Smallpox

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Smallpox
Classifications and  external resources

A child infected with smallpox
ICD-10	B03.
ICD-9	050
DiseasesDB	12219
MedlinePlus	001356
eMedicine	emerg/885
MeSH	D012899

iVariola virus (Smallpox)
Virus classification
Group: Group I (dsDNA) Family: Poxviridae Genus: Orthopoxvirus Species: Variola vera

Smallpox (also known by the Latin names Variola or  Variola vera) was a highly contagious viral disease unique to humans. it  is caused by either of two virus variants named Variola major and  Variola minor. V. major, the deadlier form, has a mortality rate of 20–40 percent, while V. minor kills 1% of its victims. Other long-term effects usually include skin scars and  occasionally include blindness due to corneal ulcerations.

After successful vaccination campaigns throughout the 19th and  20th centuries, the World Health Organization (WHO)certified the eradication of smallpox in 1979.^[1]

Smallpox was responsible for an  estimated 300–500 million deaths in the 20th century. As recently as 1967, the World Health Organization (WHO) estimated that 15 million people contracted the disease and  that two million died in that year.^[2]

Contents 1 Vaccine 2 Infection 2.1 Eurasia 2.2 The Americas 2.3 Inoculation 2.4 Vaccination 2.5 Eradication 2.6 Post-eradication 3 Famous Victims 4 See also 5 References 6 External links

[edit] Vaccine

Main article: Smallpox vaccine

Smallpox is  described in the Ayurveda books. Smallpox was ameliorated by inoculation with year-old smallpox matter. The inoculators would travel all across India pricking the skin of the arm with a small metal instrument using "variolous matter" taken from pustules produced by the previous year's inoculations. The effectiveness of this system was confirmed by the British doctor J.Z. Holwell in an  account to the College of Physicians in London in 1767.The definition for Smallpox is  a highly contagious and  infectious disease that is  often deadly.

Edward Jenner developed the smallpox vaccine using cowpox fluid (hence the name vaccination is  derived from the Latin vacca, cow); his first vaccination occurred on May 14, 1796. However, the first person recorded as vaccinating against smallpox was Benjamin Jesty, a farmer. Jesty noted the common knowledge that dairymaids who had previously contracted the less  severe cowpox never suffered with smallpox, even after nursing ill family members. During the smallpox outbreak in the summer of 1774, Jesty inoculated his wife and  her children. Jesty's wife became ill but recovered with treatment.

Jenner established the technique and  practice of vaccination.

The practice of vaccination against smallpox spread quickly in Europe and  then to America. Prior to Jenner's cowpox vaccine, many New England settlers were inoculated by means of making small incisions in the arms of the healthy patient, scraping a pustule from a smallpox victim, and  spreading the pus in the arm incisions. The inoculated patients were often sick for weeks but generally recovered. Prior to 1776, Abigail Adams from Massachusetts wrote to her husband John Adams (then in Congress in Philadelphia) that she was taking their children to Boston to be inoculated, after a severe outbreak near their rural home had killed entire neighboring families. The first smallpox vaccination in North America occurred on June 2, 1800. National laws requiring vaccination began appearing as early as 1805.

The last case of wild smallpox occurred on October 26, 1977. One last victim was claimed by the disease in the UK in September 1978, when Janet Parker, a photographer in the University of Birmingham Medical School, contracted the disease and  died. A research project on smallpox was being conducted in the building at the time, though the exact route by which Ms. Parker became infected was never fully elucidated.

Note: A recent study by the Center of Disease Control in Atlanta (CDC) have  found that every 1 in a million people are  carriers of smallpox and  could quite possibly infect many others with this horrifying disease^{[citation needed]}.

Although this disease is  believed to be eradicated, it  is still considered a potential threat, especially to children, most of whom have  not had a vaccination shot.

cultures of the virus are  kept by the  and  Prevention">Centers for Disease Control and  Prevention (CDC) in the United States and  at the Institute of Virus Preparations in Siberia, Russia. (While scientists certified it  eradicated in December 1979, WHO formally ratified this on 8 May 1980 in resolution WHA33.3) Smallpox vaccinations were discontinued in most countries in the 1970s as the morbidity and  mortality of vaccination by then exceeded the risk of infection by a disease extinct in the wild. Nonetheless, after the 2001 anthrax attacks took place in the United States, concerns about smallpox have  resurfaced as a possible agent for bioterrorism. As a result, there has been increased concern about the availability of vaccine stocks. Moreover, President George W. Bush has ordered all American military personnel to be vaccinated against smallpox and  has implemented a voluntary program for vaccinating emergency medical personnel.

