palliative care rough · • 92% would be likely to consider palliative care for a loved one if...

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ComprehensiveManagementofInfantswithSeriousCongenitalAnomalies

JonMullin,MDSamJulian,MD

Disclosures

• Therearenorelevantfinancialrelationshipstodisclose

LearningObjectives

• Developanapproachtounderstandinggoalsofcareforexpectantfamilies

• Formulatecomprehensiveplansforchildrenwithserious,life‐limiting,orlife‐threateningcongenitalanomalies

Case‐ SD

• 31yo G3P1011at344/7weeksEGA• LimitedPNC• Femalefetus• Presentsforpretermlaborevaluation• Anatomyscanshowsventriculomegaly

Case‐ SD

• MFMconsultsNeonatology• Infantdeliversbeforeconsultationdone• Neonatologyinattendance• Babyreceives

– PPVx1minute,thenCPAP– Infantwithdecreasedtonebuthasmovementsx4extremities

• AdmittedtoNICUforfurtherevaluation

Case‐ SD

• HEENTexamconcerningforholoprosencephaly– Premaxillary agenesis

• Headultrasounddemonstratesalobar holoprosencephaly• MRIconfirmsdiagnosis

• Neonatologistdoes“notsoantenatal”consultation• Patientasksaboutpalliativecareconsultation

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• Howknowledgeable,ifatall,areyouaboutpalliativecare?

www.capc.org ‐ 2011

WhatisPalliativeCare?

• Palliativecareisspecializedmedicalcarefor peoplelivingwithseriousillness.

• It focusesonproviding relieffromthesymptomsandstressofaseriousillness.

• Thegoalistoimprovequalityoflifeforboththepatientandthefamily.

• Itisappropriateatanyageandatanystageinaseriousillnessandcanbeprovidedalongwithcurativetreatment.


Onceinformed…

• 95%agreethatitisimportantthatpatientswithseriousillnessandtheirfamiliesbeeducatedaboutpalliativecare

• 92%wouldbelikelytoconsiderpalliativecareforalovedoneiftheyhadaseriousillness

• 92%believeitisimportantthatpalliativecareservicesbemadeavailableatallhospitalsforpatientswithseriousillnessandtheirfamilies.


Hospice

• Partofpalliativecarecenteredonendoflife

• Careathomeorinhospicefacilities– Painandsymptommanagement– Ongoingpsychosocialanddecision‐makingsupport– Griefandbereavementsupport

Palliative CareHospice

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Feudtner Pediatrics 2011

PediatricPalliativeCare

• Chronic,life‐limitingorlife‐threateningconditions– 0.5‐2millionchildrenintheUS

• Higherdeathratethanhealthychildren• Previouslyfatalillnesses/conditionsnowchronic

• Patientsreceivingpediatricpalliativecarearefollowedlongitudinally

• Pushforearlierreferralstopediatricpalliativecare

IOM Report 2014 IOM Report 2014

IOM Report 2014

Whatdoespediatricpalliativecaredo?

Symptommgmt(58%)• Cognitiveimpairment(47%)

• Pain(31%)• Seizures(25%)• Dyspnea(22%)

Other tasks (42%)

• Communication (48%)

• Decision Making (42%)

• Care Coordination (35%)

• Transition to home (14%)

• Limiting Interventions (12%)

• Bereavement (11%)

• Recommendations at end‐of‐life (9%)

Feudtner Pediatrics 2011

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IOM Report 2014

• Becausethisiswhatismostoftenthematchbetweenavailabletreatmentsandgoalsofcare

• Doesthispatientqualifyforapalliativecareconsult?

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DiagnosesforNeonatalReferral

• FourDomainsNeonatalPalliativeCareConsults– Genetic/Chromosomal– Organ‐systemproblems– Infectionandimmunedisorders– Complicationsofprematurity

Carter,Children,2018

Genetic/Chromosomal

• Chromosomalaneuploidies– Trisomy13,18

• Severeformsofskeletaldysplasia– Osteogenesisimperfecta(II)– Thanatophoricdwarfism

Catlin&Carter,JPerinatol,2002;Carter,Children,2018

Organ‐System

• CNS– severeneuraltubedefects

• anencephaly;holoprosencephaly• meningomyelocele;encephalocele• hydranencephaly;severehydrocephalus• exencephaly

– Neuro‐migrationaldisorders• schizencephaly• lissencephaly• polymicrogyria Catlin&Carter,JPerinatol,2002;Carter,Children,2018

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Organ‐System

• Congenitalheartdisease– PentalogyofCantrell(ectopia cordis)– Single‐ventricle(HLHS)– AnticipatedECMO– High‐outputcardiacfailure– Hydropsfetalis

Organ‐System

• Renal– Potter’ssyndrome/renalagenesis– Poly/multicystic dysplastic– Urinaryascites– Obstructiveuropathy

