RACHEL'S ENVIRONMENT & HEALTH WEEKLY

---July 9, 1998---

MAD COW DISEASE, PART 1

In 1985, cows in Britain began to die of a mysterious ailment that no one had ever seen before. The symptoms were strange. At first the cows staggered and drooled, their ears twitching. Then they began to show signs of fear, grinding their teeth, acting aggressively toward other animals. Soon they died. Farmers named the condition "mad cow disease" and the name stuck.

The disease is now known formally as "bovine spongiform encephalopathy" or BSE for short. BSE is one of a small family of diseases called transmissible spongiform encephalopathies, or TSEs. TSEs destroy neurons, the main nerve cells in the brain, creating holes that leave the brain resembling a sponge (thus "spongiform"). BSE, like the other TSEs, is an infectious disease, meaning that it can be transmitted from one cow to another.

During the 13 years since it first appeared, mad cow disease has killed more than 167,000 cows in Britain[1] and many more became infected but were slaughtered for food before symptoms appeared. Symptoms take an average of 5 years to show up after a cow is infected. Until the late 1990s, there was no test that could detect whether a cow was infected -- only the appearance of symptoms and microscopic examination of the brain could provide a definitive diagnosis. (Even today, tests require brain tissue, so they cannot be conducted on live animals.) British-type BSE has now been observed in cows in Switzerland, the Netherlands, Ireland, Portugal, France, Oman and the Falkland Islands. To date, the disease has not been observed in cows in the U.S.

When the disease first struck in Britain in 1985, health authorities insisted that British beef was safe to eat. For 10 years they defended that position aggressively, despite mounting evidence to the contrary. Then in 1996, the official story changed suddenly and the public was stunned. A panel of government scientists told Parliament in 1996 that the "most likely explanation" for 10 cases of a new TSE disease in humans was that BSE had moved from cows into people. That explanation is now widely accepted by most scientists, though airtight evidence remains elusive.

By the time of the stunning announcement in 1996, some British experts calculated that more than a million infected cows had already been consumed in Britain.[2]

In humans, the BSE-like disease is called "new variant Creutzfeld-Jacob disease," or nvCJD for short. Creutzfeld-Jacob disease (CJD) is a member of the TSE family, a brain-destroyer. CJD has been recognized for a long time as a rare disease of the elderly -- very similar to Alzheimer's disease -- but nvCJD is different. It has somewhat different symptoms, a different pattern of disintegration in the brain, and it strikes young people, even teenagers. Between 1995 and early 1998, at least 23 people died of nvCJD in Britain and at least one in France,[3] the oldest of them age 42 and the youngest 15.

In January 1997, British epidemiologists tried to estimate how large the outbreak of nvCJD might become. They concluded that the data were not sufficient to allow a precise estimate: somewhere between 75 people and 80,000 people would eventually die of the new disease, they estimated.[4] Only time will tell. More precise estimates of the size of the problem are not possible because no one knows for sure how long nvCJD "incubates," how much time elapses between infection and the appearance of symptoms.

An excellent recent book by Sheldon Rampton and John Stauber, MAD COW U.S.A.--CAN THE NIGHTMARE HAPPEN HERE? tells the story of the emergence of mad cow disease in Britain, and the scientific and political battles that have ensued.[5] Despite the evocative title, the book is thoroughly-documented and carefully-written. Without oversimplifying the details, the book recounts a complicated story of medical discoveries, strongarm tactics by the beef industry, and waffling and coverup by governments on both sides of the Atlantic -- yet it reads as easily as a detective story. As a piece of science writing -- a description of science as it plays out in the real world -- the book is an impressive accomplishment. By the end, the reader has absorbed several important lessons about public health policies distorted by big money, and about very serious threats to our first amendment right of free speech. Rampton and Stauber show that the U.S. beef industry will go to almost any lengths to try to prevent a public discussion of "mad cow" and the steps that the U.S. government still needs to take to prevent this disease from becoming an American problem.

In a nutshell: Mad cow disease developed because of a little-known but very-widespread modern agricultural practice -- farmers feeding dead cows to their cows, thus turning a vegetarian species into meat-eaters.