[edit] Infection

The disease is  only moderately infectious, far less  so than chickenpox. Unlike chickenpox, smallpox is  not notably infectious in the prodromal period—viral shedding being delayed until the appearance of the rash. Smallpox transmission is  a risk of prolonged social contact, direct contact with infected body fluids or  contaminated objects. Infection in the natural disease will be via the lungs. The incubation period to obvious disease is  around 12 days. In the initial growth phase the virus seems to move from cell to cell, but around the 12th day, lysis of many infected cells occurs and  the virus will be found in the bloodstream in large numbers. The initial or  prodromal symptoms are  essentially similar to other viral diseases such as influenza and  the common cold—fevers, muscle pain, stomach aches, etc. The digestive tract is  commonly involved, leading to vomiting. Most cases will be prostrated.

Smallpox virus preferentially attacks skin cells and  by days 12–15, smallpox infection becomes obvious. The attack on skin cells causes the characteristic pimples associated with the disease. The pimples tend to erupt first in the mouth, then the arms and  the hands, and  later the rest of the body. At that point the pimples, called macules, should still be fairly small. This is  the stage at which the victim is  most contagious.

For the general layman in the field, to distinguish between Chickenpox and  Smallpox, check the victims palms of the hand and  soles of the feet. Chickenpox does not generally and  usually present on the palms and  soles as does smallpox.

By days 15–16 the condition worsens—at this point the disease can take two vastly different courses. The first form is  of classical ordinary smallpox, in which the pimples grow into vesicles, and  then fill up with pus (turning them into pustules). Ordinary smallpox generally takes one of two basic courses. In discrete ordinary smallpox, the pustules stand out on the skin separately—there is  a greater chance of surviving this form. In confluent ordinary smallpox, the blisters merge together into sheets which begin to detach the outer layers of skin from the underlying flesh—this form is  usually fatal. If the patient survives for the course of the disease, the pustules will deflate in time (the duration is  variable), and  will start to dry up, usually beginning on day 28. Eventually the pustules will completely dry and  start to flake off. Once all of the pustules flake off, the patient is  considered cured.

In the other form of Variola major smallpox, known as hemorrhagic smallpox, a mortality of 96 percent has been reported.^[3] an  entirely different set of symptoms starts to develop. The skin does not blister, but remains smooth. Instead, bleeding will occur under the skin, making the skin look charred and  black (this is  known as black pox). The eyes will also hemorrhage, making the whites of the eyes turn deep red (and, if the victim lives long enough, eventually black). At the same time, bleeding begins in the organs. Death may occur from bleeding (fatal loss of blood or  by other causes such as brain hemorrhage), or  from loss of fluid. The entry of other infectious organisms, since the skin and  intestine are  no longer a barrier, can also lead to multi-organ failure. This form of smallpox occurs in anywhere from 3–25% of fatal cases (depending on the virulence of the smallpox strain).

The historical modes of death are  similar to those in burns, with catastrophic losses of fluid, protein and  electrolytes beyond the capacity of the body to replace or  assimilate, and  fulminating sepsis, both due to the removal of the barrier between the internal milieu and  outside world. Supportive treatments have  improved since the last large smallpox epidemics, but it  would be grossly optimistic to imagine that even with a small number of patients that the most intensive modern treatment would ensure survival even where the damage is  predominantly only in the skin. A reduction in the severity of the disease by raising immunity is  likely to make a large difference in numbers reaching the threshold of death, and  supportive treatment a small one in elevating that threshold.

[edit] Eurasia

Historical epidemics and  pandemics are  believed by some historians to have  been early outbreaks of smallpox. But contemporary records are  not detailed enough to make a definite diagnosis at this distance.

The Plague of Athens devastated the city of Athens in 430 BC, killing around a third of the population, according to Thucydides. Historians have  long considered this an  example of the disease plague, but more  recent examination of the reported symptoms led some scholars to believe the cause could have  been measles, smallpox, typhus, or  a viral hemorrhagic fever (like Ebola).

