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

Preface

ix preface For more than six decades, the hereditary bleeding disorder known as hemophilia has spurred parents and doctors to engage in a concerted effort to normalize the experience of patients: to liberate them from the imminent threat of death, to alleviate their pain and debility, and to integrate them fully into society. Crucially, blood transfusions helped physicians render bleeding manageable for patients and families in the middle decades of the twentieth century. Beginning in the 1970s, many Americans with hemophilia could say that their lives were relatively normal and be viewed as normal by others. Yet, the glory of this achievement was shortlived . The therapeutic revolution that delivered this semblance of normalcy soon brought unexpected results in the 1980s: HIV/AIDS, hepatitis C, and the notorious irony of hemophilia patients’ contracting virulent diseases borne by the biomedical treatments that they believed had liberated them. In the late twentieth century, the history of hemophilia became a narrative of technological promise and unfolding peril. While the media have widely publicized stories indicating that many hemophilia patients contracted HIV (human immunodeficiency virus) during the 1980s, the public—in many ways—still does not appreciate the depth of this tragedy. In the United States, among the more than ten thousand patients whom clinicians categorized as “severe hemophiliacs,” nearly nine out of ten acquired an HIV infection from the concentrated blood products they infused to control their bleeding disorder. What should we now make of this event? How are we to understand the fact that those hemophilia patients whose conditions were most heavily and expertly managed later proved to be at the highest risk of post-transfusion infections? What does it say about “modern” medicine and society that our most advanced , technology-intensive efforts to manage disease and promote health actually facilitated the opposite—greater debility and premature death? x Preface These questions, as well as the multiple contexts that give rise to them, are the subject of The Bleeding Disease. This history focuses on efforts to manage hemophilia in the United States with the goal of identifying what has been exemplary as well as worrisome about this enterprise. Readers should also know that my decision to write this particular work of history was borne out of circumstances I confronted during the 1990s. I first became interested in hemophilia in the fall of 1993 when Dr. Kenneth Brinkhous (1908–2000) introduced me to the problem. On meeting this vibrant hematologist on my arrival at the University of North Carolina, I could hardly have predicted the path on which it would put me. Then in his eighties, Brinkhous still ran a thriving blood research laboratory at the medical school, one that he had founded nearly five decades earlier. For three formative years, I worked in the Brinkhous laboratory as an editorial and research assistant. My qualifications for the job were hardly obvious. I was not trained as a scientist, nor did I have a prior interest in medicine or health-related work. In fact, my advanced education at that point was mostly in philosophy. Yet Brinkhous seemed certain that I could play a productive role in his laboratory, and I appreciated the job and encouragement . I soon found myself helping him prepare scientific and historical talks that he was frequently invited to give, assisting him in the revisions of the laboratory’s research papers, managing his archive and library, and facilitating his intellectual projects in various ways. Ostensibly, my reason for being in Chapel Hill was to get a graduate degree in history. I took the job with Brinkhous to help pay for my schooling as a historian, but soon found myself spending more time on the medical campus than I had planned. In 1994 I dropped my history classes completely and began working full time for this experimental hematologist. He had a way of making the activities in his laboratory seem all-encompassing. Dr. Kenneth Brinkhous had a long and distinguished career as a medical scientist, and his many successes as an experimental hematologist paralleled hemophilia’s transformation from an intractable disease in the 1930s into the manageable condition it had become by the 1970s. Born and educated in Iowa, Brinkhous began research on blood clotting as a medical student at the University of Iowa. Upon receiving his M.D. in 1932, Brinkhous joined the pathology faculty at Iowa where he made the characterization and correction of bleeding disorders a focus. In 1946, after his World [18.224.37.68] Project...