Historically, leprosy has affected humanity for over 4,000 years,[6] and was well-recognized in the civilizations of ancient China, Egypt, and India.[7] DNA taken from the shrouded remains of a man discovered in a tomb next to the Old City of Jerusalem shows him to be the first human proven to have suffered from leprosy.[8] In 1995, the World Health Organization (WHO) estimated that between 2 and 3 million people were permanently disabled because of leprosy.[9] In the past 20 years, 15 million people worldwide have been cured of leprosy.[10] Although the forced quarantine or segregation of patients is unnecessary in places where adequate treatments are available, many leper colonies still remain around the world in countries such as India (where there are still more than 1,000 leper colonies),[10]China,[11]Romania,[12]Egypt, Nepal, Somalia, Liberia, Vietnam,[13] and Japan.[14] Leprosy was once believed to be highly contagious and sexually transmitted, and was treated with mercury—all of which applied to syphilis which was first described in 1530. It is now thought that many early cases of leprosy could have been syphilis.[15] Leprosy is in fact neither sexually transmitted nor is it highly infectious after treatment, as approximately 95% of people are naturally immune[16] and sufferers are no longer infectious after as little as 2 weeks of treatment. However, before treatment was developed, leprosy was certainly contagious.[17][18]
The age-old social stigma, in other words, leprosy stigma[19] associated with the advanced form of leprosy lingers in many areas, and remains a major obstacle to self-reporting and early treatment. Effective treatment for leprosy appeared in the late 1930s with the introduction of dapsone and its derivatives. However, leprosy bacilli resistant to dapsone soon evolved and, due to overuse of dapsone, became widespread. It was not until the introduction of multidrug therapy (MDT) in the early 1980s that the disease could be diagnosed and treated successfully within the community.[20]
MDT for multibacillary leprosy consists of rifampicin, dapsone, and clofazimine taken over 12 months. Dosages adjusted appropriately for children and adults are available in all Primary Health Centres in the form of blister packages.[20] Single dose MDT for single lesion leprosy consists of rifampicin, ofloxacin, and minocycline. The move towards single dose treatment strategies has reduced the prevalence of disease in some regions since prevalence is dependent on duration of treatment.
World Leprosy Day was created to draw awareness to leprosy and its sufferers.
Classification
There are several different approaches for classifying leprosy, but parallels exist.
The World Health Organization system distinguishes "paucibacillary" and "multibacillary" based upon the proliferation of bacteria[21] ("pauci-" refers to a low quantity.)
It is characterized by one or more hypopigmented skin macules and anaesthetic patches, where skin sensations are lost because of damaged peripheral nerves that have been attacked by the human host's immune cells.
Positive
bacillus (Th1)
Multibacillary
midborderline or borderline ("BB")
A30.3
Borderline
Borderline leprosy is of intermediate severity and is the most common form. Skin lesions resemble tuberculoid leprosy but are more numerous and irregular; large patches may affect a whole limb, and peripheral nerve involvement with weakness and loss of sensation is common. This type is unstable and may become more like lepromatous leprosy or may undergo a reversal reaction, becoming more like the tuberculoid form.
Multibacillary
borderline lepromatous ("BL"), and lepromatous ("LL")
A30.4, A30.5
Lepromatous
It is associated with symmetric skin lesions, nodules, plaques, thickened dermis, and frequent involvement of the nasal mucosa resulting in nasal congestion and epistaxis (nose bleeds) but typically detectable nerve damage is late.
Negative
plasmid inside bacillus (Th2)
There is a difference in immune response to the tuberculoid and lepromatous forms.[24]
Hansen's disease may also be divided into the following types:[25]: 344–346
This disease may also occur with only neural involvement, without skin lesions.[26][27][28][29][30]<[7] This disease is also known as Hansen's Disease.
Cause
Mycobacterium leprae
Mycobacterium leprae, one of the causative agents of leprosy. As acid-fast bacteria, M. leprae appear red when a Ziehl-Neelsen stain is used.
Mycobacterium leprae and Mycobacterium lepromatosis are the causative agents of leprosy. M. lepromatosis is a relatively newly identified mycobacterium which was isolated from a fatal case of diffuse lepromatous leprosy in 2008.[2][3]
Due to extensive loss of genes necessary for independent growth, M. leprae and M. lepromatosis are unculturable in the laboratory, a factor which leads to difficulty in definitively identifying the organism under a strict interpretation of Koch's postulates.[2][32] The use of non-culture-based techniques such as molecular genetics has allowed for alternative establishment of causation.
While the causive organisms have to date been impossible to culture in vitro it has been possible to grow them in animals. Charles Shepard, chairman of the United States Leprosy Panel sucessfully grew the organisms in the footpads of mice in 1960. This method was improved with the use of congenitally athymic mice ('nude mice') in 1970 by Joseph Colson and Richard Hilson at St Goeoge's Hospital, London.
