Can Tay-Sachs Disease speak for itself? This question has followed me since I finished Shelley Z. Reuter's Testing Fate: Tay-Sachs Disease and the Right to be Responsible. The book is a critical sociological examination of Tay-Sachs Disease, an autosomal recessive neurological disorder that results in the unregulated accumulation of gangliosides or fats in the brain and nervous system. The symptoms of the disease include "change in muscle tone and eventual paralysis, an increase in water around the brain leading to enlargement of the head, hyperacusis (a heightened startle response to everyday sounds), mental delay, seizures, and social withdrawal" (2). It is "always fatal, normally by the age of three or four, and it is a death that is devastatingly protracted" (2). There is no cure for Tay-Sachs, but since 1970, genetic tests have allowed the screening of carriers of the genetic mutation and the prenatal diagnosis of fetuses.

Reuter treats Tay-Sachs as a "disease concept," that is, a disease with "social and historical implications; it is simultaneously a product and productive of history and social relationships—relationships of inclusion, exclusion, and power" (5). The book examines the historical and social life of Tay-Sachs, primarily in the United States and the United Kingdom. How Tay-Sachs has been understood, mobilized, and responded to forms one facet of "biocitizenship, " or the organization of belonging, rights, and responsibilities around biological identity. For Reuter, responsibilities and rights become conjoined in Tay-Sachs' biocitizenship, where the "right to be responsible" mobilizes behaviors like genetic screening and engaging in medicalized thinking, not merely as a duty but also as a privilege of belonging to one's race and/or nation.

The book is divided into three sections: the first examines the early days of Tay-Sachs (1880-1920) and its ties to racializing Jewish immigrant communities in the United States and England; the second examines the visual representation of Tay Sachs in medical journal articles from 1881-1950s and in contemporary YouTube videos; and the third examines the development and deployment of genetic screening in Jewish communities and wrongful birth and wrongful life suits from 1974-present day. The result is a detailed constellation of thinking about the implications of disease in terms of race, citizenship, and belonging that paints a bleak picture of agency and the possibility of an inclusive ethics of encountering the disease.

The book is rich in fascinating insights into the social life of Tay-Sachs disease. The first two chapters, (Chapter 1: Diagnosing the Genuine "Jewish Type" and Chapter 2: Governing Disease) detail the early meanings that accrued around Tay-Sachs, Jewish racialization, and Eastern European Jewish immigration to England and the United States. Amidst the forces of "assimilation and self-care" (32) and "medical racialism" (32), Jewish immigrants in the UK and the US were both subjected to disease profiling that defined them as "other" to the nations to which they emigrated. Recent immigrant Jews had to navigate settled Jewish charity organizations that often braced at the apparent lack of assimilation that threatened their strategies of racially belonging. At the same time, external national forces during the 1920s, such as demographic accounting and public health campaigns, were ramping up the surveillance of national populations. With the census and increased tracking of infant mortality, in combination with racialist theories of eugenics, the meaning of Tay-Sachs, good parenting, and racial Jewishness became closely entwined. These chapters make up the most empirically compelling section of the book, adding important historical specificity to Tay-Sachs while connecting it to the larger historical movements of disease, disability, race, and nationalism.

The second two chapters (Chapter 3: "Plainer than Words can Describe" and Chapter 4: "The Unethics of Looking at Disease-Disability") place a close focus on the work of visuality in constructing knowledge and affect around disease. Reuter argues that Tay-Sachs is accompanied by a specular logic, or the "social process of reductive visual objectification" (92), where images flatten and oversimplify the complex meaning of disease and patient's lives. Chapter 3 focuses on the use of images in medical texts from 1881-1950s to explore the ways in which medical knowledge, in its visual dimension, "directly contributed to the Jews' racialization, to the construction of this disease as Jewish, and to the wider debate around immigration," (93) while medicine remained silent on "the devastation the disease wrought for the Jewish community and especially the families" (113). There is a tension here not explored by the text, where medicine's empirical reach stopped at any actual construction of Jewishness as a distinct lived experience, even while it reinforced a racial difference that made Tay-Sachs a "Jewish disease." Reuter does point out that, "in escaping bothersome cultural details, individual cases were converted into typological abstractions" (115), which suggests an important work of racialization in making a group hyper-visible in their separateness while rendering much of the lived reality invisible. However, it is unclear whether Reuter thinks medicine ought to attempt to explain, diagnose, and describe the socio-cultural life of disease and extend its expertise to creating a normative sense of the proper familial and community relations surrounding the "devastation of disease." The concern about medicine's absence to construct familial and community relations around disease is an issue that deserves more attention, including what exactly is meant by thinking about the disease as devastating the family and community.