In Britain and the U.S., when a cow is slaughtered, about half of the animal cannot be sold for human uses -- the hide, bones, entrails, hooves, horns, fat, gristle and tough membranes are, by law, not permitted to be used in food. Obviously something else has to be done with these parts, to avoid creating a public health hazard.

Then there are "downer cows" that must be dealt with. Cows that cannot stand up, cows that collapse, and cows that die mysteriously are called "downers." Every year in the U.S. about 100,000 cows die of unknown causes. One day they are alive and the next day they are dead, and no one knows why. Something must be done with their carcasses.

In addition to downer cows there are thousands of pigs, horses, chickens, and sheep that die of unknown causes each year. To prevent public health problems, they must be disposed of. Then there is road kill -- deer, elk and other large mammals killed by motor vehicles.

In the U.S. all of these animals and animal parts end up at 280 "rendering" plants where they are ground up and boiled down. (The British call such plants "abbatoirs.") Up until the 1960s, the fat from rendering plants was generally used to make soap. But the invention of detergents, derived from petroleum, greatly reduced the demand for soap. As a result, the rendering industry had to develop new markets for its products. They hit on animal feed, and it became a great commercial success.

From the 1960s through the mid-1990s, rendering plants dried their rendered products, ground them into the consistency of brown sugar, and sold them for animal feed. Feed mills then mixed these animal by-products into various feed formulations -- about one-third for cattle, one-third for pigs and chickens, and one-third for pets.

Unfortunately, some of the animals sent to rendering plants (or abbatoirs) are sheep killed by a disease called "scrapie." Scrapie is another TSE, a member of the same family as BSE, CJD, and nvCJD -- one of those diseases that eats holes in the brain and invariably kills its victims. Scrapie takes its name from the way sheep act once they get the disease -- they rub up against a fence or a barn until they scrape away their wool, leaving raw wounds. Then they die.

Scrapie has been a well-known, though mysterious, disease of sheep for at least 200 years, but only recently have scrapie-infected sheep been fed to cows. Scientists who study mad cow disease believe that the illness crossed the species barrier from sheep to cows through contaminated feed. Indeed, the British in 1988 banned the practice of feeding animal carcasses to other animals and within seven years new cases of mad cow disease diminished quite dramatically from 900 to 1000 per week to 280 to 300 per week.[6]

The U.S., however, has been slow to act. Scrapie is well-established among sheep in this country. From October, 1988, to June, 1989, scrapie was diagnosed in 52 flocks of sheep in 20 states.[5,pg.104] Clearly, there was ample reason to ban the feeding of animal carcasses to animals in this country when the British took that action in 1988. However, the beef industry, and the rendering and feed industries, have generally opposed such precautionary measures.

TSEs have a very long incubation period. In cows, BSE takes three to eight years (average, five years) from the time of first infection to the appearance of symptoms shortly before death. CJD in humans has an incubation period of 10 to 40 years. Thus, by the time symptoms of BSE appear, many cows are likely to be carrying the infection silently. This was confirmed earlier this year in Switzerland when tests of brain tissues from 1761 cows revealed 8 infected animals without any symptoms, for a "silent" infection rate of 4.5 per 1000. This is 100 times as high as the rate of Swiss cows showing symptoms.[7] If this rate holds for Britain, it means that today there are about 460,000 British cows infected -- but symptom-free -- in a total herd of roughly 11 million.

TSE diseases are characterized by a long incubation period, and they are always fatal. Furthermore, the infectious agent is incredibly resistant to deactivation. Cooking infected meat, or even rendering it at high temperature, does not completely eliminate its infectivity. Animals get TSEs by eating infected animals or parts of infected animals, especially nerve tissues.

TSE diseases have now been identified in sheep, pigs, goats, cattle, deer, elk, mink, mice, hamsters, guinea pigs, domestic cats, puma, cheetah, eland, kudu, Arabian oryx, myland, marmosets, macaques, chimpanzees and humans. In addition, a TSE has been reported in ostriches in a German zoo.[8]

Thus one might think the U.S. Food and Drug Administration (FDA) would prohibit the feeding of any animals to any other animals, as the British did in 1988. But that is not what the FDA has done. Under pressure from the beef, rendering, and feed industries, in 1997 FDA only prohibited the feeding of ruminants and mink to ruminants.[1,8] Ruminants are animals that chew their cuds, including cattle, sheep, goats, deer and elk. Mink are included in the ban because they can get a TSE similar to mad cow disease.

