Tags

, , , , , , , , , , ,

“Tov she-barofim le-gehinom: The best doctors are destined for hell” (Babylonian Talmud, Kiddushin 82a)

The comment is made in the Talmudic Sages’ discussion of which professions should be discouraged by parents when advising their sons. It comes just after donkey-driver, camel-driver, wagon-driver, sailor, shepherd, and storekeeper are denigrated as the professions of thieves. The statement is disturbing and puzzling, particularly in the context of a theology that strives to uphold life and a culture that for millennia has revered the practice of the healing arts. There are enough jokes about Jewish mother’s fervent dream that their kids become doctors to confirm that centuries of Jewish parents have disregarded this Talmudic warning. What exactly was Abba Gurion of Zadyan warning against?

Rashi observed that the “best doctors” were healthy, wealthy, and not so devout. He worried that they would refuse to treat the poor. Other interpreters commented that doctors who think themselves superior might not consult with other doctors. The death of their patients might result. Picking up on this line of reasoning, the most “accomplished” physicians may become oblivious to observations and data that contradict held wisdom, surround themselves with nodding heads instead of inquisitive colleagues and students, and lose a sense of humility in the face of the body’s complexity. Taken this way, the Talmud is not saying “Mamas, don’t let your babies grow up to be doctors.” It is saying don’t raise them to be haughty physicians.

Although the Talmudic Sages used strong language, they did not envision a time or a circumstance when people trained to heal would create the conditions of hell on earth.  Exactly this happened during the Holocaust. Germany’s “best doctors” advocated theories of eugenics, euthanasia, Aryan superiority, anti-homosexuality, and Anti-Semitism that preceded the genocide.  The medical elite, raised by the finest families and trained at the most prestigious institutions, were the architects of the concentration camps and participants in mass murder. Most repulsively, Nazi physicians tortured and killed prisoners in horrifying ways in the name of “experimentation” to further an immoral and nightmarish version of “medical science”.

Certainly this is not the only historical episode where physicians have turned away from the profession’s best aspirations to become a menace to their fellow people (e.g. the Tuskegee and Guatemalan syphilis studies). The active participation of German physicians in the Holocaust is the most dramatic, widespread, and well-documented example. In a haunting and essential essay entitled “Without Conscience” that appeared in the New England Journal of Medicine in April, 2005, contemplating the essential role of physicians in the Holocaust, Elie Wiesel asks, “What made them forget or eclipse the Hippocratic Oath? What gagged their conscience? What happened to their humanity?”

Medical eponyms: dead, white, male and sometimes even Nazi

Medicine’s most visible way of honoring its best doctors has been to name something after them. Eponyms pervade every aspect of biomedicine: diseases, organs, anatomic structures, cells, physiologic principles, equations, surgical techniques, laboratory techniques, devices… If you can name something in medicine, you can name it after a discoverer, a promoter, an early adopter, or someone who wrote a paper about it at some point in dusty history.

The use of eponyms in medicine has fallen under heavy attack, and for good reason. The most substantive objection might be just that: eponymous names have no substance. The term “Crohn’s disease” doesn’t tell one anything about the symptoms caused by the condition, the organs it involves, or mechanisms of its pathophysiology. Eponymous naming squanders the opportunity to use a term that describes the thing being named. “Ulcerative colitis”, the name of a disease that is a close sibling of Crohn’s disease, gives one something to work with.  “Colitis” – inflammation of the colon and “ulcerative” – a description of what the inflamed colonic lining looks like. The glossary of medicine would be much more accessible to the people who need to know – patients, students, practitioners – if more of the names had intrinsic meaning.

Eponyms have also strained to resist the assault of several decades of post-modern scholarship that indict these names as reinforcing the hegemony of dead white males in medicine (and other areas of intellectual endeavor). It is a fair point. A vibrant non-white female may be discouraged from pursuing greatness in medicine if naming practice emphasizes that past glory was consistently attributed to the dominant, majority demographic. Why use names to further reinforce hierarchies in a medical culture that is already profoundly hierarchical?

