Table Of ContentEthical Considerations
Jonna D. Clark and Denise M. Dudzinski
Contents Abstract
Overthepast60years,incredibleprogresshas
Introduction.......................................... 2
been achieved in pediatric organ transplanta-
AllocationConsiderations........................... 2 tion. Some children, who historically would
CompetingResponsibilities:TheIndividual have died with end stage organ failure, are
PatientandSociety............................... 2 living for decades. Yet, imbedded in this pro-
CaseBasedDiscussions.............................. 3 gressarenumerousethicalconsiderations.The
discrepancybetweenthesupplyanddemandof
Conclusion............................................ 12
transplantableorganspersists,andquestionsof
Cross-References..................................... 12 justice remain prevalent. Transplant profes-
References............................................ 13 sionals are faced with a moral tension,
balancingtheirduty toprovidethebest medi-
calcareforeachoftheirpatients,knowingthat
their decisions directly impact outcomes for
other unknown patients. When one child
receives an organ, another child goes without
and is faced with the risks, morbidities, and
mortality associated with longer wait times.
Canthesupplyoforgansbeincreasedwithout
causing harm to children and their families?
How should parental living organ donors be
protected when they are desperate to save the
lives of their dying children? How should
scarce organs be allocated and who gets to
decide? Who is eligible for transplant and
what conditions, if any, preclude the opportu-
J.D.Clark(*)
nityforpediatrictransplant?Shouldtransplant
PediatricCriticalCareMedicine,TreumanKatzCenterfor
PediatricBioethics,DepartmentofPediatrics,University professionals ever be obligated to compel a
ofWashingtonSchoolofMedicine,SeattleChildren’s life-saving organ transplant against parental
Hospital,Seattle,WA,USA
wishes? Howdooutcome measuresandqual-
e-mail:[email protected]
ity assessments of transplant centers impact
D.M.Dudzinski
common practices and decisions? In the fol-
DepartmentofBioethicsandHumanities,Universityof
lowingchapter,throughaseriesofcasebased
WashingtonSchoolofMedicine,Seattle,WA,USA
#SpringerInternationalPublishingSwitzerland2016 1
S.P.Dunn,S.Horslen(eds.),SolidOrganTransplantationinInfantsandChildren,OrganandTissue
Transplantation,DOI10.1007/978-3-319-08049-9_1-1
2 J.D.ClarkandD.M.Dudzinski
discussions, common ethical frameworks are “God Squad,” a group of seven individuals
used to illuminate important considerations appointedtoserveontheAdmissionsandPolicy
requiredtoaddressthesedifficultquestions. CommitteeoftheSeattleArtificialKidneyCenter,
chosewhichmedicallyqualifiedpatientswithend
Keywords stage renal disease should receive hemodialysis.
Pediatric transplant ethics (cid:129) Organ donation (cid:129) Scribner’sarteriovenousshuntmeantmorepeople
Organ allocation (cid:129) Dead donor rule (cid:129) Organ could benefit from dialysis, making the resource
donation after circulatory determination of simultaneouslymorepreciousandscarce(Murray
death (DCDD) (cid:129) Organ donation after neuro- etal.1962).Howshouldthegroupchoose,know-
logicaldeterminationofdeath(DNDD)(cid:129)Liv- ingthosenotchosenwoulddie?Criticismofthis
ing organ donation (cid:129) Transplant eligibility (cid:129) group’sinconsistentandbiasedselectionprocess
Children with disabilities (cid:129) Parental refusal of ledtothedevelopmentoftheUnitedNetworkfor
treatment (cid:129) Rule of rescue (cid:129) Justice (cid:129) Equity (cid:129) Organ Sharing (UNOS) and the Organ Procure-
Efficiency(cid:129)Respectforpersons(cid:129)Medicalbest ment and Transplant Network (OPTN), which
interest havedevelopednumeroussafeguardstoensurea
national, explicit, transparent, and systematic
approachtoorganallocationintheUnitedStates
Introduction (Alexander1962;Jonsen2007).
