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October 1999 Editorial: As if Infected Beef Weren’t Scary Enough—What’s in the Nation’s Blood Supply? By Catherine Clyne

 

A “talk paper” released by the Food and Drug Administration (FDA) on August 17 1999, entitled “New Precautionary Measures to Reduce the Theoretical Risk of New Variant CJD From Blood Products,” announced the release of guidelines to blood donor centers “to exclude potential donors who have spent six or more cumulative months in the U.K. between January 1, 1980, and December 31, 1996 from donating blood.” The fear is that people who dined on British beef may be carriers of the infectious agent that causes Bovine Spongiform Encephalopathy (BSE) or “Mad Cow Disease.” BSE is understood to be transferred to humans by eating infected cow flesh and is linked to the fatal human spongiform disease Creutzfeldt-Jakob Disease (CJD). Since CJD has a long and invisible incubation period, with symptoms showing up as much as a decade after inception, it is feared that the disease could “theoretically” be passed on unwittingly by infected blood donors. According to the American Red Cross, the ban will eliminate about 2.2 percent of all blood collected, about 500,000 donors.

The recommendation that people who ate British beef nearly 20 years ago not donate blood, speaks volumes. The prion (a protein gene) that causes BSE is known to withstand incineration and is therefore not eliminated by cooking. It does not respond to current disinfectants or sterilization processes, leaving the possibility of infected medical instruments as well. It seems that the lid is barely on CJD and that it may be more prevalent than government agencies would like to admit.

The situation is reminiscent of the early 1980s when little was known about the transferal of the Human Immunodeficiency Virus (HIV), believed to be the cause of Acquired Immune Deficiency Syndrome (AIDS). Many people who regularly rely on blood products, such as hemophiliacs, and some who underwent surgery requiring blood transfusions, became infected with HIV. Scientists now know how to screen blood products for HIV; however, we are right back in that bloody nightmare with CJD. According to the Centers for Disease Control (CDC), the cause of CJD “is thought to be an unconventional filterable agent. Currently, there is no test to detect a patient’s immunologic response to the infection and the etiologic agent has not been identified. The exact mode of transmission in humans is not known.” (emphasis added) In the U.S., CJD is suspected of often being misdiagnosed as Alzheimer’s disease or other types of fatal dementia. So little is known about the disease and how it is transferred that the possibility that more people could have the disease and that blood products could be infecting people is now a scary reality.

Take for example the tragedy of Doug McEwen of Kaysville, Utah. CJD is understood to strike about one in a million people around the world, most of them over the age of 50. Mr. McEwen died of CJD this past March. He was only 30. Because he was so young, his is considered to be a rare case—only five in one billion people aged 30 or under are known to have CJD. But these people are considered to have the “new variant” of the disease, while a biopsy from McEwen’s brain seemed “similar” to “classic” CJD. It is unknown how McEwen got the disease. He reportedly hunted and ate wild game, including elk and deer, which are known to carry chronic wasting disease, another degenerative illness found in animals. However, his family members ate the same meat and they have not shown symptoms of CJD. McEwen spent some time in Canada doing missionary work well over a decade ago. He could have ingested infected meat then. Perhaps...maybe. The fact is no one knows. What is known is that a young American man died of CJD.

TThere is more to this sad story: Mr. McEwen was a frequent blood donor. He donated 22 times between 1996 and 1998. His plasma was shipped to the Bayer pharmaceutical company, which broke it down into various blood products that were then shipped to 46 countries around the world. His family is concerned that he may have unwittingly passed on the infection through his donations. In an article in USA Today (1/19/99), his wife Tracie McEwen expressed her concern: “He donated well after his symptoms started. He was at the point where he was forgetting phone numbers and names, but if I’d thought he had a fatal disease, he wouldn’t have donated.

While health authorities publicly claim that there is no concrete evidence demonstrating that CJD can be transmitted through blood transfusion and consider it only as “theoretical,” the FDA swiftly quarantined all of the blood products containing McEwen’s plasma since January 1998. However, the plasma donated before then has already been used. “They’ve tried to assure me it can’t be transmitted that way,” says Tracie McEwen, but she’s not convinced. “I don’t think they know.” Surprisingly, McEwen’s plasma was later released because he was considered to have “classic” CJD.

According to the FDA, CJD is “a rare but invariably fatal degenerative disease associated with a poorly understood transmissible agent. CJD cases occur by an unknown mechanism. It may also be acquired by exposure to infectious material; or may be familial, caused by a genetic mutation of the prion protein gene.” Basically, the neurological system deteriorates to the point that large holes are created in the brain. During an 18-year period (1979-96), 4,468 cases of CJD were reported to the CDC, although it is not mandatory to report the disease. Just imagine how many unreported and undiagnosed cases there may be.

“In 1996,” reports the FDA, “a previously unrecognized variant of CJD was described almost exclusively in the U.K., and is referred to as new variant CJD (nvCJD).” NvCJD is linked to BSE and over 40 people have died from it. According to the FDA, however, no cases of the new variant have been detected in the U.S. So, the reason that the FDA gives for the release of blood known to have been donated by people with “classic” CJD, including McEwen’s, is that “information and laboratory studies have indicated that transmission of the CJD infectious agent by blood products is highly unlikely.” (emphasis added) In contrast, however, nvCJD gives cause for alarm: “No evidence exists that the disease has been transmitted by blood transfusion, but current studies cannot exclude this possibility.” What is going on?

The World Health Organization reports that CJD is known to have been transmitted through the use of “contaminated human pituitary-derived growth hormone or gonadotropin, dura mater [brain and spinal membrane] grafts, corneal transplants, and neurosurgical instruments.” Even conventionally sterilized surgical instruments can infect. The CDC offers some suggestions for disinfection, but their overarching position is: “The minimum requirements for decontamination procedures/precautions for materials potentially contaminated with the agent causing CJD are UNKNOWN.” (caps theirs)

The American and Canadian governments have issued notices to hospitals, families and individuals warning that the blood transfusions, insulin and albumin that patients and loved ones have received may have been infected with CJD. A mother received a notice stating that “certain lots of albumin [a blood protein] were prepared from blood donated by individuals who were subsequently diagnosed with Creutzfeldt-Jacob disease (CJD). According to our records your child received some of this product before it was recalled. To date there has been virtually no evidence that your child will develop CJD. Therefore, there is little reason for concern. But we also know that the more informed you are about this situation, the more reassured you will feel.” (Note that the notice makes no distinction between nv and classical CJD.) Reassured, I’m sure. Such notices have completely changed the lives of people because they do not know if they or their loved ones are infected or whether they will come down with CJD in the next decade or so. It’s a terrifying question mark to have hanging over one’s life.

In January, advocacy groups, such as the Center for Food Safety and the Humane Farming Association, and CJD victims and their families, filed two formal petitions to the FDA and the CDC calling for aggressive monitoring of CJD cases in the U.S., and demanding that animal feed regulations ban the feeding of animal remains to farm animals. To date, no decisive action has been taken with the exception of the FDA’s recommendation to turn away blood donors who have lived in England.

Only five percent of eligible donors give blood annually, which currently is not enough to supply demands. Since our general readership falls into the low-risk category as carriers of CJD, we at Satya urge our readers to donate blood. Blood is precious and always needed. This is one act of compassion that we can make for our society.

To find a blood donor clinic near you, call the New York Blood Center at 800-933-2566. For more information on CJD and BSE, visit www.mad-cow.org.