The Antonine Plague that swept through the Roman Empire and  Italy in 165–180 is  also thought to be either smallpox or  measles. A second major outbreak of disease in the Empire, known as the Plague of Cyprian (251–266), was also either smallpox or  measles.

The next major epidemic believed to be smallpox occurred in India. The exact date is  unknown. Around 400, an  Indian medical book recorded a disease marked by pustules, saying "the pustules are  red, yellow, and  white and  they are  accompanied by burning pain … the skin seems studded with grains of rice." The Indian epidemic was thought to be punishment from a god, and  the survivors created a goddess, Sitala, as the anthropomorphic personification of the disease.^[4][5][6]. Smallpox was thus regarded as possession by Sitala. In Hinduism the goddess Sitala both causes and  cures high fever, rashes, hot flashes and  pustules. All of these are  symptoms of smallpox.

Smallpox did not enter Europe until about 581. Most of the details about the epidemic that followed are  lost, probably due to the scarcity of surviving written records of early medieval society.

[edit] The Americas

After first contacts with Europeans and  Africans, the death of a large part of the native population of the New World was caused by Old World diseases. Smallpox was the chief culprit.

In 1519 Hernán Cortés landed on the shores of what is  now Mexico and  was then the Aztec empire. In 1520 another group of Spanish came from Cuba and  landed in Mexico. Among them was an  African slave who had smallpox. When Cortés heard about the other group, he went and  defeated them. In this contact, one of Cortés’ men contracted the disease. When Cortés returned to Tenochtitlan, he brought the disease with him.

Soon, the Aztecs rose up in rebellion against Cortés. Outnumbered, the Spanish were forced to flee. In the fighting, the Spanish soldier carrying smallpox died. After the battle, the Aztecs contracted the virus from the invaders’ bodies. Cortes would not return to the capital until August 1521. In the meantime smallpox devastated the Aztec population. it  killed most of the Aztec army, the emperor, and  25% of the overall population. A Spanish priest left this description: “As the Indians did not know the remedy of the disease…they died in heaps, like bedbugs. In many places it  happened that everyone in a house died and, as it  was impossible to bury the great number of dead, they pulled down the houses over them so that their homes become their tombs.” On Cortés’ return, he found the Aztec army’s chain of command in ruins. The soldiers who lived were still weak from the disease. Cortés then easily defeated the Aztecs and  entered Tenochtitlán, where he found that smallpox had killed more  Aztecs than had the cannons. The Spaniards said that they could not walk through the streets without stepping on the bodies of smallpox victims.

The effects of smallpox on Tahuantinsuyu (or the Inca empire) were even more  devastating. Beginning in Colombia, smallpox spread rapidly before the Spanish invaders first arrived in the empire. The spread was probably aided by the efficient Inca road system. Within months, the disease had killed the Sapa Inca Huayna Capac, his successor, and  most of the other leaders. Two of his surviving sons warred for power and, after a bloody and  costly war, Atahualpa become the new Sapa Inca. As Atahualpa was returning to the capital Cuzco, Francisco Pizarro arrived and  through a series of deceits captured the young leader and  his best general. Within a few years smallpox claimed between 60% and  90% of the Inca population, with other waves of European disease weakening them further. However, some historians think a serious native disease called Bartonellosis may have  been responsible for some outbreaks of illness.

Even after the two mighty empires of the Americas were defeated by the virus, smallpox continued its march of death. In 1633 in Plymouth, Massachusetts, the Native Americans were struck by the virus. As it  had done elsewhere, the virus wiped out entire population groups of Native Americans. it  reached Lake Ontario in 1636, and  the lands of the Iroquois by 1679, killing millions. The worst sequence of smallpox attacks took place in Boston, Massachusetts. From 1636 to 1698, Boston endured six epidemics. In 1721, the most severe epidemic occurred. The entire population fled the city, bringing the virus to the rest of the Thirteen Colonies. In the late 1770s, during the American Revolutionary War, smallpox returned once more  and killed an  estimated 125,000 people.^[7]

