Tay-Sachs and “Jewish” Diseases

EARLY DISCOVERY AND RACIALIZATION

TAY-SACHS DISEASE AND ANTI-IMMIGRATIONISM

BIBLIOGRAPHY

Tay-Sachs disease (TSD) is a rare metabolic disorder prevalent in, but not exclusive to, Jews of Eastern European, or Ashkenazi, descent. Though most commonly associated with Ashkenazi Jews, TSD has also been found in some French-Canadian communities in eastern Quebec, among the French-Americans of southwestern Louisiana, and, to a lesser extent, in the general population. TSD has several forms, including the juvenile and late-onset adult variations, but the most frequently occurring (though still rare) form is the classic and fatal infantile Tay-Sachs. (Other so-called Jewish diseases include Niemann-Pick, Canavan’s, and Gaucher’s disease.)

TSD is caused by a genetic mutation that affects the body’s manufacturing of the Hex A enzyme, which functions to regulate the level of lipids (fat) in the brain and nervous system. When this enzyme is deficient, an excess of fat accumulates, leading to profound neurological deterioration. In Infantile TSD, early signs include red spots on the retinas, enlargement of the head due to the increase of water around the brain, an alteration in muscle tone, hyper-acusis (extreme sensitivity to sound), mental retardation, and social withdrawal. In time the vision becomes completely impaired and the child becomes paralyzed, with death usually occurring by the age of four or five. Infantile Tay-Sachs is considered the most severe of childhood lipid-storage disorders.

As one of the first of a series of genetic diseases identified at the molecular level using recombinant DNA technology, TSD has become widely understood in Mendelian terms to be an autosomal recessive disorder. The child of two genetic carriers or heterozygotes has a one-in-four chance of inheriting the allele for TSD from both parents and subsequently developing the disease. The chromosomal connection was determined in 1969 by the American neuroscientist John O’Brien, and screening technology for the disease was subsequently developed in the early 1970s. Since that time, Jewish individuals have participated in Tay-Sachs blood screening programs throughout the world, and along with the use of pedigrees and genetically strategic mate selection, such widespread genetic screening has led to the virtual eradication of this disease, at least among the world’s Jewish communities.

TSD is one of many diseases that are more common in an ethno-racial group and have been labeled as ethno-racial diseases. In reality, however, it is not possible to identify pure races based on gene frequencies, so there is really no such thing as a “Jewish disease” per se (nor a black, Italian, Aboriginal, or other racial or ethnic disease.) As Robert Pollack argues, “Jews are not in fact a single biological family; there are no DNA sequences common to all Jews and present only in Jews.” Thus, so-called Jewish diseases, such as TSD, are evidence rather of a shared ancestry, including “periodic massacres of such ferocity that only a small number of families were able to survive.” That a proportion of Jews possesses similar genetic mutations “suggests that the Jews whose ancestors came from the Pale—about nine of every ten Jews alive today—are the descendants of a small remnant of a few thousand families who survived a particularly devastating pogrom in the Pale of the mid-1600s” (Pollack 1999, p. 194). In other words, there are a number of single-gene traits (Tay-Sachs being one of them) that, if used as loci to regroup populations, would not yield the racial groups assigned by classical physical anthropologists.

Nevertheless, the construction of racial disease categories has long been a means of demarcating human groups. Sickle-cell anemia, for example, has been classified as a “black disease,” or a disease of “negro blood,” since it was first diagnosed in 1910 (Tapper 1999). Though it begins several decades earlier, the history of Tay-Sachs disease takes a similarly racialist trajectory, insofar as it has, since the outset of its history in the late nineteenth century, always been seen as a “Jewish disease,” despite evidence that it occurs in other populations.

The remainder of this entry will consider this perception in its historical and sociological context. In particular, it will describe the early discovery and medical racialization of TSD (its construction as a racial disease) in terms of anti-immigrationism directed at Jews, especially in the United States in the 1910s and 1920s, when both Jewish immigration and the discourse of eugenics were on the rise.

