Introduction

Between 1860 and 1864, August Hirsch published his monumental Handbuch der historisch-geographi- schen Pathologie in two volumes. In 1881 he finished an introduction to an updated edition, which Charles Creighton translated from German into English.

Our work represents a similar undertaking, but with a major difference. In the second half of the nineteenth century, the dawn of germ theory, it was still possible (as Hirsch proved) for an individual working alone to produce a compilation of this sort. Today even the contemplation of such an attempt boggles the mind. The Cambridge World History of Human Disease project was launched in 1985 as a collective effort of some 160 social and medical scien­tists to provide at the close of this century something of what the Hirsch volumes provided at the end of the preceding century. We hope that, like the Hirsch volumes, our own effort will aid future students of health and disease in grasping our present-day un­derstanding of diseases in their historical, spatial, and social dimensions.

Another important purpose of the project is to make available an understandable and accessible history of disease to social scientists and humanists in their many varieties. As historians, geographers, anthropologists, and other researchers have become increasingly aware of the importance of adding a biological dimension to their work, they have found the usual medical tomes, with their unfamiliar ter­minology and concepts, daunting indeed. We do not, however, ignore the needs of specialists in the many fields our work encompasses.

Parts I Through VIII

Part I of the work presents the major historical roots and branches of medical thought from ancient times to the twentieth century, and introduces the reader to the interplay of human migration, epidemiology, and immunology. Some may be interested to learn that despite popular notions about the antiquity of Chinese medicine, it actually trailed behind medi­cine in the West as a systematic discipline.

Part II deals with concepts of disease in the East and in the West, as well as with concepts of complex physical and mental ailments, the emphasis being on how those concepts have changed over time. As medicine has become more a science and less an art, it has helped to tame yesterday’s plagues, which capriciously brought sudden death so frequently to so many. As a result, many now have the question­able privilege of living long enough to develop can­cer or heart-related illnesses, which have sup­planted infectious disease and malnutrition in the developed world as the most important causes of death. Increasing life expectancy has also contrib­uted to the growth of that branch of medicine that deals with disorders of the mind and that, as Vem Bullough points out, has tended over time to appro­priate for itself the right to decide what is deviant in sexual as well as other matters.

Some chapters in Part III deal with the inheritance of disease. Certainly one can inherit genetic diseases just as one can inherit disease immunities. Some disease immunities are acquired, but even these can be viewed as a heritage of the disease environment of one’s birth. Children “inherit” what might be consid­ered special illnesses because of their age, and the heritage of human-modified environments has fre­quently been famine, illnesses of malnutrition, and illnesses triggered by occupation. In addition, the “heritage” of habits can often produce illness, as is made clear in the essays on substance abuse and tobaccosis (along with those on cirrhosis and emphy­sema in Part VIII).

Part IV is essentially demographic. It focuses on measuring the health of various groups by nutri­tional status, by morbidity, and especially by mortal­ity. An extremely important contribution of this sec­tion derives from the methodological questions that are raised.

The following three parts provide regional histo­ries of disease around the globe from prehistory to the present. Part V concentrates on Europe, the Mid­dle East, Africa, and most of the Americas, whereas Part VI is devoted to Asia. We have employed two types of historical division in these sections - Western idiosyncratic divisions in Part V and the more straightforward (and convenient) divisions of “ancient,” “premodem,” and “modem” for Asia in Part VI. Part VII completes the regional treatment by presenting a larger picture of changing disease ecologies. In addition to encapsulating the more de­tailed discussions of Europe, the Americas, Africa, and Asia that appear in Parts V and VI, this section deals with two more geographic areas —the Carib­bean and Australia/Oceania. Because of their diver­sity and relatively small populations, they were omitted from the history sections.

Collectively, the essays in Parts V through VII reveal how much more is known about the history of disease in Europe and the Americas than in the rest of the world. There are a number of reasons for this, but three stand out. One is that anthropologists and other investigators of diseases that afflicted our dis­tant ancestors have been considerably more active in the West than elsewhere. The second is that West­ern medical observers have historically been more empirically oriented than their philosophically in­clined counterparts elsewhere. The third reason is that Western observers have had greater opportuni­ties to observe a greater variety of illnesses.

Part VIII discusses the history and geography of the most notable diseases Ofhumankind in alphabeti­cal order, from AIDS through yellow fever. Most essays are divided by the subheadings definition, distribution and incidence or prevalence, epidemi­ology, etiology, clinical manifestations and pathology, and history and geography. However, because of the variable nature of illnesses, some essays are orga­nized in a way that is more suitable to the topic under discussion.

