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The Smallpox Story: Life and Death of an Old Diseaset ABBAS M

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The Smallpox Story: Life and Death of an Old Diseaset ABBAS M MICROBIOLOGICAL REVIEWS, Dec. 1983, p. 455-509 Vol. 47. No. 4 0146-0749/83/040455-55$02.00/0 Copyright © 1983, American Society for Microbiology The Smallpox Story: Life and Death of an Old Diseaset ABBAS M. BEHBEHANI Department of Pathology and Oncology, University of Kansas School of Medicine, Kansas City, Kansas 66103 INTRODUCTION................................................................ 455 THE EARLY PERIOD ................. .......................................... 456 Antiquity of the Disease......................................................... 456 Smallpox During the Middle Ages ................................................ 458 Smallpox During the Seventeenth and Eighteenth Centuries.......................... 458 INTRODUCTION OF VARIOLATION INTO BRITAIN AND COLONIAL AMERICA ... 458 Variolation in Asia and Africa ................................................... 458 Role of the Royal Society of London .............................................. 459 Role of Lady Mary Wortley Montagu............................................. 460 Endorsement and Practice of Variolation in Britain................................. 463 Role of the Reverend Cotton Mather in Colonial America ...... ..................... 464 Variolation in Continental Europe and Colonial America ............................ 466 JENNER SUBSTITUTES VACCINATION FOR VARIOLATION ...................... 466 Edward Jenner and his Innovation .......... ..................................... 466 Jenner's Opponents and Supporters ......... ..................................... 471 Initiation of Vaccination Programs in Europe and Worldwide Acclaim of Jenner's Innovation .............................................................. 476 Eastward and Westward Odysseys of Vaccination ....... ........................... 477 Role of Benjamin Waterhouse and James Smith in the Introduction of Vaccination to the United States ............................................................ 479 SMALLPOX AFTER THE JENNERIAN INNOVATION .............................. 482 Worldwide Prevalence .......................................................... 482 The Disease ................................................................... 482 Recent Importations of Smallpox to the United States and Europe .................... 486 Laboratory-Associated Infection in London ........................................ 488 Vaccines and Method of Inoculation .............................................. 488 Discontinuation of Vaccination in the United States ................................. 489 Adverse Reactions to Vaccination .......... ...................................... 489 GLOBAL ERADICATION OF SMALLPOX ........ ................................ 490 Concept of Eradicating Smallpox................................................. 490 Preparations for Global Eradication .............................................. 490 Problems and Setbacks ............... .......................................... 492 The 10-Year Program for Global Eradication ...................................... 494 Phase I ..................................................................... 494 Phase II ..................................................................... 495 Phase III.................................................................... 496 Certification of Global Eradication .......... ..................................... 498 Use of Vaccine After the Global Eradication ....................................... 500 POXVIRUS DISEASE AFTER THE GLOBAL ERADICATION OF SMALLPOX ........ 501 Laboratory-Associated Infection at Birmingham University ...... .................... 501 Human Infections with Animal Poxviruses......................................... 503 Monkeypox virus ............. ............................................... 503 Whitepox viruses............................................................. 505 Other animal poxviruses ............. ......................................... 505 Smallpox Scares ............... ................................................ 506 Certain Unanswered Questions................................................... 507 ACKNOWLEDGMENTS ......................................................... 507 LITERATURE CITED ........................................................... 