Eponyms raise another, more thorny issue. Affixing an eponymous name to a thing or a concept brings with it strong associations to the personal history and attributes of the named individual. Opponents of the Affordable Care Act prefer to label it as “Obamacare.” For inspiration, Republicans drew on the Democrat’s previous example of “Reaganomics”. Ideas are often named after their creator-proponents and are colored by the association (e.g. McCarthyism, Marxism, Buddhism, Christianity…). When the named individual is obscure even in the circles in question, the name can become an abstracted term, a handle devoid of intrinsic meaning. Most medical eponyms fall in this category. We usually don’t know anything about the biography of the named person. We are left to infer that the individual’s life arc intersected with the story of our understanding of the natural phenomenon, even for a brief moment in time.

The advantage of eponyms is that one word can refer to something that might take a paragraph to explain. The disadvantage is that when we hear that one word, we all might have a different explanatory paragraph in mind. It must be noted that even though they are just one word, the surnames can be a bear to spell and pronounce. I heard about five different pronunciations of “Sjogren’s” when sportscasters struggled to report Venus Williams’ withdrawal from the US Open a few weeks back due to her recent diagnosis with the systemic autoimmune disease.

Medicine is learning the hard way that if you are going to name things after people it is best to know something about them. Historical investigators have discovered that several German physicians who had diseases named after them based on work done during the pre-war decades went on to have varying degrees of affiliation with the Nazis and participation in the atrocities of the Holocaust. The effort to replace these disgraced surnames with new, descriptive names has revealed that eponyms are entrenched and habit-forming.

Rheumatology, the field of medicine that focuses on autoimmune and inflammatory diseases and seeks to better understand the immune system, has the disgraceful distinction of having a disproportionate share of Nazi doctor disease names. After a decade of painstaking historical examination revealed that Drs. Friedrich Wegener and Hans Reiter were both members of the Nazi party and were implicated in war crimes, rheumatologists are now facing the challenge of purging “Wegener’s granulomatosis” and “Reiter’s Syndrome” from our language and our literature. I think that to understand what these names represent, it is important to take a few moments to discuss what is known about the men they referred to.

Dr. Friedrich Wegener, Pathologist of the Lodz Ghetto

Friedrich Wegener was born in Varel, a village in northwestern Germany 16 km south of Wilhelmshaven, in 1907. His father was a small town surgeon. His mother was Swedish and Wegener’s early education was bilingual. Wegener began his medical studies in Munich in 1927 and appears to have been a good athlete. He was on the team that won the German schleuderball championship in 1931 (this appears to be some kind of team handball played with a leather ball hurled by an attached strap).

In the 1930s, Wegener’s career trajectory mirrored that of the growth of the Nazi party. He received his undergraduate (medical) degree from the University of Kiel in 1932. In September 1932, he joined the Sturm Abteilung (“brownshirts”), the paramilitary storm troopers of the early Nazi period. He joined the National Socialist Party on May 1st, 1933, the day that Hitler seized power. Wegener’s Nazi party membership number appeared in a 2006 article in The Lancet, the first to describe his war-time activities: 2 731 267. He joined the Nazi party’s physician organization and its Organization of Academic Teachers.

In June 1934, Wegener did an autopsy on a 38 year old man who died from kidney failure after a prolonged febrile illness. He had a sunken depression of the bridge of his nose known as a saddle nose deformity. There was inflammation of the nasal mucosa and cartilage with destruction of the nasal septum. The kidneys were large and swollen. Wegener’s histopathological examination showed a peculiar type of inflammation in the nasal mucosa and the kidneys: necrotizing inflammation with granulomas. Wegener’s medical school friend, Heinz Klinger, reported a similar case in 1931 as a “borderline variant of periarteriitis nodosa.” Wegener did studies to exclude an infectious cause of the inflammation and presented the case at a 1936 meeting in Breslau (the paper was called “About generalized septic vascular diseases”). The talk prompted much discussion and the pathologists assembled were able to recall similar cases that they had seen at autopsy.

