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  • The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress by Stephen Pemberton
  • Richard Nollan, Ph.D., M.L.S.
Keywords

hemophilia, hematology

Stephen Pemberton . The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress. Baltimore, The Johns Hopkins University Press, 2011. xviii, 377 pp., illus., $50.

The history of hemophilia reads like a classic tragedy. For over a century and a half, doctors and patients seemed to make incremental advances and then substantial gains toward the goal of ameliorating, if not curing the disease. Just when success seemed assured, the most promising therapeutic means for managing hemophilia provided an unintended pathway for the AIDS epidemic, which all but destroyed [End Page 325] everyone's beliefs in the possibility of a normal life. Stephen Pemberton tells this compelling history very well.

The narration begins with the bleeding disorder's emergence in the nineteenth century, its link to European aristocracy as an inherited disorder, and the establishment of diagnostic criteria. During the first 140 years, the disease concept evolved socially and medically to include a prolonged bleeding in the joints and soft tissues together with a family history in male sufferers. The male bias would persist into the 1950s when medicine began recognizing the disorder in female patients. In the late nineteenth century, the definition of the disease was established by the addition of a laboratory test that measured the slower clotting time in bleeders, thus establishing it as a hematological disorder. The laboratory would continue to play a key role in clarifying the pathology and treatment of the disease. Blood transfusion in the late 1930s represented the first promising therapy. The National Hemophilia Foundation was established in 1948 with the goal of helping sufferers and their families by disseminating information about the disease to its members and to society in general. The discovery of the first key defect (antihemophilic factor or factor VIII) by Kenneth Brinkhous and others at the University of Iowa was a key breakthrough that offered hemophiliacs some hope of finding an effective therapy and achieving a normal life. Normality, the author argues, was considered a key concept for marking progress in managing the disease and in rendering hemophiliacs as credibly healthy and autonomous. The advent of blood storage and transfusion technology allowed the first major advance in the treatment of hemophilia, but also offered a ready and plentiful blood supply for analysis in the laboratory and by pharmaceutical companies. In the post-World War II years, patients and families began advocating for themselves. Medical resources, including emerging pharmaceutical products such as cryoprecipitate and clotting factor concentrates, began to organize around bleeding disorders. Gradually, hemophiliacs were perceived medically and socially as able to overcome the stigma of the disease and, thus, entered into a period where it could be said that, with treatment, they could live normal, active lives. Together with the National Hemophilia Foundation, the hemophilia community was able to convince the federal government to set up funded centers around the nation that supported patients.

Pemberton's account of the disaster beginning in the 1980s is revealing and instructive. While the new technologies offered hope, they also contained the seeds of tragedy. There were inadequate standards for how to handle blood and blood products so that, as a consequence, blood-borne diseases like hepatitis B, hepatitis C, and AIDS were transmitted as an unintended consequence of the healthcare process to hemophiliacs [End Page 326] receiving blood products. The indecisiveness of health professionals and health organizations and inaction due to the vested interests of pharmaceutical companies caused a failure to recognize the signs and to arrive at a plan that would effectively protect hemophiliacs from the coming pandemic. By 1994, the skepticism about AIDS and the pressure to "wait and see" resulted in the deaths of over one-quarter of the hemophilia population. The pressure to "modernize" is assigned some of the blame for this, and more on what the author meant by this would have been welcomed. As a consequence of the AIDS epidemic, hemophiliacs shifted their focus away from achieving normality to seeking social justice.

The Bleeding Disease charts the trajectory of a single disease entity. In doing so, Pemberton gives...

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