Transition to Adult Care in Patients with Sickle Cell ...€¢ Annual screening for risk of stroke...

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?