Wegener was appointed Lieutenant Colonel in the medical corps in 1938 and moved to Breslau to work with the new head of pathology, Dr. Martin Staemmler. Staemmler was a renown theorist of “racial hygiene”, and had published a widely read book called “Nurture of Race in the National Ethnic State: An exhortation to those who feel responsible for the future of our people” in 1933. In Breslau, Wegener found a third case (Klinger’s included): a 36 year old woman with chronic rhinitis who also died of kidney failure. Staemmler was able to recall the details of an additional case that he had seen years before.  With the encouragement of Staemmler, in 1939 Wegener finally wrote up and published the four cases in a German pathology journal: “About a peculiar rhinogenic granulomatosis with marked involvement of the arterial system and kidneys.” In a review article he published in 1990, Wegener reminisced, “The disease was on the verge of being discovered. Somebody had to do it.”

The German invasion of Poland began on September 1, 1939. A letter from Staemmler on September 19th places Wegener in Lodz, Poland, newly installed there as army pathologist. Lodz was a prominent industrialized city in occupied Poland. It was also a major center of Jewish life in the Pale of Settlement, the region of Imperial Russia including Poland, Ukraine, Moldova, Belarus, and Lithuania, where Jewish settlement was allowed by Catherine the Great in 1791. The Hassidic Master’s remarkable intellectual and spiritual contributions were made here. Lodz was the first German occupied city to confine its Jewish population in a closed ghetto. Wegener’s Institute for Pathology and Forensic Medicine was initially located in a villa near the northwest border (walls?) of the ghetto. Of the 250,000 inmates imprisoned in the Lodz ghetto in 1940, less than 1,000 survived the Holocaust. Wegener’s colleagues, including two physicians named Grohmann and Kleebank, selected the Jewish prisoners who were transported from the ghetto to be murdered at the Chelmno death camp between 1941-1944. From 1944 until the end of the war, the remainder of Lodz Jewry was killed at Auschwitz.

What direct role did Wegener play in the atrocities at Lodz? In addition to his military role, Wegener appears to have served as pathologist for the municipal health office (Gesundheitsamt) which performed 50-100 autopsies per month. There is evidence that he performed autopsies on ghetto inmates who died while being transported to Chelmno. On January 25, 1941, Wegener reported autopsy findings of a child from the ghetto that died at the railway station while being deported.

Did Wegener participate in the Nazi physician’s program of torture “experimentation”? Woywodt and Matteson, authors of the ’06 Lancet report, found a 1944 letter in Wegener’s hand: “Today, your manuscript about your work on air embolism has arrived safely in my institute. I hope I will be able to concern myself with this matter in greater detail in the near future.” There are not many things that cause air embolism, gas bubbles in blood vessels that can act like clots and block blood flow. Lung trauma, crude abortion procedures that puncture the vagina or uterus, and decompression sickness in divers (“the bends”) can do it. At the Dachau concentration camp, Dr. Sigmund Rascher used a low-pressure chamber to subject prisoners to simulated altitudes of up to 66,000 feet (20,000 meters) in 1942. 80 of the 200 subjects were killed by the exposure, the survivors were executed. Rascher is rumored to have dissected the brains of still living subjects for pathological examination. We will likely never know if the air embolisms in the manuscript eluded to in Wegner’s letter were the result of Rascher’s torture.

In the later stages of the war, Wegener left Lodz and was reassigned as a field surgeon. His unit was captured by American forces and he spent some time as a prisoner of war. At the conclusion of hostilities he was released, briefly worked as an agricultural laborer, and in 1947 he successfully underwent a “de-nazification” hearing with several witnesses testifying to his honorable conduct during the war. This cleared him to resume medical practice and he found work as a pathologist in private practice in Lubeck.

