A Piece Of My Mind

A PIECE OF MY MIND

Howard

W

E ALL KNOW THE DICTUM: A PHYSICIAN WHO CARES

for himself has a fool for a doctor. Does a similar rule apply when your child has a fever, your mother has fatigue, or your spouse has unexplained weight loss? Howard is a 58-year-old psychiatrist who lives in verdant Oregon. Kind, empathetic, generous, insightful, uncomplaining. Healthy. With a wonderful family and a dog who commands a great deal of his attention. And walking his dog was how his illness began. The first symptom was thigh pain after the evening walk. The pain resembled claudication, but it did not disappear quickly with rest. Then the rigors started every evening, followed by a morning respite. The trip to North Africa suggested a tantalizing clue, but blood smears for malaria were negative. What about the dental implant, his love of gardening, or the travel a year ago to the Galápagos? The infectious disease specialist, the neurologist, and the rheumatologist each asserted that the illness belonged to a specialty other than his own. A medication effect? He was not on a statin, an obvious cause for muscle aches. Could his growing narcotic usage reflect a psychiatric diagnosis? Increasingly immobilized by shaking chills, but without fever, Howard spent a week in the community hospital. CT scans, a PET scan, an MRI, nerve conduction, a skin biopsy of an evolving rash, a muscle biopsy, and various blood tests failed to reveal a diagnosis. The only abnormalities were a slight elevation of muscle enzyme levels and a modestly elevated sedimentation rate. The hospitalist in charge of Howard’s care ordered an empirical trial of prednisone, but the diagnosis was still elusive. As the chief of rheumatology at a major university hospital, I frequently evaluate patients like Howard with complex, perplexing histories. If neurologists diagnose some diseases that they cannot treat, then rheumatologists treat some diseases that we cannot diagnose. But Howard’s saga was different in one overwhelming aspect: Howard is my younger brother. Like disease, being a physician in my family can be explained both genetically and environmentally. My father and his brother, cousins, siblings, a sister-in-law, my wife, and my children are all physicians. One brother is a neurologist, the author of three medical texts, and an expert on muscle disease. Howard’s illness seemed to nestle somewhere between neurology and rheumatology, as if the psychiatrist brother had intentionally sought a set of symptoms to force humility upon his siblings. Howard offered a ©2009 American Medical Association. All rights reserved.

reward for the correct diagnosis, but the nonphysicians in the family were doing as well as the experts. Consciously, at least, I was trying to avoid making decisions about my brother’s care. But with diagnostic studies having reached a seeming impasse and my brother still incapacitated, I began to lobby for a superior mesenteric angiogram to investigate the possibility of polyarteritis nodosa, a potentially fatal, rare disease primarily treated by rheumatologists. Unfortunately, all medical tests are imperfect and some are more imperfect than others. The angiogram can be normal, even when polyarteritis is later proved by biopsy. And when the test result is deemed abnormal, some element of subjectivity is often at play. Furthermore, the treatment for polyarteritis is cyclophosphamide, a lifesaving choice if the diagnosis is correct and a potential death sentence if the process is actually an infection mimicking the autoimmune disease. During residency, we jokingly called cyclophosphamide “vitamin C.” And if cyclophosphamide is vitamin C, polyarteritis is a vitamin C deficiency disease. So the angiogram was done and the radiology fellow said, “I think it’s normal”; and the senior radiologist said, “I think it’s normal”; and they both said, “And when the chief comes to conference tomorrow, he’s gonna read it as vasculitis.” Both physicians had an excellent perspective on the chief of radiology, because he did indeed conclude that the study showed definite vasculitis. He proceeded to solicit an opinion from an eminent emeritus professor, who echoed the chief’s conclusion. While I too reviewed the angiogram, I was less reassured when the chief indicated that his certainty was bolstered by the compelling clinical history, even though I, as a rheumatologist, found the rigors and diurnal fluctuation of symptoms unusual for a patient with polyarteritis. There’s a well-known story about an obstetrician who could predict the sex of the unborn with 100% accuracy without sophisticated tests. To every future mother he said, “I know that you are going to have a son. To show you how certain I am, I will record my prediction in writing and seal it in an envelope.” When a male child was delivered, the parents marveled at the psychic ability of the physician. But if a girl was born, the parents would understandably question the physician’s predictive powers. In response he would retrieve the sealed envelope, which always contained the prophecy: Girl. A Piece of My Mind Section Editor: Roxanne K. Young, Associate Senior Editor. (Reprinted) JAMA, September 2, 2009—Vol 302, No. 9

