Questions and answers about chronic wasting disease

April 23, 2001 Scripps Howard News Service by Gary Gerhardt

Could chronic wasting disease in deer and elk pose the same human health risk as mad cow disease? Here's what scientists know.

Q: What is chronic wasting disease?

A: Chronic wasting disease in deer and elk, mad cow disease in cattle, scrapie in sheep and Creutzfeldt-Jakob disease, or CJD, in humans all belong to a family of illnesses called transmissible spongiform encephalopathies, or TSEs.

Q: What do these TSEs do?

A: The disease agent attacks the brain and central nervous system, destroying healthy tissue. A person loses basic physical and mental abilities as the disease progresses. The word "spongiform" describes the spongelike condition of brain tissue seen in victims.

Q: Is there any cure?

A: No. The disease is always fatal.

Q: How rare is the human form and how do people get it?

A: Creutzfeldt-Jakob disease infects about one person per million each year worldwide. Typically the disease in humans, called "sporadic CJD," arises spontaneously, usually after age 50. A few cases are inherited and a few have been contracted by transplants from ill donors.

Q: How do you know you have it?

A: You don't, until late stages of the disease. It is believed to take 10 or more years to develop before memory problems and motor dysfunctions appear and advance, leading to death within about eight to 10 months.

Q: What causes TSEs?

A: Unlike bacteria or viruses that cause most disease, TSE is caused by an abnormal protein particle called a prion.

Q: Is there any way to destroy the infectious agent?

A: The prion is extremely difficult to destroy. It contains no nucleic acid (DNA or RNA) as do other bacteria and viral microbes, so there is no such element to destroy. Cooking meat, or even heat sterilization in surgical settings, does not destroy it. Using household bleach is considered an effective method of disinfecting [Not necessarily] .

Q: How does the disease spread?

A: Scientists believe that in humans it doesn't spread by traditional means of contact like the flu. They are uncertain how it spreads within other species.

They believe cows contracted the disease from eating the remains of scrapie-infected sheep added to cattle feed to increase protein content. Humans are believed to have contracted the new variant by eating meat from infected cows.

Q: What species have TSEs?

A: Scientists don't know all of the answers. Forms of the TSE have been seen in sheep, cattle, minks, humans, deer and elk, zoo monkeys, and domestic and wild cats. The only known avenue from animals to humans is through cattle.

Q: Are TSEs at all related to hoof and mouth disease?

A. No. There is no relation whatsoever. Hoof and mouth disease cannot spread to humans [Common misconception] .

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