In 1954, Godman and Churg (“Churg” would go on to appear in “Churg-Strauss Syndrome”, the name used for another rare form of vasculitis) published a paper entitled “Wegener’s granulomatosis: pathology and review of the literature” in the AMA Archives of Pathology. The unsuspecting Wegener became an academic star. A medical school was started at Lubeck in 1964 and Wegener became Professor of Pathology. By all accounts, his students admired him as a dedicated teacher. When he retired to resume private practice in 1970, the Lubeck students organized a torchlight procession. In 1986, Wegener was rediscovered a second time by investigators in the growing field of vasculitis (inflammatory disease of blood vessels). He went to international meetings and helped to found a patient support group. In 1989, Wegener received a “Master Clinician” Award from the American College of Chest Physicians for his description of the vasculitis known as “Wegener’s Granulomatosis.” Apparently, the award was specially invented by friends of Wegener – it has not been bestowed before or since. He died of a stroke at the age of 83 in 1990.

Was Wegener a war criminal?

Woywodt and Matteson searched the archives of Yad Vashem, the Simon Wiesenthal Center, the Berlin Document Center, and the British National Archives and Records Administration and found no indication that Wegener was listed or tried as a war criminal. The Polish Institute for the Prosecution of German War Crimes had a card stating that a “Dr. Wegner [misspelled], Director of the Pathological Institute at Lodz” appeared on a wanted list and that his file had been forwarded to the United Nations War Crimes Commission in May, 1944. Wegener’s name appeared on the 1948 central list of war criminals and security suspects (CROWCASS). The Allied Forces compiled many such lists. The vast majority of those named were never investigated or located, let alone prosecuted.

Wegener’s story reveals the difficulty of assessing the morality of individual action through the lenses of archival history, complimentary testimonials, and honorific obituaries. Was Wegener a well-intentioned physician-scientist merely doing what was necessary to establish a career during the horrible years of Nazi rule that were his late 20s and 30s? Was he an ideological supporter of Hitler who enthusiastically volunteered for service and helped direct the “ghettoization” and near-complete annihilation of 250,000 Lodz Jews? Even if he merely followed orders, was he a participant in atrocities? The historical record does not make clear what we would most like to know: motive and intent.

The Unambiguous Case of Hans Reiter

Transcripts from Reiter’s interrogation while he was imprisoned at Nuremberg from 1945 to 1947 reveal that as president of the Reich Health Office he acted as “quality control officer” for the involuntary sterilization, euthanasia, and murder of Germany’s mental hospital population. He helped design and implement a “study” at Buchenwald in which over 200 prisoners died after receiving an “experimental” typhus vaccine. The two ranking doctors at Buchenwald committed suicide before they could be captured and interrogated. Reiter was not one of the 23 physician defendants in the “Doctor’s Trial,” United States of America v. Karl Brandt et. al., the first of the Nuremberg Tribunals. He was released, possibly because he provided the Allies with intelligence regarding Nazi germ warfare research.

Reiter reported the case of a military officer with arthritis, eye inflammation, and urethritis in 1916. Within weeks, Fiessinger and Leroy published a more accurate description of four cases in a French journal. The recognition of the pattern of inflammatory disease of the joints, eyes, and urethra in the medical literature goes back to the early 1500s with more complete reports by Stoll in 1776 and Brodie in 1818. The syndrome that came to be known as “Reiter’s Syndrome” is an inflammatory disorder that is triggered by bacterial infections, most often Chlamydia but also intestinal bugs. It occurs in genetically susceptible individuals, particularly those with the B27 subtype of the HLA gene. Of course, Chlamydia is a sexually transmitted disease. The reports of military officers with the syndrome during World War I may well have been due to action in the brothels while on leave from action in the trenches.

Although Reiter’s was not the first, most accurate, or largest case series, he received eponymous recognition due to a 1942 paper published by two rheumatologists at MGH which referred in the title to “so-called Reiter’s disease”. Reiter had a peaceful post-war life and received accolades including the Great Medal of Honor of the Red Cross. JAMA published a glowing obituary following his death in 1969 which referred only in passing to his “National Socialist” affiliation.

How best to confront the past?