In Part VIII readers will encounter some disease entities discussed under archaic names, because they occur with some frequency in historical works. In certain cases we know what disease entity or entities they were intended to describe. The term catarrh, for example, was used in the past (and occa­sionally is still used) to label a variety of conditions that produced an inflamation of the mucous mem­branes of the head and throat. In other instances, such as chlorosis, which was proabably often ane­mia, we kept the name because it specifically signi­fies a “disease” recognized in the past as one that struck mostly young women. However, in the case of other ephemeral diseases such as sweating sickness, typhomalarial fever, and the plague of Athens, we had no choice but to use the archaic terms because to this day we can only guess what they were.

Most of these ailments are found in Hirsch under their now archaic names. He did not, of course, dis­cuss AIDS or other newly discovered, extremely deadly infections such as Ebola virus disease, Lassa fever, or Legionnaires’ disease, or illnesses such as Alzheimer’s disease that were not recognized as spe­cific clinical entities when Hirsch wrote.

Indexes, Overlap, and Illustrative Materials

By means of two detailed indexes we have attempted to make the information in this work as useful, and accessible, as possible. The larger, general index pro­vides many cross-references and historical syn­onyms for diseases. The second index lists proper names and supplies the dates and a brief biographi­cal sketch of all historical figures in medicine men­tioned by more than one author. Thus, it is possible to consult an entry on, say, the perception of disease in Asia during the eighth century (Parts I and II); to turn to another entry on the impact of smallpox and other epidemic diseases in eighth-century Japan (Part VI); to read another entry that discusses small­pox as a disease entity and provides its history; to discover something about Edward Jenner in the in­dex of names; and then, using the general index, to trace the course of smallpox over time and around the globe.

The fact that this is possible means that there is some overlap. Yet from the outset it was decided that each essay should stand on its own. Thus, some repe­tition was not only inevitable but even desirable. Indeed, given the variety of methods and approaches employed by the contributors, what might be thought to be duplication is often scrutiny of a ques­tion with different lenses. Still, much overlap has been avoided because of the different approaches.

Overview

Despite the diversity, some consensus can be gleaned from the essays, and the epidemiological overview presented here is an attempt to highlight some new findings and old answers as well as the many peren­nial questions that remain unanswered.

Hunter-gatherers and early agriculturalists of the Old World, although hardly disease free, are gener­ally held to have been free of epidemic diseases as well as of many other illnesses now regarded as “diseases of civilization.” In fact, there is some agree­ment that the cradle of many epidemic viral ail­ments, such as smallpox, was ancient South Asia, which was among the first regions to develop civiliza­tions large enough to support these ailments. From there the diseases traveled east to China and then accompanied Buddhist missionaries to Korea and Japan.

Much evidence suggests that the West was rela­tively free of eruptive fevers such as smallpox, mea­sles, and rubella until the first millennium A.D., when they finally settled in. These fevers are not mentioned in Greek texts, and as Stephen Ell points out in his study of the disease ecologies of Europe, the military fortunes of the Romans and their ene­mies do not seem to have been influenced by the kinds of diseases that decimated later European armies - or at least this influence was not felt until very late in the Empire’s decline. But at that time, the eruptive ailments had apparently taken root, as is indicated by a change in Roman strategy and by the fate of wave after wave of invaders who enjoyed initial success only to go finally into sharp decline.

It is now clear that leprosy was a serious health problem for Europeans during much of the Middle Ages. Excavations of leper cemeteries leave no doubt that those interred were suffering from that disease and not something else such as yaws, as earlier writers have argued. The reason or reasons for the disappearance of leprosy remain subject to dispute. Some researchers believe that it had to do with the rise of tuberculosis - a disease that provides some immunity to leprosy; others argue that the plague killed so many lepers that the disease itself died out.

As for the plague, a great number of the questions regarding its course in Europe remain unanswered. It is generally accepted that it originated in the Himalayan borderlands between India and China and that the plague of Justinian (542-3), which reached Europe by 547, was in fact bubonic plague. But why it disappeared from Europe for some 800 years remains a mystery. Plague certainly seems to have been active in China during this period. The circumstances of its reappearance are also obscure. Our authors note that it may have reached the West from the Middle East. But the most likely explana­tion is that a plague epidemic began in China in 1331, found its way to the Crimea by 1346, and then diffused over Europe. Yet how this disease could linger on for centuries, very possibly without a sylvatic focus and with little evidence of widespread rat mortality, has not been explained. Nor have the circumstances of the eventual disappearance of plague from western Europe in the late seventeenth and early eighteenth centuries been determined. Ex­planations ranging from a mutation of the bacillus to the rise of strong governments able to regulate trade (and, often by accident, disease) continue to be advanced.

