Book-43278.Pdf

HOW THE COWS TURNED MAD MAXIME SCHWARTZ HOW THE COWS TURNED MAD Translated by Edward Schneider UNIVERSITY OF CALIFORNIA PRESS Berkeley Los Angeles London The publisher gratefully acknowledges the generous contribution to this book provided by the French Ministry of Culture. Comment les vaches sont devenues folles, by Maxime Schwartz, is published in English translation by arrangement with Éditions Odile Jacob, © Éditions Odile Jacob, Mars 2001. © University of California Press Berkeley and Los Angeles, California University of California Press, Ltd. London, England © 2003 The Regents of the University of California Library of Congress Cataloging-in-Publication Data Schwartz, Maxime, 1940–. [Comment les vaches sont devenues folles. English] How the cows turned mad / Maxime Schwartz; translated by Edward Schneider. p. cm. Includes bibliographical references and index. ISBN 0-520-90087-1 1. Prion diseases—History. I. Title. EA644 .P93.53913 2003 616.8'3—dc21 2002075514 CIP Manufactured in the United States of America 10 09 08 07 06 05 04 03 10 987654 321 The paper used in this publication meets the minimum requirements of ANSI/NISO. Z39.48-1992(R 1997) (Permanence of Paper). ' CONTENTS Prologue 1 1: THE SHEEP ARE STRANGELY DIZZY 4 2: MOLECULES AND MICROBES 13 3: MAD DOGS AND EARTHWORMS 18 4: SCRAPIE UNDER THE MICROSCOPE 24 5: CREUTZFELDT, JAKOB, AND OTHERS 31 6: SCRAPIE IS INOCULABLE 38 7: AND GOATS, AND MICE 45 CONTENTS VI 8: SCRAPIE IS CONTAGIOUS 51 9: KURU AND THE FORE PEOPLE OF PAPUA NEW GUINEA 58 10: THE WALL COMES DOWN 67 11: FROM PEARL NECKLACE TO DOUBLE HELIX 73 12: THE PHANTOM VIRUS 81 13: A TRAGEDY IN THE MAKING 87 14: ONE CASE PER MILLION 93 15: PRIONS 98 16: APRIL 1985 104 17: THE “KISS OF DEATH” 110 18: THE RETURN OF THE SPONTANEISTS 119 CONTENTS VII 19: TO GROW—AND TO DIE 128 20: LESSONS LEARNED 136 21: HAVE THE COWS GONE MAD? 142 22: FROM COWS TO HUMANS 153 23: FROM COWS TO SHEEP? FROM HUMANS TO HUMANS? 163 24: THE SECRET IN THE CLOSET 169 25: UNMASKING “THE DISEASE” 176 26: HAVE WE CONQUERED “THE DISEASE”? 180 27: 2001 184 Epilogue 199 Notes 205 CONTENTS VIII Bibliography 215 Chronology 217 Acknowledgments 225 Index 227 PROLOGUE Thus, we see infection in a new light which cannot fail to be a cause of concern for humankind—unless, in the course of its evolution over the centuries, nature has already come upon every opportunity to produce infectious or contagious diseases, which is highly unlikely. Louis Pasteur, 1881 There shall be new diseases. That is an inevitable fact. Another fact, no less inevitable, is that we will never be able to detect them from the outset. By the time we have some idea of those diseases, they will already be fully formed—in their adulthood, so to speak. They will appear like Athena, springing fully armed from the forehead of Zeus. Charles Nicolle, director of the Institut Pasteur of Tunis and winner of the 1928 Nobel Prize for medicine, 1933 UNKNOWN TO THE public at large until recently, Creutzfeldt-Jakob disease (CJD) is now the subject of daily media attention, especially in western Europe. What exactly is this disease that seems to pose a threat to us all? What is the meaning of terms we hear, such as “sporadic,” “in- herited,” “iatrogenic,” and “new variant”? What is CJD’s relationship to “mad cow disease”—bovine spongiform encephalopathy (BSE)— and to the sheep disease known as scrapie? If it can be transmitted from cattle to humans, why not from sheep to humans? As an infectious disease, can it be transmitted from human to human? What is the 1 PROLOGUE 2 causative agent? It is said to be neither a bacterium nor a virus—so what is it? Can it be detected in infected animals or humans? Can it be eliminated with the right drugs? Is there a vaccine? Why have the cows gone mad? If it is due to their animal-based feed, as some assert, can government prohibitions put an end to the epidemic? And in the mean- time, what should we do to protect ourselves? Can we eat beef? Can we drink milk? How many human victims will there be? Dozens? Or hundreds of thousands? Many of us have asked these questions and more. At the moment, unfortunately, science has only partial answers, and the lack of scientific certainty only fuels anxieties—and wild imaginings. In France, the “mad cow crisis” grew to incredible proportions toward the end of 2000. It was without doubt a defining event of the final months of the twentieth century. A number of reasons have been suggested for this. Setting aside the isolated incidents that have garnered widespread publicity, there were indeed objective reasons for concern: predictions by epidemiologists that there could ultimately be more than a hundred thousand victims in the United Kingdom alone, the growing number of reported cases of BSE in French cattle, and a scientific finding that suggested the disease could be transmitted through