HEMOPHILIA AND HIV: A DOUBLE CHALLENGE

Before the current policies for screening HIV in blood supplies were instituted, many people were exposed to the virus through intravenous transfusions of blood or blood products. People with hemophilia routinely need certain blood-clotting components, and so for several years, primarily 1978 through 1985, many were inadvertently put at extremely high risk for acquiring HIV through the public blood supply. Hemophiliacs now represent one percent of all people with AIDS in the U. S., but that equals four percent of people with hemophilia. Another 50 percent, about 10,000 people, are asymptomatic but HIV seropositive.

Hemophilia is a blood disorder involving deficiencies in one of two types of the body's coagulation proteins, factor VIII or factor IX. Deficiencies in factor VIII are more common and more serious. While both can be inherited, at least one third of all instances are new genetic mutations. Women who carry the hemophilia genes do not manifest the deficiency, but transmit hemophilia to approximately half of their male children and carrier genes to half of their female children. Men with hemophilia cannot transmit the deficiency to sons, but do transmit carrier status to all their daughters.

No matter which individuals in an affected family may carry genes for hemophilia, all members will eventually cope in varying degrees with the disorder. And the enormous prevalence of HIV infection in a community already coping with chronic health concerns has had severe medical and emotional consequences.

Physicians Who Have Learned To Balance Both

Physicians who follow hemophiliac patients have historically nurtured a close relationship with them. Over a period of years the patient and doctor may meet many times, from controlling a hemorrhage crisis in the emergency room to working out a pain-relief program which avoids anti-coagulants. Obviously, these physicians were destined to become familiar with HIV diagnoses and therapies.

We spoke to Brad Lewis, M. D., who cares for hemophiliac and HIV+ patients at Alta Bates Hospital in Berkeley. He described to us ways in which health concerns related to hemophilia can be affected or unaffected by HIV infection. In at least one way, people with hemophilia were better prepared than most to cope with a health crisis -- frequent hospitalizations and intermittent illness were familiar experiences. But for the same reasons, HIV at least doubles the emotional and economic stress on their lives. In terms of the physiological interaction of hemophilia and HIV, Dr. Lewis shared these observations:

* For reasons not fully understood, people with hemophilia and HIV are rarely troubled by Kaposi's sarcoma or active CMV infections.

* The hemorrhaging allowed by the clotting deficiency frequently causes serious joint pain. AZT appears to exacerbate this pain.

* HIV infection is often associated with decreased numbers of platelets, another of the body's clotting agents. This situation, called idiopathic thrombocytopenic purpura (ITP), is distinct from the mechanisms which cause hemophilia, but each can make bleeding caused by the other harder to manage.

* Hemophiliacs experience higher rates of liver-related problems, such as chronic hepatitis, resulting from repeated infusions of pooled blood products. Some of the drugs currently used in the treatment of HIV and AIDS are also taxing on liver functions, increasing the danger of toxicity for anyone already coping with liver dysfunction. However, Dr. Lewis notes that while liver enzymes are usually elevated in people with hemophilia, they are also usually stable and not necessarily indicative of progressive liver disease. He noted that monitoring a patient's levels of immune globulin may help predict if their liver abnormalities are stable or deteriorating.

(Chronic hepatitis is a serious problem for many hemophiliacs, and often for HIV+ gay men and I. V. drug users as well. Interferon has been studied as a treatment for hepatitis, and for HIV as well. Another substance called glycyrrhizin, currently used in Japan to treat liver disease, is being studied as a possible complementary therapy for a number of conditions involving hepatic dysfunction. AIDS TREATMENT NEWS is now collecting information on glycyrrhizin, which we discussed over three years ago in issue #17, November 7, 1986; the treatment is attracting attention because of new research in Japan.)

To improve treatment development for the HIV+ hemophiliac population, Dr. Lewis suggested a few changes in the manner that clinical trials are designed. For one, entry criteria, particularly liver function parameters, could be relaxed for candidates with hemophilia. Additionally, the locations of HIV clinical trials should be expanded to established hemophilia treatment centers, in order to reach potential participants where they already receive healthcare, rather than expecting them to travel hundreds of miles to the nearest ACTG (AIDS Clinical Trail Group) site.

Dr. Lewis added that HIV and hemophilia each require specialized care, meaning that patients coping with both need access to medical care which is also familiar with both.

Interview: Skip Harris

AIDS TREATMENT NEWS interviewed Skip Harris, a Bay Area activist in the HIV community, and president of the local chapter of the Hemophilia Society from 1983 to 1985. He is familiar with each community's concerns, and with the general crisis in U. S. health care.

DS: How do you identify yourself in two different communities, Skip?

SH: I'm very public about my condition. When I was young, starting about eight years old, we ran blood drives every other year. I was always helping with the publicity for that -- it was so nice asking people for help with something that didn't involve money!

