HansensDisease

ByEricChowandTaniaRoman
M&I103:Parasites&Pestilence
Dr.ScottSmith;Spring2008

INTRODUCTION
Mycobacteriumleprae,thebacteriumthatcausesleprosywasdiscoveredin1873
byGerhardHenrikArmauerHansen(BacteriaGenomes2008).Alsoknownas
Hansensdisease,leprosyhasalonghistoryofassociatedstigma,whichpreventspeople
fromseekingmedicaltreatmentandoftenthwartspublichealthinterventiontocurb
furtherspreadofthedisease.Forthisreason,publichealthinterventionsshouldinclude
effortstoreducestigmaviaaneducationalawarenessprogramandtrainingoflocal
healthofficials.Ourprojectaimstoestablishspecialskinailmentunitswithinexisting
clinicsinLondrinaCityofBrazilwiththegoalsofdiagnosing,treatingandeducatingthe
generalpopulationofthecity.
BACKGROUND
LeprosyisahumandiseasecausedbythebacillusMycobacteriumleprae(Figure
1).M.lepraeisanacidfastbacterium.Asoneoftheslowestgrowingbacteriaknown
anditsinabilitytogrowindependently,successfulinvitrocultivationhasneverbeen
achieved.Althoughfoundinthesamegenusasthetuberculosisbacterium
(Mycobacteriumtuberculosis),thetwodiseasescausedifferentsymptoms.
Pathology
ItishypothesizedthatM.lepraeinfectsanewhostbywayofskinorupper
respiratorytract,butmostexperimentssuggestthelatterasthemorelikelypossibility
(Shepard1960).M.lepraecausesachronicdiseaseoftheperipheralnerves,skinand

mucosalmembranesofthebodyandhasanincubationperiodofabout35years(Hart
2003).Ifinitialsymptomsareleftuntreated,thenpermanentdamagemayresultinmany
partsofthebodyincludingtheeyesandouterextremities.

Fig.1TheabovepictureshowstheM.lepraebacteriainhumantissue1.
Theseverityofthediseasecanexistonaspectrumofsymptomsdependingonthe
hostsimmuneresponse,geneticsandthenumberofbacteriathatinitiallyinfectthe
body.Intuberculoidorpaucibacillaryleprosy(PB),rashesdeveloponafewspotsofthe
body.Thesespotsareoftenflatandwhiteincolorwithacharacteristicnumbnessdueto
thenervedamagecausedbythebacteria.Themoresevereformofthediseaseisknown
aslepromatousleprosyormultibacillaryleprosy(MB)(Figure2).Theinfectedindividual
willoftendevelopsimilarrashestothosewithtuberculoidleprosyexceptthatthese

HansensDiseaseTreatment.NationalParkServiceWebsite.2008.Availableat:
http://www.nps.gov/kala/historyculture/hansens2.htm. Accessed: 20 May 2008.
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rashesareoftenraisedandmaybecharacterizedasbumps.Patientswiththisformof
leprosymayalsoexperiencenumbnessandweaknesstoentiremusclegroups.Thethird
formpresentsacombinationofsymptomsintermediatetobothtuberculoidand
lepromatousleprosy,hencethename,borderlineleprosy.Withouttreatment,thedisease
caneitherimprovetoamoretuberculoidformoradvancetothelepromatoussymptoms
(Leprosy:Merck2003).Skinrashesmaybecomepermanentandleadtosomecasesof

