FROM LEPROSY TO HANSEN-S DISEASE | Kickoff

FROM LEPROSY TO HANSEN-S DISEASE

12 May

More   than   any  other   disease,  leprosy  demonstrates  the  difference between the biological  nature  of illness and  the attributes ascribed  to the  sick.  Indeed,  it  is fair  to  say  that  leprosy  and  Hansen’s  disease (the modern  name for true leprosy) stand for different ideas more than different  diseases. The word leper is still commonly  used to mean  one who is hated  and shunned  by society.

Medieval  attitudes  towards  the leper were based  on biblical pas- sages pertaining  to ‘‘leprosy,’’ a vague term applied to various chronic, progressive  skin afflictions,  from  leprosy  and  vitiligo to  psoriasis  and skin  cancer.  The  leper,  according  to  medieval  interpretations of  the Bible, was ‘‘unclean’’ and,  therefore,  a  dangerous  source  of physical and  moral  pollution.  Biblical rules  governing  leprosy  demanded  that persons  and  things  with  suspicious  signs of leprosy  must  be brought to the priests for examination. Leprosy  was said to dwell not  only in human  beings, but  also in garments  of wool or linen, objects made of skins, and  even in houses.  Diagnostic  signs included  a scaly eruption, boil, scab, or bright  spot.  When  the signs were ambiguous,  the priest shut the suspect away for as long as two weeks for further  observations. When  a final judgment  had  been reached,  the leper was instructed  to dwell  in  isolation   and  call  out  as  a  warning  to  those  who  might approach him, ‘‘unclean, unclean.’’

Islamic  teachings  also  reflected  fear  of  leprosy.  ‘‘Fly from  the

leper,’’ warned  the  Prophet   Muhammad, ‘‘as you  would  fly from  a lion.’’ According  to  Islamic  law  and  custom,  people  suffering  from leprosy  were  not  allowed  to  visit  the  baths.  It  was  said  that  foods touched  by a leper could transmit  the disease and that  no plants would grow in soil touched  by the bare feet of a leper.

A brief survey of modern  findings about  this disease may help us

understand the ambiguities  and complexities of the medieval literature concerning  leprosy. Mycobacterium  leprae, the bacillus that  causes the disease, was discovered  in 1874 by the Norwegian  physician  Gerhard Hansen  (1841–1912). Hansen  had  observed  leprosy  bacilli as early as

1871, but  it was very difficult to prove that  the bacteria  found  in skin scrapings  of  patients  actually  caused  the  disease.  No  animal  model was available  and the putative  leprosy bacillus refused to grow in arti- ficial media. It took almost one hundred  years for scientists to overcome these obstacles. To honor  Gerhard Hansen  and avoid the stigma asso- ciated with the term leper, the disease was renamed  Hansen’s disease.

What is most surprising about  Hansen’s disease is the fact that it is

only  slightly  contagious.  Many  people  having  extended  and  intimate contact  with lepers, such as spouses, nurses,  and  doctors,  do not  con- tract  the  disease,  whereas  others  with  little  contact  become  infected. Where leprosy remains  endemic, almost  all people have been exposed to the infectious  agent,  but  only a small percent  actually  contract  the disease. Those who do seem to have a deficiency in their immune system that  makes them unusually  susceptible M. leprae. Evidence of the lim- ited degree of contagiousness of leprosy today does not, of course, prove that  the disease was not more contagious  in the past. Nevertheless,  lep- rosy could  not  have been as contagious  as medieval  writers  assumed. Religious  and medical authorities  argued  that  leprosy could be spread

leprosy

A man with leprosy (photographed in Manila  in 1899).

by the glance of a leper or an unseen leper standing  upwind of healthy people.

