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BrandiPernell,DNP,PNP-BCChristyBemrich-Stolz,MD,MSPH
UniversityofAlabamaatBirminghamDivisionofHematology/Oncology
October20,2017
TransitiontoAdultCareinPatientswithSickleCellDisease
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
SicklecellinAlabama
• Mostcommonhemoglobinopathy inAL– 1/12AfricanAmericanswithsicklecelltrait– 1/350AfricanAmericansarebornwithSCD
• Screeningdonebyhighperformanceliquidchromatography(HPLC)onheelstick
• Hemoglobinopathy screeningstartedin1988
Children’sSickleYouthNetwork(CYSN™)
• Multidisciplinaryteam:hematologists,nursepractitioners,nurses,socialworkers,psychologist,schoolliaison
• Providescareformorethan1000patientswithsicklecelldisease(SCD)inAlabama
• Manages>80patients/monthonchronictransfusion
Children’sSickleYouthNetwork(CYSN™)CatchmentArea
Children’sSickleYouthNetwork(CYSN™)StandardsofCare
• Infectioncontrol(prophylacticpenicillin)• Annualscreeningforriskofstroke(transcranialdoppler)
• Patient/familyeducation• Hydroxyureatherapy• Chronicbloodtransfusiontherapy(UAB)• Screeningforcommoncomorbidities:asthma,kidneydisease
• Transitionreadiness
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
WhatisSickleCellDisease?
• Anautosomal recessiveredbloodcelldisorder
• PrimarilyaffectsindividualsofAfricandescent
• Characterizedby:o earlyredbloodcellhemolysis - ->anemiao widespreadinflammationo recurrentepisodesofpaino riskforinfectionandend-organdamage
Pathophysiology ofSCD
CommonSCDcomplications
MortalityinSCD• 1973autopsyreview:estimatedmediansurvivalof14.3y• CooperativeStudyofSicklecelldisease(CSSCD)1979-87
(Leikin),deathratehighestin1-3yo,probabilityoflivingpast20yo85-95%
• PlattCSSCDfollowupin1994:medianageofdeath42yformale,48yforfemale
• Lanzkron(2013):improvedmortalityinchildrenwithSCDwithincreasingmortalityinadults1979-2005
DiggsLM,AnatomiclessonsinsicklecelldiseaseinSicklecellidsease:diagnosis,management,educationandresearch1973Leikin SLetalMortalityinchildrenandadolescentswithsicklecelldiseasePediatrics1989PlattetalMortalityinsicklecelldisease:-Lifeexpectancyandriskfactorsforearlydeath.NEJM1994Lanzkron MortalityratesandageatdeathfromsicklecelldiseaseUS1979-2005Publichealthrep2013
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
MostcommoncomplicationsaffectingadolescentsandyoungadultswithSCD
• Stroke• Ironoverload• Chroniclungdisease• Pulmonaryhypertension• Chronickidneydisease• Venousthromboembolism
Potentialimpactofastrokeduringearlyadulthood
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
Transition≠Transfer
WhatisTransition?• “Purposeful,plannedmovementofadolescentswithchronicmedicalconditionsfromchild-centeredtoadult-orientedhealthcare”
• Goalofmaximizing“lifelongfunctioningandpotentialthroughtheprovisionofhigh-quality,developmentallyappropriatehealthcareservicesthatcontinueuninterruptedastheindividualmovesfromadolescencetoadulthood.”
Blum R. W. Introduction. Improving transition for adolescents with special health care needs from pediatric to adult-centered health care. Pediatrics. 2002;110(6 Pt 2):1301-1303.A consensus statement on health care transitions for young adults with special health care needs. Pediatrics. 2002;110(6 Pt 2):1304-1306.
WhyistransitionimportantinSCD?
• 93%ofchildrenwithsicklecelldiseasewilllive18yearsold
• Increasedmorbidity
• Increasedriskforprematuremortality
ImbalancebetweennumberofadultsprovidersandnumberofadultswithSCD
AdultprovidersforSCD(Primarycare,Hematologists)AdultswithSCD
WhyistransitionimportantinSCD?• 2012Dickerson:Youngadultswithsicklecellanemiahave:– TwicethenumberofERvisitsandinpatientstaysperyearthanthose10-17yoor31-45yo
– Highestpercentageoffrequentacutecarevisits(>10visitsperyear)andreturntoacutecarewithin14days
• 2010Quinn:WithinDallasNewborncohort,mostdeaths(6/7)inpatients18-23yooccurredwithin2yearsoftransitiontoadultcare.
Dickerson A. K., Klima J., Rhodes M. M., O'Brien S. H. Young adults with SCD in US children's hospitals: Are they different from adolescents? Pediatr Blood Cancer. 2012.Quinn C. T., Rogers Z. R., McCavit T. L., Buchanan G. R. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115(17):3447-3452.
WhyistransitionimportantinSCD?
• Hamideh 2013:“Mortalityratesinpatientswithsicklecelldiseaseincreasedfrom0.6inthe15-19yeargroupto1.4/100,000inthe20-24yeargroup”
• Blinder2013:“Patientstransitioningto adult care receivedlesstransfusionsandhydroxyurea,lessironchelationtherapy,hadhigherhealthcarecostsandsufferedfrommorefrequentSCDrelatedcomplicationsthanpediatricpatients.”