Fig.2PatientwithmultiplewhiterashesthatsuggestanMBleprosyinfection 2.
facedisfigurement.Furtherdamagetothenervesmayleadtosevereweakeningof
musclegroupcontrolofthehandsandfeetalsoknownasclawedhandsorfootdrop.
Itwasoncethoughtthatitwasthediseasethatcauseslimbstofalloff,however
thisisnotthecase.Asthediseaseprogresses,symptomsbecomemoresevere,leadingto
Smith,DScott.Leprosy.eMedicineWebsite.2006.Availableat:
http://www.emedicine.com/med/topic1281.htm.Accessed:19May2008.
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thelossofsensationoftheouterextremitiesduetoperipheralnervedamage.
Additionally,thepatientcannotfeelpainortemperaturemakingcutsandburnseasyto
gounnoticed.Constantdamagetotheseareasmayultimatelycausethelossoftheseparts
(Figure3).Infectioncanalsoaffecttheeye,andifuntreated,blindnessmayoccur.Feet
sores(Figure4)arealsocommonsymptomsaswellasdamagetothenasalpassages.
Diagnosisofleprosycanbeeasilydonethroughtherecognitionofthe
characteristicrashesandtheaccompanyingnumbness.Bloodtestsandtheculturingof
bacteriumareunsuccessfulduetotheinabilityofbeingabletoculturethesebacteriain
vitro.Duetothehighnumberofbacteriainlepromatousleprosyinfectedindividuals,
skinlesionandnasalsecretionsmearscanbetakenandviewedunderthemicroscope.In
alaboratorysetting,samplesofthebacteriacanbeamplifiedusingpolymerasechain
reactions(PCR)andM.lepraespecificprimerstoconfirmtheidentityofthebacterial
infection.

Fig.3Leprosypatientshandsthathavebeenrepeatedlydamagedduetolossof
nervesensationontheextremeties3.
Treatmenthasbeencomplicatedduetotheemergenceofdrugresistantstrainsof
M.leprae.Untilrecently,dapsonewasusedasthemaindrugtocureinfectionsofM.
leprae,howevertheWorldHealthOrganization(WHO)recommendstheuseofmulti
drugtherapy(MDT)especiallyforthosewiththeMBformofthedisease.Inadultswith
PBleprosy,therecommendeddosagesare600mgofRfampicinonceamonthand100
mgofDapsonedailyforadurationof6months.ForMBpatients,thedosageis
recommendedat600mgofRifampicinonceamonth,100mgofDapsonedaily,300mg

Smith,DScott.Leprosy.eMedicineWebsite.2006.Availableat:
http://www.emedicine.com/med/topic1281.htm.Accessed:19May2008.
3

ofClofazimineonceamonthand50mgofClofaziminedailyfor1year(WHO
recommended2008).Therearereportedsideeffectstothesedrugsandpatientsshouldbe
awarethatthesesymptomstypicallydiminishacoupleofmonthsaftertreatmenthas
stopped.

Fig.4Patientdevelopingafootsorecausedbythedamagefromleprosyinfection4.

Epidemiology
Verylittleisknownaboutthediseaseinitsnaturalhabitat.Itissuspectedthatthe
armadillomaybeareservoirforthediseasehoweverstudieshaveshownthatthebacteria
canalsoinfectnonhumanprimatesandmice.Leprosyisendemicworldwidehowever
mostoftodayscasesofleprosyinfectionsarefoundinthedevelopingcountries,withthe
highestprevalenceinIndia.Brazil,MyanmarandNepalalsocontinuetohavealarge

Smith,DScott.Leprosy.eMedicineWebsite.2006.Availableat:
http://www.emedicine.com/med/topic1281.htm.Accessed:19May2008.
4

Fig.5Mapoftheprevalenceofleprosyworldwidein20075.
numberofcases.Atthebeginningof2006,theWHOrecordedapproximately219,826
newcasesofleprosyworldwide,whichisadramaticshiftfrom1993whenthenumberof
newcasesthatyearwasabout590,933(Newcasedetection2005).InBrazil,there
were43,933newlydetectedcasesofleprosyin1998withatotalof72,953totalcases
(Cassandra,2002).Continuedpublichealtheffortshavedecreasedtheprevalence
worldwide,howevertherecontinuestobelocationsthathavenotbeenreached.
LeprosyandStigma