FDA is still allowing the feeding of pigs to other animals, and the feeding of blood and gelatin from rendering plants to all animals. For example, many calves in the U.S. are being raised on a diet of dried blood taken straight from rendering plants. Pigs and chickens are still being fed rendered animal products. There are sound scientific arguments why this policy represents a form of Russian roulette being played with the health of the American public. Given that we are dealing with infectious diseases that invariably kill, the precautionary principle (see REHW #586) seems the only appropriate policy.

More next week. --Peter Montague


---July 2, 1998---

THE Y2K PROBLEM, PART 2

Y2K is the shorthand name for Year 2000, a computer problem that may affect both environmental and human health in every industrialized or industrializing country. Many thousands of operating computers currently represent the year by two digits: 25 is 1925 and 98 is 1998. When January 1, 2000 rolls around, these computers will assume 00 means 1900, not 2000, unless their software is fixed. Computers that have this "Y2K date problem" are called "noncompliant."

If you were born in 1935, a computer this year would determine that your age is 98-35=63. However, two years from now that same computer may determine that your age is 00-35=-35. At that point the computer may stop working, or it may pass this incorrect information on to others, including other computers.

This seemingly-simple problem has large consequences.

BYTE magazine, a technical computer journal, calls Y2K "a crisis without precedent in human history."[1] FORTUNE magazine calls it "the biggest screwup of the computer age"[2] and says it may cost $1 trillion to fix. (The Vietnam War cost half that much, $500 billion.) The Electric Power Research Institute (EPRI) --a trade association for electric utility companies --says the Y2K problem will begin to disrupt businesses, including electric utilities, a year before the new century begins: "Major disruptions in technical and business operations could begin as early as January 1, 1999. Nearly every industry will be affected," EPRI says.[3]

If the disruptions don't begin January 1, 1999, they may begin July 1, 1999, when fiscal year 2000 begins for 46 out of the 50 states, or on October 1, 1999, when fiscal year 2000 begins for the federal government. But most of the problems will probably surface after midnight December 31, 1999.

Charles Rossetti, commissioner of the U.S. Internal Revenue Service (IRS), told the WALL STREET JOURNAL April 22, 1998, that Y2K is a "very, very serious problem." "There's no point in sugarcoating the problem," he said. "If we don't fix the century-date problem, we will have a situation scarier than the average disaster movie you might see on a Sunday night. Twenty-one months from now, there could be 90 million taxpayers who won't get their refunds, and 95% of the revenue stream of the United States could be jeopardized."[4] Mr. Rossetti went on to say he is confident that these problems will not occur because IRS computer experts will prevent them. Critics of IRS are not so sure.[5]

In addition to many thousands of noncompliant computers needing to be fixed, there are millions of noncompliant "embedded systems" --computer chips embedded in other equipment such as photocopiers, telephones, elevators, traffic lights, electric generating plants, and nuclear missiles --that also need to be fixed or replaced.

The deadline for having everything fixed --December 31, 1999 --is just over 500 days away, and it is an unusual kind of deadline because it cannot be ignored or extended. FORTUNE magazine reported April 27, 1998, that, on average, large corporations are only 34% of the way through the job of making their systems compliant.[2]

Government agencies are doing only slightly better. The Government Accounting Office (GAO) said in March, 1998, "Time is running out for solving the Year 2000 problem. Many federal agencies will not be able to renovate and fully test all of their mission-critical systems and may face major disruptions in their operations. At the same time, systems that have been renovated and tested may encounter unanticipated Year 2000 problems."[6]

The GAO gave examples of what might go wrong:

** The nation's air transportation may face major delays and disruptions because the airlines may not be able to file flight plans with the Federal Aviation Administration.

** Taxpayers may not receive timely tax refunds because the Internal Revenue Service (IRS) may be unable to process their tax returns.

** Payments to veterans and retirees may be delayed or disrupted by the failure of mission-critical systems supporting the nation's benefit payments systems. [In other words, people may not receive their social security or disability checks in a timely fashion.]

