked and those that had little to offer beyond the body’s own recuperative powers. Medicine entered the twenty-first century stocked with a pharmacopeia of potent and effective tools to treat a broad range of diseases.

  Much of the research of the past few decades has examined which therapies to use and how to use them. Which medication, what dose, for how long? Which procedure? What’s the benefit? These are all questions commonly asked and that can now be regularly and reliably answered. Treatment guidelines for many diseases are published, available, and regularly used. And despite concerns and lamentations about “cookbook medicine,” these guidelines, based on a rapidly growing foundation of evidence, have saved lives. These forms of evidence-based medicine allow patients to benefit from the thoughtful application of what’s been shown to be the most effective therapy.

  But effective therapy depends on accurate diagnosis. We now have at our disposal a wide range of tools—new and old—with which we might now make a timely and accurate diagnosis. And as treatment becomes more standardized, the most complex and important decision making will take place at the level of the diagnosis.

  Often the diagnosis is straightforward. The patient’s story and exam suggest a likely suspect and the technology of diagnosis rapidly confirms the hunch. An elderly man with a fever and a cough has an X-ray revealing a raging pneumonia. A man in his fifties has chest pain that radiates down his left arm and up to his jaw, and an EKG (electrocardiogram) or blood test bears out the suspicion that he is having a heart attack. A teenage girl on the birth control pill comes in complaining of shortness of breath and a swollen leg, and a CT scan proves the presence of a massive pulmonary embolus. This is the bread and butter of medical diagnosis—cases where cause and effect tie neatly together and the doctor can almost immediately explain to patient and family whodunit, how, and sometimes even why.

  But then there are the other cases: patients with complicated stories or medical histories; cases where the symptoms are less suggestive, the physical exam unrevealing, the tests misleading. Cases in which the narrative of disease strays off the expected path, where the usual suspects all seem to have alibis, and the diagnosis is elusive. For these, the doctor must don her deerstalker cap and unravel the mystery. It is in these instances where medicine can rise once again to the level of an art and the doctor-detective must pick apart the tangled strands of illness, understand which questions to ask, recognize the subtle physical findings, and identify which tests might lead, finally, to the right diagnosis.

  To the doctors caring for Crystal Lessing, it was not clear if the mystery of her illness was going to be solved in time to save her life. Certainly there was no shortage of diagnostic data. There were so many abnormalities it was difficult to distinguish between the primary disease process and those that were the downstream consequences of the disease. The doctors in the ER had focused on her uncontrolled bleeding. Why wasn’t her blood clotting? Was this disseminated intravascular coagulopathy (DIC)—a mysterious disorder that frequently accompanies the most severe infections? In this disease the fibrous strands that make up a clot form willy-nilly inside blood vessels. These tough strands slice through red blood cells as they course through the artery, releasing the oxygen-carrying contents and strewing the torn fragments of cells into the circulation. Yet careful examination of Crystal’s blood didn’t reveal any of these cell membrane fragments. So it wasn’t DIC.

  And why was she yellow? Hepatitis was the most common cause of jaundice in a young person. But the ER physician found no evidence of any of the several viruses that can cause hepatitis. Besides, the blood tests they’d sent to check how well her liver was working were almost normal. And so, they concluded, it wasn’t her liver.

  Once Crystal was transferred to the ICU, the doctors there had focused on the bloody diarrhea. She’d had two courses of powerful antibiotics for a dental infection before the diarrhea and fever started. That fit the pattern for an increasingly common infection with a bacterium called Clostridium difficile, or C. diff., as it’s known around the hospital. The use of antibiotics can set the stage for this bacterial infection of the colon, which causes devastating diarrhea and a severe, sometimes fatal, systemic illness.

  The ICU team had looked for the dangerous toxin made by the C. diff. bacteria but hadn’t found it. Still, that test can miss up to 10 percent of these infections. In fact, it’s standard practice to retest for the bacterial toxin three times before believing that the disease isn’t present when suspicion for the disease is high. The ICU team started Crystal on antibiotics to treat C. diff. anyway—the story of antibiotics followed by bloody diarrhea made that their leading diagnosis.

  But Dr. Wagoner, the resident caring for the patient, was unsatisfied with the diagnosis. Too many pieces didn’t seem to fit. The antibiotics and diarrhea made sense but the diagnosis left too many of her symptoms unexplained.

  That Friday afternoon—forty-eight hours after Crystal was admitted to the hospital—Wagoner did what doctors often do when faced with a complex case: he reached out to a more experienced physician. Despite all the available technology, the tools doctors often rely on most are the most old-fashioned—a phone, a respected colleague, a mentor or friend.

  Dr. Tom Manis was one of the most highly regarded doctors in the hospital. A nephrologist, he was called in because of Crystal’s kidney failure. But as Wagoner presented the patient to the older doctor, it was clear he was hoping that Manis could help them figure out more than just the kidney.

  As Manis read through the chart, he too became alarmed. Wagoner was right—this diagnosis didn’t fit well at all. For one thing, C. diff. colitis is usually a disease of the sick and elderly. The patient was young and had been healthy. But even more to the point, C. diff. wouldn’t account for the profound jaundice and the anemia that persisted despite multiple transfusions. So Manis did what the resident had done—“I called every smart doctor I knew,” and told them each the perplexing tale of Crystal Lessing—again, using those irreplaceable tools, a phone and a friend. One of those friends was Dr. Steven Walerstein, the head of the hospital’s Department of Medicine.

  It was early evening by the time Walerstein had a chance to see the patient. He didn’t read her chart. He never did in tough cases like this. He didn’t want to be influenced by the thinking of those who had already seen her. Far too often in these difficult cases something has been missed, or misinterpreted. And even if they had collected all the pieces, they had clearly put the story of this illness together incorrectly.

  Instead he went directly to the patient’s bedside.

  Walerstein introduced himself to the young woman and her mother. He pul
led up a chair and sat down. Getting the whole story is essential but it can take time. Can you tell me what happened, from the beginning? he asked the sick girl. Like the classic detective in a mystery novel, he asked the victim to go over the crime once more. “I’ve told this story so many times,” Crystal protested. Her voice was thick with fatigue, her words slurred. Couldn’t he just read it in her chart? No, he told her gently but firmly. He needed to hear it from her, needed to put it together for himself. Slowly the girl began telling her story once more. Her mother took up the tale when the girl became confused or couldn’t remember.

  Once the two women had gone through the events that brought each of them to the ICU, Walerstein asked the mother for a little more information about her daughter. Crystal had just graduated from college, she told him. She was working as a nanny while she tried to figure out what she wanted to do with her life. She didn’t smoke or drink or use drugs. And she’d never been sick. Never. She roughly brushed away tears as she described her daughter to this kindly middle-aged doctor. He nodded sympathetically. He had a daughter.

  Then Walerstein turned back to the young woman in the bed. Her yellow skin was now hot and dry. Her lips were parched and cracked. Her abdomen was distended and soft, but he could feel the firm edge of the liver, normally hidden by the rib cage, protruding a couple of inches below. She moaned again as he put pressure on this tender and enlarged organ.

  Only then did he allow himself to look through her chart. He skipped over the notes and buried himself in the myriad abnormal test results that had been collected over her two days in the intensive care unit.

  Walerstein was a general internist, admired for his broad knowledge of medicine and his clinical acumen. If he didn’t know the answer right off the bat, he was known to ask questions that would lead to the answer. And this young woman needed an answer, or she would die. Having thoroughly examined the patient and her chart, Walerstein took a moment to step back and look for some kind of pattern buried in the chaos of numbers and tests.