navigang the gray: clinical decision- making with families

Naviga&ngtheGray:ClinicalDecision-MakingwithFamiliesandtheirChildrenwithMedicalComplexityintheFaceof

Uncertainty

EmilyGoodwinMD,AssistantProfessorofPediatrics,Children’sMercyHospital,UniversityofMissouri,KansasCitySchoolofMedicine,KansasMO

KathleenHuthMDMMSc,InstructorofPediatrics,BostonChildren’sHospitalandHarvardMedicalSchool,BostonMA

NancyMurphy,MD,ProfessorandChief,DivisionofPediatricPMR,UniversityofUtahHealthSciencesCenter,SaltLakeCityUT

AACPDMIC27;Cincinna&2018

FINANCIAL DISCLOSURE AACPDM 72nd Annual Mee8ng October 9-13, 2018 Speaker Names: Emily Goodwin MD Kathleen Huth, MD, FRCPC, MMSC Nancy Murphy MD 1. Disclosure of Relevant Financial Relationships We have no financial relationships to disclose. 2. Disclosure of Off-Label and/or investigative uses: We will not discuss off label use and/or investigational use in our presentation.


Learningobjec&ves

1.  Describetheimportanceandlimita&onsofevidence-basedprac&ceinthecareofCMCandneurodevelopmentaldisabili&es.

2.  Demonstrateanapproachtograyscalethinkingappliedtocommonclinicalissuesthroughcase-baseddiscussions.

3.  Applythe4basicprinciplesofhealthcareethicstochallengingdiagnos&candtreatmentdecisions,inpartnershipswithfamilies.

4.  UsetheICFframeworktoguideclinicaldecision-makingforCMCandtheirfamilies,understandingtheimportanceofgoal-directedcare.


Course Overview

1.  Defini&onsandapplica&onsofgrayscaledecision-making2.  HowdowebestfeedaCMC?

Evidence,goalsofcare,anddecision-making.

3.  HowdowebestmanagepaininaCMCandquadriplegicCP?Evidence,goalsofcare,anddecision-making.

4.  Applyingtheprinciplesinterac&vely(3casestoconsider)5.  Q&A


Naviga&ngtheGray:Defini&ons,FrameworksandApplica&ons

NancyMurphy,MDProfessorandChief,DivisionofPediatricPMR

UniversityofUtah,SaltLakeCityUT


Framingthedecisions

Grayscalethinking

•  Easierorharder•  Fasterorslower• Closerorfurther• Be`erorworse• Moreorless

Blackorwhitethinking• Rightorwrong• Goodorbad• Alwaysornever• Perfectordisastrous• Allornothing


Consideryourrecentclinicaldecisions

• ConvertGTtoGJ,orreviseaslippedfundoplica&on?•  Systemicorintrathecalbaclofen?•  Spinalfusionforadvancedscoliosiswithgoalofcomfort?• Where’sthelinesbetweenmedicalneglect-medicaliza&on-medicalchildabuse?

• Otherexamples?


Evidence

• Givesusconfidenceinourclinicaldecisionmaking• Armswithagreattoolsaseducators• Protectsusfrompayerdenials;empowersustomakeappeals• Protectsusfromli&ga&onshouldtherebeanadverseoutcome•  Increasesadherencewithinterven&onswhenfamiliesarewell-informed

•  It’sthe“goldstandard.”


h`ps://pct.libguides.com/EBM/levels-of-evidence.AccessedAugust19,2018.


Evidence-basedprac&ce

•  EBPistheintegra&onofclinicalexper&se,pa&entvalues,andthebestresearchevidenceintothedecisionmakingprocessforpa&entcare.

• Clinicalexper&seisgroundedincumulatedexperience,educa&onandclinicalskills.

•  Thepa&entbringstotheencounterhisorherownpersonalpreferencesanduniqueconcerns,expecta&ons,andvalues.

•  Thebestresearchevidenceisusuallyfoundinclinicallyrelevantresearchthathasbeenconductedusingsoundmethodology.

h`p://guides.mclibrary.duke.edu/c.php?g=158201&p=1036021.AccessedAugust19,2018.AACPDMIC27;Cincinna&2018

Evidencebasedprac&ce


4PrinciplesofMedicalEthicsAutonomyPeoplehavetherighttocontrolwhathappenstotheirbodies.Aninformed,competentadultcanrefuseoraccepthealthcareastheywish.