Organ‐System

• GI– Short‐gutsyndrome– TPNdependence– Liverfailure– Biliaryatresia


Organ‐System

• Structuralanomalies– Giantomphalocele– Congenitaldiaphragmatichernia– Pulmonaryhypoplasia

• Space‐occupyingthoracicmass• BPS/CPAM

– Inoperableconjoinedtwins


InfectionandImmuneDisorders

• SCID• SevereperinatalHSVdisease• ToxoplasmosisorCMVinfections• Zika virus

ComplicationsofPrematurity

• SevereIVH• Ventilator‐dependentBPD• SevereNEC

Carter,Children,2018

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WhyinvolvePalliativeCare?

• Gettoheartofparentalfearsandwishes• Providesstrongpatientandparentadvocate• LongitudinalcareinsideandoutsideNICU• Assistancewithhomepreparation• Assistancewithmedicationsatendoflife

31

Second Trimester

Birth

Obstetrics Maternal Fetal Medicine

Pediatric Subspecialty Care

Neonatology

Surgical Intervention

Complex Care

WhenshouldIinvolvePalliativeCare?

Case‐ SD

• HasA/Bs – escalatedtoNIPPV• Toleratesfeedings– escalatedtofullvolume

– NoPOinterest– Hassomerefluxaswell

• Initialtempinstability,resolvesover3weeks

Case‐ SD

• Goalsofcare– slowlyelucidatingbutnotfinal• Parentswanttotakeherhome

• Preparationsforgastrostomymade• Concernsaboutrespiratorystatusremain

PrimaryvsSpecialtyPalliativeCare

• Primary– Symptommanagement– PsychosocialSupport– BasicDiscussions

• Prognosis• Goals• AdvanceCarePlanning

• Specialty– Refractorysymptoms– Complexpsychosocialsituations– ChallengingDiscussions

• Uncertainorcomplicatedprognosis• Conflictsbetweenfamilyand/orclinicians

Quill NEJM 2013

PrimaryvsSpecialtyPalliativeCare

• Primary– Symptommanagement– PsychosocialSupport– BasicDiscussions

• Prognosis• Goals• AdvanceCarePlanning

• Specialty– Refractorysymptoms– Complexpsychosocialsituations– ChallengingDiscussions

• Uncertainorcomplicatedprognosis• Conflictsbetweenfamilyand/orclinicians

Quill NEJM 2013

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GoalsofCareDiscussions

1. Tellusaboutyourchild,whatiss/helikeasaperson?2. Whatisyourunderstandingofyourchild’sillness?3. Inlightofyourunderstanding,what’smostimportant?4. Whatareyourhopingfor?Whatareyourworries?5. Howdoyoumaintainyourstrength?

Tellmeaboutyourself/yourchild

• Setstone/firstimpression• Disarming• Differentthanstandardopeningofmedicalencounter

– Oftenrequiresre‐stating

• Followups:– Whatdoesagooddaylooklike?– Tellmeaboutherpersonality

Whatisyourunderstandingof…?

• Oftenthisquestionillustratesneedfordiscussionofprognosis

• Bestcase,worstcase,mostlikely– Setbumpersinlightofuncertainty– Increasesyourcredibilityasoutcomeunfolds– Setsyouuptotalkabout“worsecase”scenarios

HopesandWorries

• Respondingto“unrealistic”hopes?– Idotoo– I’msorry– Iwish…

• EmotionalDistancing

Hope

• Patientsremainhopefulaboutthefutureeveniftheyhavebeentoldthatthereisnopossibilityofcure

• Understandingofpoorprognosisallowspatientsandtheirfamiliestoformulatealternativehopesbyfocusingonoutcomesthatarepossible

• Patientsandtheirfamiliescanmultiplesimultaneoushopes:– Longlife– Meaningfullife– Minimalsuffering– Lovewithinafamily Wright et al. JAMA. 2009

Apatira et al. 2008Feudtner et al. NEJM. 2009

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HopefortheBestPreparefortheWorst

• Giveequaltimetobothhopingandpreparing

• Alignwithhopes

• Encouragedualagenda– donotimpose

• “Woulditbehelpfultotalkaboutifthingsdon’tgoaswehoped?”

