ByDr.S.L.Kate EmeritusMedicalScientist,DepartmentofPediatrics B.J.MedicalCollege,Pune,India October23,2000 Note:ThisarticleistakenfromImmunohaematologyBulletinwiththekindpermissionofDr.Kate.

HealthProblemsofTribalPopulationGroups fromtheStateofMaharashtra
S.L.Kate
Introduction Tenpercentofthetotalpopulationof theStateofMaharashtrabelongsto tribalpopulationgroups.These groupsremainisolated,livingin remoteforestandhillyareasfarfrom civilization.Majorityofthemhave poorhealthstatus,peculiarhealth needsandawideprevalenceofred bloodcellgeneticdisordersthat complicatestheirhealthproblems further.Moreover,theinadequate healthinfrastructureintribalareasto dealwithsuchcomplicatedhealth problemsisamatterofgraveconcern. Preamble Aspertherecentinformation availablefromtheGovt.sources, MaharashtraStaterankssecondin populationandareawisethirdinthe country.Asperthe1991census,the totalpopulationoftheStateis80 millionandthisis9.33percentof India'spopulation.Thetotaltribal populationintheStateis7.4million thataccountstoabout9.3percentof thetotalpopulationoftheState.

TherearethreemountainrangesintheStateknownasSahyadri,SatpudaandGondwanranges.Intotal, thereare47scheduledtribalpopulationgroupsintheStateandmajorityofthemareinhabitantsofthese geographicallydifficultterrain.[Note:"Scheduledtribalpopulations"isatermappliedbytheBritish colonialiststogroupsinIndiawhobyandlargesuccessfullyresistedtheircontrolinthe19th century. "Clans"mightbeamoreappropriateterm.KRB]Outofthese47groups,17aremajortribalgroups.Inthe SahyadrirangestherearetheMahadeoKoli,Katkari,Warli,MalharKoliandKokanagroups.Among Satpudaranges,Bhil,Pawara,KorkuandTadviarethemajorgroups.TheMadia,Gond,Pardhan,Halbi Otkar,andAndhaarefoundintheGondwanrange. Thesetribalgroupsdifferfromeachotherinvariousaspects.Theydifferinthelanguagetheyspeak,in theirculturalpatternandsocioeconomiccategories.Asthemajorityofthesetribalslivingintheremote forestareasremainisolated,untouchedbycivilization,theyarelargelyunaffectedbythedevelopmental processesgoingonintherestoftheState.Therefore,thesegroupsremainbackward,particularlyinhealth, educationandsocioeconomicaspects.Weareworkinginthefieldoftribalhealthforthelastseveralyears andthehealthproblemsofthetribalpopulationintheStatecanbesummarizedasfollows: 1. Deficiencyofessentialcomponentsindietleadingtomalnutrition,proteincaloriemalnutritionand micronutrientdeficiencies(vitA,ironandiodine)arecommon.Goitreofvariousgradesisalso endemicinsomeofthetribalareas. 2. Waterborneandcommunicablediseases:Gastrointestinaldisorders,particularly dysenteryandparasiticinfectionsareverycommon,leadingtomarkedmorbidityandmalnutrition. Malariaandtuberculosisstillremainaprobleminmanytribalareas,whilethespectrumofviraland venerealdiseaseshavenotbeenstudiedindepth. Highprevalenceofgeneticdisordersmostlyinvolvingredbloodcells:Geneticallytransmitted disorderslikesicklecellanaemia,glucose6phosphatedehydrogenasedeficiencyanddifferentforms ofthalassaemiaarealsocommon.Allthesedefectsleadtotheearlydestructionofredbloodcellsand addtotheoverallanaemia. Excessconsumptionofalcohol:ThebrewingofalcoholfromMohuaflowerandfruitshasbeen practicedtraditionally.However,theswitchovertocommerciallyavailableliquorislikelytobea majorthreat. Superstitionsparticularlyrelatedtohealthproblems. Extremepoverty. Theinadequatehealthinfrastructureforthesepeculiarhealthneedsofthetribalsisalsoamajorfactor. Thereremainsaconspicuouslackofmaternalandchildhealthservicesamongthehillytribalareasand consequently,thetribaldemographicscenarioisoneofhighfertility,highmaternalandinfantmortality rates.ThedatapublishedbythePublicHealthDept.oftheState(Table1)showsthehealthsituationin tribalareas.

3.

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5. 6.