BeneficenceDothemostgoodforeverypa&entineverysitua&on.

Non-maleficenceDonoharm.Watchfordoubleeffect,whereatreatmentintendedforgooduninten&onallycausesharm.

JusCceBeasfairaspossiblewhenofferingtreatmentstopa&entsandalloca&ngscarcemedicalresources.

h`ps://www.dummies.com/health/medical-ethics-for-dummies-cheat-sheet/.AccessedAugust19,2018.AACPDMIC27;Cincinna&2018

Afunc&onalapproachtocare Goal-directedmedicalplanofcareCarecoordina&onFamilysupports


WHO, 2001

Interna&onalClassifica&onofFunc&on(ICF)

Body Function & Structure

Health condition

(disorder or disease)

Activity Participation

Environmental Factors Personal Factors


ChildrenwithMedicalComplexity(CMC)

• CMCareasubsetofthe18%ofallUSchildrenwhohavespecialhealthcareneeds

• CMChavethehighestdegreeofmedicalfragilityandmostintensivehealthcareneeds

• Onenwithtechnology-dependenciesandhighpsychosocialcomplexity

•  Lessthan1%ofallUSchildren


ExamplesofCMC

• Perinatalcondi&onsandprematurity• Congenitalanomaliesandchromosomaldisorders• Chroniclungdisease• Neurologicimpairments•  Technologydependencies• Progressiveneuromusculardisorders• Metaboliccondi&ons• Childrenwhohistoricallywouldnothavesurvivedwiththeircondi&ons


Lessthan1%ofallUSchildren…

• Accountfor1/3ofallpediatrichealthcarecosts•  5%ofCMCaccountfor50%ofthetotalspend• Accountfor42%ofallUSpediatrichospitaliza&ons,and71%ofthe30-dayunplannedhospitalreadmissions

• Childrenwithneurologicimpairmentsaccountfornearly30%ofhospitalchargeswithinchildren’shospitals

• Howcanprovidersnavigatethegraywiththesechildrenandfamilies?


ToolsforNaviga&ngtheGray

1.  Useevidence-basedprac&cetoguideclinicaldecisionmaking.2.  Whenevidenceislacking,applythe4principlesofmedicalethicsin

discussionsanddecisionsofgoal-directedcare.3.  Takeafunc&onalapproachtocare,alwayswithclearlystatedgoals.4.  Promotehealthcarevaluebypartneringwithpa&ents,parentsand

providerstoensurethebestoutcomesatthelowestmostappropriatecost.


[email protected]


Case:FeedingSafetyinaCMC

KathleenHuth,MD,FRCPC,MMSCPhysician,ComplexCareService

InstructorinPediatricsBostonChildren'sHospitalandHarvardMedicalSchool


Case: Feeding safety in a CMC

•  2andahalfyearoldgirlwithspas&cquadriplegia,GMFCSIII• Chroniclungdiseaseofprematurity,inhaledbudesonidedaily•  Twohospitaliza&onsforpneumoniainthepastyear•  Sialorrheamanagedwithglycopyrrolate• Weightz-score-2.5• Meal&mesareprolongedandasourceofstressforthefamily.• Parentsdescribeoccasionaldribblingoffoodfromherlips.•  Lotsofanxietyaroundthepossibilityofrequiringenteralgastrostomytubefeeds.


How would you evaluate feeding safety in this child? A.  Clinicalfeedingevalua&onB.  VideofluoroscopicswallowingstudyC.  Fiberop&cendoscopicexamina&onofswallowingD.  Noneoftheabove


What is the evidence? Whereisitmissingorconflic&ng?


Clinical feeding evalua8on

•  DiagnosCcaccuracyoftheclinicalfeedingevaluaConindetecCngaspiraConinchildren:asystemaCcreview.