Hans Reiter would meet anyone’s definition of a Nazi war criminal. Friedrich Wegener’s record is deeply troubling but somewhat ambiguous. Reiter made no significant scientific contribution with his report on reactive arthritis. Wegener published an early and tentative report on four patients. It is fair to say that he had little idea of what he was looking at, other than that it was new to science. Reacting with glacial pace, the rheumatology community is finally making moves to repudiate the eponyms of “Wegener” and “Reiter”. An account of the record of Reiter’s interrogation at Nuremberg did not appear in the rheumatology literature until 2000. An editorial by a group including Dr. Ephraim Engleman, one of the authors of the 1942 paper, formally retracting the use of “Reiter’s syndrome” did not appear until 2007.

The Lancet rejected the paper on Wegener’s history when it was first submitted in 2000. The editors’ explanation for the “not acceptable in present form” decision was: “We understand that he was imprisoned after the war for activities undertaken under the Nazi regime, and that was why he was not practicing as a pathologist.” Seriously? With “Wegener’s granulomatosis” in universal use and no mention in the medical literature of his Nazi activities, The Lancet acted as though the first detailed analysis of the historical and scientific record was old news. It took six more years of painstaking research before The Lancet was persuaded that the medical community needed to know about Wegener’s past.

Coming up with descriptive names to replace eponyms has been a medical and organizational challenge. A new name for the entity of arthritis, uveitis, and urethritis triggered by infection was pretty easy: “reactive arthritis.” The term had already been widely used in the literature, seems to describe the mechanism of the disease and rolls off the tongue nicely. The disease formally known as “Wegener’s” is more complicated and so was the re-naming process. An international group of 13 vasculitis experts met in November, 2010 to reach consensus on an alternate name. The drum roll announcement appeared in Arthritis and Rheumatism in April, 2011: “granulomatosis with polyangiitis (Wegener’s)” with the acronym of GPA. The parenthetical survival of “Wegener’s” was felt to be necessary for clarity given the eponym’s firmly entrenched status and to facilitate on-line literature searches. The experts expressed hope that the trailing “(Wegener’s)” can be phased out over a period of “several years”.

The disease entity now known as granulomatosis with polyangiitis is a rare disease, very rare in children. Despite being so unusual (we recently discussed another enigmatic vasculitis, Takayasu arteritis), we currently follow about 10 children with the disease in our clinic. We consider the diagnosis in other patients who won’t end up having the condition. So yes, we have plenty of opportunities to talk about the disease every day. I’ve only heard “GPA” referred to in jest, as that cumbersome new name. In formal talks, chapters in textbooks published this year, conversations with patients and families, email list-serves, and cafeteria shop-talk we continue to say “Wegener’s.”

I try, but do not always find the opportunity, to teach residents and students the story behind the term “Wegener’s.” I think this is preferable to merely telling them that the term is no longer in use. I have found it difficult to correct my colleagues’ usage, particularly my senior colleagues. The right way to address this will be to put it on the agenda – a discussion of our use of eponyms at a team meeting.

The haunting questions

My grandparents survived the Holocaust in Poland (now Ukraine). My mother was born in a displaced persons’ camp in Berchtesgaden, Germany. She was the first Jewish baby born in the hospital at the foot of the mountain where Hitler’s Eagle’s Nest was perched. It was a nerve-wracking delivery. My mom was born at 32 weeks, very premature by 1946 standards. My grandmom told the story that the doctor who delivered my mom in the morning was arrested for being an SS officer that afternoon. The doctor did his job. But what was his intent?

Wiesel is left to answer his question – What happened to the Nazi doctor’s humanity? – with another question: “Am I naïve in believing that medicine is still a noble profession, upholding the highest ethical principles?” For a source of hope, he draws on his memory of the physicians he knew in the concentration camps who tried desperately to relieve the suffering.

Who are the “best doctors”? Our best hope of fulfilling the aspirations of this and other noble professions might be that we continue to ask the haunting questions. And that we ask them of ourselves.

Advertisements