Table1:HealthStatusIndicatorsinTribes Indicators Infantmortalityrate Amongthegeneticproblems,we haveconductedandindepthstudy onsicklecelldisorders.Best Crudedeathrate 7.9 13 examplesofgeneticpolymorphism Maternalmortality 2 Notavailable clearlyconnectedwithdiseaseare rate sicklecellanemia,thalassemiaand LBWbabies 28% 40% glucose6phosphatedehydrogenase FamilySize 3.8 4.2 deficiency.Thesearesinglegene defectsoftheblood,totallyconfined DeliverybyTBA 86% 12% toredbloodcells.Sicklecell anaemiaandthalassemiaarethemajorpublichealthproblemsinourcountry(1,2,4,6,9,10).Thalassemiais prevalentamongstallpopulationgroupsirrespectiveofcaste,religionandcreed.However,sicklecell disorderismostlyconfinedtosocioeconomicallybackwardgroups,likescheduledcastes(SC),scheduled tribes(ST)andnomadictribes(NT). 59 110 SickleCellDisorder Thisisahereditaryblooddisorderduetodefectivehemoglobinstructure.Hemoglobinisatetramer consistingoftwopairsofnonidenticalglobinandpolypeptidechains,eachchainbeingassociatedwith onehemegroup.Thehemegroupisanironcontainingpigmentresponsibleforoxygentransportinred bloodcells.Therearefourpolypeptidechains(alpha,beta,gammaanddelta)intheglobinportion.HbA consistsof2alphaand2betachainsHbFconsistsof2alphaand2gammachainsandHbA2consistsof2 alphaand2deltachains.Thealphachainsconsistof141aminoacidswithspecificsequences,whilebeta, gammaanddeltachainsconsistof146aminoacids.Thesequencesofaminoacidsinthealpha,beta, gammaanddeltachainsaredifferentfromeachotherandveryspecificforthatchain.Sicklecell hemoglobinsicklingandhencethemutanthemoglobiniscalledsicklecellhemoglobin(HbS). TheGeneticsofSickleCellHemoglobin Itiswelldocumentedthatthegeneforsicklecellhemoglobinislocatedontheshortarmofchromosome11 andhasanautosomalrecessiveinheritance.Hence,itcanmanifestintwoformsviz.heterozygous(carrier) andhomozygous(sufferer).Whentwocarriersmarry,thechanceofhavingahomozygouschildis25% everypregnancy. Diagnosis Diagnosisofsicklecellcarriersandsufferersispossiblebycarefulclinicalexaminationofthepatient supportedbylaboratoryinvestigations.FordevelopingcountrieslikeIndia,simple,rapidandinexpensive yetreliablelaboratorytestsarenecessary,particularly,whenscreeninghastobecarriedoutonlarge populationslocatedingeographicallydifficultareas.SuchtechniqueshavebeendevelopedinourDept. thataresuitableforfieldworkandrequireminimumamountofblood(onlyafewdrops).Theresultsare availablewithinhalfanhourandthetestisverycosteffective.Thetestsinclude: Situationin Maharashtra TribalSituation

 1. Solubilitytest 2. ElectrophoresisatalkalinepHusingcelluloseacetatemembraneassupportingmedium. Duringthelasttenyears,weconductedpopulationgeneticsurveysindifferenttribalareasofMaharashtra usingtheserapidcosteffectivelaboratorytechniques.Theprevalenceofsicklecelldisorder(carrierstate)is shownintable2. PopulationgeneticsurveydatafromourDept.indicatesthattheprevalenceofsicklecelldisorderinthe overallpopulationofMaharashtraislessthan0.1%whileitisveryhighamongstthetribalpopulation groupsfromNandurbarandGadchirolidistrictsoftheState.Thesametribalpopulationgroupsresidingin theneighboringstatesofGujarat,MadhyaPradeshandAndhraPradeshhaveasimilarprevalence.Our epidemiologicaldatasuggests: 1. PrevalenceofsicklecelldisorderisveryhighamongtribalpopulationgroupsBhilandPawarafrom NandurbarDist.andamongsttheMadia,PardhanandOktarpopulationfromGadchiroliDist.I.e.20 20%. 2. ThehighestrecordedprevalenceisamongtheOktargroupi.e.35%. 3. ThesicklecellgeneispracticallyabsentamongtheMahadeoKoli,Thakarandothertribalgroups fromWesternMaharashtra. 4. Theoverallprevalenceamongtribalpopulationsisabout10%forthecarrierstateand0.5%for sufferers. Table2:Summaryofthedistributionofsicklecelltrait amongdifferenttribalgroupsofMaharashtra Sr. No 1 2 3 4 5 6 7 8 9 10 Tribal Group Otkar Pardhan Pawara Madia, Gond Bhil Halbi Rajgond Korku Tandvi District SickleCell Carriers(%) Gadchiroli Nanded,Yeotmal Dhule,Jalgaon Gadchiroli,Yeotmal Nandurbar Gadchiroli Gadchiroli Amravati Jalgaon 35 33.7 25.18 20.8 20.6 13.93 13.88 10.88 9.49 8.33 SickleCellAnemia Alltheclinicalcomplicationsrecordedin theliteraturearefoundamongpatients fromtribalpopulationgroups.The clinical,molecularandgeneticaspectsof sicklecelldiseasehavebeenreviewed (7).Althoughsomegeneralizationabout thegenotypephenotypecorrelationmay bemade,sicklecellpatientssharing identicalgenotypesexhibitconsiderable heterogeneityinclinicalsymptoms.Some ofthecommonsymptomsseeninpatients sufferingfromsicklecellanaemia include:

MalharKoli Thane

11 12 13 14 15 16

Kolam Warli Katkari Kokana Andha Mahadeo Koli

Yeotmal Thane Pune,Raigad,Ratnagiri Dhule,Nasik Nanded Pune,Nasik

8.33 8.04 5.90 3.50 1.97 0.81

*Anaemia *IntermittentJaundice *Severejointpains *Recurrentinfections

Thesesymptomsusuallyappearbetween theagesoffourandfiveandseverity 17 Thakur Pune,Thane,Raigad, 0.00 increaseswithage.Mostofthepatients Ahmednagar donotrequirebloodtransfusion.Careful history,presenceoftheabovementioned symptomsandcasteconstitutebasisfordiagnosisofsicklecellanemia. Treatment Nogeneticdiseasecouldbesimplerthansicklecellanaemia.Itisasimplediseasebutthereisnocure.In theabsenceofanycure,majorityofthesicklecellpatientshaveamiserableandshortlifespan.Instancesof suddendeathhavealsobeenreportedinsuchcases.Theavailabletreatmentstrategiescanbebroadly dividedintofivecategories:antisicklingagents,vasoactivedrugs,enhancingfetalhemoglobinproduction, bonemarrowtransplantationandgenetherapy.Unfortunatelyneitherthediagnosticnortheabove mentionedtreatmentfacilitiesareavailableintribalareasandallthesearebeyondtheirreach.Althoughthe tribalhealthproblemisgrave,theHealthDepartmentisunabletoprovidethemwithanyhealthcarefor severalreasons.Teachersandmedicalstudentswhohavesufficientknowledgeaboutthesubjectaremost unwillingtoworkintribalareas.Ultimately,thepatientsapproachtraditionalhealersknownas"Bhagat"in whomtheyhavecompletefaithandconfidence.Sincethereisnospecifictreatmentavailableforthe disease,theonlyalternativeisprevention.Therefore,wecanundertakesuchpreventativemeasureslike healtheducationandgeneticcounseling.StudiescarriedoutinCyprus,GreeceandtheUKforsimilar typesofdiseasesusingsuchpreventativestrategieshaveshownpromisingresults(5).Apreliminaryreport onthepreventionofsicklecelldisorderinIndiaiscited(3,8). SickleCellDisorderandtheStateofMaharashtra Studiescarriedoutbyourcentreandotherscientistsfromdifferentinstitutionsindicatethattheoverall prevalenceofsicklecelldisorderindifferenttribalpopulationsis10%forcarrierstateand0.5%forthe sufferer.ThetotaltribalpopulationfromtheStateofMaharashtraaspertherecentGovt.recordis90lakhs attheendofthe20th century[Note:lakhequals100,000.KRB].Basedonthisnumber,theexpected carriersofsicklecellwouldbe9lakhsandexpectednumberofsicklecellhomozygoteswouldbe45,000. TheprevalenceisveryhighamongtheBhilandPawaratribalgroupsfromtheNandurbarDistrictandthe Madia,Pardhan,OtkarandGondfromtheGadchiroliDistrict.Itisestimatedthattherearemorethan 10,000sicklecellpatientsintheNandurbarDistrictitself.TheGadchiroliDistrictisexpectedtohavemore than5,000.Takingintoconsiderationtheproblemofsicklecelldisorder,theIndianCouncilofMedical Research(ICMR)launchedapreventativeprogramonatrialbasisamonghighrisktribalpopulationsinthe StateofMaharashtra.Weestablishedtwocommunitycontrolprogrammecentres,oneintheNandurbar DistrictandtheotherintheGadchiroliDistrict.ThemajoractivitiesoftheCentresare:

i. ii. iii. iv. v. vi. vii.

Providediagnosticfacitlitiesforsicklecelldisorderandothergeneticdefects. Followupofsicklecellanemiapatients. Carryoutpopulationgeneticsurveyprogramstodetectcarriersandsufferers. Healtheducation. Geneticcounselling. Marriagecounselling. Provideprenataldiagnosiswheneverrequired.