Various indeterminate patterns  exist between the two polar  forms of Hansen’s disease, known as tuberculoid and lepromatous. If infected people  mount  a partial  immune  response,  the disease will assume  the tuberculoid form,  which is not  infectious  or dangerous  to others.  The early symptoms  of tuberculoid leprosy include skin lesions and loss of sensation  in  the  affected  areas.  Unfortunately, the  loss  of  sensation and  the  weak  immune  response  may  eventually  result  in  damage  to tissues. About  80 percent  of all cases take  this form. Patients  with the tuberculoid form mount  a partially  effective cell-mediated immune response to the infection, although  this does not eliminate the bacteria. Individuals  with the more severe lepromatous form have essentially no immune  response.  The  bacteria  multiply  freely and  large  numbers  of bacteria  are discharged  in the nasal  secretions.  The lepromatous form is characterized by skin lesions consisting  of raised  blotches,  nodules, and lumps. Eventually,  the thickening  of the skin of the forehead  and

face, exaggeration of natural lines, and loss of facial hair produce the so- called ‘‘lion face.’’ As the disease progresses, ulcerating skin lesions lead to destruction of cartilage and bone. About  30 percent of all victims of Hansen’s   disease   eventually   develop   crippling   deformities   due   to damaged  joints, paralysis  of muscles, and loss of soft tissue and bone, especially the fingers and toes. Loss of sensitivity caused by nerve dam- age results in repeated  injuries and infections.

Leprosy  seems  to  have  been  rare  in  Europe  before  the  fall  of

Rome. Although  the disease was creeping into the Mediterranean arena by the sixth century, the Crusades  of the eleventh and twelfth centuries created   ideal   conditions   for   a  major   invasion.   Indeed,   there   are estimates  that  by the end of the twelfth century  one out  of every two hundred  Europeans was infected  with  leprosy.  The  high  incidence  of leprosy among pious persons, especially Crusaders  and pilgrims return- ing from the Holy Land, was a potential  source of embarrassment to the Church. The Crusades were part of massive movements of human popu- lations that  broke down ancient barriers  and carried infectious diseases to new populations. However,  after reaching  its peak in the thirteenth century,  leprosy all but disappeared  from Europe.

Priests,  doctors,  and  lepers were involved  in the  examination  of alleged medieval lepers. If incriminating  signs of leprosy were found— bright  spots,  depigmented  patches,  sores, thickened  skin, hoarse  voice, and ‘‘lion face’’—the accused was found guilty. A funeral service, rather than an execution, followed the verdict. Although  the rites of exclusion could be held in a church, performing  them in a cemetery with the leper standing  in a grave made the symbolic death and burial more dramatic. Having sprinkled earth on the leper’s head, the priest declared him dead to the world, but reborn  to God.  Although  hated  by all men, the leper was said to be loved by God and could, therefore, look forward to com- pensation  in the next world.  The rules governing  lepers and  marriage reflect the ambiguity  of the leper’s status.  Despite  the leper’s symbolic death, leprosy was not necessarily accepted as a cause for the dissolution of marriage.  Indeed, the Church  decreed that  a man with leprosy could require a healthy  wife to continue  sexual relations.

Feared  by all and condemned  to live in isolation,  lepers were also the conspicuous  targets  of religious charity.  Thousands of leper houses were established by various religious orders throughout Europe.  Where lepers were granted the special privilege of begging for alms, other impoverished  people  apparently pretended   to  be  lepers  in  order  to receive alms or be admitted  to leper houses.  Miserable  as these places might be by modern  standards, they were presumably  better  than  the alternatives.

Sometimes lepers were the objects of ‘‘heroic charity,’’ such as that of Queen Mathilda, who expressed her piety by bringing  lepers into her own rooms where she fed them and washed their feet. On finding her so

Leprosy is now known as Hansen’s

Leprosy is now known as Hansen’s disease.

occupied, her brother  was filled with revulsion and warned her that King Henry might not enjoy being intimate  with a woman who spent her days washing the feet of lepers. Mathilda’s  piety was, however, so contagious that she soon had her brother kissing the lepers. In contrast, Philip the Fair of France,  thought  that  lepers should be buried alive or burned  to death rather  than subjected to merely symbolic rites of isolation  and burial.

When  in public,  lepers were supposed  to wear a special costume

and  warn  others  of their  approach with a bell, or rattle.  Lepers  were not allowed to speak to healthy people, but could point with a long stick

to indicate  items they wished to purchase.  (Presumably,  money taken from  a leper was not  a troublesome  source of contagion.)  As always, justice or injustice was not equally distributed. Enforcement of the rules of exclusion varied from benign neglect to extreme brutality.  The role forced upon the leper was not that of a sick person, but that of a scape- goat  for  all  of  medieval  society.  Perhaps,  there  were  also  economic motives  at  both  the  state  and  the  family level for  the  persecution  of lepers, who often lost their right to property  and inheritance.