A second animal model was developed by Eleanor Storrs at the Gulf South Research Institute. Dr Storrs had worked on the nine banded armadillo for her PhD and reasons that because this animal had a lower body temperature than humans that it might be a suitable animal model. The work started in 1968 with material provided by Waldemar Kirchheimer at the United States Public Health Leprosarium in Carville, Louisiana. These experiments proved unsucessful but additional work in 1970 with material provided by Chapman Binford, medical director of the Leonard's Wood Memorial was sucessful. The papers describing this model lead to a dispute of priority. Further controversy was generated when it was discovered that wild armadillos in Louisana were naturally infected with leprosy.
Genetics
Several genes have been associated with a susceptibility to leprosy.
The mechanism of transmission of leprosy is prolonged close contact and transmission by nasal droplet.[7] The only animal other than humans that is known to contract leprosy is the nine-banded armadillo.[33] The bacterium can also be grown in the laboratory by injection into the footpads of mice.[34] There is evidence that not all people who are infected with M. leprae develop leprosy, and genetic factors have long been thought to play a role, due to the observation of clustering of leprosy around certain families, and the failure to understand why certain individuals develop lepromatous leprosy while others develop other types of leprosy.[35] It is estimated that due to genetic factors, only 5% of the population is susceptible to leprosy.[36] This is mostly because the body is naturally immune to the bacteria, and those persons who do become infected are experiencing a severe allergic reaction to the disease. However, the role of genetic factors is not entirely clear in determining this clinical expression. In addition, malnutrition and prolonged exposure to infected persons may play a role in development of the overt disease.
The incubation period for the bacteria can last anywhere from two to ten years.
The most widely held belief is that the disease is transmitted by contact between infected persons and healthy persons.[37] In general, closeness of contact is related to the dose of infection, which in turn is related to the occurrence of disease. Of the various situations that promote close contact, contact within the household is the only one that is easily identified, although the actual incidence among contacts and the relative risk for them appear to vary considerably in different studies. In incidence studies, infection rates for contacts of lepromatous leprosy have varied from 6.2 per 1000 per year in Cebu, Philippines[38] to 55.8 per 1000 per year in a part of Southern India.[39]
Two exit routes of M. leprae from the human body often described are the skin and the nasal mucosa, although their relative importance is not clear. It is true that lepromatous cases show large numbers of organisms deep down in the dermis. However, whether they reach the skin surface in sufficient numbers is doubtful. Although there are reports of acid-fast bacilli being found in the desquamating epithelium (sloughing of superficial layer of skin) of the skin, Weddell et al. had reported in 1963 that they could not find any acid-fast bacilli in the epidermis, even after examining a very large number of specimens from patients and contacts.[40] In a recent study, Job et al. found fairly large numbers of M. leprae in the superficial keratin layer of the skin of lepromatous leprosy patients, suggesting that the organism could exit along with the sebaceous secretions.[41]
The importance of the nasal mucosa was recognized as early as 1898 by Schäffer, particularly that of the ulcerated mucosa.[42] The quantity of bacilli from nasal mucosal lesions in lepromatous leprosy was demonstrated by Shepard as large, with counts ranging from 10,000 to 10,000,000.[43] Pedley reported that the majority of lepromatous patients showed leprosy bacilli in their nasal secretions as collected through blowing the nose.[44] Davey and Rees indicated that nasal secretions from lepromatous patients could yield as much as 10 million viable organisms per day.[45]
The entry route of M. leprae into the human body is also not definitively known: the skin and the upper respiratory tract are most likely. While older research dealt with the skin route, recent research has increasingly favored the respiratory route. Rees and McDougall succeeded in the experimental transmission of leprosy through aerosols containing M. leprae in immune-suppressed mice, suggesting a similar possibility in humans.[46] Successful results have also been reported on experiments with nude mice when M. leprae were introduced into the nasal cavity by topical application.[47] In summary, entry through the respiratory route appears the most probable route, although other routes, particularly broken skin, cannot be ruled out. The CDC notes the following assertion about the transmission of the disease: "Although the mode of transmission of Hansen's disease remains uncertain, most investigators think that M. leprae is usually spread from person to person in respiratory droplets."[48]
In leprosy both the reference points for measuring the incubation period and the times of infection and onset of disease are difficult to define; the former because of the lack of adequate immunological tools and the latter because of the disease's slow onset. Even so, several investigators have attempted to measure the incubation period for leprosy. The minimum incubation period reported is as short as a few weeks and this is based on the very occasional occurrence of leprosy among young infants.[49] The maximum incubation period reported is as long as 30 years, or over, as observed among war veterans known to have been exposed for short periods in endemic areas but otherwise living in non-endemic areas. It is generally agreed that the average incubation period is between three and five years.