Hamideh. Sickle cell disease related mortality in the US 1999-2009 PBC 2013Blinder. Age related treatment patterns in sickle cell disease patients and the associated sickle cell complications and healthcare costs. PBC 2013
SCDQualityGuidelines(Wang2011)
AdolescentswithSCD:• Shouldhavea“transitionplantoadultcareincludingawrittentransfersummaryofmedicalhistory”
• Shouldreceivecounselingregardingthetransitionprocess
HealthPeople2020Goal(Dept.ofHealthandHumanServices)
Increasetheproportionofyouthwithspecialhealthcareneedswho’shealthcareproviderhasdiscussedtransitionplanningfrompediatrictoadultcare
GotTransition
• AgreementbetweentheMaternalChildHealthBureauandthenationalalliancetoadvanceadolescenthealth
• Advocatesforsixcoreelementsofhealthcaretransition
Sixcoreelementsoftransition1. Transitionpolicy
– Developpolicy– Educatestaffonapproach– Postpolicyanddiscusswithyouthandfamilies
2. Transitiontrackingandmonitoring– Developdatabaseandusetotrackprogressofpatients
3. Transitionreadiness– Conducttransitionreadinessassessmentsbeginningatage14
– Developgoalsandactionsanddocument
TransitionReadinessAssessmenttemplateforSCD-DISEASEKNOWLEDGE
AmericanSocietyofHematology
IknowwhattypeofsicklecelldiseaseIhave.Iknowmymedicalneedsandcanexplainthemtosomeone.IknowwhatahematologistisandwhyIgotoone.Iknowwhattodoincaseofamedicalemergency.Iunderstandwhatcausesapainepisode.Iunderstandhowdrugs,alcoholandtobaccoaffectsicklecelldisease.IhavefriendsthatIcantalktoaboutsicklecelldisease.Iknowaboutnecessaryscreeningexams(echoannually,kidneyfunctionannually,retinalexams,etc.).Iknowhowtogetbloodworkandx-rays.
Iknowwhatmymedicationsarefor.Iknowthenamesanddosesofmymedications.Iremembertotakemymedicationswithoutmyparentremindingme.IfillprescriptionsbeforeIrunoutofmedications.Iamawareofwhathydroxyureaisandhowitpreventssicklingofmyredbloodcells.IknowhowtopreventapainepisodeandwhattodoifIhavepain.
TransitionReadinessAssessmenttemplateforSCD-MEDICATIONMANAGEMENT
AmericanSocietyofHematology
Imakemyowndoctors’appointments.Iknowhowtogetmedicalcarewhenthedoctor’sofficeisclosed.IfilloutmyownmedicalhistoryformIkeeptrackofmyownmedicalinformation.Ikeeptrackofmydoctors’andotherappointments.Imakealistofquestionsbeforemyvisitwithmydoctors.Ianswerquestionsonmyownduringmedicalvisits.Iarrangemyowntransportationtomedicalappointments.
TransitionReadinessAssessmenttemplateforSCD-APPOINTMENTS
Icarrymyowninsurancecard.Iunderstandmyinsuranceplan.
TransitionReadinessAssessmenttemplateforSCD
INSURANCE
PRIVACYINFORMATIONIunderstandhowhealthcareprivacychangesatage18,whenIamlegallyanadult.
Sixcoreelementsoftransition
4.Transitionplanning• Developandupdateplanofcare• Prepareforadultapproachtocareat18yoincludinglegalchanges
• Determineneedfordecisionmakingsupport• Planwithpatient/parenttimingoftransfer• Assistyouthinidentifyingadultprovider• Provideinsuranceandselfcareresources
Sixcoreelementsoftransition
5.Transferofcare� Confirmdateoffirstadultprovidercontact� Transferwhenpatient’sconditionisstable� Completetransferpackage� Continuetoseeinpediatricsettinguntilyoungadultisseenintheadultsetting
Sixcoreelementsoftransition
6.Transfercompletion� Contactyoungadult3-6mpost-transfertoconfirmtransferandobtainfeedback
� Communicatewithadultpracticeandofferconsultationassistanceasneeded
� Buildcollaborativepartnershipswithadultprimaryandspecialtycareproviders
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
EXPLORINGADULTCAREEXPERIENCESANDBARRIERSTOTRANSITIONINADULTPATIENTSWITHSICKLECELLDISEASE
Bemrich-stolz CJ,Halanych JH,HowardTH,HilliardLM,LebensburgerJD.ExploringAdultCareExperiencesandBarrierstoTransitioninAdultPatientswithSickleCellDisease.Int JHematol Ther.2015;1(1)
StudyMethodology
• Patients>18yofromUABsicklecellclinic(nolimitongenotype)
• Semi-structuredinterviews– Timingandpreparationfortransfer– Developmentofindependence– Feelingsaboutandexperiencesinpediatricandadultcare
• ReviewandcodingoftranscriptsbyCBandJL
Patientdemographics
• 10participants• 24-55yo• Allpatientshadinsurance• 9/10ondisability• 9/10HSgraduates
Pre-transitionconcernsLeavingcomfortablepediatricsetting[10,11,14,15]
Goingtoadoctorunfamiliarwithtreatingsicklecell
disease[11]
Willmyparentsallowpatientstobecome
independent[11,15]
HowwillIpayformedicaltreatment[10]
WillIbetreatedasanadult[10]Willadultprovidersunderstandhowsicklecelldiseaseaffectsmeasanindividual[10,14]
LivingwithSCDasanadult
LivingwithSCDasanAdult–ThemesIdentified
� Physicianmistrusto “Treatmelikeyouwouldwantsomeonetotreatyou.Don’t
groupmeinacategoryofotherSCDpatients.”o “Dr.Xismyprimarydoctorbuthedoesn’treallyknowanything
aboutsicklecelldiseasesoIhavetotellhimwhattodo..”o “he(PrimaryMedicalDoctor)wasreallymeantome.Hewasa
littlebitarrogant.Itwaslikehewasblamingmeforthesoreonmyleg(legulcers).”