Chapter 3, while importantly turning the reader's attention to the use of portraiture in early medical texts on Tay-Sachs, does not actually include any portraiture, a decision the author explains in terms of the "unethics" of specularism, which crystallizes in a concern for the lack of informed consent for those whose images are reproduced. The author's reinforcement of the ethics of informed consent stands in paradoxical contrast with the rest of the book, which serves as a critique of medical thinking and liberal agency. Informed consent is based on the notion that individuals, when given all relevant information, can make a fully rational decision based on their own best interests. The questions of why we ought to affirm informed consent as the ethics of visuality or why informed consent would transform problematic uses of imagery is never discussed by the author and is connected to a larger issue of the theoretical perspective of the text, discussed below. The chapter does make a compelling argument that while visuality is supposed to produce "the objective, the real, and the truth" (94), in actually, it works to transform what subjective and partial realities inhabit these universal categories. In the context of Tay-Sachs, racialist thinking and medical objectivity appear to be neutral and natural rather than ideological.

Chapter 4 retains a focus on the visual but shifts domains to the use of YouTube as a medium used by non-profit medical research organizations and individual families to produce social meanings of Tay-Sachs today. Reuter demonstrates that while Tay-Sachs is no longer primarily mobilized in the service of medical racialization, visual storytelling now primarily circulates around "disease-disability," (23) a preventable and undesirable state "to be avoided at all costs" (118). But despite the shift in narrative, as Reuter points out, "what has remained constant throughout the history of this disease category is its deployment as a way to delineate, exclude, and regulate the other" (118). Reuter cites nineteen videos and argues that the videos share a "reorientation of exclusion" (119) based on the "unethics of looking" (119). Reuter uses the phrase, unethics of looking, to "refer to a mode of viewing that takes for granted that she or he who appears has less or no capacity to think or act (especially when the diseased or disabled are concerned), that she or he therefore need not be consulted or engaged as to his or her preferences and wishes but is, rather, always already present, available, and willing to be the object of someone's diminishing gaze" (122). Reuter draws on several well-known works that deal with how gazing and staring is contextualized by disability, such as Rosemarie Garland-Thomson's work on freak shows and Christopher Smit and Paul Longmore on telethons, to argue that an audience's desire to consume disability as strange, other, and worthy of pity and prevention does not allow for an understanding of disability as part of an inclusionary social body.

In constructing the meaning of Tay-Sachs videos, Reuter, in a rare first-person move, acknowledges a tension in her analysis of the YouTube videos and then quickly dismisses its import: "I can offer no alternative to using these images and I recognize that they serve certain valuable purposes—personal, collective, and scientific. However, to acknowledge the value of these videos is not to say that they do not raise ethical issues all the same, and the fact that there may not be an alternative that is equally as effective does not preclude our reflecting critically on their effect" (119). What I find interesting about this reflection is twofold. First, while in examining medical portraiture, the personal, collective, and scientific value of the images are of central concern, in part because the author can be critical of the negative value of each. The bracketing of the personal, collective, and scientific value here, however, precludes the reader from reflecting on a more complex picture of the meaning produced by these images that may include normatively productive value. Second, this chapter begins to crystalize an argument that comes into full fruition in the final substantive chapter (Chapter 5: The Right to Be Responsible) that the socio-cultural life of Tay-Sachs has wholly eradicated the agency of actors responding to the disease. These two points are related: by not engaging with the complex and contradictory meanings produced by the YouTube videos and attempting to isolate ethics from wider socio-cultural complexity, the book produces a black-and-white picture of agency that over-determines what meaning and action children with Tay-Sachs and their caregivers create in the context of the disease. What would happen if, instead of arguing that the caregivers (often parents) and children with Tay-Sachs have no agency, Reuter attempted to look for, following Charles Lee, a kind of constrained but ingenious and "contaminated" agency (2015). There may not be an ethically pure way to represent infants and young children with Tay Sachs, but to argue that the representations on YouTube deny the possibility of any ethical relationship with children with Tay Sachs denies the complicated chains of empathy that one can find both in the videos themselves and in the comments. For instance, in the YouTube video entitled, "Parenting Children with Life-Limiting Illness," one set of parents say that their aim was that their daughter with Tay-Sachs could "live the full arc of her life"; in another, called "Nathan's Story", the parents say, "we want Nathan to have a legacy." While these statements are often embedded in problematic disease-disability representations, many of the videos are also an attempt to respect, represent, and extend the agency of children with Tay-Sachs. The contradictions and contaminations here provide a lesson for the possibility of affective connections to be both enabling and constraining and resists a pure ethics, such as informed consent, as the standard for the ethics of visuality.