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A PIECE OF MY MIND

Knowing this apocryphal tale, I should not have been so surprised that the official reading of the angiogram stated, “While there are a few suspicious vessels in this angiogram, we would not base a diagnosis of vasculitis solely upon this study.” In essence, the radiologists were predicting either a boy or a girl. Such hedging is altogether frequent in our profession. Although diagnoses are sometimes conveyed to the patient as indisputable, every medical pronouncement has an element of uncertainty. I had sent my brother to two different rheumatologists. The community rheumatologist who was consulted is superb, although it was also clear he was uncomfortable making decisions in the midst of such a medical family. And the university rheumatologist is extremely talented, but I am his immediate supervisor. He saw my brother relatively early in the illness, when an autoimmune process like polyarteritis was low on the list of possible diagnoses. The junior professor thus pronounced his confidence that Howard’s illness was not rheumatologic. As the illness evolved and the test results accumulated, I sought my colleague’s counsel, but he would undoubtedly feel uncomfortable disagreeing with me. The hospital has a food chain: the student asks the intern; the intern turns to the resident; the resident relies on the staff, who in turn consults with the chief. As the chief, I could seek advice from my junior colleagues, medical literature, and national experts. So I described Howard’s history to two world-renowned vasculitis experts, who both encouraged the use of cyclophosphamide, although a consultant 2000 miles away is different from a consultant at the bedside. Howard was relieved to learn that the Harvard radiologists were convinced that he had polyarteritis. I never told Howard that the Harvard physicians said that the renal vessels—that the Oregon doctors deemed abnormal—were judged in Massachusetts to be entirely normal. Instead the Harvard physicians made the diagnosis based on putative abnormalities of the mesenteric artery. I have written several essays in my life, some published, some not. I want to write a confessional called “I’m a Killer,” about my patients who have had fatal reactions from a medication I prescribed. In an unpublished essay, I describe the role of a physician as being akin to waiting tables in a restaurant. In an idealized world, we elaborate on a menu of

930 JAMA, September 2, 2009—Vol 302, No. 9 (Reprinted)

options, telling the patient of the advantages and disadvantages of each dish. My father’s medical practice was far more paternalistic: an arm around the shoulder and a bit of autocratic advice. I tried the restaurant approach with my brother, only to discover that he and his physician wife wanted me to choose the entre´e. As I see it, medical decisions should be made dispassionately. But both the physician and the patient have emotions. The physician must set this emotion aside and make a decision based on logic. And the physician must ease the emotional stress of the patient because that anxiety can ricochet through the decision-making process and perturb its logic. I knew that by making a decision for my brother, my opinion would never be devoid of emotion, and, in a sense, it could not be logical. But Howard was now bedridden and in pain, unable to work after two months of searching for a diagnosis. Confronted with ambiguous data on which to base a lifethreatening decision involving my family, I opted for cyclophosphamide and watched as it coursed through my brother’s veins. When I wrote his orders, I faced a series of decisions beginning with whether I should take responsibility for my brother in the setting of uncertainty, some controversy, and definite risk. Once I made the decision to take charge, the choice of medication and dosage was straightforward by comparison. Some degree of uncertainty touches every offer of medical advice. Touching too firmly creates a barricade. For me, surmounting the obstacle of uncertainty to provide medical care for my family was hard. Not providing medical care for my family would have been harder still. James T. Rosenbaum, MD Portland, Oregon [email protected] Editor’s Note: Howard has responded promptly to cyclophosphamide. He has now received 3 cycles of this immunosuppression, discontinued narcotics, and nearly eliminated prednisone. He works full time and plays basketball more skillfully and more energetically than his brothers. His dog is very, very happy. Funding/Support: The author receives funding support from the Stan and Madelle Rosenfeld Family Trust, the Fund for Arthritis and Infectious Disease Research, and Research to Prevent Blindness. Additional Contributions: I am grateful to Lisa S. Rosenbaum, MD, Sandra Lewis, MD, Jennifer L. Rosenbaum, and Joe Ensign-Lewis, who critically reviewed the manuscript. Dedication: This essay is dedicated to the memory of my father (and Howard’s father), Edward E Rosenbaum, MD, who passed away May 31, 2009.

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