Prevention
In a recent trial, a single dose of rifampicin reduced the rate at which contacts acquired leprosy in the two years after contact by 57%; 265 treatments with rifampicin prevented one case of leprosy in this period.[50] A non-randomized study found that rifampicin reduced the number of new cases of leprosy by 75% after three years.[51]
BCG offers a variable amount of protection against leprosy as well as against tuberculosis.[52][53]
Treatment
Historical treatments
The disease was known in Ancient Greece as elephantiasis (elephantiasis graecorum). The Bible (Mathew 11,5) suggested that leprosy was curable and the practice of laying on of hands or of relics developed from this. Saint Giles, Saint Martin, Saint Maxillian and Saint Roman were associated with this practice. Several monarchs were also associated with this practice: among these were Robert the First of England, Elizabeth the First, Henry the Third and Charlemange.
At various times blood was considered to be a treatment either as a beverage or as a bath. That of virgins or children was considered to be especially potent. This practice seems to have originated with the Ancient Egyptians but was also known in China where people were murdered for their blood. This practice persisted until at least 1790 when the use of dog's blood was mentioned in De Secretis Naturae. Paracelus recommended the use of lamb's blood and even blood from dead bodies was used.
Snakes were also used being mentioned by Pliny, Areteus of Capadocia and Theodorus. Gaucher recommended the use of increase doses of cobra venom. Boinet in 1913 tried increasing doses of bee stings (up to 4000). Scorpions and frogs were used occasionally instead of snakes. The excreta of Anabas (the climbing fish) was even tried.
Alternative treatments included scarification with or without the addition of irritants including arsenic and hellebore. Castration was also practiced in the Middle Ages. Despite its lack of obvious benefit removal of the ovaries or Fallopian tubes in women or vasectomy in men was carried out in the United States for many years.
Hot baths and in particular sulfur springs were tried especially in Japan. After the discovery of the causative organism it was noted that the lesions were largely confined to parts of the body where the temperature was normally below 37 degrees. This led in the 1930s to the introduction of sauna like treatments similar to those tried earlier. Unsurprisingly the results were no better than before.
Radiotherapy, electric currents and phototherapy were also tried: radiotherapy while providing some analgesic relief did nothing to treat the underlying disease. Various drugs including thymol and strychnine were tried with little effect.
Chaulmoogra oil
Perhaps the most popular of the pre modern treatments of leprosy was chaulmoogra oil. This agent has been used since ancient times for the treatment of leprosy. One Indian legend relates that Rama acquired leprosy and was cured by eating the fruit of the Kalaw (a species of the genus Hydnocarpus) tree. He went on to cure the princess Piya with the same fruit and the pair returned to Benares to spread the word of their discovery.
The oil has long been used in India as a Ayurvedic medicine for the treatment of leprosy and various skin conditions. It has also also been used in China and Burma. It was introduced into the Western formulary by Frederic John Mouat, professor at the Bengal Medical College. He tried out the oil as an oral and as a topical agent in two cases of leprosy and reported significant improvements in 1854 paper in the Indian Annals of Medical Science. He suggested that further work was required before this could be recommended.
This paper caused some confusion. Mouat indicated that the oil was the product of a tree Chaulmoogra odorata which had been described in 1815 by William Roxburgh, a surgeon and naturalist, while he was cataloging the plants in the East India Company’s botanical garden in Calcutta. This tree is also known as Gynocardia odorata. For the rest of the 19th century this tree was thought to be the source of the oil. In 1901 Sir David Prain identified the true chaulmoogra seeds of the Calcutta bazaar and of the Paris and London drugs sellers as coming from Taraktogenos kurzii which is found in Burma and Northeast India. The oil mentioned in the Ayurvedic texts was from the tree Hydnocarpus wightiana known as Tuvakara in Sanskrit and chaulmugra in Hindu and Persian.
The first parenteral administration was given by the Egyptian doctor Tortoulis Bey, personal physician to the Sultan Hussein Kamel. He had been using subcutaneous injections of creosite for tuberculosis and in 1894 tried out subcutaneous injection of chaulmoogra oil in a 36 year old EgyptianCopt who had been unable to tolerate oral treatment. After 6 years and 584 injection the patient was declared cured.
The first scientific analysis of this oil was carried out by Frederick B. Power in London in 1904. He and his colleagues isolated a new unsaturated fatty acid from the seeds which they named 'chaulmoogric acid'. They also investigated two closely related species species - Hydnocarpus anthelmintica and Hydnocarpus wightiana. From these two trees they isolated both chaulmoogric acid and a closely related compound 'hydnocarpus acid'. They also investigated Gynocardia odorata and found that it produced neither of these acids. Later investigation showed that 'taraktogenos' (Hydnocarpus kurzii) also produced chaulmoogric acid.
The next difficulty with the use of this oil was of administration. Taken orally it is extremely nauseating. Given by enema may cause peri-anal ulcers and fissures. Given by injection the drug caused fever and other local reactions. Despite these difficulties a series of 170 patients were reported in 1916 by Ralph Hopkins, the attending physician at the Louisiana Leper Home in Carville, Louisiana. He divided the patients into two groups - 'incipient' and 'advanced'. In the advanced cases at most a quarter showed any improvement or arrest of their condition. In the incipient cases he reported an improvement or stabilization of the disease in 45%; 4% died and 8% died. The remainder absconded from the Home apparently in improved condition.
