Authorities appear to be baffled by the rise of one of the scariest illnesses around, writes Ann Eveleth
THE horrible death of a West Rand man from a sickness linked to the one which sparked Britain’s “mad cow disease” scare prompted a flurry of anxious denials this week from South African authorities.
Ironically, the authorities’ stance repeated that adopted for years by the British government – right up until March last year, when it admitted a “probable” link between the fatal human ailment, Creutzfeldt-Jakob Disease, and consumption of meat contaminated by its mad cow equivalent, Bovine Spongiform Encephalopathy (BSE).
Creutzfeldt-Jakob is rapidly gaining a reputation as one of the scariest illnesses around, with some scientists claiming its effect will be worse than Aids. One of the world’s top neurologists, Oliver Sacks, says it represents “a whole new order of disease”.
And amid all the bluster in South Africa this week, officials conceded the one thing they were certain of about the disease is they actually know very little. At least 30 South African cases have been recorded in the past 10 years, all identified over the past 12 months after research began.
Victor Lund (76) died in a private Krugersdorp hospital last week: his brain had been reduced to a sponge in a matter of weeks. Two months earlier, he had been fine, said his neurologist, Kevin Rosman.
Days after Lund’s Canadian-based son, Rob, raised the public alarm, health and agriculture department officials – and the red meat industry – played down any link to mad cow disease.
Officials said South African Creutzfeldt- Jakob cases were of the “classic” form, which had not been linked to beef. Agriculture minister Derek Hanekom said: “To date there is no evidence of the presence of BSE in beef cattle in South Africa.”
While Hanekom said it was too early to comment on Lund’s case, other officials fell over themselves in a string of contradictory statements:
* Dr Cornelius de Hoog, who treated Lund, told the Mail & Guardian he had died from Creutzfeldt-Jakob. He told another newspaper, however, “the diagnosis was never confirmed”.
* Rosman said Lund had not suffered from the BSE-linked illness, but conceded that no scientific tests had been undertaken to prove this. “The only way to definitely diagnose it is to do a biopsy,” he said. “But no responsible surgeon would do that because then you have to throw your tools away.”
Rosman added: “As long as you don’t eat him it’s all right.”
* Dr Theo van der Venter, director of the Department of Health’s food-control unit in Pretoria, said BSE-linked cases “tend to affect younger people and have different symptoms”.
Scientists have, however, identified only 18 cases of the mad cow-linked form of the disease. While most of these occurred in young Britons, the medical journal Lancet late last year reported that the new Creutzfeldt-Jakob strain had also been found in older victims.
In part, South African officials’ nerves have been jarred by the size of the stakes.These increased dramatically last year when the European Union imposed a global ban on imports of British beef after the reluctant admission by the British government.
Hundreds of thousands of British cattle have been slaughtered in a drive costing billions of pounds. Other countries whose cattle have contracted the BSE disease have also suffered bans or restrictions on their beef exports.
The South African red-meat industry pounced on Britain’s beef problems as a major marketing opportunity. The ban on British beef imports is still in force in South Africa.
The problem, however, is it may already be too late to protect South African beef- eaters. Scientists believe many victims of the BSE-linked Creutzfeldt-Jakob strain contracted the disease from eating contaminated beef in the mid-1980s — when South Africa imported significant amounts of British beef products. Prior to the ban, South Africans consumed between 3 500 and 4 000 tons of beef a month from Europe, most of it from Britain.
Dr Pieter Kempen, general manager of South Africa’s Meat Board, said the chances that infected British beef could enter the South African food chain are “very slim”.
But he said the government’s failure to recall British beef from local shelves when it banned UK beef could still leave consumers exposed. The board had discovered British beef in some butcheries just four months ago.
“I advised the Department of Agriculture to recall all British beef,” Kempen said, “but they said it was out of their control. It’s difficult to quantify how much is still floating around.”
Hanekom said his department has “taken all precautionary measures to protect the public and consumers of beef” from BSE- infected meat.
It will, however, be years before South Africa can fully judge the impact of BSE and its human equivalent on public health.
In a recent issue of The New Yorker magazine, Oliver Sacks described his first encounter with Creutzfeldt-Jakob disease as far back as 1964: “We had no idea of the truly singular nature of Creutzfeldt-Jakob, its affinities with other human and animal diseases, or that it would turn out to be the archetype, the epitome, of a whole new order of disease. We never for a moment thought of it as infectious; it was only in 1968 that it was shown to be a transmissible disease.”
The cause of 85% of the “classic” Creutzfeldt-Jakob cases is also still unknown. “There’s no definite proof of a link, but that’s why the disease is so dangerous,” Kempen said. “All we know is that we don’t know.”