The Bleeding Disease: Hemophilia and the Unintended Consequences of Medical Progress
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By the 1970s, a therapeutic revolution, decades in the making, had transformed hemophilia from an obscure hereditary malady into a manageable bleeding disorder. Yet the glory of this achievement was short lived. The same treatments that delivered some normalcy to the lives of persons with hemophilia brought unexpectedly fatal results in the 1980s when people with the disease contracted HIV-AIDS and Hepatitis C in staggering numbers. The Bleeding Disease recounts the promising and perilous history of American medical and social efforts to manage hemophilia in the twentieth century.
This is both a success story and a cautionary tale, one built on the emergence in the 1950s and 1960s of an advocacy movement that sought normalcy―rather than social isolation and hyper-protectiveness―for the boys and men who suffered from the severest form of the disease.
Stephen Pemberton evokes the allure of normalcy as well as the human costs of medical and technological progress in efforts to manage hemophilia. He explains how physicians, advocacy groups, the blood industry, and the government joined patients and families in their unrelenting pursuit of normalcy―and the devastating, unintended consequences that pursuit entailed. Ironically, transforming the hope of a normal life into a purchasable commodity for people with bleeding disorders made it all too easy to ignore the potential dangers of delivering greater health and autonomy to hemophilic boys and men.
for AIDS. Seizing the moment in front of the cameras, other representatives from the plasma industry also endorsed Alpha’s plan and indicated their willingness to follow suit.40 Evatt and others at the CDC welcomed Alpha’s readiness to serve its customer base (the hemophilia treatment centers and their patients) by implementing measures to reduce the spread of AIDS through transfusions of plasma products. As Donald Francis later recalled, “Some [of the commercial plasma people] were certainly
quite wary of doctors who wished to draw their blood: “So assured are the members of this family of the terrible consequences of the least wound, that they will not suffer themselves to be bled on any consideration, having lost a relation by not being able to stop the discharge occasioned by this operation.”14 Moreover, the family had consulted several physicians of “acknowledged merit” over the years, and these physicians had employed “various remedies … to restrain the hemorrhages,” including
Haemostasis 1 (2003): 206–21. There was some initial skepticism about Owren’s findings, and Armand Quick disputed them aggressively. See Armand J. Quick, “Components of Prothrombin Complex,” American Journal of Physiology 151 (November 1947): 63–70; Ratnoff, “Why Do People Bleed?” pp. 610–14. 39. Pavlovsky believed the results pointed to the existence of an anticoagulant factor in the plasma that inhibited the capacity of the patient’s blood to clot. See Alfredo Pavlovsky, “Contribution to the
“National Hemophilia Foundation Correspondence, 1954,” Kenneth M. Brinkhous Papers, Francis Owen Blood Research Laboratory, University of North Carolina at Chapel Hill. 47. See the editorial, “Blood Coagulation and the Clinical Pathologist,” Journal of Clinical Pathology 6 (February 1953): 1–2. 48. See, e.g., the recommendations in hematologist Benjamin Alexander’s “Medical Progress: Coagulation, Hemorrhage and Thrombosis,” New England Journal of Medicine 252 (1955): 432–42, 484–94, 526–35.
York Times, January 25, 1955, p. A27; “Record,” Time 65 (February 7, 1955): 48. 72. Hemophilia continues to be extraordinarily costly. In 2001, for example, Duke Hospital spent five million dollars to treat one patient with acquired hemophilia. More the 95% of the cost was for blood products. See Ron Winslow, “Intensive Care: One Patient, 34 Days in the Hospital, a Bill For $5.2 Million, He Had Internal Bleeding; Doctors at Duke Spared No Effort to Control It,” Wall Street Journal, August 2,