Given the apparent usefulness of this agent the search was on for improved formulations. Victor Heiser the Chief Quarantine Officer and Director of Health for Manila and Elidoro Mercado the house physician at the San Lazaro Hospital for lepers in Manila decided to add camphor to a prescription of chaulmoogra and resorcin which was typically given orally at the suggestion of Merck and Company in Germany to whom Heiser had written. They found that this new compound was readily absorbed without the nausea that had plagued the earlier preparations.
Heiser and Mercado in 1913 then tried the oil by injection out on two patients whom appeared cured of the disease. Since this treatment was administered in conjunction with other materials the results were not clear. A further two patients were treated with the oil by injection without other treatments and again appeared to be cured of the disease. The following year Heiser reported a further 12 patients but the results were much more mixed.
Less toxic injecteble forms of this oil were sought after. Between 1920 and 1922 a series of papers were published describing the esters of these oils. These may have been based on the work of Alice Ball - the record is not clear on this point and Ms Ball died in 1916. Trials of these esters were carried out in 1921 and appeared to give useful results.
These attempts had been preceded by others. Merck of Darmstadt had produced a version of the sodium salts in 1891. They named this sodium gynocardate in the mistaken belief that the origin of the oil was Gynocardia odorata. Bayer in 1908 marketed a commercial version of the esters under the name 'Antileprol'.
To ensure a supply of this agent Joseph Rock, Professor of Systematic Botany at the College of Hawaii was sent to Burma. The local villagers located a grove of trees in seed which he used to establish a plantation in 2,980 trees on the island of Oahu, Hawaii between 1921 and 1922.
The oil remained a popular treatment despite the common side effects until the introduction of sulfones in the 1940s. Debate about its efficacy continued until it was discontinued.
Modern treatment
MDT patient packs and blisters
Promin was first synthesised in 1908 by Emil Fromm, professor of chemistry at Albert-Ludwig University in Freiberg im Breisgau, Germany. Its anti strepococcal activity was investigated by Gladstone Buttle at Burroughs Wellcome and Ernest Fourneau at the Institut Pasteur.
Until the development of promin in the 1940s, there was no effective treatment for leprosy. The efficacy of promin was first discovered by Guy Henry Faget and his co-workers in 1943. Later dapsone was developed. However, it is only weakly bactericidal against M. leprae and it was considered necessary for patients to take the drug indefinitely. Moreover, when dapsone was used alone, the M. leprae population quickly evolvedantibiotic resistance; by the 1960s, the world's only known anti-leprosy drug became virtually useless.
The search for more effective anti-leprosy drugs than dapsone led to the use of clofazimine and rifampicin in the 1960s and 1970s.[54] Later, Indian scientist Shantaram Yawalkar and his colleagues formulated a combined therapy using rifampicin and dapsone, intended to mitigate bacterial resistance.[55] Multidrug therapy (MDT) and combining all three drugs was first recommended by a WHO Expert Committee in 1981. These three anti-leprosy drugs are still used in the standard MDT regimens. None of them are used alone because of the risk of developing resistance.
Because this treatment was quite expensive, it was not quickly adopted in most endemic countries. In 1985 leprosy was still considered a public-health problem in 122 countries. The 44th World Health Assembly (WHA), held in Geneva in 1991, passed a resolution to eliminate leprosy as a public-health problem by the year 2000—defined as reducing the global prevalence of the disease to less than 1 case per 10,000. At the Assembly, the World Health Organization (WHO) was given the mandate to develop an elimination strategy by its member states, based on increasing the geographical coverage of MDT and patients’ accessibility to the treatment.
The WHO Study Group's report on the Chemotherapy of Leprosy in 1993 recommended two types of standard MDT regimen be adopted.[56] The first was a 24-month treatment for multibacillary (MB or lepromatous) cases using rifampicin, clofazimine, and dapsone. The second was a six-month treatment for paucibacillary (PB or tuberculoid) cases, using rifampicin and dapsone. At the First International Conference on the Elimination of Leprosy as a Public Health Problem, held in Hanoi the next year, the global strategy was endorsed and funds provided to WHO for the procurement and supply of MDT to all endemic countries.
MDT anti-leprosy drugs: standard regimens
Between 1995 and 1999, WHO, with the aid of the Nippon Foundation (Chairman Yōhei Sasakawa, World Health Organization Goodwill Ambassador for Leprosy Elimination), supplied all endemic countries with free MDT in blister packs, channelled through Ministries of Health. This free provision was extended in 2000 with a donation by the MDT manufacturer Novartis, which will run until at least the end of 2010. At the national level, non-government organizations (NGOs) affiliated to the national programme will continue to be provided with an appropriate free supply of this WHO supplied MDT by the government.