•  Calvoetal.,DevMedChildNeurol2016

•  Sixstudiesexaminingthediagnos&caccuracyofCFEusingVFSSand/orFEESasagoldstandard

• Methodswerevariedandconsideredlowquality•  CFEstriallingliquidconsistenciesmightprovidebe`eraccuracyes&matesthanCFEstriallingsolidsexclusively

•  “cri&callackofevidence”ontheaccuracyofCFEindetec&ngoropharyngealaspira&on


Videofluoroscopic swallow study (VFSS) Advantages Disadvantages

Visualizeoral,pharyngealandupperesophagealphasesofswallowing

Contrastmedium—issuestolera&ngit,whetherthereisconcordancewithtypicallyconsumedliquids

Assessanatomyandphysiology Radia&onexposure

Canseetheeffectoftherapeu&cmaneuvers

Ques&onablegeneralizabilityofjudgmentsbasedononeperiodof&me

Arvedsonetal.Instrumentalassessmentofpediatricdysphagia.SeminSpeechLang2017


VFSS

•  InfantvideofluoroscopicswallowstudytesCng,swallowingintervenCons,andfutureacuterespiratoryillness

•  Coonetal.,HospPediatr2016

•  576infantsweretestedwithaVFSSintheirfirstyearoflife,receivingatotalof1051VFSSsintheirfirst3yearsoflife.

•  Morethan60%ofinfantsreceivedafeedinginterven&on.•  thickeningfeeds,NG/NJfeeds,gastrictubeplacement,fundoplica&on

•  Primaryoutcome:EDvisitorhospitaladmissionforacuterespiratoryillness(ARI),occurringbetweenthefirstVFSSandthirdbirthday.

•  Thickening/nasaltubefeedings,comparedwithnointerven&on,werenotassociatedwithadecreasedriskofsubsequentARI.

•  Exceptforinfantswithsilentaspira&onwhoreceivedthickenedfeedings


Fiberop8c endoscopic evalua8on of swallowing (FEES)

Advantages Disadvantages

Noradia&on Cannotassessoralphaseofswallowing

Canbedoneatthebedside Maynottoleratecamerainser&on

Canassesssecre&onmanagement LessavailablethanVFSS

Evaluateconcernsrelatedtoupperairwayobstruc&onorvocalcordparesis.

Ques&onablegeneralizabilityofjudgmentsbasedononeperiodof&me

ArvedsonJC.Feedingchildrenwithcerebralpalsyandswallowingdifficul&es.EurJClinNutr.2013AACPDMIC27;Cincinna&2018

Arvedsonetal.Instrumentalassessmentofpediatricdysphagia.SeminSpeechLang2017AACPDMIC27;Cincinna&2018

Challenges with VFSS and FEES interpreta8on

Pisegnaetal.Parametersofinstrumentalswallowingevalua&ons:Describingadiagnos&cdilemma.Dysphagia2016AACPDMIC27;Cincinna&2018

Case con8nued: Feeding safety in a CMC

•  Theyfamilymetwithadie&cianandhavebeensupplemen&ngcalorieswithanutri&onaldrink,thoughsheisnotconsistentlyachievinggoalintake.

• Atherfollow-upappointment,parentsnoteshesome&mesbecomescongestedandhas“gurgly”breathinganerdrinking.Youwitnessthisinyourclinic.

•  Shehasgainedanaverageof4g/dayoverthepastmonth.•  Shehasnormalvitalsignsandiswell-hydratedonexam.• Parentsreiteratetheirhopesthattubefeedscanbeavoided.


How would you provide nutri8onal support to this child? A.  ArrangeregularfeedingtherapyB.  RecommendthickeningherfeedsC.  Nasogastrictubefeedsandini&ateevalua&onforgastrostomytube


Feeding interven8ons •  FeedingintervenConsforchildrenwithcerebralpalsy:areviewoftheevidence

•  Snideretal.,PhysOccupTherPediatr2011.

•  Feedinginterven&onsincluded:oralsensorimotorfacilita&on,foodconsistency,posi&oning,oralappliances,andadap&veequipment.

•  21studieswereincludedinthefinalanalysis,•  Outcomesmainlyfeedingsafetyandefficiency.1studyshowedheightandweightincrease.