EARLY DISCOVERY AND RACIALIZATION

The symptoms of TSD were first described in 1881 by the British ophthalmologist and surgeon Warren Tay (1843– 1927). At a meeting of the Opthalmological Society of the United Kingdom (recorded in the first volume of its Transactions), he reported that, upon examination of his patient’s eyes with an ophthalmoscope, he had observed an inexplicable “brownish-red, fairly circular spot” at the center of the optic discs. In the society’s Transactions of 1884, Tay published a similar description—the third instance of the disorder in the same family.

A few years later, in 1887, the American neurologist Bernard Sachs (1858-1944) made a similar report, though he had not yet heard of Tay’s discoveries. Sachs began his medical career in New York, where he had a private practice for the treatment of mental and nervous diseases. By 1887 Sachs was widely regarded as one of America’s leading clinical neurologists and had been appointed as an instructor at the New York Polyclinic Hospital. It was there that he reported on his first case of “arrested cerebral development” (as he described it), which he presented before the American Neurological Association and then reported in The Journal of Nervous and Mental Disease. Sachs also described a red spot in the eyes of his two-year-old patient, a little girl he referred to as “S.”

In 1896, in a report in the same journal, Sachs named the condition “Amaurotic Family Idiocy” (though it would not be long before doctors began to refer to the disease as Tay-Sachs). Still unaware of Tay’s earlier reports, the fact that Sachs chose to include “familial” in the disease name is significant, because it reflected the growing practice of tracing patients’ lineage as part of the diagnosis of TSD. In other words, it was already becoming apparent to doctors that the disease was hereditary. What was less clear to them, however, was how inheritance worked exactly. (Although Mendel had already published his work on pea plants, it would be decades before his findings were formally taken up.)

The emergent nature of the understanding of heredity led physicians to entertain various causation theories, including the possibility that TSD was transmitted through breast milk. This theory was rejected because, as Dr. Sachs observed (in Osler’s Modern Medicine, 1910), several of his young patients had not been “nursed by their own mothers, but by wet-nurses of a different race and different nationality.”

With this observation, Sachs introduced a key element of TSD, namely the perception that it was exclusive to the apparently overly inbred “Hebrews,” especially those from Russia. In fact, it was not long after Sachs’s first case report in 1887 that TSD was declared a Jewish disease. Indeed, by 1895, Dr. Sachs had reported in A Treatise on the Nervous Diseases of Children for Physicians and Students that of the cases known so far, the condition had been observed almost entirely among Jews.

Many doctors agreed that “genuine TSD” was exclusive to the Jews. In 1910 the physicians Hildred B. Carlyll and F. Mott recorded in the Proceedings of the Royal Society of Medicine that the Jews possessed an “inborn lack of specific vital energy of the nerve-cells, due to a racial inherited failure of the germinal determinants of the nervous system.” In contrast, the cases with special features of interest were those in children who, according to the physician R. Sattler, “can not like all others reported be said to have a Jewish parentage” (Sattler 1914). These remarks suggest that the Jewish heritage of patients with Tay-Sachs was the defining feature of the disease. Thus, evidence of TSD in Gentile patients was met with skepticism, especially if the doctor believed that there was Jewish blood in the patient’s history.

However, not all doctors were prepared to accept the classification of TSD as exclusively Jewish, nor the notion that Jews were biologically inferior, and to this end they adapted the discourse of race science in ways that served their own interests (Rafael Falk, 1998; Eric L. Goldstein, 1997; and Mitchell Hart, 2001). For some, such as the British Jewish biologist and physician Redcliffe N. Salaman (1874–1955), the goal was to counter anti-Semitism by advocating Zionism. Salaman used the tools and language of genetics and inheritance to show that the Jews were distinct and in need of their own Jewish state. Amaurotic Family Idiocy was, for him, a key indicator of Jewish raciality: As he wrote in a 1911 article titled “Heredity and the Jew,” published in the Journal ofGenetics, according to “all the authorities” TSD was “probably unknown outside the Jewish people.” Salaman later argued before the 1921 Second International Congress of Eugenics (published as Eugenics in Race and State: Second International Congress of Eugenics, 1921, Vol. 2, edited by Charles B. Davenport) that Jewish Tay-Sachs was evidence in favor of the Jews’ “ethnic differentiation.”