[edit] Inoculation

By that time, a preventive treatment for smallpox had finally arrived. it  was a process called inoculation, also known as insufflation or  variolation. Inoculation was not a sudden innovation, as it  is known to have  been practiced in India as early as 1000 B.C.^[8] The Indians rubbed pus into the skin lesions. The Chinese blew powdered smallpox scabs up the noses of the healthy after discovery that this inoculated non-immune people by a Buddhist nun. The patients would then develop a mild case of the disease and  from then on were immune to it. This technique is  known as variolation and  although variolation had a 0.5-2% mortality rate, this was considerably less  than the 20-30% mortality rate of the disease itself. The process spread to Turkey where Lady Mary Wortley Montagu, wife of the British ambassador, learned of it  from Emmanuel Timoni (ca. 1670–1718), a doctor affiliated with the British Embassy in Istanbul. She had the procedure performed on her son and  daughter, aged 5 and  4 respectively. They both recovered quickly and  the procedure was hailed as a success and  reported to the Royal Society in England. Timoni, from the University of Padova, Italy and  a member of the Royal Society of London since 1703, published “an account, or  history, of the procuring the smallpox by incision” in December 1713 in the Philosophical Transactions. His work was published again  in 1714 in Leipzig and  was followed by those of Pylarino (1715), Leduc (1722), and  Maitland (1722).

In 1721, an  epidemic of smallpox hit London and  left the British Royal Family in fear. When they read about the success of Lady Wortley Montagu’s efforts, they wanted to use inoculation on themselves. Doctors told them that it  was a dangerous procedure, so they decided to try it  on other people first. The subjects they used were condemned prisoners. The doctors inoculated the prisoners and  all of them recovered in a couple of weeks. So assured, the British royal family inoculated themselves and  reassured the English people that it  was safe.

But inoculation still had its critics. Prominent among them were religious preachers who claimed that smallpox was God’s way of punishing people and  that inoculation was a tool of Satan^{[citation needed]}. This resistance only encouraged Montagu and  the others to work even harder. By 1723 inoculations were extremely common in England, but even scientific opposition (such as the Fellow of the Royal College of Physicians Pierce Dod) continued for some time.

In 1721, Onesimus (Oh-NES-ih-mus) was the slave of a Boston preacher when smallpox came to Boston via a ship arriving from Barbados.<^[9] His owner, Cotton Mather asked his slave if he ever had smallpox. Onesimus said, “Yes and  no,” and  explained a technique from his homeland in Africa, thought to be in Sudan. He explained that pus from an  infected person was deliberately rubbed into a scratch or  cut of a non-infected person, and  when successful, the person had immunity. This remedy from an  African slave was the precursor to inoculations. Cotton Mather, the son of a former Harvard University dean, was waging a campaign of his own to promote the process, although religious resistance to inoculation was very strong. At one point, Cotton Mather was in danger from a crowd that wanted to hang him. After six patients died from the procedure, he was called a murderer. But, when the population of Boston returned after the end of a smallpox epidemic in 1722, he was an  instant hero. Out of the population of Boston, 7% had died from smallpox. Out of the 300 people that chose to inoculate themselves, only 2% died. In 1750, the English magazine, Gentleman's Magazine, reprinted a 1725 pamphlet that argued in support of smallpox inoculations. By 1774, it  was considered odd not to choose inoculation. Onesimus was later freed by Mather, not for his knowledge and  help in combating smallpox, but because Mather considered him to be disobedient.

Even though inoculation was a powerful method of controlling smallpox, it  was far from perfect. Inoculation caused a mild case of smallpox which resulted in death in about 2% of the cases. it  was also difficult to administer. Sick patients had to be locked away to prevent them from transmitting the disease to others. Thus George Washington initially hesitated to have  his Revolutionary War troops inoculated during a smallpox outbreak in February 1777, writing, “should We inoculate generally, the Enemy, knowing it, will certainly take Advantage of our Situation;” but the virulence of the outbreak soon prompted him to order inocculation for all troops and  recruits who had not had the disease.^[10]

In 1757, a young boy in England by the name of Edward Jenner was inoculated. He suffered from the disease for an  entire month. Even though he recovered, he was determined to find a better method of preventing smallpox.

[edit] Vaccination

At the age of thirteen, Jenner was apprenticed to Dr. Ludlow in Sodbury. He observed that people who caught cowpox while working with cows were known not to catch smallpox. He assumed a causal connection. The idea was not taken up by Dr. Ludlow at that time. After Jenner returned from medical school in London, a smallpox epidemic struck his home town of Berkeley, England. He advised the local cow workers to be inoculated. The farmers told him that cowpox prevented smallpox. This confirmed his childhood suspicion, and  he studied cowpox further, presenting a paper on it  to his local medical society.