MDT remains highly effective, and patients are no longer infectious after the first monthly dose.[7] It is safe and easy to use under field conditions due to its presentation in calendar blister packs.[7]Relapse rates remain low, and there is no known resistance to the combined drugs.[7] The Seventh WHO Expert Committee on Leprosy,[57] reporting in 1997, concluded that the MB duration of treatment—then standing at 24 months—could safely be shortened to 12 months "without significantly compromising its efficacy."
Efforts to overcome persistent obstacles to the elimination of the disease include improving detection, educating patients and the population about its cause, and fighting social taboos about a disease whose patients have historically been considered "unclean" or "cursed by God" as outcasts. Where taboos are strong, patients may be forced to hide their condition (and avoid seeking treatment) to avoid discrimination. The lack of awareness about Hansen's disease can lead people to falsely believe that the disease is highly contagious and incurable.
The ALERT hospital and research facility in Ethiopia provides training to medical personnel from around the world in the treatment of leprosy, as well as treating many local patients. Surgical techniques, such as for the restoration of control of movement of thumbs, have been developed.
Current treatment recomendations
Pauci-bacillary leprosy(1-5 skin lesions) Treat with rifampicin and dapsone for 6 months
Multi-bacillary leprosy (>5 skin lesions) Treat with rifampicin, clofazimine and dapsone for 12 months
Worldwide, two to three million people are estimated to be permanently disabled because of leprosy.[9]India has the greatest number of cases, with Brazil second and Burma third.
In 1999, the world incidence of Hansen's disease was estimated to be 640,000. In 2000, 738,284 cases were identified.[59] In 1999, 108 cases occurred in the United States. In 2000, the World Health Organization (WHO) listed 91 countries in which Hansen's disease is endemic. India, Myanmar and Nepal contained 70% of cases. India reports over 50% of the world's leprosy cases.[60] In 2002, 763,917 new cases were detected worldwide, and in that year the WHO listed Brazil, Madagascar, Mozambique, Tanzania and Nepal as having 90% of Hansen's disease cases.
According to recent figures from the WHO, new cases detected worldwide have decreased by approximately 107,000 cases (or 21%) from 2003 to 2004. This decreasing trend has been consistent for the past three years. In addition, the global registered prevalence of HD was 286,063 cases; 407,791 new cases were detected during 2004.
At highest risk are those living in endemic areas with poor conditions such as inadequate bedding, contaminated water and insufficient diet, or other diseases (such as HIV) that compromise immune function. Recent research suggests that there is a defect in cell-mediated immunity that causes susceptibility to the disease. Less than ten percent of the world's population is actually capable of acquiring the disease.[62] The region of DNA responsible for this variability is also involved in Parkinson disease,[citation needed] giving rise to current speculation that the two disorders may be linked in some way at the biochemical level. In addition, men are twice as likely to contract leprosy as women.[citation needed] According to The Leprosy Mission Canada, most people-–about 95 % of the population-–are naturally immune.[62]
Disease burden
Although annual incidence—the number of new leprosy cases occurring each year—is important as a measure of transmission, it is difficult to measure in leprosy due to its long incubation period, delays in diagnosis after onset of the disease and the lack of laboratory tools to detect leprosy in its very early stages. Instead, the registered prevalence is used. Registered prevalence is a useful proxy indicator of the disease burden as it reflects the number of active leprosy cases diagnosed with the disease and receiving treatment with MDT at a given point in time. The prevalence rate is defined as the number of cases registered for MDT treatment among the population in which the cases have occurred, again at a given point in time.[63]
New case detection is another indicator of the disease that is usually reported by countries on an annual basis. It includes cases diagnosed with onset of disease in the year in question (true incidence) and a large proportion of cases with onset in previous years (termed a backlog prevalence of undetected cases). The new case detection rate (NCDR) is defined by the number of newly detected cases, previously untreated, during a year divided by the population in which the cases have occurred.[citation needed]
Endemic countries also report the number of new cases with established disabilities at the time of detection, as an indicator of the backlog prevalence. However, determination of the time of onset of the disease is generally unreliable, is very labor-intensive and is seldom done in recording these statistics.
Global situation
Europe
The Hospital-Colónia Rovisco Pais (the Rovisco Pais Hospital-Colony) was founed in Portugal in 1947 as a national center for the treatment of leprosy. It was renamed in 2007 as the Centro de Medicina de Reabilitação da Região Centro-Rovisco Pais. It still retains a leprosy service in which 25 ex patients live. Between 1988 and 2003 102 patients were treated in Portugal for leprosy.[64]
The Sanitorio de Fontilles (Fontilles Sanatorium) in Spain was founded in 1902 and admitted its first patient in 1909. In 2002 the Sanitorio had 68 in-patients in the Sanatorium, and more than 150 receiving out-patient treatment. A small number of cases continue to be reported[65]
Two indigenous cases were reported from Greece in 2009.[66]
As reported to WHO by 115 countries and territories in 2006, and published in the Weekly Epidemiological Record the global registered prevalence of leprosy at the beginning of the year was 219,826 cases.[68] New case detection during the previous year (2005 - the last year for which full country information is available) was 296,499. The reason for the annual detection being higher than the prevalence at the end of the year can be explained by the fact that a proportion of new cases complete their treatment within the year and therefore no longer remain on the registers. The global detection of new cases continues to show a sharp decline, falling by 110,000 cases (27%) during 2005 compared with the previous year.