For others, such as Dr. Maurice Fishberg, race science provided a means of advocating for Jewish assimilation into U.S. society. As an anthropologist, the medical examiner for the United Hebrew Charities, and himself a Russian Jewish immigrant to New York, Fishberg was a strong opponent of Zionism and in his 1911 treatise The Jews: A Study of Race and Environment he used race science to show that Jews were no more diseased than or inferior to other inhabitants of New York.

TAY-SACHS DISEASE AND ANTI-IMMIGRATIONISM

For those who saw the Jews as a problematic racial group—biological arguments served a different set of interests; specifically, they were used to argue against Jewish immigration to the United States from eastern Europe. Second only to Italians, eastern European Jews (of varying degrees of religiosity) were increasingly constructed as troublesome and problematic as their numbers grew (between 1881 and 1914, more than two million Jews entered the United States). Various articles on eugenics and public health that appeared in periodicals such as Popular Science Monthly (e.g., Alfred C. Reed’s “Immigration and the Public Health” and J. G. Wilson’s “The Crossing of the Races”) typified the view that Jewish immigrants had undesirable habits—such as inbreeding or “racial incest” (Friedman, quoted in Reed 1913, p. 325)—and were inherently “clannish” (Wilson 1911, p. 494–495) because they continued to practice their distinct religion, speak their own language (Yiddish), and had a system of organization based on their communities of origin, newspapers, theater, and more (Waxman 1999). These perceived faults amplified the Jews’ tendency to isolate themselves rather than assimilate American standards, as well as exacerbate their marginal status as “the Other.” Central to their marginalization was their construction as a “community of the ill” and a “collective at risk”—a stereotype that reinforced a widely held perception that race and disease were linked (Gilman 2003) and reason enough to implement restrictive immigration policies. Reports of Tay-Sachs from this period suggested that the alleged link between race and disease was not limited to infectious conditions such as tuberculosis, typhus, and cholera, but came to include genetic disorders such as TSD as well (Kraut 1994; Markel 1997).

In fact, the organized eugenics movement that had emerged in the United States by 1910, with its belief that social ills were the result of defects in heredity, along with

the anti-immigrationist opinion that “the gates [had been] left open too long” (as the well-known nativist Edward Alsworth Ross put it in his Roads to Social Peace, The Weil Lectures on American Citizenship [1924]), led to the growing view that America’s immigration problem was “at heart a biological one” (Higham 1992, p. 151). Kenneth M. Ludmerer (1972) similarly describes the emergence of genetic theories that marked southern and eastern European immigrants as biologically inferior and warned that race-mixing would lead to the nation’s overall decline. These sorts of arguments directly informed American policy to restrict immigration. With a growing resentment directed at Jews (and other immigrants) who were seen to be taking what few jobs were still available, initial temporary legislation to restrict immigration to certain quotas passed through Congress in 1921. By 1924 a more permanent piece of legislation, the Johnson-Reed Act, further restricted immigration.

It is not coincidental that the TSD cases reported in the years leading up to and following this legislation contained some of the most explicit racialist and nativist rhetoric, portraying “the Jew” as profoundly susceptible to nervous diseases (including Tay-Sachs) due to an inferior racial constitution. Consistent with this was the tendency for doctors to include in their reports of TSD specifics about their patients’ foreign origins, often noting how long it had been since the patient’s family had immigrated to the United States, or simply describing the patients, as Drs. Carlyll and Mott did in 1910, as being from “alien families.” Some doctors emphasized that although there had been reports of the disease from eastern Europe, it was really a problem for immigrant families in America. What they were implying by this was that the foreign-born population was bringing their health problems with them to American soil. There is certainly no mistaking this sentiment in Dr. Earl Tarr’s observation in a 1916 report in the Louisville Monthly Journal of Medicine and Surgery that his colleagues “frequently stated that the condition is rare in Russia and that it occurs in children [only] after they have emigrated to the United States.” Not surprisingly, this was seen as burdensome to American society. In contrast, Gentile patients demonstrating symptoms of TSD were usually described simply as being of American extraction, and often as showing no evidence of consanguinity (i.e., of inbreeding).

