rev bras hematol hemoter. 2015;37(1):5–6
Revista
Brasileira
de
Hematologia
e
Hemoterapia
Brazilian
Journal
of
Hematology
and
Hemotherapy
w w w . r b h h . o r g
Scientific
Comment
The
invisibility
of
sickle
cell
disease
in
Brazil:
lessons
from
a
study
in
Maranhão
夽
Marcos
Borato
Viana
∗UniversidadeFederaldeMinasGerais(UFMG),BeloHorizonte,MG,Brazil
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Articlehistory:
Availableonline20November2014
Sickle cell disease (SCD) is a genetic disorder in which hemoglobinS (Hb S) predominatesin red blood cells.It is consideredasignificantpublichealthissueinBrazil.1–3
Sicklecellanemia(SCA,HbSS)isthemostcommon sub-type of SCD in the world. Although its clinical course is variable,patientswithSCAgenerallyhave themostsevere phenotype.SCDalsoincludestheheterozygouscombination ofHbSwithotherhemoglobinvariants(HbSC,HbSD-Punjab, andothers).ThecombinationofHbSwiththalassemia(Hb S/0andHbS/+thalassemia)leadstoothersubtypesofSCD withavariable relative incidencedepending onthe ethnic compositionofthepopulation.4,5
Therelative death rate due tohemoglobin disorders in underfive-year-oldchildrenallovertheworldisreportedtobe 3.4%ofalldeaths.6Morbidityandmortalityareespeciallyhigh indevelopingcountries.7Evenindevelopedcountries,SCDis stillasignificantcauseofmortality,particularlyinadolescents andadults.8–10
Thereareonlytwonewborn-screeningcohortstudies in Brazil,whichhavereportedthedeathrateforchildrenwith SCD.In bothstudies,it wasvery high comparedtofigures reportedindevelopedcountries.InMinasGerais,3thecrude death rate for 1396 children (all subtypes) diagnosed in a seven-year period was 5.6%. The Kaplan–Meier estimated probabilityofdeathatfiveyearsofageforchildrenwithHbSS orHbS/0thalassemiawas10.6%(standarderror:1.4).InRio
DOIoforiginalarticle:http://dx.doi.org/10.1016/j.bjhh.2014.11.009.
夽
SeepaperbyLimaetal.onpages12–16.
∗ Correspondenceto:DepartamentodePediatria,UniversidadeFederaldeMinasGerais(UFMG),Av.AlfredoBalena,190,sala267,30130-100
BeloHorizonte,MG,Brazil.
E-mailaddress:vianamb@gmail.com
deJaneiro,1thecrudedeathratefor912children(allsubtypes) inaten-yearperiodofnewbornscreeningwas4.2%.Themain causesofdeathinbothcohortswereinfection(includingacute chestsyndromewhichinchildrenisindistinguishablefrom pneumonia)andacutesplenicsequestration.
Unfortunately,thecrudedeathrateforthenewborncohort inMinasGeraishasnotsignificantlydecreasedovertheyears. InarecentreportthatwillbepublishedintheJornalde Pedi-atria (RJ),11 the deathrate in the last seven-yearperiodof observationwas5.12%comparedto5.43%(p-value=0.72)in thefirstsevenyearsofthestudy.
In this issue of the Revista Brasileira de Hematologiae Hemoterapia(RBHH),Limaetal.12analyzetrendsinmortality andhospitaladmissionratesforpatients(notonlychildren) withSCDina14-yearperiod,comparingthedatabeforeand after the introduction of a newborn screening program in Maranhão,anortheasternstateinBrazil.Thetotalnumber ofrecorded hospitaladmissionsincreased from 128in the firstseven-yearperiod(‘pre-newbornscreening’–1999–2005) to 840 in the ‘post-newborn screening’ period (2006–2012). Therateofhospitalizationrelativetothetotalpopulationin Maranhãoincreasedfrom0.315(pre)to1.832(post)per100,000 persons,indicatingaratio5.82timeshigherandshowinga growth in trend(p-value=0.04). The medianage at admis-siondroppedfrom 11.4yearsto8.7years(p-value=0.0002). Themortalityrateincreasedfrom0.115to0.216,1.88times
http://dx.doi.org/10.1016/j.bjhh.2014.11.001
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rev bras hematol hemoter. 2 0 1 5;37(1):5–61200
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1998 2001 2004 2007 2010 2013
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Figure1–Hospitaladmissionsforchildrenandadultswith themaindiagnosisofsicklecelldiease(CIDD57)inMinas Gerais,Brazil,from1999to2012(n=8028).
higher(p-value=0.59 – non-significant).Themedianage at deathincreasedfrom tenyearsto14 years(p-value=0.67– non-significant).Inconclusion,theauthorsstatethat“thekey reasonfortheapparentparadoxofincreasedmortalityand hospitalization rates after the implementation of neonatal screeningistheincreased‘visibility’ofsicklecelldisease”.
TheinvisibilityofSCDisalsoevidentwhentheauthors comparethehospitalizationrateforSCDinMaranhão(1.832) withthoseinBahia(1.8),SãoPaulo(6.0), andRiodeJaneiro (7.0).13 Considering the relative proportionof Black people inthe totalpopulation,Limaetal.haveestimatedthatthe expectednumberofpatientsshouldbeverysimilarinthese states(9–11thousand)andsothehospitalizationrateshould beaboutthesame,whichisempiricallynottrue.Inthis com-parison,thelowerhospitalizationrateforMaranhãoandBahia relativetoRiode Janeiroand São Pauloisdue notonlyto under-reportingofpatients(the‘invisible’disease),butalso, probably,tolowerlevelofhealthcareforpatientswithSCDin thesepoorerstatesdemonstrating“thesocialdifferencesthat existbetweenregionsinBrazil”.12
In MinasGerais, we have observed two other pieces of evidence of the invisibility of SCD. In the aforementioned study,11theinclusionofthewordsickle(“falciforme”)onthe deathcertificatesofchildrenwhowereknowntobepatients with SCD sincebirth (they tested positive inthe newborn screeningandhadbeenfollowedupintheFundac¸ãoCentro deHematologiaeHemoterapia deMinasGerais– HEMOM-INAS) increasedfrom the incredible figure of42.1% in the firstseven-yearperiodtoastilllowfigureof60.5%inthelast period.SimilartothedataofMaranhão,wehaveobserveda steepincreaseinhospitaladmissions(n=8028)forchildren and adults registered in the Hospital Information System (SIH)oftheBrazilianNationalHealthService(SUS)from1999 to2012(datanotpublishedyet–Figure1).14
Inconclusion,continuouseducationalprogramsdirected tohealthprofessionalsandtofamiliesandpatientswithSCD shouldbeboostedinordertoincreasethe‘visibility’ofthe dis-easeandtodecreasethemortalityandmorbiditycausedbyit. Also,aswehavepreviouslystated,3“the[Brazilian]Ministry ofHealth’sprogramtoprovideintegratedhealthcarefor peo-plewithSCDisanidealtowardwhichpatients,theirfamilies andtheprofessionalsinvolvedintreatingthemmustworkin ordertoachievetheobjectiveofimprovingthecurrentliving conditionsandhealthstatusofthesepeople”.
Conflicts
of
interest
Theauthordeclaresnoconflictsofinterest.
Acknowledgments
Theauthor thanksGabrielaRicardode AquinoSantos, Fer-nandaAraújoAvendanha,GabriellaOliveiraLimaeAnaPaula Pinheiro Chagas Fernandesfor havingsharedoriginal data preliminarydisplayedattheXXIIISemanadeIniciac¸ão Cien-tíficadaUFMG(reference#14).
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