•  Sensorimotorinterven&onsprovidedregularlyoverweekstomonthsaswellasposi&oningtechniquesmayimproveoral-motorskills.

•  Adjus&ngfoodconsistencymayalsohelpimprovefeedingperformance.•  “currentlevelofevidenceispoor”,smallsamplesizes,limiteddatapar&cularlyformul&modalapproachesorlongtermoutcomes.


Oromotor therapy

•  IntervenConsfororopharyngealdysphagiainchildrenwithneurologicalimpairment

• Morganetal.,CochraneDatabaseSystRev2012.

•  Primaryoutcomes,whichwerephysiologicalfunc&onsoftheoropharyngealmechanismforswallowing,respiratoryinfec&onsanddietconsistency.

•  3studiesmetinclusioncriteria;evalua&ngoralsensorimotorinterven&onsandlipstrengtheningexercises

•  Studiesaffectedbya`ri&on,detec&onbias,smallsamplesizes•  “insufficienthigh-qualityevidence…toprovideconclusiveresultsabouttheeffec&venessofanypar&culartypeoforal-motortherapy.”


Thickened feeds

•  Thickenedliquidsasatreatmentforchildrenwithdysphagiaandassociatedadverseeffects:asystemaCcreview

• Gosaetal.,InfantChildAdolesc2011

•  6studiesexaminedtheeffectsofthickenedliquidsonswallowingorpulmonaryoutcomesandreportedmixedfindings.

•  16studiesexaminedadverseeffects—amongpediatricpa&entswithGER,therewasnosignificantincreaseintherateofadverseeffectsfromtheuseofthickenedliquids.

•  “insufficientevidencebaseforthispopulartreatmentop&on”


Tube feeding

•  Gastrostomyfeedingincerebralpalsy:AsystemaCcreview.•  SleighandBrocklehurst,ArchDisChild2004.

•  Twocohortstudies,15caseseries,andeightcasereportsmetinclusioncriteria.•  Weightgainresultedfromgastrostomyfeedinginmostcases.•  Complica&onsreportedincludedincreasedGERandaspira&on.

•  Gastrostomyfeedingversusoralfeedingaloneforchildrenwithcerebralpalsy.•  Gantasalaetal.,CochraneDatabaseSystRev2013.

•  NoRCTsiden&fied.•  “considerableuncertaintyabouttheeffectsofgastrostomyforchildrenwithcerebralpalsyremains"


Gastrostomy tube: Exploring impact

•  Commonconcerns•  Missingmeal&mesocializa&onexperiences•  Restrictedmobility/inabilitytoleavehome•  Gtubesiteissues•  Aytudesofothers•  Sleepdisrup&on•  “unnatural”

•  Impactofgastrostomytubefeedingonthequalityoflifeofcarersofchildrenwithcerebralpalsy.

•  Sullivanetal.,DevMedChildNeurol2004

•  12monthsanergastrostomy,significantimprovementsinsocialfunc&oning,mentalhealth,energy/vitality,andgeneralhealthpercep&onwerereported.

•  Significantreduc&oninfeeding&mesandreducedconcernabouttheirchild'snutri&onalstatus.


4PrinciplesofMedicalEthics

AutonomyPa&ent/familyvaluesanddecision-makingaroundfeedingtheirchildanerbeinginformedoftherisks/benefits.

BeneficenceProvidingnutri&onalsupportinawaythatisposi&ve,func&onalforthefamily,andreducesstress.

Non-maleficencePreven&ng/mi&ga&ngriskofaspira&on

JusCceSelec&nginves&ga&onsbasedondiagnos&cyieldandtriallinginterven&onswithclearlyestablishedgoals


WHO, 2001


Body Function & Structure

Health condition

(disorder or disease)

Activity Participation

Environmental Factors Personal Factors


Feeding safety and the ICF framework

OropharyngealdysphagiaPulmonarystatusNutri&onalstatus

Feedingtube/equipmentFamilystructure

Meal&meenvironmentSocialinterac&ons

Child’sage,self-feedingability

OromotorskillsFeedingskills


A func8onal approach to feeding safety

•  Interdisciplinaryteamapproachconsideringmul&plefactors•  Pulmonarystatus,GIissues,Oralsensorimotorskills,Behaviouralissues,Familyinterac&ons