Among scholars of diseases in the Americas, there is a noticeable tendency to accept the notion that much larger Indian populations developed in isola­tion from the rest of the world than has previously been believed. Indeed, even the now nearly empty Amazon basin was teeming with them. Like the Old World hunter-gatherers, these people were not dis­ease free. Intestinal parasites, a form of tuberculosis that may have become extinct, encephalitis, and hepatitis tormented them along with some kind or kinds of treponemal infection, and in certain locales, such uniquely American ailments as Carrion’s dis­ease and Chagas’ disease were present. But despite the fact that some populations were dense enough to host them, neither the Old World epidemic diseases, nor malaria or yellow fever, infected the native Americans. If the Indians lacked pathogens to kill them, they seem to have made up for it by violence, because burial sites often reveal that trauma was an important cause of death.

European diseases changed this state of affairs - abruptly in the case of Caribbean Indians, some­what more slowly in the Inca and Aztec empires, and substantially more slowly among other groups in Brazil and North America. But it is generally con­ceded that, despite locale, about 90 percent of the population eventually died out before demographic recovery began. Ann Ramenofsky has pinned down at least 13 diseases that arrived in the Americas with the Europeans and Africans during the first two centuries after the discovery of the American continent: viral diseases including influenza, mea­sles, mumps, rubella, smallpox, and yellow fever; bacterial ailments embracing pneumonia, scarlet fe­ver, pertussis, anthrax, and bubonic plague; typhus, whose causative microorganism stands midway be­tween a virus and bacteria; and one protozoal infection — malaria. There is general agreement that, outside of Brazil, smallpox was the most devas­tating disease, though there is puzzlement as to how a relatively benign disease suddenly became deadly in Europe as well as the Americas in the sixteenth century. But all authors concerned with the matter point out the devastating impact of disease after disease sweeping over a people and the social disloca­tion caused by the death of adults who normally provided food and care for the young and the old. Malaria is blamed for having depopulated much of the interior of Brazil, which brings us to still other Old World ailments, and to Africa.

If it is generally conceded that Asia was the cradle of many of the illnesses so far discussed (i.e., dis­eases that probably reached humankind via domesti­cated animals), there is no doubt that Africa was the cradle of another group of illnesses - those of wild animals and thus diseases that in many instances antedated the human species. These African dis­eases include some malarial types and other proto­zoal infections, such as African trypanosomiasis, which first infected our primate predecessors, as well as viral infections such as yellow fever and dengue.

Malaria, of course, has plagued India and China for millennia. But Africans and those of African descent living elsewhere around the globe are al­most completely refractive to vivax malaria. This suggests that they have had the longest experience with what is generally believed to be the most an­cient of the malarial types to affect humans. Indeed, because black Africans are so refractory to vivax malaria, the disease has disappeared from almost all of sub-Sahara Africa. By contrast, falciparum malaria, which is the most deadly of the malarial types, is also the newest; it too seems to have an African origin (or at least to have plagued Africans the longest), because Africans have by far the great­est variety and highest frequencies of genetic de­fenses against it.

Nonetheless, falciparum malaria spread out across the Sahara desert to take up residence around the Mediterranean and to become a serious threat in classical times, whereas vivax malaria had diffused much earlier over much of the globe. Vivax malaria doubtless reached the New World in the blood of early Spanish conquistadors, and falciparum malaria in the blood of the first slaves to be imported, if not before. Yellow fever, by contrast, was confined to Af­rica until the slave trade brought it to the Americas in the mid-seventeenth century. It seems to have be­gun tormenting European cities only in the eigh­teenth century, and never became established in Asia despite a plethora of suitable vectors, as well as mon­key and human hosts. One possible explanation for the latter is that Asia supports so many other group B arborviruses that there has been no room for another.

In any event, such was not the case in the Ameri­cas. Both malaria and yellow fever joined in the slaughter of Indians; in the Caribbean this African wave of disease, coming hard on the heels of the European wave, almost obliterated them. But Afri­can diseases also killed whites, while sparing blacks, who seemed immune to both. These differences in susceptibility suggested to the Europeans that nei­ther Indians nor whites could survive hard labor in warmer regions of the hemisphere. This brought about an accelerated slave trade and, of course, an accelerated flow of African pathogens to the New World. Indeed, much of tropical and subtropical Amer­ica became more an extension of the African disease environment than of the European, until late in the nineteenth century. Even smallpox arrived from Afri­can, as opposed to European, reservoirs.