The problem of absolutist notions of agency carries over to the final substantive chapter, Chapter 5: The Right to Be Responsible, which is the theoretical heart of the book. The chapter's empirical focus is split between the development of genetic screening and the mobilization of Jewish communities in the United States and Britain to successfully manage their own genetic identities and court decisions in wrongful death and wrongful life suits brought against physicians in the cases of infants born with Tay-Sachs. The examination of the development and deployment of genetic screening is thoughtful and detailed, and as with the first two historical chapters, contributes to a nuanced understanding of the intersections between disease, medicine, and community belonging. But the question Reuter is centrally concerned with is normative and centers on the meaning and practices of responsibility. Reuter asks the question, "can the responsibilized and geneticized biocitizen exercise agency that is unentangled by sociocultural constraints or imperatives" (140-141)? She quickly answers the question in the negative, concluding that genetic testing and the litigation it inspires is "a misrepresentation of choice, as choice is always already constrained by the biopolitics of risk…choice in this context is in fact the unfreedom to choose—unfree in the sense that an individual's choices are constrained or mediated by circumstances and structures, most notably the moral obligation to others to be responsible" (150). While Reuter does good, careful work examining what these structures are and how they call for particular forms of responses, both the notion of agency developed here and the depiction of individual actors are less helpful for developing a comprehensive sociological picture of the social life of Tay-Sachs disease. Unlike Rayna Rapp (1999), who recognizes that pregnant persons and their partners work as "moral pioneers" in response to genetic testing, who must forge actions and meanings out of novel choice situations, Reuter leaves no space for individual negotiation with the terms of the given. Relatedly, if unfreedom exists whenever choices are "constrained or mediated by circumstances and structures," then Reuter's concept of agency rests on a fictional notion of the unencumbered individual totally free from relations with others or the world. The work would have benefitted by a more generous interpretation of how and why people negotiate not just the decision to genetically screen, but also why they might avail themselves of litigation if their child was born with Tay-Sachs. One can speculate that perhaps some parents do not actually consider their children to be wrongfully born or wrongfully alive, but rather need financial support to pay for medical care to sustain the lives of their children and must use the blunt tools of the law strategically for survival.

This leads me to return to my first question, Can Tay-Sachs speak for itself? For all its achievements, the book's critical sociology leaves the status of Tay Sachs itself ambiguous. In part, the problem is one unique to Tay-Sachs disease; when we are speaking of a disease that affects infants and results in the death of very young children, the attempt to narrate Tay-Sachs from individual disease experience is difficult if not impossible. The imaginative world of the disease, from the tangible experiences of caregivers and intimates to the narrative world of physician reports and YouTube videos and to the interpretive world of eugenicists and sociologists, is, in many ways, the only access to the disease experience that can be explored with traditional sociological methods. But the failure to apprehend Tay-Sachs directly also derives from the fact that the book resists a discussion of the normative questions associated with the responsibility that surrounds Tay-Sachs. The refusal of the normative world of Tay-Sachs is a privilege reserved for the scholar who does not have to navigate the necessity of decision making occasioned by a world in which Tay-Sachs is partially understood and genetic screening is a reality for pregnant people. Such a privilege does allow the sociologist to treat the world of Tay-Sachs as a laboratory, where the observer can be both detached and critical to provide new insights into a lived reality that may not be apparent for those living that life. But that privilege might also hide important dimensions that may undermine the theoretical conclusions about responsibility, agency, and belonging in relation to disease-disability.

In the end, Testing Fate: Tay-Sachs Disease and the Right to be Responsible is an engaging and carefully documented piece of scholarship. For anyone interested in the complicated history and social life of a disease that still stands in for many as the worst genetic diagnosis for a child, Reuter's work is a wonderful starting point. The theoretical work tends towards some absolutist conclusions that do not match the complexity of the empirical world presented in the text. A more empathetic theoretical treatment would have allowed the author to not only document the problems with racialized medicine, responsibilized biomedicine, and exclusionary terms of belonging, but also to try and explore how constrained agency operates in relation to Tay-Sachs disease. In the end, Reuter has provided a useful resource for anyone interested in understanding how genetic diseases came to be understood and mobilized in political context.

Works Cited

  • Lee, Charles T. 2016. Ingenious Citizenship: Recrafting Democracy for Social Change. Durham: Duke University Press. https://doi.org/10.1215/9780822374831
  • Rapp, Rayna R. 1999. Testing Women, Testing the Fetus: The Social Impact of Amniocentesis in America. New York: Routledge.
Return to Top of Page


Copyright (c) 2017 Claire McKinney

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Beginning with Volume 36, Issue No. 4 (2016), Disability Studies Quarterly is published under a Creative Commons Attribution-NonCommercial-NoDerivatives license unless otherwise indicated. 

If you encounter problems with the site or have comments to offer, including any access difficulty due to incompatibility with adaptive technology, please contact the web manager, Maureen Walsh. Disability Studies Quarterly is published by The Ohio State University Libraries in partnership with the Society for Disability Studies.

ISSN: 2159-8371