The mystery is the most terrifying thing about the chronic disease of attrition.
Its threat to human health is unknown. There is no cure or remedy, and it seems that it has not got anywhere.
Chronic wasting disease is not a classical disease caused by a virus or bacteria, but by a radical protein that alters the shape of other proteins in a way that leaves holes in a victim's brain until it causes death.
CWD is a version that affects deer, elk and elk. Mad cow disease (bovine spongiform encephalopathy) affects livestock. Scrapie affects the sheep. There are at least three versions that affect humans. There is even a feline version.
Collectively, they are known as transmissible spongiform encephalopathies, or TSEs. His story is a fascinating mix of politics, marketing, suffering and greed.
The oldest known TSE is Familial Fatal Insomnia. It is hereditary and was documented for many years in a single Italian family. His first known victim was a Venetian doctor in 1765. The victims first noticed symptoms in the mid-50s, when one day they began to sweat profusely. Your pupils narrow to the size of punctures, and lose the ability to sleep. Death due to fatal familial insomnia is said to be horrible. Edgar Allen Poe reflected on it in his poem, The Assignation .
Kuru is another form that exists between the Fore tribe of New Guinea and propagates through cannibalism.
Creutzfeldt-Jakob disease is the best known of the human variants, and the one that gave acceptance to the mainstream of the prion theory.
Dr. Stanley Prusiner, a neurologist and biochemist, won the Nobel Prize in 1997 for isolating radical proteins as the cause of CJD, a human variant that in 2005 killed Truman Ball, a well-known Little Rock real estate agent. CJD is sporadic. It does not follow any lineage, but it occurs with scattered irregularities.
Like all researchers in marginal disciplines, Prusiner struggled to obtain grant money in the first days of his mission. He felt the need for a simple and catchy buzzword to record his concepts in the public consciousness. He combined and juxtaposed "infectious protein" to create a new word, "prion." Like the word "evolution", it temporized a timeless condition and captivated the commercial media.
To his great fortune, Prusiner's revelations coincided with an outbreak of mad cow disease in Europe. That helped open the money gate and the research publicity. To the dismay of his companions, most flowed to Prusiner.
Outbreaks of TSE cause economic and political havoc. An outbreak of scrapie in the 1700s almost destroyed the English lamb industry and, by extension, the Spanish merino wool industry.
In the 1990s, the British government was so intimidated by the possible collapse of its meat and milk industries that it hesitated much longer than it should to prevent BSE-infected canals from entering the food supply. Some 200,000 cows infected with BSE and up to 1.6 million symptomatic cows entered the human food chain in a period of six years.
Despite clinical evidence to the contrary, the British government insisted that there was no human risk in eating infected cow cows. When he finally acknowledged the obvious, the government ordered the slaughter of 3.3 million cows. The European Union banned the importation of English meat, and the total economic impact in pounds sterling was estimated in billions.
It is widely believed that the epidemic was caused by cows that ingested protein infected with scrapie that was included in food supplements. That is also one of the ways in which chronic disease spreads throughout the United States.
Prusiner's prion theory was and remains heretical to classical epidemiology and still faces fierce, if diminishing, resistance. However, Prusiner is the standard bearer and most of the scientific community follows his example.
The enigmatic Prusiner has offered other controversial theories. One is that prions can be responsible for Alzheimer's disease and Parkinson's disease. He has also postulated that prions are the same regardless of species, but that they could be radicalized by species-specific genetic triggers.
Supporting this theory is the conventional belief that some deer, for example, are genetically susceptible to Crohn's disease, but that no deer is genetically resistant to Crohn's disease.
There is no cure or remedy in sight for any TSE. Even if one becomes available, it will not be practical, practical or even remotely profitable to administer it to a wild, deer-free population on a county-wide scale, much less on a national scale.
CWD is with us forever, and if it is a risk to human health, unfortunately we will learn it in the most difficult way.
Sports on 08/04/2018