•  Not”allornothing”•  Oralskillsandaspira&onriskchangesdependingoncontextandover&me.Considerimpactoffa&gue,URTI,seizuredisorder,GERD,medica&onsthataffectsecre&ons,dentalhygiene,posture/tonemanagement

•  Goalisn’tnecessarilytotaloralfeeding.Iden&fywhatfeedingispossible,withappropriateparametersandsupports

•  Discusstheevidenceandlimita&onsofproposedevalua&onsandtreatment•  Priori&zepulmonaryhealthandop&mizingnutri&onandhydra&onstatuswhileensuringalignmentwithfamilygoals.

ArvedsonJC.Feedingchildrenwithcerebralpalsyandswallowingdifficul&es.EurJClinNutr.2013AACPDMIC27;Cincinna&2018

Andrewetal.Feedingdifficul&esinchildrenwithcerebralpalsy.ArchDisChildEduc2012.AACPDMIC27;Cincinna&2018

[email protected]


Naviga&ngtheGray:CaseExampleChronicPain


EmilyJGoodwin,MD,FAAPClinicalAssistantProfessorChildren’sMercyHospital

UniversityofMissouriKansasCitySchoolofMedicine

Case: Chronic Pain •  12yofemalewithGMFCSVCerebralPalsy•  SheisdependentonG-tubefornutri&on,hasosteopenia,cor&calvisualimpairment,scoliosiss/pspinalfusion,andspas&citymanagedbybaclofenpump.

•  Shehasbeenhavingunexplainedepisodesofchronicpainnearlydailyforthepastfewmonths.

•  Painbehaviors:inabilitytobeconsoled,tachycardia,moaning,restless,archesback.Occursmostevenings.

•  Pa&entisnon-verbalandunabletousecommunica&ondevice.Nolongersiyngcomfortablyinherwheelchairandimpac&ngschoolandac&vi&esofdailyliving.Nooneissleeping.

•  Examisunrevealing.X-rayimagingshowsbilateralcoxavalga,migra&onpercentage40%bilaterally,allspinalfusionhardwareintact,nofractures,mildtomoderatestool.


How would you approach treatment op8ons in addi8on to non-pharmacologic measures? A.  FurtherDiagnos&cevalua&ons(x-rays,endoscopy,pHstudies,

assessbaclofenpump)B.  Stepwiseempiricmedica&ontrials(H2antagonist,PPI,cons&pa&on

treatment,analgesics,gabapen&noids,opioids,adjustbaclofenpump,tricyclican&depressants,alphaagonists)

C.  Offerproceduralinterven&ons(botulinumtoxin,phenol,surgicalop&onsforhipsubluxa&on)

D.  Noneoftheabove


Case: Chronic Pain sources

Children(Basel).2018Jan;5(1):13.Publishedonline2018Jan18.doi:10.3390/children5010013AACPDMIC27;Cincinna&2018

Case: Chronic Pain •  Iden&fica&onofsourceandtargetedtreatmentidealbutnotalwayspossible

•  Nostandardapproach

•  Empirictrialsnotwithoutadverseeffects •  Pa&entandfamilyimpact/&me•  Medica&onsideeffects


Hauer&Houtrow.PainAssessmentandTreatmentinChildrenwithSignificantImpairmentintheCentralNervousSystemPediatrics2017,139(6)

Oravaetal(2014).Chronicpainassessmenttoolboxforchildrenwithdisabili&es:Sec&on1.0:Toolboxbackground.Toronto,Ontario:HollandBloorviewKidsRehabilita&onHospital.Retrievedfrom:h`p://hollandbloorview.ca/toolbox

KingsnorthetalChronicPainAssessmentToolsforCerebralPalsy:ASystema&cReview.PediatricsOct2015,136(4)e947-e960;DOI:10.1542/peds.2015-0273

Suggested guidelines for pharmacologic management of recurrent pain behavior episodes.45,93–95,105–107.