Leprosy:GlobalSituation.WorldHealthOrganizationWebsite.2005.Availableat:
http://www.who.int/lep/situation/en/.Accessed:18May2008.
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Oneofthesalientcharacteristicsofleprosyisitsassociatedstigmaandtheeffects
ithasonearlydetectionandadherencetotreatment.SinceMedievalEurope,leprosyhas
beenthecauseoftheoutcastofmanyofitscarriers,whichwereforcedintoleprosy
isolationcenters.Manycenturiesafter,incontemporaryBrazilthestigmaandbelief
systemsurroundingleprosyisstillverywellingrainedinsociety.Becauseourproposed
healthinterventionaimsatreducingthestigmaassociatedwithleprosy,itisimportantto
understandtheelementsofBrazilianculturethatinfluencepatientexperienceandshape
popularknowledgeandbeliefaboutleprosy.
InBrazil,thereanumberofcommonlyheldbeliefsaboutleprosy,particularlyin
thewayitistransmitted.Manypeoplebelievethatleprosyishighlycontagiousandthat
itcanbeacquiredthroughcasualcontact,fromhavingvisitedaleprosariumorfroma
sexualencounter.Otherbeliefsassociateleprosywithunsanitaryconditions,dogs,or
eatingfish.Leprosyisalsocommonlyknownasthefallingoflimpsdiseaseandis
thoughttobeincurable.Moreover,theCatholicchurchandtheEvangelicalreligions
stronglyreinforcetheideathatleprosyisaformofdivinepunishment.Ontheother
hand,AfroBrazilianreligionslookatleprosyastheresultofsorceryorwitchcraft(White
2002).Also,povertyincreasesthesusceptibilitytoleprosysinceahighlevelof
inequalityandpopulationgrowthgivesrisetoovercrowding,whichfacilitatesaerosol
transmissionofM.leprae.InBrazil,asignificantpercentageofleprosypatientslivein
favelas,orslums,thusperpetuatingtheassociationofleprosywithpovertyand
augmentingthestigmaassociatewiththiscondition(White2005).

Giventhenegativeconnotationsthatmanyofthefolkbeliefsaboutleprosycarry,
manypeopleareextremelyhesitanttoeitherseektreatmentoncetheysuspectleprosy,or
tocontinuetreatmentoncetheystartexperiencingsideeffectsthatmightexposetheir
conditiontothepublic.Thishasseriousmedicalimplicationsbecausethisisthemain
causeofmorbidityamongstleprosypatientssincethelossofsensationresultsin

Fig.6DuringorafterMDT,manyleprosypatientsexperiencealeprosyreaction,
whichisnotasideeffectbutthebodysownnaturalimmuneresponsetoMDT.
Nonetheless,thisreactionoftentimesdiscouragespeoplefromcontinuing
treatment6.
inevitableinjuriestothebody.Furthermore,stigmastronglyinfluencescompliancewith
treatment,especiallysinceMultidrugtherapyhasmanysideeffects,whichindicatethat
thepersonhasleprosy.ThedrugClofazamine,forexample,causestheskintobecome
darkeronindarkerpeople,itacquiresadarkerreddishcolor,whichiseasilyidentifiedas
leprosy.Forthesereasons,healthinterventionsthataddresstheissueofstigmacausedby

Leprosy:ManagementofComplications.WorldHealthOrganizationWebsite.2005.
Availableat:
http://www.wpro.who.int/sites/leprosy/treatment/treatment_complication.htm.Accessed:
18May2008.
6

