The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress

1 The Emergence of the Hemophilia Concept

18 chapter one The Emergence of the Hemophilia Concept Hemophilia’s unusual visibility in the twentieth century reflected its cultural status as a disease of heredity and blood. As a bodily marker of both identity and kinship, hemophilia has provided experts and laypeople with opportunities to express their thoughts and anxieties about matters of class, race, ethnicity, and gender. Thus, in covering the Second International Congress of Eugenics in 1921, the New York Times quoted biologist Charles Davenport as saying of hemophilia and other traits: “Already there is being developed a well-defined conscience in the matters of cousin marriages and matings into families with grossly defective members .” By linking hemophilia to eugenic prohibitions against “cousin marriages ,” Davenport was playing off American anxieties about class and racial difference at a moment when eugenicists also sought to identify what types of hereditary traits should mark immigrants as unfit for participation in American society. As told by the reporter, Davenport and other leading eugenicists sought strict immigration laws to ensure “the healthy racial progress of the American people.”1 Markers of social identity—class, ethnicity, and race—should therefore matter to anyone interested in understanding the history of hemophilia and its management. And because scientists have long regarded hemophilia as a sex-linked hereditary trait, gender plays a particularly important role in this story. Throughout the history of hemophilia, experts have been no less inclined than laypeople to hold the characteristic class, gender, and racial The Emergence of the Hemophilia Concept 19 views of their era. Even in the clinic and laboratory, hemophilia has been signified in ways that reinforced and even performed widely held, if not hegemonic, ideas about class, gender, or racial identity. It is impossible, then, to appreciate the range of social and cultural experiences that have circulated around hemophilia without first understanding the history of the disease concept itself. This chapter details how the medical community came to share an understanding of the disease concept known as hemophilia and, in doing so, introduces the biological and social aspects of a condition that was a sustained subject of fascination and concern for both experts and laypeople in the past century. Although physicians first articulated our current portrait of hemophilia’s clinical and hereditary aspects in the mid-nineteenth century , it would take them another century to begin managing hemophilia in ways that effectively addressed the primary bleeding problems associated with it. Hemophilia’s transformation into a manageable disease in the second half of the twentieth century should be seen not only against the backdrop of sustained medical and scientific interest in hemophilia between 1800 and 1940 but also in terms of the range of social and cultural matters that knowledge of the disease raised for experts and laymen alike. By design, our modern hemophilia concept has provided a way of marking progress in efforts to understand and treat disease; it has circulated as a signifier for a variety of social and cultural phenomena as knowledge of that disease has evolved. Some of these changes in our knowledge of hemophilia (e.g., the recognition of sex-linked inheritance and the growing realization of the functional mechanisms of blood clotting) have had a profound and lasting impact on efforts to manage the disease. Subtler, however, are the ways that particular circumstances have shaped interpretations of hemophilia beyond the treatment of individual patients or families. For instance, what difference did it make that the first medical reports of hemophilia came from the fledgling nation of the United States in the early 1800s? Or that German clinicians operating in urban hospitals standardized our formal conception of the disease in the mid-nineteenth century? What role did hemophilia’s early status as a hereditary disease play in the formation of the science of heredity and the notorious eugenics movements of the twentieth century? And what practical effects, if any, did hemophilia ’s place within the new medical and life sciences of the twentieth century have on efforts to manage the disease? 20 the bleeding disease As with all diseases, physicians have used the hemophilia concept to describe a distinct clinical reality, one that presumably captures the illness experience of the bleeding patient at the same time that it helps everyone comprehend what natural course of events is occurring in the patient’s body and/or mind. This means that at any given moment, the concept of...