Given the ambiguity  of the early signs of leprosy, how was it pos- sible to ‘‘discover’’ lepers? Theoretically,  lepers were supposed to report their  disease  to  the  authorities,  but  ‘‘closet  lepers’’  were  probably detected  and  exposed  by suspicious  neighbors.  Medieval  descriptions of all diseases contain highly stylized and speculative material expressed in terms  of humoral  pathology.  The  divergence between  modern  and medieval observations is, however, more striking in the case of leprosy than  for other  diseases. In the absence of immunological  and bacterio- logical tests, even the most skillful modern diagnostician may find it dif- ficult to distinguish  leprosy from  other  diseases that  produce  chronic, progressive  skin  lesions.  Although   some  changes  in  clinical  patterns may  occur  over  time,  it seems likely that  medieval  authors,  whether physicians or priests,  often  described  what they expected to see rather than  what they actually saw.

Many  medical  authorities   assumed  that  leprosy  was  caused  or

transmitted by improper  sexual acts,  such as intercourse  with a men- struating  woman, or contact  with a healthy woman who had previously had intercourse  with a leper. The presumption of a link between leprosy and ‘‘moral defilement,’’ specifically lechery, persisted into the twentieth century. However, sex and sin were not the only causes of leprosy recog- nized by medieval physicians. The disease could be inherited from a lep- rous  ancestor  or acquired  from  the bite of a poisonous  worm,  rotten meat,  unclean  wine, infected air, and corrupt  milk from a leprous  wet nurse.  Various  diets were commonly  recommended  to prevent  or cure leprosy;  almost  every food  fell under  suspicion  at  one  time or  other. Indeed, many years of research convinced Sir Jonathan Hutchinson (1828–1913) that  eating rotten  fish caused leprosy.

Although  the only useful medieval response to leprosy was the iso- lation of the afflicted, physicians, quacks, and the Bible offered hope of miraculous  cures. According to Matthew,  Jesus healed a leper simply by touching  him  and  saying ‘‘be thou  clean.’’ In  contrast  to  this  instan- taneous  cure, Naaman, who was cured  by the Prophet  Elisha,  had  to wash  himself in the  Jordan  River  seven times.  Bartolomeus Anglicus (fl. 1250) admitted  that  leprosy  was hard  to cure, except of course  by the help of God,  but  he did suggest a remedy made  from  the flesh of a black snake  cooked  in an earthen  pot  with pepper,  salt, vinegar, oil, water, and a special ‘‘bouquet garni.’’ Because this powerful snake soup

would make the patient  dizzy and  cause his body to swell, theriac  was needed to counteract undesirable  side effects. Eventually,  the patient’s flesh would peel and his hair would fall out,  but these problems  would subside.  An  equally  promising  remedy  from  a fifteenth-century leech- book  combined  a bushel of barley and half a bushel of toads  in a lead cauldron.  The mixture was simmered until the flesh of the toads fell from the bones. The barley brew was dried in the sun and then fed to newly hatched  chicks. The chicks were roasted  or boiled and fed to the leper.

Driven by fear and hope, desperate  lepers might attempt  even the most gruesome of cures, be it eating human  gall bladders  or bathing  in blood.  Because many transient  skin lesions were probably  mistaken  for Hansen’s disease, appeals  to saints, baths,  bizarre potions,  and strange diets were sometimes followed by miraculous  cures of the post hoc ergo propter hoc variety. This well-known logical fallacy, in which sequence is confused with cause, has all too often secured the reputation of useless remedies and healers.

Perhaps,   the  most  surprising  aspect  of  medieval  leprosy  is the

virtual disappearance of the disease from Europe by the fourteenth cen- tury.  Obviously,  this change was not  the result  of any medical break- through.  Even  the  cruel  measures  taken   to  isolate  lepers  were  of dubious  efficacy in breaking  the chain of transmission because the dis- ease has a long latent  period  during  which susceptible individuals  may be exposed to infection.  Changing  patterns  of commerce, warfare,  and pilgrimages may have broken  the chain of contagion  by which leprosy reached Europe from areas where the disease remained, and still remains, endemic.

