Getting Help to Hemophiliacs Is No Simple Task

AIDS first gained notoriety as a disease largely confined to homosexual men and intravenous drug users. But another, less visible segment of the population has been hit especially hard: hemophiliacs.

Medical researchers estimate that 92% of people classified as severe hemophiliacs have already been exposed to the AIDS virus, principally those who received transfusions in the period between the discovery of the disease in the late 1970s and the development of adequate screening tests.

It wasn’t until 1985 that testing became available and blood samples were routinely scanned for the antibodies that typically form in response to the human immunodeficiency virus (HIV).

Nationwide, about one person in 10,000 has hemophilia, a blood disorder that disrupts the body’s natural clotting

mechanism. But attempting to determine how many hemophiliacs live in San Diego County, and how many may be infected with the AIDS virus, is not easy.

“Almost every one of them (severe hemophiliacs) is antibody-positive,” said Lance Clem, a San Diego AIDS Project worker. “It’s an incredible situation.”

Clem estimated that 90 to 200 hemophiliacs live in the county. But compiling exact figures is difficult because hemophilia is not a condition requiring a report to health officials, he said.

Clem said hemophiliacs rarely seek help from the AIDS Project, despite the high probability that they have been exposed to the virus and may need testing or other support services.

“The project traditionally and historically, and perhaps somewhat inaccurately, is perceived as representing only gay males,” said Clem. “Hemophiliacs develop the HIV infection as a result of the blood products they use, and there must be some of them who blame gay men for their condition. That might be the reason why they’re reluctant to get involved with the AIDS Project--they’re resentful.”

“On the other hand, there are those (hemophiliacs) who realize it just happened, and there was no intent to taint the blood product. It’s a very, very touchy subject for them.”

Highly Purified Blood Product Offers Hope

The first cases of AIDS were reported in the United States in about 1978. But it was almost a decade before reliable testing was developed, and it wasn’t until October, 1987, that federal officials announced approval of a highly purified blood product--developed at Scripps Clinic and Research Foundation in La Jolla--that is expected to substantially decrease hemophiliacs’ risk of contracting AIDS.

The county Department of Health Services, which has been tracking AIDS cases since 1981, reports that, as of June 1, 878 county residents had been determined to have the disease. Of those, 36 contracted the virus through transfusions of contaminated blood or blood products, and six of them are hemophiliacs, according to department epidemiologist Dr. Michele Ginsberg.

Tracy Mahoney, health educator at the Hemophilia Council of California AIDS Project in San Diego, expressed doubt that the county’s figures are accurate. Mahoney said some doctors who don’t usually deal with AIDS patients may not be adept at recognizing the disease’s symptoms.

“As a result, there may be some underreporting going on,” she said.

Mahoney agreed that hemophiliacs as a group are tough to catalogue and even tougher to help.

“There may be as many as 100 hemophiliacs in San Diego County who have been exposed to the virus,” she said. “But there’s no way to make them go get tested.”

Hoping to Attract Hemophiliacs’ Attention

The hemophilia council AIDS project, which is not affiliated with the San Diego AIDS Project, opened its doors in North Park in January. There are only two employees, Mahoney and mental health counselor Laurie Pendleton, but together they hope to attract the attention of San Diego’s hemophiliac community and point them in the direction of AIDS awareness.

A private, nonprofit agency financed by the state, the project has concentrated so far on getting the word out to hemophiliacs who may have ignored the fact that they are at risk.

The estimate that 92% of severe hemophiliacs are HIV-positive is tempered by the fact that less than half of all hemophiliacs are classified as “severe,” Mahoney said. Those who are may require two or three transfusions of a special blood-clotting serum a week.

Another misconception about hemophiliacs is that they will bleed profusely from cuts or wounds, putting people near them at risk of being contaminated with the AIDS virus. Most hemophiliacs are more prone to internal bleeding, Mahoney said.

“They have blood-clotting platelets and other substances that will cause a surface clot on their skin,” she said. “So, if you’re around someone with hemophilia and they get a little paper cut or even a larger knife cut, there’s no danger that person will bleed all over the place.”

‘It’s a Very Scary Time, It Really Is’

“We have had a few instances of prejudicial behavior toward persons who are HIV-positive,” Mahoney said. “They lump it all together and treat people who have anything to do with AIDS as lepers. . . . It’s a very scary time, it really is.”

Mahoney explained that hemophilia is a genetic condition passed from mother to child through the mother’s X chromosomes. Although it originates with the mother, it is almost always passed to male offspring, she said. Females may be carriers, but don’t manifest the condition as often as males.

In about one-third of the cases, the female does not inherit the condition from her mother, but develops it as a spontaneous genetic mutation, Mahoney said.

Hemophiliacs are missing one or more of the clotting factors in the blood. At one time, they received transfusions of “whole blood” to make up for the deficiency, but the process has been refined so that now only the necessary clotting factor is used. According to Mahoney, it takes more than 20,000 pints of blood, pooled together, to extract a single “lot” of the clotting factor.

“Because hemophiliacs have received blood products gathered from over 20,000 people, they have been exposed (to AIDS) at an extremely high rate,” she said.

It was a few years after the first cases of AIDS began appearing in the United States that the connection was drawn between the virus and the blood system, she said.