Winter 2000 - Factor Nine News

Bovine spongiform encephalopathy (BSE) is a similar disease that has recently begun to occur in cattle. It is also known as “Mad Cow Disease”. BSE was essentially unknown until the early 1980’s when an epidemic of Mad Cow Disease began among cattle in Britian. The epidemic was apparently caused by feeding cattle with animal by-products, including brain and nerve tissues which are now known to carry the agent that causes BSE and the other types of diseases such as CJD. Britain ended the BSE epidemic in the mid-1990’s by banning the use of animal by-products in cattle feed and by slaughtering thousands of suspected cattle. However, around that same time a new disease in humans began to be seen there. The disease had very similar symptoms to CJD but it was occurring in young people.

Extensive research on this new disease has shown that it is caused by the same agent that causes BSE. The disease is now known as “variant CJD” or vCJD, and it is assumed to have come from some kind of exposure to cattle with BSE. There is a good chance that at least some of the cases were caused by eating beef from BSE-infected cattle, but so far that has not been definitely confirmed. So far, there have been at least 80 cases of vCJD, most of them in Britain, but also several scattered around the rest of Europe. There have been no known cases of either BSE or vCJD in the U.S.

CJD, BSE, and vCJD are part of a group of diseases known as transmissible spongiform encephalopathies or TSE’s. The “spongiform” part of the name refers to the fact that the diseases eat holes in the brain causing it to eventually look like a sponge. Human TSE’s were first studied in a group of native people in New Guinea who were afflicted with a form of dementia called kuru. Kuru was eventually traced to their practice of eating the brains of their deceased relatives, which they actually did as a sign of respect for the deceased’s wisdom. The earliest TSE known in animals is scrapie which afflicts sheep. It is widely believed that scrapie was the source of BSE. It was transmitted to cattle through the use of sheep by products in their feed.

The reason we are interested in this is that vCJD behaves very differently than CJD. It is not currently known whether vCJD can be transmitted by blood or blood products. However, researchers in Scotland conducting a study on BSE were recently able to transmit the disease to a sheep by a blood transfusion taken from another sheep that they had infected with BSE. This is only one sheep and does not prove anything yet, but it is the first evidence that BSE, and possibly vCJD, might be able to be transmitted by blood. This is not cause for alarm, but it definitely is cause for consideration and more research. Even before this was known, blood banks and plasma processors have been taking precautions against vCJD.

Today in Britain, all plasma products are imported from countries where BSE does not occur. None come from blood donated by British residents. In the U.S. the FDA has banned collecting blood donations from anyone who has spent a cumulative period of six months or more in Britain between 1980 when the BSE epidemic began and 1996 when it was over. A number of studies over the past several years have shown that plasma products are very safe from exposure to CJD, even if it were to occur in blood. Almost nothing is known, however, about vCJD. As might be expected, a large amount of research is going on in Britain, and this year the Red Cross opened the first lab in the U.S. that is qualified to work with vCJD. The U.S. is free of BSE and vCJD and both the FDA and the U.S. Department of Agriculture have very strict rules to make sure it stays that way. The new research is trying to find out more about these diseases and the agents that cause them, to find ways of preventing their spread, and to find cures.

This all takes time, but the pace of research is far faster than it was during the AIDS crisis. The number of cases involving transfusion by blood products is still believed to be zero. We should all be aware, but the risk seems to us to be very small. Today it is much less likely that anyone will be affected because of a blood transfusion or the use of plasma products. You are more likely to suffer from not using the products if you need them. This may seem like a worrisome time with new diseases appearing all too often, but we are actually very fortunate that medical science has progressed to where it is today. We can now find problems very quickly. Less than 100 years ago, before antibiotics were discovered, people routinely died from simple bacterial infections. Now we can deal with diseases like AIDS and Ebola and at least limit the numbers of casualties. Diseases like the TSE’s are the next frontier, and there is no reason to think that they will not be conquered as well. We will keep you well informed as that happens.

The Coalition has a copy of this scientific article if you would like to receive one.