Defusing the timebomb of human BSE

Defusing the timebomb of human BSE

July 14, 2001 Belfast News Letter by John Von Radowitz

IT'S not a pleasant thought, but anyone who ate a lot of burgers, meat pies and cheap mince in the 1980s is likely to have been exposed to mad cow disease.

A whole generation of impoverished university students would have fallen into that category. So would a great many children, especially from less well-off families.

Until it was banned from human consumption in 1989, large amounts of brain and spinal cord tissue found its way into beef products like burgers.

Some of it came from animals with BSE, and could potentially pass the disease on to humans in the form of variant CJD.

Many people are almost certainly, to this day, walking around with the infectious agent in their bodies.

The incubation period of vCJD is like a slow-burning fuse. When detonation occurs, perhaps 20 or more years after exposure, the victim will start showing symptoms of dementia, deteriorate rapidly and die within a year.

At present there is no way of telling, or even guessing with any accuracy, how many people may be harbouring the disease.

The only way it can be identified for sure is by examining the brain after death. Less definite diagnosis can be made by analysing a surgically removed piece of brain, or testing the tonsils after their removal.

However, this might soon be about to change. Scientists around the world are racing towards developing routine tests that can detect vCJD in blood or urine. They say vCJD testing might be a real possibility a year from now.

When these tests become available it will have as profound an effect as the introduction of HIV testing.

People who test positive will have to face the prospect of dying at an unknown time in the future of a horrible disease for which there is no cure.

As occurred with HIV, carriers of vCJD might be feared and discriminated against. They might find it difficult to get life insurance.

Counselling would have to be introduced to help people cope with the shock of a positive diagnosis.

Variant CJD testing would also have a major impact on blood donations. The National Blood Service says blood stocks could fall by 50 per cent as contaminated supplies are discarded and people stop giving blood because of fears of being tested.

The most widely accepted theory is that the disease is spread by rogue prion protein molecules that have changed shape and become dangerous.

Prion is a microscopic protein particle thought to be the infectious agent responsible for scrapie - a fatal disease found in sheep and goats - and other degenerative diseases of the nervous system.

One of the most hopeful techniques has been developed by Swiss scientists working for Serono, Europe's biggest biotech company.

They have exploited the "chain reaction" effect in which rogue prions multiply. The process is speeded up in a test tube by repeatedly incubating rogue prions with "normal" prion protein.

Scientists found the presence of one rogue prion can be exaggerated by using it to alter the shape of large numbers of ordinary prions. Thus the prion is "amplified" to a point where it can be detected.

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