Whether the Indians gave syphilis to the rest of the world is a question taken up by a number of authors in this work. The biological anthropologists report that the treponemal lesions found in the bones of pre-Columbian Indians are probably not those of syphilis (as previously thought), or at least not of syphilis as we know it today. Moreover, the ability of Indians to resist the disease, which has been taken by some as proof of its pre-Columbian presence in the Americas, can be explained to some extent by the prevalence of pinta and perhaps nonvenereal syphilis - both milder forms of treponemal disease that nonetheless would have provided some cross­immunity. Also, our authors take into account the opinion of European physicians who claimed that the syphilis they saw at the beginning of the six­teenth century was simply a more virulent form of an old disease they had always treated. In view of these circumstances it is tempting to speculate that two treponemal infections, one from the Old World and the other from the New World, somehow fused to become the disease that ravaged Europe for more than a century before becoming more benign.

Somewhere around the beginning of the eigh­teenth century, Europe’s population began to in­crease and, despite fits and starts, has continued to do so. How much of this growth can be credited to im­proved nutrition, the abatement of disease, increas­ing fertility and decreasing infant and child mortal­ity, medical intervention, the growth of nation-states and improved public health, and changes in human attitudes and behavior has long been a subject of considerable debate. Stephen Kunitz believes that all are important factors and that no single one provides a full explanation. Nonetheless, he, along with other authors considering the problem in Asia, does stress the importance of the growth of cities in which dis­eases could become endemic and thus transformed into childhood ailments.

Meanwhile, from the 1770s onward, the natives of Australia and Oceania were subjected with increas­ing ferocity to the same trial by disease that had begun almost two centuries earlier in the Americas. And according to David Stannard, the results were similar, at least in Hawaii, where he places the popu­lation decline at 90 percent and blames smallpox, as well as other epidemic illnesses and venereal dis­ease, for both increased mortality and reduced fertil­ity. Elsewhere the process was often more gradual and is unfortunately still ongoing in some places, such as Brazil and Colombia.

As the world’s cities grew in importance, so did tuberculosis, in both Asia and the West. Our authors leave no doubt about the relationship between urban­ization and tuberculosis. Yet the disease began reced­ing while urbanization was still accelerating and long before medicine had acquired its therapeutic “magic bullet.’’ This leaves still another unresolved medical mystery, although it would seem that the explanation lies somewhere within the parameters of improving nutrition and the development of resis­tance to the illness.

Crowded nineteenth-century cities with poor sani­tation and impure water supplies were natural tar­gets for another of the plagues from India - Asiatic cholera. In his essay, Reinhold Speck demonstrates the role of the British army in unwittingly unleash­ing the disease. He traces each of the pandemics, straightens out the problem of dating one of them, and shows how cholera did much to emphasize the importance of public health and sanitation programs.

The end of the 1800s brought an understanding of the role of vectors in a number of diseases, including malaria and yellow fever, and this along with an increase in the production of quinine permitted the Europeans finally to venture beyond the coasts into the interior of Africa. Tropical medicine became an integral part of colonizing efforts, although as both Maryinez Lyons and K. David Patterson make clear, the initial aim was not so much to help those colo­nized as it was to preserve the health of the coloniz­ers. Moreover, in modifying environments to suit themselves, the latter inadvertently facilitated the spread of such illnesses as African trypanosomiasis, onchocerciasis, schistosomiasis, and leishmaniasis.

The twentieth century dawned with the principles of germ theory still being digested by the world’s medical community. As our authors on Asia and Africa indicate, Western medicine has (perhaps fortu­nately) not always supplanted local medicine but rather has often coexisted with it. Nonetheless, the effectiveness of Western medicine in combating trauma and illnesses such as rabies, cholera, ty­phoid, gangrene, puerperal fever, and yaws was quickly recognized.

Yet Western medicine was completely over­whelmed as influenza became the next great plague to sweep the earth just as World War I was winding down. As Alfred Crosby emphasizes, although the case mortality rate for the disease is low, the disease killed upward of 30 million across the globe, making it perhaps the “greatest single demographic shock that the human species has ever received.” Nor did the dying cease after the pandemic was over: As R. T. Ravenholt reveals in his essay on encephalitis lethar­gica, this illness was a peculiar sequela to the so- called Spanish flu.