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leprosyinaculturalspecificcontextwillbehighlyefficaciousindealingwiththefactors
thatprecludeearlyleprosydetectionandadherencetotreatment(White2007).
LeprosyControlPrecedence
Duetoleprosyshighassociatedmortalityandmorbidity,therehavebeena
numberofcollaborativeeffortsgearedtowardsitscontrolandabatement.Oneofthe
mostrecentstepstowardsthisgoalwasin1991,whentheWorldHealthAssemblycalled
foraglobalefforttoeliminateleprosyasapublichealthproblembytheendofthe
secondmillennium(Visschedijketal,2000).Eliminationwasdefinedasalevelof
prevalencebelowonecaseper10,000people.Oneimportantelementofthisworldwide
effortwastheLeprosyEliminationCampaigns(LECs),whichtrainedhealthworkersin
casefinding,educatedcommunitiesinordertoincreaseawareness,andperformedactive
casefindingandpatienttreatment.
Thesecampaignshadconsiderablesuccessinmanycountries,especiallysincethe
diminishingstigmaassociatedwithleprosyresultedinabetteroutlookforpatients(Naff,
2006).In1997and1998LECswereheldin29IndianStatesandUnionTerritories.
Morethan500,000healthworkerswereinvolvedand454,290newcasesweredetected
(Visschedijketal,2000).SimilarlyinNepalmorethan11,000newcaseswere
identifiedduringthe1998nationalcampaign,whiletheincidencenumbertheprevious
yearwasaround7,500(MinistryofHealthNepalDepartmentofHealthServices1998).
Furthermore,casestudieshaveshownthatcommunityhealtheducationprograms
thatfocusedonreducingstigmaandincreasingacceptanceofleprosyareeffectivein
promotingmorefavorableattitudestowardsleprosy.Forexample,inBangladeshlower

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levelsofprejudicetowardsleprosywereobservedinaruralcommunitywhichhad
receivedcommunityeducationascomparedtotheanotherruralcommunitywithoutthe
healtheducationprogram.Inthevillagethatdidnotreceivehealtheducation,over75%
ofthefamiliesreportedthattheywouldnotshareamealwithaleprosysuffererand94%
citedthattheywouldnotpermittheirsontomarryagirlwhoiscuredofleprosy(Wong
andSubramanian,2003).

Also,culturespecifichealtheducationprograms,suchastheoneimplementedin

Malaysiainthelate1980s,havebeenfoundtoincreaseacceptanceofleprosymessages
andimprovedthepublicsknowledgeandattitudestowardsleprosy.Othercountries
havebeenworkingwithtraditionalandreligioushealerstoprovideforleprosypatients,
mostlikelybecausemanypatientsconsultthemfirstbeforeseekingtreatmentfrom
westernhealthcaresystems.Infact,astudyinNigeriashowed59%ofthepatients
consultedthefolkmedicinesectorasthefirststepintheirhealthseekingroutine.
Moreover,useofthemassmediatochangeattitudeofthemassestowardsleprosy
increasecasedetentioninSriLankain1995(WongandSubramanian,2003).
OtherhealthinterventionstookplaceinIndia,whenaftertheadventoftheMulti
drugTherapy(MDT)in1982,theNationalLeprosyEradicationProgram(NLEP)was
launchedin1983withtheobjectiveofarrestingthediseaseinallknowncasesofleprosy.
However,coveragewithMDTremainedlowduetoarangeoforganizationalissues,and
afearofthediseaseandassociatedstigma(NCMH,2005).Nonetheless,othernational
effortsinIndia,suchasTheBombayLeprosyProject,whichwaslaunchedmorethan20
yearsago,havebeensuccessfulinusingtherapeuticmanagementforthepreventionof

