Book Review

Blood Saga: Hemophilia, AIDS, and the survival of a community

N Engl J Med 2000; 342:666-667March 2, 2000

Article

Blood Saga: Hemophilia, AIDS, and the survival of a community
By Susan Resnik. 292 pp. Berkeley, University of California Press, 1999. $29.95. ISBN: 0-520-21195-2

In 1984, the Centers for Disease Control (CDC) announced that, between the years 1979 and 1984, 90 percent of patients with hemophilia who were treated with clotting-factor concentrates had been infected with the human immunodeficiency virus (HIV). A former director of education at the National Hemophilia Foundation, Susan Resnik has written a fascinating page turner about one of medicine's darkest periods. She describes the role well-meaning physicians and organizations trapped in fear and denial had in worsening the situation. In the final chapters of her book, Resnik shows how the hemophilia community survived — renewed in spirit, strong and more united than ever.

The comprehensive approach to the treatment of hemophilia was initiated by Shelby Dietrich, a pediatrician in Los Angeles, in 1957. The limiting factor of the program was the lack of a potent, concentrated source of clotting factor (factor VIII) that could be used at home at the first sign of bleeding. This limitation was overcome by the discovery of cryoprecipitate by Judith Graham Pool in 1965. By 1968, Hyland Laboratories had received a license to market pooled factor VIII that had been refined and concentrated from cryoprecipitate. In 1973, Peter Levine and colleagues showed that the treatment of patients with hemophilia at home resulted in a net savings in health care costs by reducing the numbers of emergency room visits, hospitalizations, and orthopedic surgeries. Resnik details the critical role of “Dr. Marcus Victor,” her code name for an ambitious and talented platelet physiologist, in advancing the care of patients with hemophilia. After a two-year struggle, “Victor” and other physicians, with the solid backing of the National Hemophilia Foundation, were successful in getting Congress to authorize funding for 26 comprehensive hemophilia-treatment centers.

The establishment of hemophilia-treatment centers around the country ushered in a new, “golden” era for patients with hemophilia and their families. Patients even began engaging in athletics, and those with the mild form of the disease were gradually allowed to become involved in strenuous sports, even contact sports, as confidence increased in the miraculous power of factor VIII concentrates to prevent bleeding. However, in more than 90 percent of those treated with the concentrates, hepatitis developed. But the phenomenal improvement in lifestyle far outweighed concern about any future problems. A few “dinosaurs,” such as Oscar Ratnoff, decided there was cause for concern. He restricted patients to treatment with the much safer but less convenient cryoprecipitate. A method of pasteurization that inactivated most viruses was discovered, but it increased the cost of the concentrates, and its routine use was not recommended.

In 1981, the first cases of AIDS were reported, all in gay men. By the summer of 1982 three patients with hemophilia had contracted AIDS, and the National Hemophilia Foundation issued an advisory to its members in which it passed on the information it had received from the CDC, adding that “at this time the risk of contracting this immunosuppressive agent is minimal.” Leaders in the blood-banking community refused to view the problem as a potential disaster. One investigator from the CDC exploded: “How many people have to die? . . . Give us the threshold of deaths that you need in order to believe that this is happening.” By the following year, there were so many cases that no one any longer questioned the extreme danger to the hemophilia community. By January 1988, the National Hemophilia Foundation announced that all products available for the treatment of hemophilia were free of contamination with HIV. But for most patients with hemophilia it was too late: HIV infection had already occurred.

The final chapters of Resnik's book chronicle the rising rage and anger of patients with hemophilia who were infected with AIDS, not only because of what happened to them, but also, in many instances, because of what happened to their wives and children. Lengthy, protracted lawsuits against the manufacturers of the concentrates were eventually successful in getting some compensation for the affected patients and their survivors. Surprisingly, physicians and national advisory groups were not the defendants in such suits, nor were they prosecuted outright, as occurred in France. The improvements in treatments for AIDS and the advent of gene therapy in the late 1990s renewed the hope and optimism of the hemophilia community.

Resnik's book forcibly brings home the wisdom of the ancient dictum “first, do no harm.” She is careful in her criticism of physicians and organizations but does not fail to point out appropriately the important role that denial, paternalism, and hubris had in the disaster that befell the hemophilia community. I strongly recommend this book to those with hemophilia and their families, as well as to physicians, nurses, and others involved in the care of patients with hemophilia. I would also highly recommend it to medical students. Young women and men embarking on a career in the brave new world of gene therapy will find much in this book to contemplate.

James P. Crowley, M.D.
Memorial Hospital of Rhode Island, Pawtucket, RI 02860