Strength

• Opportunitytoassesspsychosocialdynamic• Imbeddedsocialandfamilyhistory• Spiritualassessment

• It’softenthesediscussionsthatunmaskkeydriverstodecision‐makingandopportunitiesforholisticsupport

HelpfulPhrases

• “Lovingparents…”• “Somefamilies…”• “Iwonder…”• “Iwish…”• “Iworry…”• “Woulditbehelpfulif…?”• “Yes,and…”

SharedDecisionMaking

• Pinnacleoffamily‐centeredcare

• Usefulinchildrenwithchronicconditionsorseriousillness

• Collaborativecommunicationandexchange– Familysharesgoalsandvalues– Medicalteamsharesinformationandexperience– Leadstorecommendation,and/orjointdecision

Katz, 2013

GuidedByGoalsofCare

Goals of Care

ApproachLive as long as

possibleLive as long as possible and as well as possible

Live as comfortably as

possible

DifferentialDiagnosis √ √ √

Assessment Intensive Targeted Limited‐target

Treatment Intensive TargetedFlexible and at times empiric

Follow‐up Intensive Intensive Intensive

TreatmentPathways

• Gastrostomyplacement• Tracheostomyplacement• Decisiontoaggressivelytreatseizures

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Pediatric Palliative Care

PerinatalEnd‐of‐Life

Care

Prognostic Uncertainty(prepare for both

scenarios)

‐Anencephaly‐Exencephaly‐Renal agenesis

‐HLHS?‐Holoprosencephaly‐Mild CDH‐Omphalocele



Care


scenarios)



Most Everything Else!



Care


scenarios)




Dependent on Goals of Care

Case‐ SD

• Gastrostomyplaced,shetoleratesextubation• Stillhasspellsrequiringvigorousstimulationtorecover

• Limitedcodedecisionmade• Respiratorystatusremainsunclear

ParentsExpectationsofCaring:• Maintainingbelief

• Knowing

• Beingwith

• Empowering

• Doingfor

Swanson, Nursing Research, 1991Kavanaugh. Palliative and Supportive Care,2015

WUSMFetalCareCenter

N=244;2017Data

Discharge home/transfer55%

Terminate pregancy

7%

FDIU9%

Post natal death12%

Lost to follow‐up17%

Outcome Normal6%

CNS11%

Chromosomal/Syndromic

13%

GI13%

GU10%

Multiple gestation16%

Thoracic5%

Other14%

Not entered12%

Diagnoses

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Cardiac26%

Genetic26%

Neuro13%

Resp19%

Skeletal10% Renal

6%

2018PPCConsults

N=30;2018Data

2017

2018

0

2

4

6

8

10

FDIU Death<24 hours

Death24 hours‐2 wks

2 wks‐6 mo Living

2017 2018

56

PerinatalPalliativeCareOutcomes

“Blueprint”forAntenatalPalliativeCareConsultation

Consultation

• Coreconceptsofpalliativecareconsultationholdtrue

• AssessUnderstanding– Prognosis– Comfort‐basedandintervention‐basedplansofcare

• Discussionofinformationanddecision‐makingpreferences

UniqueAspects

• Addressconcernsaboutsiblings,otherfamilymembers

• Shatteredexpectations,isolation,differingparentalattachment

• Discussionofinternetandsupportgroupuse

• Discussionoftermination?

Kett, Acta Paediatrica, 2017



Care


scenarios)





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Care


scenarios)





PartneringatEnd‐of‐Life

• Delivery– Typeofdeliverymayinfluenceexperience

• Locationofcare– DeliveryRoom– PostpartumvsAntepartum– Homewithhospice?

• Planforsymptommanagement

Denney‐Koelsch, J Pall Med, 2015

PartneringatEnd‐of‐Life

• Lactationcareand/ormilkdonation• MemoryMaking

– HandandFootprints– Photographs– Religiousorspiritualconsiderations– Siblinginvolvement

• Discussionofautopsy/genetics

Denney‐Koelsch,JPallMed,2015

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Care


scenarios)





65



Care


scenarios)





Lookingahead…

• Discussionof“futureconsiderations”– “Isthereanythingthatwouldbeworsethandeath?”

• Plantseedsfordiscussionsoflimitinginterventions– “Theremaybechallengingdecisionsahead,like…”

• Anticipatoryguidanceaboutchildrenwithseriousillness

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Care


scenarios)







Care


scenarios)





PrognosticUncertainty

• Explicitnaminganddiscussionofuncertainty• Avoidoverlyhypotheticaldiscussions• Navigateexternalpressures

• Discussionandguidancealongthespectrumofoutcomes

Conclusions

• AntenatalPCConsultationprovidesopportunitiesfor:– CollaborationwithcolleaguesinMFM,Neo,nursing,etc.– AdaptPCtoearlierdiagnosisofpediatricseriousillness– Exquisitesupportaroundend‐of‐lifecareperinatally– Earliestinvolvementofpediatricpalliativecare

Conclusions

• NeonatalPCConsultationprovidesopportunitiesfor:– CollaborationwithcolleaguesinNeonatology,pediatricsubspecialists,nursing,etc.

– Doesnotlimitmedicalmanagement– Allowsunificationofparentalandmedicalgoals

palliative care rough · • 92% would be likely to consider palliative care for a loved one if...

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