Table 1 shows that global annual detection has been declining since 2001. The African region reported an 8.7% decline in the number of new cases compared with 2004. The comparable figure for the Americas was 20.1%, for South-East Asia 32% and for the Eastern Mediterranean it was 7.6%. The Western Pacific area, however, showed a 14.8% increase during the same period.
Table 2 shows the leprosy situation in the four major countries which have yet to achieve the goal of elimination at the national level. It should be noted that: a) Elimination is defined as a prevalence of less than 1 case per 10,000 population; b) Madagascar reached elimination at the national level in September 2006; c) Nepal detection reported from mid-November 2004 to mid-November 2005; and d) D.R. Congo officially reported to WHO in 2008 that it had reached elimination by the end of 2007, at the national level.
Current situation in the People's Republic of China
The People's Republic of China has many leprosy recovered patients who have been isolated from the rest of society. In the 1950s the Chinese Communist government created "Recovered Villages" in rural remote mountaintops for the recovered patients. Although leprosy is now curable with the advent of the multi-drug treatment, the villagers remain because they have been stigmatized by the outside world. Health NGOs such as Joy in Action have arisen in China to especially focus on improving the conditions of "Recovered Villages".
History
Etymology
The word leprosy comes from ancient Greek λέπρα [léprā], "a disease which makes the skin scaly", in turn a nominal derivation of the verb λέπω [lépō], "to peel, scale off". The word came into the English language via Latin and Old French. The first attested English use is in the Ancrene Wisse, a 13th-century manual for nuns ("Moyseses hond..bisemde o þe spitel uuel & þuhte lepruse." The Middle English Dictionary, s.v., "leprous"). A roughly contemporaneous use is attested in the Anglo-Norman Dialogues of Saint Gregory, "Esmondez i sont li lieprous" (Anglo-Norman Dictionary, s.v., "leprus").
Historically, individuals with Hansen's disease have been known as lepers; however, this term is falling into disuse as a result of the diminishing number of leprosy patients and the pejorative connotations of the term. Because of the stigma to patients, some prefer not to use the word "leprosy," though the term is used by the U.S. Centers for Disease Control and Prevention and the World Health Organization.
Historically, the term Tzaraath from the Hebrew Bible was, erroneously, commonly translated as leprosy, although the symptoms of Tzaraath were not entirely consistent with leprosy and rather referred to a variety of disorders other than Hansen's disease.[69]
In particular, tinea capitis (fungal scalp infection) and related infections on other body parts caused by the dermatophyte fungus Trichophyton violaceum are abundant throughout the Middle East and North Africa today and might also have been common in biblical times. Similarly, the related agent of the disfiguring skin disease favus, Trichophyton schoenleinii, appears to have been common throughout Eurasia and Africa before the advent of modern medicine. Persons with severe favus and similar fungal diseases (and potentially also with severe psoriasis and other diseases not caused by microorganisms) tended to be classed as having leprosy as late as the 17th century in Europe.[70] This is clearly shown in the painting The Regents of the Leper Hospital in Haarlem 1667 by Jan de Bray (Frans Hals Museum, Haarlem, the Netherlands), where a young Dutchman with a vivid scalp infection, almost certainly caused by a fungus, is shown being cared for by three officials of a charitable home intended for leprosy sufferers.[citation needed] The use of the word "leprosy" before the mid-19th century, when microscopic examination of skin for medical diagnosis was first developed, can seldom be correlated reliably with Hansen's disease as we understand it today.[citation needed]
India
The Oxford Illustrated Companion to Medicine holds that the mention of leprosy, as well as cures for it, were already described in the Hindu religious book Atharva-veda.[71] Writing in the Encyclopedia Britannica 2008, Kearns & Nash state that the first mention of leprosy is in the Indian medical treatise Sushruta Samhita (6th century BC).[72]The Cambridge Encyclopedia of Human Paleopathology (1998) holds that: "The Sushruta Samhita from India describes the condition quite well and even offers therapeutic suggestions as early as about 600 BC"[73] The surgeon Sushruta lived in the Indian city of Kashi by the 6th century BC,[74] and the medical treatise Sushruta Samhita—attributed to him—made its appearance during the 1st millennium BC.[72] The earliest surviving excavated written material which contains the works of Sushruta is the Bower Manuscript—dated to the 4th century AD, almost a millennium after the original work.[75] In 1881, around 120,000 leprosy patients existed in India. The central government passed the Lepers Act of 1898, which provided legal provision for forcible confinement of leprosy sufferers in India.[76] In 2009, a 4,000-year-old skeleton was uncovered in India that was shown to contain traces of leprosy.