After the influenza epidemic, the developed world enjoyed perhaps its first extended respite from epi­demic disease since classical times. Medical science made great strides in understanding and control­ling infectious illnesses. Because of the emphasis on germ theory, however, the etiologies of nutritional ailments remained elusive for a time, but eventu­ally those of beriberi, pellagra, rickets, and scurvy were unraveled and the role of vitamins discovered. In the tropical and subtropical worlds, Rockefeller programs were launched to rid these regions of long-standing illnesses, and if hookworm disease and yellow fever were not eradicated as had been confidently expected, at least much was learned about their control.

Rickettsial diseases have always been trouble­some for armies. Typhus has been credited with de­feating Napoleon in Russia, and during World War I the disease killed some 2 million to 3 million soldiers and civilians. But in World War II and its aftermath, important advances were made in combating rickett­sial diseases. In addition, new drugs were developed against malaria.

As the authors writing on the ailments of infants and children make evident, the young have histori­cally suffered most from epidemic and endemic dis­eases. But in the past several decades this has been changed by the development of antibiotics and by accelerated worldwide programs of vaccination. In fact, as Alfred Crosby points out, in his essay on smallpox, one of these programs led by the World Health Organization appears to have ended the ca­reer of that disease, which killed so many for so long. Poliomyelitis, which briefly loomed as another of the world’s great plagues, was also brought under con­trol by similar programs, although as H. V. Wyatt reminds us, such efforts are not without some risk and the disease lingers in many parts of the world.

Unfortunately, other diseases of the young con­tinue to be prevalent, especially in the developing world, where protein-energy malnutrition, respira­tory infections, dysenteries, and helminthic and pro­tozoal parasites, separately and working in concert, kill or retard the development of millions each year. As our specialists make clear, much headway must be made in that world against other potentially crip­pling ailments such as yaws, leprosy, and ophthal­mia. Some malarial strains have become drug resis­tant, and continued dengue epidemics, carried by the same vectors that spread yellow fever, raise the very real possibility of yellow fever making a come­back in the Western Hemisphere, as it seems to be doing in parts of Africa.

In the developed world, chronic diseases such as cancer, heart-related illnesses, and Alzheimer’s dis­ease have supplanted infectious diseases as the im­portant killers, and increasingly the role of genes in the production of these diseases has come under scru­tiny. In addition, medicine has concentrated on un­derstanding the genetic basis for such diseases as cystic fibrosis, Down syndrome, epilepsy, favism, hemophilia, Huntington’s disease, leukemia, multi­ple sclerosis, muscular dystrophy, Parkinson’s dis­ease, sickle cell anemia, and Tay-Sachs disease. Some of these, such as sickle-cell anemia, favism, and possibly Tay-Sachs disease, are an unfortunate result of the body’s evolutionary attempt to protect itself against other diseases.

As this research has gone forward, it has become clear that many other disease conditions, such as lactose intolerance and diabetes, are strongly influ­enced by heredity. Leslie Sue Lieberman argues that in the latter instance a “thrifty gene” left over from the feast and famine days of our hunter-gatherer ancestors may be at work. Genes also play a vital role in selecting cancer and heart disease victims or even gout patients, although in these cases genetic predisposition can often be modified by behavioral changes - and to that extent these illnesses can be viewed as human made. No wonder, then, that there has been a recent upsurge of concern about the air we breathe and the food and other substances we take into our bodies.

It may well be that environmental factors are bringing us back face to face with epidemic illnesses. For example, as more and more individuals are enter­ing our shrinking rain forests, new and deadly vi­ruses are being released into the rest of the world. In fact, AIDS, which almost certainly originated in non­human primates, has become a plague of such propor­tions that it may eventually be ranked along with

the Black Death, smallpox, cholera, and influenza as among the most disastrous epidemic scourges of hu­mankind. In addition, as Wilbur Downs shows, other extraordinarily lethal viruses such as Ebola, Mar­burg, and Lassa also lurk in these rain forests and pose a serious threat to us all.

When Hirsch wrote in the preceding century, few ιmderstood and appreciated the extent to which hu-

7 mans have created their own disease ecologies and their own diseases by the ways they live and the ways they manipulate their environments. As we approach the twenty-first century, we have finally begun to acquire that Imderstanding. Whether at the same time we have acquired a sufficient appre­ciation of what we have done is another matter.

Kenneth F. Kiple