blood transfusions. What was broadly viewed as a genuine panic spread throughout Europe with the late November appearance of BSE cases in Germany and Spain, countries previously thought to be untouched by the disease. The collapse of confidence in products of bovine origin spread to all agricultural products. People no longer knew what they could safely eat—and the economic and political consequences are well known. The confusion was exacerbated by a parallel crisis: the transmission of Creutzfeldt-Jakob disease to children who had been treated with human growth hormone. That tragedy surfaced in 1985, when the first cases were identified in the United States. And, sadly, it has continued, especially in France, where new cases come to light each year. The pub- PROLOGUE 3 lic is asking how physician-administered treatments to make these children grow could infect them with a lethal disease. Creutzfeldt-Jakob disease is frightening because it is always fatal, because it involves the central nervous system and hence the conscious- ness—the very personality—of its victims, and because we all share a vague fear of contracting it at some point from one source or another. Lest we fall prey to obsessive fear, we must rationally assess the often alarmist information that comes our way. Thus, we need to know more about this mysterious illness—“The Disease”—and must try to under- stand where it comes from and how it spreads. When we delve into the origins of this worrisome illness and into the growth of knowledge about it, we find a sort of detective story with its roots in the distant past. It was already lurking in the eighteenth century. First identified among English sheep, The Disease killed all it touched. It was like a criminal, donning ever-changing disguises to elude its pursuers: We have been on its trail for three centuries. That trail begins in Britain, and continues in many other countries such as France, Germany, the United States, Switzerland, Austria, Israel, Aus- tralia, and two islands at opposite ends of the Earth—Iceland and New Guinea. Our hunt has made use of the latest scientific advances as they emerged, but its route has also led us through Stone Age civilizations. Many times, almost as if it sensed that it was about to be found out, The Disease has counterattacked, claiming many victims and spread- ing fear. Have we finally tracked it down? Many people think so, but others are doubtful. We shall see. 1 THE SHEEP ARE STRANGELY DIZZY THE EIGHTEENTH CENTURY, which was to conclude with the American and French Revolutions, was also the Age of Enlightenment. The convic- tion was growing that scientific progress was intended to enable humans to control the world around us: Had we not learned to control thunderbolts, thanks to the lightning rod? This was the era of Lin- naeus, Buffon, and Diderot, and we began cataloguing nature’s riches and seeking to employ them in a more rational way. This approach was seen particularly in the areas of agriculture and animal husbandry. Improved productivity was the order of the day. Landowners organized and agricultural societies and academies were founded, where questions of farming were discussed and where news and information were exchanged and documents published. In Eng- land, the enclosure policy was broadly implemented, evicting small- scale farmers to the benefit of big landowners and providing the latter with the resources for long-term investment. In the sphere of animal husbandry, major efforts were made to improve feed and conditions, and to select the most productive breeds. Sheep farming was the first beneficiary of this modernization because wool production was a major industry not only in England but 4 THE SHEEP ARE STRANGELY DIZZY 5 throughout Europe. It is estimated that a quarter of the English popu- lation was involved in wool production or the wool trade in one way or another. And that sector was to remain important. Toward the end of the nineteenth century, an eminent French veterinarian, while noting that sheep farming was on the rise for purposes of meat production, wrote this: Wool is among the pillars of the well-being of modern-day societies. We may thus venture to say that the people who produce the most wool will be the richest and perhaps the most powerful. For more than fifty years, wool production has rained showers of gold upon Europe: For Germany and Russia, it has provided hitherto unknown material well-being and the hope of future prosperity. As for England, is it not its countless merinos that must be deemed ac- countable for the wealth of its colonies and the magnificence of its trade beyond compare?1 Given the care lavished upon these wool-bearing creatures, is it any surprise that the diseases that could affect them were also the object of attention? They had to be catalogued in the hope of being able to con- quer them.

Load more