当前位置: 首页 > 期刊 > 《新英格兰医药杂志》 > 2004年第20期 > 正文
编号:11306571
A Great Case
http://www.100md.com 《新英格兰医药杂志》
     "It's a really great case," the neurology resident said. "Gerstmann's syndrome." I was a third-year medical student, and neurology was my first clinical rotation. The resident listed the four findings associated with the disorder: agraphia, right–left disorientation, finger agnosia, and acalculia. "Due to a tumor in the parietal lobe," he explained.

    We entered the patient's room. A disheveled man in a hospital gown looked at us uncertainly. The resident had the man attempt a series of tasks and maneuvers demonstrating all the elements of the syndrome's tetrad.

    "What a great case," I said as we left. The resident smiled.

    Internal medicine followed neurology. A cachectic drug user was admitted in the middle of the night with spiking fevers. "Listen to his heart," the intern instructed. I placed my stethoscope over the shrunken chest. Cacophony flooded my ears. "His valves are chewed to nothing," the intern said. It was acute bacterial endocarditis, and the intern recited some of its devastating complications: brain abscess, heart block, endarteritis. No doubt, I thought, it was a great case.

    Over the course of the year, I learned that there were subsets of great cases. Some were great puzzles. On rounds, master clinicians with encyclopedic knowledge would weave together seemingly loose ends of information — threads from the history, the physical examination, and laboratory tests — and form a whole cloth of diagnosis. The very best of these great cases were called "fascinomas" — arcane diseases that tested your acumen and evoked awe at the strange forms maladies could take. I recall a middle-aged woman with months of headaches supposedly due to "sinusitis" who turned out to have histiocytosis. The resident beamed as he described the multiple and subtle presentations of the proliferating Langerhans' cells that eroded bone and invaded the brain.

    Other cases were great because of the muscular drama they brought. In surgery, these were called "womps." A man with a gunshot wound to the gut was rushed into the emergency room. All hands were on deck, elbow deep in blood, putting in catheters, inserting an endotracheal tube, palpating the lacerated organs. A woman with a retroperitoneal sarcoma that had snaked up her abdomen, penetrated the diaphragm, and gripped her heart underwent an 11-hour dissection requiring teams from surgical oncology, thoracic surgery, and cardiac surgery. Such cases were great because they afforded a live tour of human anatomy.

    As our clinical rotations came to an end, we discussed where to intern. Choosing well involved considering not only the location of the hospital and its staff's commitment to teaching, but also whether there was "amazing pathology," a range of disease wide and deep enough to yield "great cases" along with the regular fare of internal medicine: peptic ulcer disease, adult-onset diabetes, alcoholic cirrhosis.

    As a house officer, I was drawn to the specialty of hematology because it seemed to be filled with great cases that came cloaked in a special beauty. Under the microscope, blood and marrow from patients with acute promyelocytic leukemia or the Sézary syndrome or thalassemia looked like wonderful works of art, with dazzling colors and intriguing shapes, offering motifs that might have come from Miró or Seurat. These aesthetically great cases were further enhanced by the cerebral disciplines of molecular genetics and protein chemistry.

    When I became an attending physician, I centered ward rounds on great cases. They provided a chance for students to shine, to show off the knowledge they had gained about the pathophysiology of the disease. Great cases also gave interns and residents the opportunity to demonstrate physical findings at the bedside, holding up the patient's legs to show erythema nodosum or rotating the trunk to show Turner's sign, and gave me a chance to be illuminated in their reflected light.

    On a steamy July 4th weekend 21 years ago, my wife, Pam, and I brought our first child, Steven, into the emergency room of Boston's Children's Hospital. Steve was nine months old. For nearly two days, he had been crying bitterly and refusing to nurse. At the onset of these symptoms, we were visiting my in-laws in Connecticut. An avuncular pediatrician in their town had told us not to worry, that our baby had a simple viral gastroenteritis. Pam, an internist and endocrinologist, thought differently. And by the time we returned to Boston, it was clear that she was right. Something was seriously wrong. Steve's breathing was rapid, and his face was without color. Every minute or so, he drew his knees to his chest and flailed his arms at his sides. He looked like he was going to die.

    A surgical resident in the emergency room began taking the history. Suddenly, an intern in scrubs burst into the room. "What have you got in here? What is it? A good case?"

    I fixed on the intern's expectant eyes and lost control. "Who the hell are you? My son is not a `good case!'"

    The intern stood frozen.

    "Get out of the room!" I bellowed. "Out!"