AsummaryoftheworkisshowninTable3. Table3:SicklecelldisorderfromNandurbarandGadchiroli Centres District Taluka Totalpopulation screened Totalsufferers detected Prevalenceofsickle celldisorder Totalpopulationof thedistrict Expectedcarriers Expectedsufferers Nandurbar Dhadgaon 4116 Gadchiroli Aheri 6 2912(including SC/ST) 525 37 20% 47,000SC/ST 94,000 >5,000 Eventhoughprevalenceisveryhigh inthedistrict,parents,patientsand privatemedicalpractitionershave verylimitedinformationaboutthe disease.Wehaddetaileddiscussions withtheparentsofsickle homozygouschildren.About50%of theparentsshowedkeeninterestin thesubject.Whenprovidedwiththis information,theyweresurprisedto knowthattheycouldhaveavoided suchsufferingsoftheirchildren. Withthisbackground,weorganized severaldiscussions,meetingsand arrangeddemonstrationsinvillages onsicklecelldisease.

Totalvillagesselected 8

Totalcarriersdetected 824 44 21% 1,00,000onlyST 2,10,000 >10,000

Oursurveyshowedthatinevery villagethereareatleastoneortwopatientssufferingfromsicklecelldisease.Peopleofthevillagewere awareofthesufferingofthesepatientsandhadadeepsenseofsympathyandcompassionforthem.We explainedtothemaboutthehereditarynatureofsicklecelldiseaseandthatnocurativetreatmentis availablealthoughitcanbeprevented.Thishadagoodimpactonthegeneralpopulationandwasthemajor beginningforcreatingcommunityawarenessforthecounsellingprogramme. Wedistributedidentitycardsgivingthesicklecellstatustoeveryindividualandexplainedtheconceptof marriagecounsellingtopreventthedisease.Theyalsoreadilyacceptedtheconceptoffamilyplanningto preventthebirthoffurtheraffectedchildren.Somesuchparentshavenowbecomecounsellorsforsickle cellanemiaintheirvillages. Wehadalsoorganizedseverallecturesforschoolandcollegestudentslivinginthehighriskareas, explainingtothemtheprinciplesofgenetics,modeofinheritance,highprevalenceofsicklecelldiseasein tribalareas,givingthemdetailedinformationonsicklecelldiseaseincludingcontrolandtreatment.The studentsweretestedfortheirsicklecellstatusandwegavethemcounsellingcardsexplainingtothemthat

marriagecounsellingistheonlysolutionforthedisease.Allmalestudentscollectedthecardsbutabout 20%ofthefemalestudentsdidnotturnupeventocollectthecards,infearthatincasetheywerecarriersor sufferersofsicklecell,itmaybecomeknowntoothers.Thiswasdespiteourpromisetokeeptheirsickle cellstatusconfidential. Simultaneously,weorganizedlecturesonsicklecelldisorderforthelocalGovt.andprivatemedical practitioners,includingparamedicalworkers,explainingtothemabouttheprevalence,diagnosticmethods, possibletreatment,complicationsandpreventionaspectsofthedisease.Althoughwecarriedoutthese activitiesforalimitedperiod,ourexperiencesuggeststhatwehavedefinitelycreatedawarenessamong peopleaboutsicklecelldisordersintheareaswherewehaveestablishedcommunitycontrolprogrammes. However,itisnecessarytocontinuewithsuchactivitiesandwithourexperiencewearehopefulthatwith devotionfrommedicalscientistsfrommultidisciplinaryfields,peoplewillacceptmarriagecounselling programmes. References 1. BalgirRS.,SharmaPK:DistributionofsicklecellhemoglobininIndia.Ind.J.Hematol6:1(1988). 2. BhatiaHM.,RaoVR:InGeneticAtlasofIndianTribes.ICMRPublication,Mumbai(1988). 3. GangakhedkarRR:Healtheducationinsicklecelldisease.ImmunohaematologyBulletin(ICMR): 20(6)1(1989). 4. KateSL.,PhadkeMAetal:Impactofgeneticdisordersonhealthproblemsamongtribalpopulation groupsofMaharashtra.In:AnOverviewofTribalResearchStudies.TribalResearchInstitute11:99 (1995). 5. MichaelA.,SophiaK.,MinagH:HowThalassemiawascontrolledinCyprus.WorldHealth Forum,7:291(1986). 6. ModelBC.,PetrouMTheproblemofhemoglobinopathiesinIndia.Ind.J.Hematol.1:5(1983). 7. MohantyD:SickleCellAnemiaTheIndianScenario.Ind.J.Hematol.16,1:1(1998). 8. PandePL.,ChaudhuriNK.,JainDC:AwarenessregardinghemoglobinopathiesamongGondsof Jabalpur.In:TribalHealthBulletin5(2):36(1999). 9. RaoVR:GeneticsandepidemiologyofsicklecellanemiainIndia.ImmunohaemtologyBulletin 11(1):39(1988). 10. RaoVR.,GorakshakarAC:Sicklecellhemoglobin,betathalassemiaandG6PDdeficiencyintribes inMaharashtra.Genegeography4:131(1990).