507 INTRODUCTION disfigured innumerable millions of people Smallpox, a disease of antiquity, was one of throughout the world. Variolation, the immuni- the greatest scourges of mankind. In endemic zation of susceptible individuals with material form and in waves of epidemics, it killed and taken from smallpox lesions, was practiced in T Dedicated to Ray, Allen, and Bita. who, unlike their Asia and Africa for many centuries; it was father, recollect smallpox only in connection with a childhood introduced into Britain by Lady Mary Wortley vaccination. Montagu in 1721. As variolation was not a safe 455 456 BEHBEHANI MICROBIOL. REV. protective measure, Dr. Edward Jenner intro- duced vaccination (using cowpox virus instead of smallpox virus) into Britain in 1796. As a result of widespread vaccination, smallpox de- clined steadily in Europe and North America. Elsewhere, however, death from smallpox pre- vailed. In January 1967, The World Health Orga- nization (WHO) initiated a program for the glob- al eradication of smallpox. This was achieved in October 1977 when the last person to acquire naturally occurring smallpox in the world recov- ered from this disease in Somalia, Africa. Cur- rently, there is concern about the emergence of naturally occurring animal poxviruses (e.g., monkeypox virus, which also infects humans) as possible widespread agents of human poxvirus disease. I became well familiar with the ravages of FIG. 1. Pharaoh Rames V (died 1157 B.C.) (courte- smallpox during my childhood in Persia and sy of the WHO, Geneva, Switzerland). have ever since been anxiously following the progress of global efforts to eliminate this dis- ease from the entire world. Now that smallpox is thousands of years; evidence of the preventive dead, it is therefore with a great sense of relief measure of variolation is found in the Sanskrit and muchjoy that I recount the fascinating story text "Sacteya," attributed to Dhanwantari. A of the long fight with this dreadful scourge. special god, Kakurani, was recognized for small- pox in the Brahmin mythologies. In China, THE EARLY PERIOD smallpox was known since 1122 B.c. during the Chou (Tcheou) dynasty (1122 to 255 B.c.), and Antiquity of the Disease the nasal route of variolation was practiced Descriptions of smallpox appear in the earliest during the Sung dynasty (960 to 1280 A.D.) (29). Egyptian, Indian, and Chinese writings. The Thucydides, the Athenian chronicler, described mummy of Pharaoh Ramses V (Fig. 1) (discov- an outbreak of a disease with certain similarity ered in 1898 and currently at the Cairo Museum, to smallpox which occurred in Athens around Egypt), who reportedly died of an acute illness 430 B.C. He further stated that the plague started at the age of 40 years in 1157 B.C., shows a in Ethiopia, spread to Egypt and Libya, and striking rash of yellowish blisters or pustules later was introduced into Athens by a ship which closely resembles that of smallpox. The coming from North Africa and docking at Pirae- pustules measure 1 to 5 mm in diameter and are us (the port of Athens) (95, 101). However, found on the lower face, neck, shoulders, arms, medical historians have speculated endlessly and the lower part of the abdomen and scrotum. about the nature of the aforementioned plague. No rash is manifest on the chest and upper part It is striking that smallpox is mentioned neither of the abdomen. The palms and soles have not in the Old and New Testaments nor in the been examined. Donald R. Hopkins, Assistant classical Greek (including the Hippocratic writ- Director for International Health at the Centers ings) and Roman literatures. The disease de- for Disease Control, Atlanta, Ga., was allowed scribed by Eusebius as occurring in 302 A.D. in on 8 November 1979 by special permission of Syria appears most likely to be smallpox.. Mari- the late President Mohammad Anwar Al-Sadat us, the Bishop of Avenches, first used the word to collect and examine specimens from this variola in his "Chronicle" of 570 A.D., but it is mummy to scientifically determine whether Pha- not certain that he was describing smallpox raoh Ramses V had died of smallpox. Electron since no clinical description was given. Howev- microscopic and other immunological and viro- er, it is possible that the disease described by logical studies of tiny pieces of skin on the Gregory of Tours in 580 A.D., which fits the shroud, which appeared to be normal, interven- description of smallpox well, was the same as ing skin rather than directly affected skin, did that described by Marius 10 years earlier (29). not reveal evidence ofpoxvirus. However, since obtaining a piece of the mummy's skin contain- Smallpox During the Middle Ages ing one or more of the visible lesions was not The Persian physician Rhazes (Fig. 2), who in permitted, the negative results do not preclude 910 A.D. wrote the first differential and graphic death by smallpox (53). description of symptoms of smallpox and mea- The disease was present in India for many sles in his classic monograph A Treatise on VOL. 47, 1983 SMALLPOX 457 .--, o -, POr ,,,:o- ,--- 000 .Ik- - ".o I',' . 11 v p -,,. T 14 FIG. 2. Rhazes (Abu Bakr Mohammad Ibn Zakariya Al-Razi) (865 to 925 A.D.) (courtesy of Parke, Davis and Co., Detroit
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