    It took several minutes to regain my composure. "I'm sorry," I said to the resident. He finished taking the history from Pam and then proceeded with the physical examination. "Rushed bowel sounds, then quiet. Very classic," the resident said. Steve had an intussusception, the telescoping of one segment of bowel into another. His desperate flailing was due to an acute intestinal blockage. As it happened, the intussusception could not be reduced by a barium enema. So, in the early hours of the morning, a senior surgeon was called to operate urgently. The bowel, it turned out, was at the point of rupturing. The surgery saved our son's life.

    In the aftermath, I imagined how, on rounds the next morning, the surgical house staff would share their stories of the night on call. Steve would be advanced from "a good case" to "a great case": a misdiagnosis by an older Connecticut pediatrician followed by the astute pickup by a PGY3 and then the dramatic findings at surgery. But for me and for Pam, the experience had no resonance of "a great case." There was no intellectual pleasure in solving a clinical puzzle, no charge of exhilaration from the drama of the operation. Instead, there was terror, raw and palpable, as we realized how close we had come to burying our first son.

    For many years after that perilous July 4th weekend, I would fix my face and try not to cringe when the students and house staff greeted me on morning rounds with the exclamation that they had "a great case" to present. The words and phrases we doctors use mold our thoughts and emotions, our actions and reactions to our patients and to each other. But I was reluctant to explain the reason for my discomfort. I did not want to inhibit the awe and excitement the ward team was expressing. So I would simply nod and, during the discussion, assiduously avoid using what had for me become tainted words.

    Not long ago, three friends of mine, all in their 50s as I am — one a physician, another a journalist-colleague, and the third a neighbor — received diagnoses of non-Hodgkin's lymphoma involving the intestine. "This is a great case," one of the hematology fellows said to me when the physician's biopsy was reviewed. I replied with silence. A friend my own age with intestinal lymphoma. Unexpectedly, seemingly capriciously, a bout of abdominal discomfort had proved to be not acid reflux but a clone of malignant B cells. It could easily have been me. And that truth erased any intellectual excitement I might have felt.

    I began to talk about who my friend was, his wife and his children, how he exhibited extraordinary calm in the face of intensive treatment. The hematology fellow seemed anxious to return to the subject of the molecular pathology of B-cell lymphoma, which genes were shuffled, why the apoptotic machinery of the cells had broken down.

    I recalled how impatient I had been at that stage of training when an older attending physician started to speak about the social and psychological circumstances of his patient. It all seemed a mere distraction, so tangential to the pathogenesis and clinical management.

    As more and more friends and family members of my generation became ill, the convenient illusion that there is a wide gulf between physician and patient was eroding. But to abandon this illusion would be detrimental, because it permits us to stand at the bedside without flinching at some of the most gruesome and threatening maladies that afflict men and women. As doctors age, I suspect that our shift of focus from the purely clinical to a perspective incorporating the emotional and spiritual reflects the realization that our powers are limited, that in the midst of human biology, we are also seeking knowledge about resilience and courage, attributes that we hope will be ours when disease strikes us.

    A few months after my conversation with the hematology fellow, I was sitting at dinner with Pam, as we reviewed with each other the events of our day. "I was referred a great case," she said. I was taken aback by her use of the phrase. She registered my reaction, but she continued. "A woman in her late 50s, with a blood pressure of 240 over 120, who had been dizzy and flushing. One doctor concluded that she was hypertensive and beginning menopause and prescribed standard medications for her blood pressure, including an ACE inhibitor. Then a consulting nephrologist, concerned about her kidneys, ordered an abdominal scan. And there it was: an adrenal mass. It turned out to be a pheochromocytoma."

    It had, I told myself, key elements of "a great case": the initial misdiagnosis, the confluence of disparate symptoms and signs of an unusual disease, the instance when standard therapies can be paradoxically harmful, the complex coordination of medical and surgical management. But still I resisted the appellation.

    "What makes it a great case?" I asked.

    Pam replied that it had provoked an animated discussion among the students and house staff, that important teaching points had been made. But then she reflected more deeply. "A great case because you not only make the diagnosis — you do something fundamental about it. You can really help."

    This conception came closer to the equipoise that had been eluding me. It was foolish to deny the profound intellectual excitement that came from medicine. It was also a hollow form of medicine that was practiced without factoring in the ultimate outcome. But that balance required exceptional circumstances. It necessitated knowing the outcome and could therefore be experienced only with hindsight, whereas thoughts and feelings are sparked from the start and demand a language right at the outset if they are to be shared.

    I still find myself unable, except in retrospect, to retrieve the language of my youth and speak about "a great case." It is as if medicine at this stage of my life has split into two streams — a current of marvelous biology and an undertow that pulls at the soul. From the bank where I stand, it is hard to imagine that these two streams can ever again flow as one.

    Source Information

    From the Beth Israel Deaconess Medical Center and Harvard Medical School, Boston.(Jerome Groopman, M.D.)