If leprosy all but  vanished  from Europe  with minimal medical or public health advances, could it not be totally eradicated  today through deliberate efforts? There are good reasons to consider Hansen’s disease a logical candidate  for a global eradication campaign.  Unlike  bubonic plague, leprosy does not seem to have a natural animal reservoir. There- fore, breaking the chain of person-to-person transmission should eventually  eliminate the disease. Leprosy  was one of six infectious dis- eases that the World Health Organization selected as targets of a world- wide public health campaign launched in 1975. However, malaria, schistosomiasis,  filariasis,  leishmaniasis,  trypanosomiasis, and  leprosy do not present  a major  threat  to wealthy nations  or individuals.  Thus, they do not  receive the attention that  has been awarded  to smallpox, poliomyelitis, measles, and other  preventable  infectious diseases.

Because  the  early  symptoms  of  Hansen’s  disease  are  similar  to many  other  ailments,  its victims may  be misdiagnosed  and  subjected to inappropriate treatments for long periods  of time. The drugs  most frequently  used to  treat  leprosy  are  dapsone,  rifampicin,  and  clofazi- mine;  often  all three  drugs  are  given as a  multidrug  regimen  for  six months  to  two  years.  Despite  the appearance of strains  of M.  leprae

resistant  to each of these drugs,  public health  authorities  argue that  if a  partial  course  of therapy  were instituted  for  all lepers,  the  disease could  be eradicated.  Even if the afflicted  individual  is not  completely cured,  drug  treatment renders  the  patient   noninfectious   and  breaks the chain  of transmission. Unless  resources  are  allocated  for  a major assault  on  leprosy,  the  worldwide  incidence  of the  disease will inevi- tably  increase  and  multidrug   resistant  Hansen’s  disease  will become more  difficult to treat.

The World Health Organization estimates that more than one hun- dred years after the discovery of the causative agent for leprosy, about

15 million people are still suffering from the disease. The number  could actually be much higher because Hansen’s disease is often misdiagnosed or unreported. Many patients still think of the diagnosis as a curse. About

90 percent  of all cases were found  in ten  countries:  India,  Indonesia, Brazil, Democratic Republic of the Congo, Guinea, Madagascar, Mozambique, Myanmar, Nepal,  and  Tanzania, but  Hansen’s  disease remains a significant public health problem in other impoverished areas. For  example, in Somalia, during  the floods that  affected over sixty vil- lages and over one hundred  thousand people in 2000, hundreds  of lep- rosy victims were among  those forced out of their homes and villages. Some  lepers  were  so  debilitated  by  the  disease  that  they  had  to  be carried  in wheelbarrows  and  carts  pulled  by donkeys.  Most  of those suffering  from  leprosy  had  been  living in perpetual  quarantine  since the 1980s when they were exiled to two remote  villages. Villagers and the  authorities  in nearby  towns  quickly  expressed  concern  about  the sudden  influx of leprosy victims.

The Kalaupapa Peninsula,  on the island of Molokai,  Hawaii,  was once a place of permanent exile for thousands of victims of Hansen’s dis- ease. About  eight thousand people have been exiled to Kalaupapa since

1865 when King Kamehameha V signed an act to prevent the spread of

leprosy. Mandatory exile, as well as all admissions to Kalaupapa, ended in 1969. By 2003, only about 40 elderly patients remained. The survivors were too disabled or disfigured by the disease to leave a place that once was little more  than  a prison.  The former  leper colony became a National Historical   Park.   Another   well-known   American   leper  hospital   was founded in Carville, Louisiana,  in 1913. Facing strict, life-long isolation, patients described Carville as more like a prison or ‘‘living cemetery’’ than a hospital.  By the late twentieth  century,  almost  all cases of Hansen’s disease in the United States were found in immigrants  who had contrac- ted the disease in areas where it remains endemic.

Politics and poverty  account  for much of the difficulty in mount-

ing a global campaign against leprosy, but research on Hansen’s disease has also been hindered  by the reluctance  of M. leprae to multiply  and be  fruitful  in  laboratory animals  and  artificial  media.  Research  has been  facilitated  by  the  discovery  that  the  bacilli  will multiply  in  the

nine-banded  armadillo,  the  footpads  of mice, and  several nonhuman primates.

Random Posts

Comments are closed.