February 13, 2004 The Seattle Times by Sandi Doughton

Now they're trying to decide what to do about it.

"We shouldn't have been surprised, but it was quite a shock to hear it," Dr. Robert Rohwer, of the Veterans Administration Medical Center in Baltimore, told a U.S. Food and Drug Administration (FDA) advisory panel in a packed meeting yesterday.

Rohwer briefed the committee on his own studies, which show that even blood plasma, originally thought to be safe, can carry the disease in animals.

The 14-member group is reviewing the nation's blood-safety rules in the wake of America's first mad-cow case and the news that a British man died of the human version of the disease after receiving blood from another victim.

Though the panel is not expected to vote on specific proposals during the two-day meeting, the United Kingdom is considering a ban on blood donations from anyone who has received a transfusion.

Such a step here could undermine the nation's already-tenuous blood supply, said Mike Fitzpatrick, chief policy officer for America's Blood Centers. Most blood centers operate on no more than a two- to three-day inventory, he said.

FDA officials said no additional safeguards may be needed because the United States has adopted rules to exclude blood donors who lived in the U.K. and other parts of Europe during the years the mad-cow epidemic was raging there.

"We've taken the theoretical risk of blood-borne infection seriously for years," said Dr. David Asher of the FDA's Center for Biologics Evaluation and Research.

But Hatte Blejer, of Alexandria, Va., shook some of the committee members with the story of her late husband, Daniel, a lifelong blood donor who died five weeks ago of Creutzfeldt-Jakob Disease (CJD), a condition similar to the human form of mad-cow disease.

Experts believe people can contract the human form of mad-cow, called new variant CJD, by eating beef from an infected cow. The 140 known cases are concentrated in the U.K. The only U.S. case is a woman who lived most of her life in England.

The classical form of CJD, which Blejer's husband had, is thought to be caused by genetic mutations that arise spontaneously in roughly one person in a million. Both diseases kill victims after eating holes in their brains.

"Unless you have seen someone die over 12 months, as I did, you don't know the human impact of this disease," Blejer told the panel.

As soon as she found out what was wrong with her husband, Blejer contacted local blood banks, where he had donated three times a year for 25 years. She also tried to alert hospitals where he had undergone seven brain operations.

She discovered there was no federal system in place to warn blood banks and other medical facilities when a former donor or patient is diagnosed with CJD.

"If I had not contacted them, the Red Cross would never have known," she said.

As it was, there was little the blood bank could do, since the donated blood had been used. Blejer said she was never able to find out whether the surgical instruments used on her husband were reused, potentially spreading the brain infection to other people.

The U.S. tries to screen people at risk of classical CJD from the blood-donor pool with a questionnaire, which asks about relatives who may have had the disease and about human-tissue implants, which may transmit it.

So far, there's no evidence that classical CJD can spread through transfusions, though Rohwer said he would be surprised if it didn't.

British researchers are on the alert for any other transfusion-related cases of new variant CJD. Fifteen people who died of the disease donated blood before they got sick, and scientists have tracked those blood products to 48 recipients. Many have died of unrelated causes, but 17 are alive, said Dr. Robert Will, of the U.K.'s National CJD Surveillance Unit.

In the case reported in December, a 62-year-old man came down with the human version of mad-cow disease 6 years after receiving blood donated by an apparently healthy, 24-year-old. The younger man died of new variant CJD 3 years later.

That means the disease can be transmitted long before symptoms appear, Will said. And the long incubation period means more people may yet develop the disease as a result of transfusions.

"We simply can't know for several more years," he said.

White blood cells seem to carry about half of the infectivity in blood. Because of that, the U.K. and Japan require that white blood cells be removed from all donated blood. U.S. blood banks remove white cells from about 60 percent of blood.

Rohwer is co-founder of a private company working on a system to filter the mad-cow agent from blood plasma, the straw-colored fluid left after red and white cells are removed. Plasma is used to produce a wide range of products to treat clotting problems and other blood disorders.