[77] The discovery was made at a site called Balathal, which is today part of Rajasthan, and is believed[by whom?] to be the oldest case of the disease ever found.[78] This pre-dated the previous earliest recognized case, dating back to 6th-century Egypt, by 1,500 years.[79] It is believed that the excavated skeleton belonged to a male, who was in his late 30s and belonged to the Ahar Chalcolithic culture.[79][80] Archaeologists have stated that not only does the skeleton represent the oldest case of leprosy ever found, but is also the first such example that dates back to prehistoric India.[81] This finding supports one of the theories regarding the origin of the disease, which is believed to have originated in either India or Africa, before being subsequently spread to Europe by the armies of Alexander the Great.[78]
Regarding ancient China, Katrina C. D. McLeod and Robin D. S. Yates identify the State of Qin'sFeng zhen shi 封診式 (Models for sealing and investigating), dated 266-246 BC, as offering the earliest known unambiguous description of the symptoms of low-resistance leprosy, even though it was termed then under li 癘, a general Chinese word for skin disorder.[82]This 3rd century BC Chinese text on bamboo slip, found in an excavation of 1975 at Shuihudi, Yunmeng, Hubei province, not only described the destruction of the "pillar of the nose", but also the "swelling of the eyebrows, loss of hair, absorption of nasal cartilage, affliction of knees and elbows, difficult and hoarse respiration, as well as anesthesia."[82]
Japan has had a unique history of segregation of patients into sanatoriums based on leprosy prevention laws of 1907, 1931 and 1953, and hence, it intensified leprosy stigma. The 1953 law was abrogated in 1996. There are still 2717 ex-patients in 13 national sanatoriums and 2 private hospitals as of 2008. In a document written in 833, leprosy was described as "caused by a parasite which eats five organs of the body. The eyebrows and eyelashes come off, and the nose is deformed. The disease brings hoarseness, and necessitates amputations of the fingers and toes. Do not sleep with the patients, as the disease is transmittable to those nearby." This was the first document concerning infectivity.[83]
Rome
In the West, the earliest known description of leprosy there was made by the Roman encyclopedist Aulus Cornelius Celsus (25 BC – 37 AD) in his De Medicina; he called leprosy "elephantiasis".[82] The Roman author Pliny the Elder (23–79 AD) mentioned the same disease.[82] Although "sara't" of Leviticus (Old Testament) is translated as "lepra" in the 5th century AD Vulgate, the original term sara't found in Leviticus was not the elephantiasis described by Celsus and Pliny; in fact, sara't was used to describe a disease which could affect houses and clothing.[82] Katrina C. D. McLeod and Robin D. S. Yates state that sara't "denotes a condition of ritual impurity or a temporary form of skin disease."[82]
Numerous leprosaria, or leper hospitals, sprang up in the Middle Ages; Matthew Paris, a Benedictine Monk, estimated that in the early thirteenth century there were 19,000 across Europe.[84] The first recorded Leper colony was in Harbledown. These institutions were run along monastic lines and, while lepers were encouraged to live in these monastic-type establishments, this was for their own health as well as quarantine. Indeed, some medieval sources indicate belief that those suffering from leprosy were considered to be going through Purgatory on Earth, and for this reason their suffering was considered holier than the ordinary person's. More frequently, lepers were seen to exist in a place between life and death: they were still alive, yet many chose or were forced to ritually separate themselves from mundane existence.[85] The Order of Saint Lazarus was a hospitaller and military order of monks that began as a leper hospital outside Jerusalem in the twelfth century and remained associated with leprosy throughout its history. The first monks in this order were leper knights and they originally had leper grand masters, although these aspects of the order changed over the centuries.
Radegund was noted for washing the feet of lepers. Orderic Vitalis writes of a monk, Ralf, who was so overcome by the plight of lepers that he prayed to catch leprosy himself (which he eventually did). The leper would carry a clapper and bell to warn of his approach, and this was as much to attract attention for charity as to warn people that a diseased person was near.
Germany
Leprosy was almost eradicated in most of Europe by 1700 but sometime after 1850 leprosy was re introduced into East Prussia by Lithuanian rural workers immigrating from the Russian empire. The first leprosarium was founded in 1899 in Memel (now Klaipėda in Lithuania). Legislation was introduced in 1900 and 1904 requiring patients to be isolated and not allowed to work with others.
Hawaiian Islands
The date of introducton of leprosy into these islands is debated. The most commonly held view is that it was introduced by the Chinese immigrants in the mid 1850s but some historical records suggest that seafarers may have introduced it at an earlier date.
Great Britain
The last known case in Great Britain was acquired in 1798 in the Shetland Islands.
