Lessons from Hemophilia

Recently I had the pleasure of spending some time with Corey Dubin, thinker, activist, and president of the Committee of Ten Thousand. Corey is a really interesting person (this article gives a decent overview of his past activities), child of the 60s, has amazing Zardoz hair (click at your own risk), and finally, he's a member of the "Triple H club": hemophiliac, HIV+, and hepatitis+, and has been for many many years. What happened to Corey Dubin was not an accident of fate, genetics, or public policy. Rather, it was the direct consequence of decisions made about the American blood supply, and his experience has important lessons to teach us about what counts as an acceptable risk in a highly connected world.

First a little context. Not all that long ago, hemophilia was an invariably fatal disease. Internal bleeding caused extremely painful swelling, blood corroded the bones and damaged the organs, and it was rare for somebody with the condition to live beyond their teens. The most famous historical hemophiliac was Prince Alexei Nikolaevich Romanov, who's condition played a minor, but significant role in the Russian Revolution, as it allowed Rasputin to rise in the court and alienated the Tsar from his most natural supporters in the aristocracy.

The 1960s saw the first effective treatments for hemophilia, with the discovery of Cryoprecipitate and then the concentrated blood-clotting proteins Factor VIII and IX. With these treatments, hemophiliacs were able to lead normal lives. Science and medicine had triumphed in reduced hemophilia from a fatal disease to a chronic condition. Of course, this lead to a whole new industry in supplying blood products. Plasma was collected from paid donors, mixed into very large batches containing blood for over 30,000 donors, processed into Factor, and then sold to doctors and patients.

This system worked fine until the early 80s, when a virulent new disease emerged on the marginal fringes of society. Homosexuals, IV drug users, and hemophiliacs were dying of strange lesions and secondary infections. The Center for Disease Control soon realized that it was a blood-born disease, but lacked the political clout to make the Food and Drug Administration and pharmaceutical companies act. The FDA vacillated, refusing to take Factor off the market for several years, and knowingly allowed contaminated blood to be shipped overseas. The end result was that an entire generation of hemophiliacs were infected with a fatal disease.

The point here is not that regulators made terrible, and in some cases unethical decisions in the midst of the AIDS crisis-although they did (and if you find this interesting, I highly recommend the documentary Bad Blood). The point is that the blood system was set up to fail.

The blood supply was contaminated from the beginning with hepatitis. Everybody involved knew so, but they believed that hepatitis was a fair trade for a cure for hemophilia. Perhaps they were right, but through a combination of greed, arrogance, and laziness authorities ignored techniques that could have purified the blood supply; things as simple as running plasma through columns of detergent. Similarly, mixing donor samples into large batches increased profits, but also increased the transmission rate by orders of magnitude. A single bad donor could infect thousands of people.

We have to be very careful about what counts as an "acceptable risk." New technologies present novel risks, and do not have adequate safety mechanisms. Risk is part of the process of innovation, but technologies that do not become safer over time deserve a critical revaluation. The other lesson is that we are all connected. Hemophiliacs are intimately connected to thousands of strangers through the blood supply, but to a lesser extent, we all have the same problems of trust and reliability. The food supply is highly commoditized, which means that food poisoning affects the entire nation, and account for an estimated 48 million illnesses, 128,000 hospitalizations, and 3000 deaths. As we become more dependent on internet-enabled and 'cloud' services, we become more vulnerable to hackers. The stability of countries on the other side of the world can shake the US economy, as proven by repeated oil price shocks. And pollution does not respect national boundaries; we all breathe the same air and drink the same water.

There is no cure for risk. Regulation is an inherently difficult task: the barrier of specialized expertise and the lure of industry money can eventually lull even the most dedicate watchdog agency into passivity. Independent citizens' groups and hard-hitting journalism are the only long-term antidotes to regulatory capture, and they require continual social investment and support. When industry or the experts say that "this is too complex" or that "this will be too expensive", we should demand clearer explanations and sensible alternatives. To do otherwise is to invite disaster. Maybe not today, maybe not tomorrow, but eventually.

Even if the blood supply had been safe in the 1980s, some hemophiliacs would have been exposed to AIDS and some would have died, but the scale of the human tragedy would have been far lower. To this day, the Center for Disease Control uses hemophiliacs as the 'canary in the coal mine' for signs of contamination in the national blood supply. But the story of hemophiliacs and the blood supply also serves as a lesson about techno-social systems and 'normal accidents', and how they can be prevented. Good system design and careful monitoring saves lives.