DS: How is the hemophilia community dealing internally with HIV -- is there a single consensus or strategy, or many different approaches?

SH: There are a variety of strategies; unfortunately the most pervasive that I've seen is one of denial. It results partially from our training. Around 1970 there was a dramatic development in the treatment of hemophilia, when the use of fresh-frozen plasma gave way to concentrates of the missing clotting factor. The concentrate was much more effective, and the medical community then tried to teach hemophiliacs that they could lead a relatively normal life. Well, HIV came along, and many of us found out we were infected. But most people weren't sick, so they went on living a "normal life". It was common then for doctors involved with HIV to say "don't do anything until you're sick," a sentiment easily and unfortunately echoed by doctors dealing with both HIV and hemophilia.

DS: Well, since early intervention has proven to be a more effective response to HIV, has that idea found acceptance among people with hemophilia?

SH: Yes, our Northern California chapter of the Hemophilia Society, and I believe the New Jersey chapter as well, are very aggressive about getting such information to their members through publications. I feel very fortunate having moved here by chance before the epidemic, and having access to HIV information developed by the San Francisco gay community. I think it's been a leader in the world. Even though it's smaller, it's been much more together, more politically active and involved than those in larger cities like New York or Los Angeles.

DS: Do hemophilia communities around the country look to the Bay Area as a model, like HIV communities do?

SH: Yes, and not just in HIV or hemophilia, but in health care generally, California is a leader. I'm fortunate to be part of an aggressive push in the local hemophilia community. For example, we reprinted several pages of text from Project Inform's compound Q study. Otherwise, there was no real source of news regarding Q for our members outside of the Bay Area. I know at least one person with hemophilia who has tried compound Q.

DS: In light of that, I wonder if the hemophilia community feels included or excluded in terms of HIV research or funding, or news in general.

SH: Well, the hemophilia community amazes me in terms of its diversity. People often think of it as a white, middle class disease. But it's an experience just as varied as the world at large in terms of race, color, creed and economic group.

DS: How did people respond to the recent "shortage" of clotting factor and the out-of-sight cost of medical care ... particularly the combined costs of clotting factor and HIV treatments?

SH: Clotting factor's price makes HIV treatment look like pocket change. We were very angry when the price went up about 600 percent. People are enraged into numbness. The manufacturers tried to come up with all kinds of "pretty" reasons. But as far as I'm concerned, they just did what any drug pusher does to an unruly client -- cut off their supply for awhile, let 'em squirm.

DS: It's a dynamic parallel to the profits wrought by Burroughs Wellcome, and Lyphomed, and all the pharmaceutical giants. It hardly seems to matter who their target patient population is; they just try to wring out as much as they possibly, possibly can. What do you think the breadth of HIV treatment knowledge is among people with hemophilia?

SH: Not good. Because of conditioning, the system, the doctors. Many hemophilia physicians weren't prepared to deal with HIV, and yet didn't want to give up control of the patients by referring them out.

DS: That's an interesting contrast to the many doctors who refer PWA's elsewhere precisely because they won't work with HIV patients.

SH: Yes, they've dumped responsibility on some "HIV specialist." But a number of the hemophilia specialists were actually too proprietary to consult an HIV specialist.

DS: Now, ten years into the epidemic, do you think the average physician caring for hemophiliac patients is familiar with AZT dosing, or the use of acyclovir, or the approval of EPO and aerosol pentamidine, etc.?

SH: Probably most are familiar with AZT and aerosol pentamidine, not necessarily with acyclovir and EPO.

DS: If you could make one or two things go in a different direction for people with hemophilia and HIV, what would you want?

SH: I'd want much more education disseminated through all the local chapters of the Hemophilia Society...just last year I spoke to a nurse in Chicago who works with hemophilia and who didn't know what aerosol pentamidine was, probably because Cook County wouldn't pay for it. Secondly, the government needs to fulfill a commitment to make treatment available. This year treatment was finally the recipient of some of the giant fundraising benefits. For a long time I found it very disturbing that AIDS prevention was the only idea addressed by those benefits, never AIDS treatment. The Reagan administration's policy toward AIDS very deliberately was to write off those who were already infected.

DS: Right, and that hasn't substantially changed.

SH: No -- Bush has talked more about treatment but hasn't put his money where his mouth is. I really think the value of coalitions will grow in the future, coalitions of all the communities affected by HIV.

DS: When a couple of us from AIDS TREATMENT NEWS attended the national "AIDS and Minorities" conference last August, we sensed very much a common realization that this is not a Black or Latino or gay or hemophiliac problem. If there is any silver lining to the epidemic, it's that people understand they deserve health care, no matter what. Why should someone have to struggle for either clotting factor or AZT? It's stupid.

SH: And what happens with health care will make or break this country.