After the end of the 17th century, Norway and Iceland were the only countries in Western Europe where leprosy was a significant problem. During the 1830s, the number of lepers in Norway rose rapidly, causing an increase in medical research into the condition, and the disease became a political issue. Norway appointed a medical superintendent for leprosy in 1854 and established a national register for lepers in 1856, the first national patient register in the world.[86]
He observed a number of non refractile small rods in unstained tissue sections. The rods were not soluble in potassium lye and they were acid and alcohol fast. In 1879 he was able to stain these organisms with Zeihl's method and the similarities with Kock's bacillus (Mycobacterium tuberculosis) were noted. There were three significant differences between these organisms: (1) the rods in the leprosy lesions were extremely numerous (2) they formed characteristic intracellular collections (globii) and (3) the rods had a variety of shapes with branching and swelling. Thse differences suggested that leprosy was caused by an organism related to but distinct from Mycobacterium tuberculosis.
He worked at St. Jørgens Hospital in Bergen, founded early in the fifteenth century. St. Jørgens is today a museum, Lepramuseet, probably the best preserved leprosy hospital in Northern Europe.[89]
There were cases of leprosy in Atlantic Canada at the end of the 19th century. The patients were first housed on Sheldrake Island in the Miramichi river and later transferred to Tracadie[disambiguation needed]. Catholic nuns (the religieuses hospitalières de Saint-Joseph, RHSJ) came to take care of the sick. They opened the first French-language hospital in New-Brunswick and many more followed. Many hospitals opened by the RHSJ nuns are still in use today. The last hospital to house lepers in Tracadie was demolished in 1991. Its lazaretto section had been closed since 1965. In a century of existence, it had housed not only Acadian victims of the disease, but people from all over Canada as well as sick immigrants from Iceland, Russia and China, among other nations.[90]
Saint Damien was a Roman Catholic missionary-priest who contracted leprosy while serving the leper colony at Molokaʻi and continued to serve the lepers until he died from the effects of the disease himself. He was canonized on October 11, 2009 at a Vatican ceremony presided over by Benedict XVI
Christian sacred scripture has a number of references such as Moses's sister ([1]), Moses ([2]), commander of the army of Syria Naaman the Syrian and later the prophet Elisha's servant Gehazi ([3]), and several people Jesus healed ([4], [5]), though the afflictions mentioned and symptoms described are now thought not to be, or not exclusively to be, the result of Hansen's Disease (see discussion at 'Tzaraath').
In Shakespeare's Hamlet, the ghost of old king Hamlet, prince Hamlet's father, claimed to have been poisoned by a 'leprous distillment', as he contracted several symptoms similar to those of leprosy such as pustulating boils.
In the novel Shantaram by Gregory David Roberts, the main character, Lin, often dealt with members of a leper colony in Mumbai as part of his time living in a slum.
In the original book (and movies based on the book) Ben Hur, the mother and sister of Judah Ben-Hur, the protagonist, contract leprosy while in prison.
Rachel Kalama in the novel Molokai is sent away from Honolulu to live on the leper colony in Molokai[citation needed].
In the novel Healing Water by Joyce Moyer Hostetter, a Hawaiian teenager struggles to survive the leper colony of Molokai in the 1860s[citation needed].
The Island is a historical novel written by Victoria Hislop, much of which is set in a leper colony on the Mediterranean island of Spinalonga
Caius Merlyn Brittanicus[citation needed] from The Camulod Chronicles contracts leprosy while trying to save a bleeding leper from freezing to death.
The protagonist of the book The Leper by Steve Thayer is an American soldier who is infected during World War I and contracts the disease
In Tess Gerritsen's 2003 novel The Sinner, Maura's post-mortem tests on an unidentified body found in an abandoned Boston warehouse show this victim to be Indian leprosy victim born perhaps 40 or 50 years before the date of the corpse being studied[citation needed]
In the TV-series Monk, S05-E10, Derek Bronson, of Bronson Technologies suffers from leprosy[citation needed].
In The Simpsons episode "Little Big Mom", Lisa fools Bart and Homer into thinking they have leprosy. Ned Flanders sends them to the island of Molokai for treatment.
Dante from The Last Vampire series by Christopher Pike appeared to Sita to suffer from leprosy[citation needed].
In the "Monty Python" sketch "Conquistador Coffee Campaign", an advertiser for a coffee company is questioned by his boss about his changing an ad for instant coffee to "instant leprosy", along with a few other unappealing ads.
In the film "Princess Mononoke", one of the main characters, Lady Eboshi of Iron Town took care of several lepers, who made weapons for her.
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^Montestruc E, Berdonneau R (1954). "2 New cases of leprosy in infants in Martinique". Bull Soc Pathol Exot Filiales (in French). 47 (6): 781–3. PMID14378912.
^Fine PE, Smith PG (1996). "Vaccination against leprosy—the view from 1996". Lepr Rev. 67 (4): 249–52. PMID9033195.
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