GAO reported June 10, 1998, that 24 government agencies are only 40% of the way toward their goal of Y2K compliance.[7] GAO said it had published 40 reports on government computers during the past two years: "The common theme has been that serious vulnerabilities remain in addressing the federal government's Year 2000 readiness, and that much more action is needed to ensure that federal agencies satisfactorily mitigate Year 2000 risks to avoid debilitating consequences." GAO concluded, "As a result of federal agencies' slow progress, the public faces the risk that critical services could be severely disrupted by the Year 2000 computing crisis."

No one knows what will happen as we approach the year 2000. We do know that many manufacturing processes are dependent upon computers, especially in the chemical process industries. FORTUNE magazine said recently, "The precision and interdependence of process controls in chemical plants, for instance, make a Rube Goldberg fantasy contraption look simple. Let a single temperature sensor in the complex chain of measuring instruments go cuckoo because of a year 2000 problem, and you'll get a product with different ingredients than you need--if it comes out at all."[2]

Even the nation's defense apparatus could be adversely affected. The GAO reported June 30 that the U.S. Navy is far behind in fixing its Y2K problems and concluded, "Failure to address the year 2000 Problem in time could severely degrade or disrupt the Navy's day-to-day and, more importantly, mission-critical operations." GAO said the Navy does not even know how many of its computers have Y2K problems, so it doesn't know how big the task ahead may be.[8]

Why is this seemingly-simple problem so difficult? Merrill Lynch, the financial management firm, says there are four reasons:[9]

1. Pervasiveness. Computers that depend on dates are present in every kind of technology --manufacturing systems, medical equipment, elevators, telephone switches, satellites, and even automobiles.

2. Interdependence: Computers exchange information among themselves. "A single uncorrected system can easily spread corrupted data throughout an organization and even affect external institutions," Merrill Lynch says.

3. Inconsistency: Computer languages do not store and use dates in a consistent way. Dates are labeled, stored, and used in different ways from program to program and even within a single program. Therefore, identifying and correcting dates requires close inspection of the computer code line by line.

4. Size: Most large corporations and government agencies use thousands of programs containing millions of lines of computer code. Each line of code must be inspected manually and, if necessary, fixed.

There are additional reasons why this is a particularly difficult problem.

** Many business computer programs that run on the largest ("mainframe") computers are written in an obsolete language called COBOL. COBOL hasn't been taught for 10 years, so there is a distinct shortage of COBOL programmers.[2,10]

** Indeed, there is a shortage of all programmers to work on Y2K problems. Swiss Re (a firm that insures insurance companies against major losses) says, "A total of well over three million programmers would be needed to solve the millenium [date] problem in the US. In actual fact there are only around two million of them at present."[11]

** When computer code is re-written, new errors are introduced at an average rate of one new error in every 14 lines of re-written code. Thus even "Y2K compliant" code may not work right when the time comes.[2]

Therefore, we believe it is reasonable to conclude that portions of the nation's critical infrastructure (water, electricity, telecommunications, and transportation) may be disrupted for a period --perhaps a few days, but conceivably longer. Essential government services may also be disrupted.

We could be entirely wrong. However we believe it is sensible to hope for the best but prepare for the worst.

Individuals might take precautions to protect their families. They need water, food, shelter, and a cash reserve.[12] They need paper records of bank accounts and insurance policies, in case computerized records are lost. But even more importantly, communities need to begin now to think about ways to mitigate these problems. All is not lost. Much trouble can be averted by focused efforts now. Awareness is the first issue. (A recent survey of 643 individuals found that 38% had never heard of the Y2K problem. Among the 400 (62%) who HAD heard of it, 80% said they believed it would be fixed before the year 2000 arrived. This contrasts with an earlier poll of technology and business executives charged with fixing Y2K problems: only 17% of them said they thought the problems would be fixed before the year 2000.)[13] People need to be told.

Coordinated action is the second issue. People need the resources to fix their own computers.[1] Third, communities need to think creatively about ways to help those who are most vulnerable: people who rely on social security, veterans benefits, and private pensions, for example. What will happen if their funds are delayed? Local governments, churches, and civic groups, could begin now to bring communities together to find ways to avert serious problems that might occur. Approached properly, Y2K could become a catalyst for positive community growth and development in the best sense of those words.[14] --Peter Montague

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