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disabilities.Theprojectsadministratorsattributesitssuccesstoitsreductioninthe
socialstigmainthepatientsfamiliesandtheeducationofthecommunity(Ganapati,
2003).
Thesecasestudiesshedlightontheimportanceofreducingstigmaassociated
withleprosyinorderimprovetheeffectivenessofotherhealthinterventions.Innovated
approaches,liketheextensivetrainingofcommunityhealthworkers,needtobe
implementedtoimprovecommunityawarenessandparticipation.Inthismanner,
suspectedleprosycasesdonotfearstigmaandreporttoahealthfacilityattheearliestfor
timelydiagnosisandprompttreatment(NCMH,2005).
ProjectLocation
Becauseareductioninstigmaassociatedwithleprosywouldbeextremely
beneficialinanareawithhighleprosyendemicitysuchasLondrina,locatedinthestate
ofParanainSouthernBrazil,wechosethiscityasourprojectlocation.Londrinaisthe
secondlargestcityinthestateandcountswithabouthalfamillionhabitants.Most
importantly,descriptivestudyaimedtoevaluatetheleprosycontrolprograminLondrina
from1997to2001showedthattheseprogramslackedandfailedtoprovideinformation
aboutleprosy,andhadasignificantpatientdropoutrate(Barro,2004).Thereforeapilot
programwouldbehelpfulinaddressingtheweaknessesofprevioushealthinterventions
viaaculturalsensitiveapproach.

PROJECTDESIGN

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TheprojectwillinitiallybeginwithaspecialskinailmentclinicinLondrinaCity,
Brazil.Wewillgatherinformationbycreatingafocusgroupinordertogainabetter
understandingofcommonlyheldbeliefssurroundingleprosy.Fromthisinformation,the
initialgroupwillbeabletofinetunetheprojecttomeettheculturalneedsofthepeople.
Additionally,localhealthofficialsandvolunteerswillbetrainedindiagnosingand
prescribingthemedicationnecessarytotreatpatientssoastoensurethefuture
sustainabilityoftheprogram.
Clinic
Inordertoeliminatethestigmathatmightresultfromestablishingacliniconly
treatingleprosy,wewillopenaspecialskinailmentunitaimedatjoiningoneofthe
existingcommunityhealthcentersorhospitalsinthearea.Theclinicwillberunbythe
medicalpersonnelthathavealreadybeentrainedindiagnosingleprosy.Althoughleprosy
isnotaskindisease,itsearlyclinicalsignsconsistofsuperficialrashesofthedermis.
Thusbyopeningageneralskinclinic,patientscanfeelateasewhenseekingmedicalcare
withoutfearofgoingintoaclinicspecificforleprosy.Thisclinicwillprovidefree
diagnosesandtreatmentforthosewithleprosy.MDTisprovidedforbytheWHOatno
cost.
Tobetterintegratetheprogramintothelocalpublichealthservicesandto
encouragecommunityinvolvement,wewilltrainlocalhealthofficialsandvolunteersas
theprimarycaregiversfortheskinunit.Trainingwillbefeasibletobeachievedbecause
thediagnosisofleprosydoesnotrequirespecialtoolsorskills.Oftentimes,thedisease
canberecognizedthroughtheinitialskinrashesandlesionsandtreatmentcanbe

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prescribedthereafter.ThustrainingwillnotonlyinvolvetheWHOsPowerPoint
presentationonthediagnosisofleprosy,butalsoclinicaltrainingbyshadowingthegroup
ofhealthprofessionalandmedicalprovidersduringdailyrounds.
Bytheendofthisinitialpilotprogram,wehopetousetheinformationgathered
fromthefocusgrouptobettercatertowardstheneedsofthecommunity.Whatwewill
learnintheseinitial6monthsoftheprogramwillallowustobetteranswermisguided
questionsaboutleprosyandfurtherreducestigmabyreachingouttopeoplethroughout
theentirecity.Theinformationgatheredfromourresearchmayalsoallowusto
personalizeourteachingtechniquesallowingforamorethoroughimpactonthe
communityatlarge.Throughtheeducationandcontinuedtrainingoflocalvolunteers,
suchacampaigntoeliminateleprosycanbesustained.Inthefuture,wehopethatwe
willbeabletoexpandtheseskinailmentunitssothatpeoplewillhaveeasyandfree
accesstohavetheirleprosyinfectionsdiagnosedandtreated.

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