o “Whenyougototheemergencyroom…theythinkitsanopinionbutitsnot,itjusthurts.”
o “They…treateduslikepeopleoffthestreet.Oh,she’sjustcominginheretogetafix.Theworstthingisthattheydon’ttreatyouwithrespectandkindness.Theytreatyoulikeacrackhead becauseofwhatyouhave.”
o “Whenyougettobeanadult,theytendnottobelieveyouasmuch.”
LivingwithSCDasanAdult–ThemesIdentified
� Employmento “Youcan’tgointheretellingthemthatyouhavethese
crisesandIhavetobeout”.o “It’sverydifficulttoholdemploymentbecauseofthe
stresslevel.”
� Relationshipsatworkandathome:o “wherehaveyoubeen,onvacation?”o “(hedidnot)understandthecycleofpainandwhatit
does.”o “IfIsay‘ohmygodmyleghurtssobad’thanhe’dsay‘well
minehurtstoo’IjustsayOKdad,butit’sadifferenttypeofhurt.”
Patientandfamilyfears:
• Leavingthecurrentprovidero “Itwaslikesomeonepulledtherugoutunderme”
• Establishingtrustwithanewprovidero “WhowasgoingtobemydoctorandwasshegoingtobenicefromthetimewhenIcomeover?”
• Navigatinganewhealthcaresystemo “Iwasdefinitelyconcernedaboutswitchingbecausethat’sallIknew—Children’sandthedoctorsIwasseeingthere.IwasdefinitelyscaredtomoveovertoUAB”
Conclusions
• Patientsperceivesignificantdistrustfromphysicianso InterventionstoimprovephysicianopinionsregardingptswithSCD
• ContinuedparentinvolvementoMaybeusedasatransition
• Lackofphysicianknowledgeo Improvededucationefforts
Conclusions
• Difficultyidentifyingdoctorso Appropriatecareparadigm?
• Difficultymaintainingemploymento Furtherresearchneeded
• Questionsregardingnumbersofpatientswithsicklecello 2ptsdiagnosedasadultso 2ptswithnohealthcarecontactformanyyears
Objectives• Children’sSickleYouthNetwork(CYSN™)
• Pathophysiology ofsicklecelldisease
• Commoncomplicationsaffectingadolescentsandyoungadultswithsicklecelldisease
• CoreElementsofTransition
• FindingsfromaqualitativestudyonSCDtransition
• Multidisciplinaryrolesintransitioninsicklecelldisease
Factorsthatinfluencesuccessoftransitiontoadultlife
Transition
Family/Relationships
Health
Education
Vocation
Lifeskills
Self-efficacy
TransitionProcess
<12yo• Encourageparentstoteachchildrenabouttheirdiseaseincludinggoodhealthpractices
12-14yo• Beginencouragingpatientstolearnmoreabouttheirdisease
15-18yo• Patienttransitionplanningandeducation
>18yo• Transfertoadultcare
TransitionProcess
• Engagepatientsintheconversationregardlessofage
• Makeeveryclinicvisitaneducationalvisit• Followschoolprogresstoensurestayingatappropriategradelevel
• Encouragedreamswhilemaintainingafocusongoalswithinreach
Selfefficacy
Importantthingstoknow
<19yo Adulthood�Medicaideligibilitybasedonparentalincome
�Unlimitedinpatientandoutpatientvisits
�Dentalservices� Yearlyeyeexamsandeyeglassesevery2years
� Parkingisfreeorvalidatedforpatients/parentsatCOAforappointments
� COAsocialworkers/KID1willprovidetransportationassistance.
�Unlessdisabled,nolongereligibleforMedicaid
� Limitedto14combinedoutpatientandinpatientvisits
�Nodentalservices� Eyeexamsevery3years,noeyeglasses
�Nofreeparking�NoKID1ortransportationassistance
Transitionresources
• www.gottransition.org• http://www.sickkids.ca/Good2Go/index.html• http://hctransitions.ichp.ufl.edu/hct-promo/
Questions?