Reading the early reports of TSD against the backdrop of racial and anti-immigration ideology makes it possible to see how this disease category was deployed and how medical discourses both shape and are shaped by cultural concerns. Tay-Sachs represented a threat that was not confined to the children in whose bodies it manifested in the immediate present; rather, it was seen as having the potential to be a permanent and damaging feature of American society as long as Jewish immigrants continued to have unfettered access to the nation. Thus, the early history of TSD was as much about foreignness and immigration as it was about health and disease.

SEE ALSO Diseases, Racial; Sickle Cell Anemia.

BIBLIOGRAPHY

Falk, Rafael. 1998. “Zionism and the Biology of the Jews.” Science in Context 3–4: 587–607.

Fishberg, Maurice. 1911. The Jews: A Study of Race and Environment. New York: Charles Scribner’s Sons.

Gilman, Sander. 2003. Jewish Frontiers: Essays on Bodies, Histories, and Identities. New York: Palgrave Macmillan.

Goldstein, Eric L. 1997. “‘Different Blood Flows in Our Veins’: Race and Jewish Self-Definition in Late Nineteenth Century America.” American Jewish History 86 (1): 29–55.

Hart, Mitchell B. 2001. “Racial Science, Social Science, and the Politics of Jewish Assimilation.” Isis 90: 268–297.

Higham, John. 1992 (1955). Strangers in the Land: Patterns of American Nativism, 1860–1925. New Brunswick, NJ: Rutgers University Press.

Kraut, Alan M. 1994. Silent Travelers: Germs, Genes, and the “Immigrant Menace.” New York: Basic Books.

Ludmerer, Kenneth M. 1972. Genetics and American Society: A Historical Appraisal. Baltimore, MD: Johns Hopkins University Press.

Markel, Howard. 1997. Quarantine! East European Jewish Immigrants and the New York City Epidemics of 1892. Baltimore, MD: Johns Hopkins University Press.

Paul, Diane B. 1995. Controlling Human Heredity: 1865 to the Present. Atlantic Highlands, NJ: Humanities Press.

Pollack, Robert. 1999. The Missing Moment: How the Unconscious Shapes Modern Science. Boston: Houghton Mifflin.

Reed, Alfred C. 1913. “Immigration and the Public Health.” Popular Science Monthly Vol. LXXXIII: 320–338.

Reuter, Shelley Z. 2006. “The Genuine Jewish Type: Racial Ideology and Anti-Immigrationism in Early Medical Writing about Tay-Sachs Disease.” Canadian Journal of Sociology 34 (1): 291–323.

Ross, Edward Alsworth. 1924. Roads to Social Peace. The Weil Lectures on American Citizenship. Chapel Hill: University of North Carolina Press.

Salaman, Redcliffe N. 1921. Eugenics in Race and State: Second International Congress of Eugenics. Vol. 2, edited by Charles B. Davenport. Journal of Heredity 12: 219–223.

———. 1911. “Heredity and the Jew.” Journal of Genetics.

Tapper, Melbourne. 1999. In the Blood: Sickle Cell Anemia and the Politics of Race. Philadelphia: University of Pennsylvania Press.

Waxman, Chaim I. 1999. “The Sociohistorical Background and Development of America’s Jews.” In Jews in America: A Contemporary Reader, edited by Roberta Rosenberg Farber and Chaim Waxman. Hanover, NH: Brandeis University Press.

Wilson, J. G. 1911. “The Crossing of the Races.” Popular Science Monthly November, 79, 29: 486–495.

Shelley Z. Reuter