Julie Hauer et al. Pediatrics 2017;139:e20171002

©2017 by American Academy of Pediatrics AACPDMIC27;Cincinna&2018

Case: Chronic Pain

•  SurgicalInterven&ons•  Nopreferredprocedurefornon-ambulatorypa&entswithpainfulchronicsubluxatedordislocatedhips(arthodesisnotrecommended,FHR,VO,THAareop&ons).Painreliefbetween88-93%,complica&onsbetween24-35%


KolmanSE,RuzbarskyJJ,SpiegelDA,BaldwinKD.SalvageOp&onsintheCerebralPalsyHip:ASystema&cReview.JPediatrOrthop.2016Sep;36(6):645-50

Case: Chronic Pain

•  Limitedevidenceforuseofmanymedica&onsinchildrenfromchronicpain.NoRCTs

• Gabapen&noids(Gabapen&n,Pregabalin)•  HauerJM1,SolodiukJC.Gabapen&nformanagementofrecurrentpainin22nonverbalchildrenwithsevereneurologicalimpairment:aretrospec&veanalysis.JPalliatMed.2015May;18(5):453-6.doi:10.1089/jpm.2014.0359.Epub2015Feb6.

•  N=22,21(91%)hadsignificantdecreaseinsymptoms/painbehaviors•  CooperetalAn&epilep&cdrugsforchronicnon-cancerpaininchildrenandadolescents.Cochranereview2017

•  noevidencetosupportorrefutetheuseofan&epilep&cdrugstotreatchronicnon-cancerpaininchildrenandadolescents.



h`p://guides.mclibrary.duke.edu/c.php?g=158201&p=1036021.AccessedAugust19,2018.

Hipsurgeryrecommendedbutunclearifthisis

THEsourceofpainbehaviors

Familywishestoavoidsurgery

Somemedica&onsfoundtobehelpful


ChronicPaintreatmentandMedicalEthicsAutonomyFamilyhopestoavoidanesthesiaandsurgery,valuesdecisionmakinganerbeinginformedofbenefitsandrisks

BeneficenceCana`empttotreatpainmedicallywithoutknowingsource

Non-maleficenceLimitedevidence(RCTs)forsomemedica&ons,mayhaveadverseeffects.Unknownifhipsaresourceofpainbehaviors,manysurgicalandanesthesiarisks

JusCceManytreatmentop&ons,cantrialsomesystema&callywithcleargoals(pa&entcomfort,par&cipa&on)


Afunc&onalapproachtocare Goal-directedmedicalplanofcareCarecoordina&onFamilysupports



Spasticity and Pain behaviores,

40% MP hips, near skeletal

maturity

Chronic Pain

Relies on wheelchair

Pain limits participation in

school and therapy

Wheelchair not fitting comfortably

Non-verbal, enjoys school and family time

AACPDMIC27;Cincinna&2018ICFWHOFramework

Case: Chronic Pain

• Mul&disciplinaryCareconferenceisheld• Unclearifhipsaresourceofpain•  risksandbenefitsofsurgeryandproceduralinterven&onsandmedica&onsarediscussed

• Botulinumtoxintrialedwithoutimprovement• Gabapen&noidstrialedwithoutimprovement• Ul&matelytransi&onedtohomeboundschoolingwithcommunityac&vi&esini&atedtominimizepainwithtransfers

• Alpha2agonisttrialedandprovidesbenefitAACPDMIC27;Cincinna&2018

[email protected]


GrayscaleDecisionMakingInterac&veDiscussion


AutonomicDysfunc&onSpas&city&Dystonia ProgressiveNeuromuscularScoliosis

WhatistheEvidence?ApplyEthicalPrinciplesFunc&onalApproach

Interac&veDiscussion


AutonomicDysfunc&onSpas&city&Dystonia ProgressiveNeuromuscularScoliosis

What’syourtreatmentplan?

Whatfactorsinfluenceyourdecisions?


Acknowledgements

SpecialthankstomembersoftheAACPDMComplexCareEduca&onsubcommi`eeontheComplexCareCommi`eefortheircontribu&ons

andassistancewiththispresenta&on


Thankyou.


navigang the gray: clinical decision- making with families

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