Perhaps there was already an  informal public understanding of some connection between disease resistance and  working with cows. The “beautiful milkmaid” seems to have  been a frequent image in the art and  literature of this period.

In 1796 Sarah Nelmes, a local milkmaid,, contracted cowpox and  went to Jenner for treatment. Jenner took the opportunity to test his theory. He inoculated James Phipps, the eight-year-old son of his gardener, not with smallpox but with cowpox. After an  extremely weak bout of cowpox, James recovered. Jenner then tried to infect James with smallpox but nothing happened—the boy was immune to smallpox.

Jenner reported his observations to the Royal Society. Further work was suggested, and  Jenner published a series of 23 cases, including his son Edward, none suffered severely from smallpox. Two years later a society to oppose vaccination had been established in Boston, Massachusetts^{[citation needed]} — an  indication of rapid spread and  deep interest. By 1800 Jenner’s work had been published in all of the major European languages. The process was performed all over Europe and  the United States. The death rate was close to zero with the process, which became known as vaccination and  was continued to around 1974 in the UK. A typical death rate at that time was roughly one per million, making vaccination against smallpox with vaccinia the most dangerous immunisation widely provided in modern times.

The Balmis Expedition (1803) carried the vaccine to Spanish America, the Philippines and  China under commission of the Spanish Crown.

Some years before Dr. Jenner, Benjamin Jesty, a farmer at Yetminster in Dorset (he later moved to and  is buried at Worth Matravers) is  recorded as observing the two milkmaids living with his family to have  been immune to smallpox and  then inoculating his family with cowpox to protect them from smallpox. This has never been adequately verified, however, and  the question of who first initiated smallpox inoculation/vaccination has not been settled to this day.

Louis T. Wright^[11], an  African-American and  Harvard medical school graduate (1915), introduced intradermal vaccination for smallpox for the soldiers while serving in the Army during WWI.^[12]

[edit] Eradication

Jenner said, “The annihilation of smallpox—the dreadful scourge of the human race—will be the final result of vaccination.” Jenner’s dream was ultimately realized. Around the world, attempts would be made to annihilate smallpox. In 1842, England banned inoculation, later progressing to mandatory vaccination instead. In the United States, from 1843 to 1855 first Massachusetts, and  then other states required vaccination. This alteration in the relationship of State to citizen was not universally approved.^[13] Protests notwithstanding, coordinated efforts against smallpox went on and  the disease continued to diminish in the wealthy countries. In poorer countries, vaccines and  the necessary infrastructure were less  affordable and  available.

In 1958 the Soviet Union called for the eradication of smallpox from the planet. At that point, 2 million people were dying every year. In 1967, an  international team was formed under the leadership of an  American, Donald Henderson. To eradicate smallpox, each outbreak had to be stopped from spreading, by isolation of cases and  vaccination of everyone who lived close by. This process is  known as ring vaccination. The initial problem the WHO team faced was inadequate reporting arrangements, as many smallpox cases were not notified to the authorities.

Before WHO could begin to eradicate smallpox it  was necessary to establish that smallpox carriers did not exist, and  that smallpox did not exist in the wild, as diseases such as yellow fever did. The fact that there has been no case of smallpox since 1977 has confirmed these two facts as amongst the most certain in medical science.

A network of experts was established around the world. When the disease emerged, assistance was offered to local governments to vaccinate everyone in the area.

Eradicating smallpox required huge effort and  concentration. In India and  Bangladesh, religion and  round-the-clock civil wars became obstacles. In fear of offending the goddess associated with the disease, many Hindus refused the vaccine. Civil war was the bigger problem. Troop movements and  crowded encampments have  long been associated with spread of disease, including smallpox.

The team’s answer to civil war was to vaccinate all the troops, with or  without the permission of generals commanding the armies. The team placed themselves in great danger by doing this, and  the WHO even told them to stop. Surprisingly, none of the team members were hurt in the process.

Natural events also impeded the vaccination team’s efforts. The monsoon rains burst dams and  dikes. The rain and  flooding forced people to flee, once again  allowing smallpox to spread. This outbreak took the team a whole year to stop.

