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Introduction. Hemophilia as Pathology of Progress
- Johns Hopkins University Press
- Chapter
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1 Introduction Hemophilia as Pathology of Progress Knowledge about hemophilia is not uncommon. In the first half of the twentieth century, this physician’s term of art spread into popular usage as journalists reported its effects among the royal families in Great Britain, Germany, Spain, and Russia. Because of hemophilia’s colorful reputation among educated laypeople in the 1930s, students of heredity in the United States often mentioned it to familiarize the reading public with the principles of Mendelian genetics and sex-linked traits. Eugenicists likewise found it useful in their promotions of “better breeding” and “fitter families.” From the 1940s through the early 1960s, the word gained traction among advocates of hematological science, transfusion medicine, and voluntary blood donation. As the hemophilia patient’s demand for blood plasma grew in postwar America, the public learned that this hereditary disorder afflicted many ordinary families, not just aristocratic ones. By the mid-1960s, hemophilia was a focus of the emergent biotechnology industry , and it even had a role in policy and legislative debates about affordable health care in the early 1970s. More recently, Americans witnessed the tragic coupling of hemophilia and AIDS in the tainted blood scandals of the 1980s and 1990s. The word hemophilia has resonated in surprisingly diverse ways in the twentieth century, marking something deeper in modern medicine and society than just its increased visibility. Hemophilia has traveled far in the past century precisely because we, as a modernizing society, have 2 the bleeding disease transformed the disease and rendered it manageable. Just as insulin helped transform diabetes (type I) from an acute condition into a chronic, manageable disease, biomedical knowledge and technologies have played a critical role in transmuting how people experience hemophilia.1 Hemophilia ’s significance within the developed world has only multiplied as our technology-intensive efforts to manage it have enjoyed success. But it is the paradoxical terms of that success, particularly the new opportunities and dangers that medical management has entailed, that speaks to the enduring relevance of this bleeding disorder and its history. Efforts to manage hemophilia as both a medical and a social problem concern not only the long-standing and deeply felt aspiration to “normalize” the experience of patients with bleeding disorders but also a wide range of issues in modern American medicine and society that have surfaced in conjunction with the rising incidence of heart disease, cancer, stroke, diabetes, kidney failure, and other chronic conditions over the past century.2 In short, the management of hemophilia is a subject that informs and reflects our increasingly biotechnological way of life, and we ignore it at our own peril. A Chronicle of Perilous Progress Although hemophilia is among the oldest hereditary diseases on record, it emerged as a subject of sustained medical attention only in the nineteenth century. Since the 1890s, clinicians have highlighted hemophilia’s three cardinal characteristics: frequent and prolonged bleeding into joints and soft tissue, a family history of uncontrollable bleeding among male family members, and a laboratory test that indicates that the patient’s blood is slow to clot. Even though the reading public knew hemophilia as little more than a strange malady of European royalty during the 1920s and 1930s, patients, families, and their physicians knew it as a devastating hereditary disease characterized by prolonged bleeding, disabling pain, and social isolation. Whether it was regarded as a curiosity or merited the seriousness befitting its tragic impact on certain families, hemophilia’s visibility remained limited before the age of transfusions and mass blood donation. The widespread use of transfusions for hemophilia that required very public calls for fresh blood and plasma substantially raised the cultural visibility of hemophilia in the United States in the two decades after World [3.94.102.228] Project MUSE (2024-03-28 10:04 GMT) Introduction 3 War II. In fact, the need for blood donors prompted families and physicians to work together to provide hemophilic children with the attention and resources they required to treat their condition effectively. Hemophilia communities emerged in the 1950s as the National Hemophilia Foundation (established in 1948) and other family-based advocacy groups aided patients with bleeding disorders.3 Expectations about hemophilia also changed for the better as physicians and scientists developed increasingly powerful hematological tools for diagnosing and treating bleeding disorders . Postwar physicians were envisioning a life of medical management for their pediatric hemophilia patients and the prospects of healthier lives. Patients along with their families embraced what modern medicine both...