The last major European outbreak of smallpox was the 1972 outbreak of smallpox in Yugoslavia. After a pilgrim returned from the Middle East, where he had contracted the virus, an  epidemic infected 175 people, causing 35 deaths. Authorities declared martial law, enforced quarantine and  undertook massive revaccination of the population, enlisting the help of the WHO and  Donald Henderson. In two months, the outbreak was over. Prior to this, there has been a smallpox outbreak on May-July of 1963 in Stockholm, Sweden, brought from the far east by a Swedish sailor; This has been dealt with by quarantine measures and  vaccination of local population ^[14].

The last naturally occurring case of Variola Minor was diagnosed in Somalia on a cook named Ali Maow Maalin on the date of October 26, 1977. The last naturally occurring case of the more  deadly Variola Major had been detected in October 1975 in a two-year-old Bangladesh girl, Rahima Banu. In the end, 300 million United States dollars had been applied to the eradication process.

[edit] Post-eradication

In 1978, there was evidently an  escape of smallpox from containment in a research laboratory in Birmingham, England. A medical photographer, Janet Parker, died from the disease itself, and  the Professor responsible for the unit, Professor Henry Bedson, killed himself. In light of this accident, all known stocks of smallpox were destroyed, except the stocks at the United States Center for Disease Control (CDC) and  the Russian  and  Biotechnology">State Research Center of Virology and  Biotechnology (also known as the Vector Institute) in Siberia, where a regiment of troops guards it. Under such tight control, smallpox would, it  was thought, never be let out again. Even though the destruction of virus stocks was ordered in 1993, 1994, 1995, and  1996^{[citation needed]}, they have  not yet been destroyed, since a number of researchers still wish to retain the stocks for scientific purposes.

It is  also feared that additional stocks of the virus may exist in research collections, the product of the accumulatory nature of microbiologists. Additional collections of the virus almost certainly exist as the result of certain military and  biological warfare programs, such as those undertaken at the Vector Institute, which maintained stocks separate from those held by the Moscow Institute for Viral Preparation.

In March 2003 smallpox scabs were found tucked inside an  envelope in a book on Civil War medicine in Santa Fe, New Mexico.^[15] The envelope was labeled as containing the scabs and  listed the names of the patients that were vaccinated with them. Assuming the contents could be dangerous, the librarian who found them did not open the envelope. The scabs ended up with employees from the National Centers for Disease Control, who responded quickly once informed of the discovery. The discovery raised concerns that smallpox DNA could be extracted from these and  other scabs and  used for a biological attack. Even with Variola sequenced, assembling a virus from scratch remains challenging.

[edit] Famous Victims

Famous victims of this disease include Ramesses V^[16], the Shunzhi Emperor and  Tongzhi Emperor of China (official history), Mary II of England, Louis XV of France and  Peter II of Russia. Henry VIII's fourth wife, Anne of Cleves, survived the disease but was scarred by it, as was Henry VIII's daughter, Elizabeth I of England in 1562, Guru Har Krishan 8th Guru of the Sikhs in 1664, Peter III of Russia in 1744 and  Abraham Lincoln in 1863. Joseph Stalin, who was badly scarred by the disease early in life, would often have  photographs retouched to make his pockmarks less  apparent.

[edit] See also

• Biological warfare
• Vaccination
• Vaccinia
• Virus
• Population history of American indigenous peoples

[edit] References

[edit] External links

• Smallpox Fact Sheet from the  and  Prevention">CDC
• Smallpox Biosafety The Genetic Engineering of Smallpox: WHO’s Retreat from the Eradication of Smallpox Virus
• CDC.gov—‘Bioterrorism Agents > Smallpox Info for Specific Groups: What Everyone Should Know,’  and  Prevention">CDC
• WHO.int—‘Smallpox: Historical significance,’ World Health Organization (WHO)
• Smallpox summary by AFIP (Armed Forces Institute of Pathology)
• Bioterrorism Category A Agents—Information Resources
• Smallpox Images and  Diagnosis Synopsis
• Inoculation for the Small-Pox defended—1750 article from Gentleman's Magazine
• Smallpox as a potential WMD
• Photographs of smallpox patients
• Why Blame Smallpox?
• History of Smallpox in South Asia