Despite significant improvements in transplant
Remarkableadvancesinpediatrictransplantmed- medicine,thediscrepancybetweenthesupplyand
icine are highlighted throughout this text book. demand of transplantable organs persists, leading
Sincethefirstsuccessfulsolidorgantransplantsin transplantprofessionalstoconstantlygrapplewith
the 1950s and 1960s, solid organ transplantation issuesofjustice.AccordingtoUNOS,asofAugust
isnowevolvingasthestandardofcareformany 2015, 122,428 people were awaiting transplants,
children with end stage organ failure, leading to including 1,876 children (age lessthan 18 years).
the prolongation of many lives that historically Yet,in2014,onlyatotalof29,532transplantswere
wouldhavebeenlost(Grenvik1988).Thefunda- performed, 1,795 of which were in children
mentalquestinmedicine,tosavelifeandstaveoff (UNOS2015).Howshouldweallocatetheavail-
death, continues to drive these success stories ableorgansinanequitablemannerwhilemaximiz-
(Strong and Lynch 1998). Yet, hidden between ing the benefit of a scarce resource? Who should
are the stories of loss, where children have been have priority and on what grounds? How do we
subjectedtomultiplesurgeries,toxicmedications, ensure that listing and evaluation practices are
and devastating infections. Other children have equitable across transplant centers? How should
diedwithouttheopportunityfortransplantevalu- we optimize patient outcomes when the risks of
ation (Fox and Swazey 1998). Attention to the innovativetherapiesarepoorlyunderstood?Asthe
ethical issues endemic to transplantation allows field of pediatric transplant medicine progresses,
ustohonorthepatientswhohavetaughtus,lived the constant inclusion of ethical analysis ensures
through complications, or died as well as those equal consideration of the medical and the social
whoneverhadafairchanceatatransplant. implicationsoflistingandtransplantationpractice.
AllocationConsiderations CompetingResponsibilities: The
Individual Patient and Society
Imbedded in transplant medicine are numerous
ethical questions (Aulisio et al. 2007). Albert Al Jonsen scrutinized the Rule of Rescue, cap-
Jonsen, a philosopher and ethicist, argues that tured in the following statement: “the dominant
the origin of bioethics is rooted in the history of tradition is for the physician to provide the best
transplant medicine (Jonsen 2007). In 1962, the care of which he is capable for those who either
EthicalConsiderations 3
seek his services or are assigned to his responsi-
bility;byandlargethisisdonewithoutregardthe Case#1:PediatricOrganDonation
fortheconceivablybroaderissueofwhethertreat- andtheDeadDonorRule(continued)
ment is justifiable on social grounds.” (Jonson extremely poor neurologic prognosis.
1986) Yet, as technology advances, health care According to the local organ procurement
costs rise, and organ scarcity persists, there is a organization (OPO), Janie is a potential
ubiquitous tension between the transplant team’s candidatefororgandonationaftercircula-
obligation to their own patients and to other tory determination of death (DCDD). Her
unknown patients in need of their services. Cer- parentsarehopefulthattheirdaughtercan
tainly, transplant teams have an obligation to givethe“giftoflife”asafinalexpressionof
advocate for their own patients. However, like their daughter’s generous nature and in
publichealth,transplantmedicinedoesnotafford hopes that “something good” might come
the luxury of exclusive dedication to one’s own out of this tragedy. However, they want to
patients(Kohnetal.2011;McKieandRichardson holdtheirdaughterforatleastanhourafter
2003; Osborne 1994; Strong and Lynch 1998). she dies so that she “passes to heaven in
While some argue that clinicians should not theirarms.”Unfortunately,holdingherfor
make societal level decisions at the bedside anhourimmediatelyfollowingherdeathis
(VeatchandRoss2015),manyargueitwouldbe notcompatiblewithDCDD,andtherefore,
irresponsible not to do so. When one child shecannotbeanorgandonor.Herparents,
receives a cadaveric organ, another child goes frustrated by the circumstances, inquire
without, burdened by all the risks associated whyherliverandkidneyscannotberecov-
with a longer wait time. Physicians reasonably ered prior to withdrawal of the ventilator,
struggle with this moral tension, feeling that pri- whichwouldallowhertobeadonor,after
oritizingotherpatientsisaformofabandonment which they could withdraw the ventilator
ofone’sownpatient. and hold her for an hour immediately fol-
lowingherlastbreath.
Case Based Discussions
This case highlights long-standing debates
Each case below highlights common ethical about the Dead Donor Rule. The Dead Donor
issues in pediatric transplantation. These cases, Rule (DDR) codifies two professional obliga-
whilehypothetical,areallbasedonactualclinical tions: (1) vital organsshould only be taken from
cases.Thecasesrangefrompediatricorgandona- deadpeopleand(2)livingpatientsshouldnotbe
tion (Cases 1–2) to pediatric transplantation killedfororbyorganprocurement(Bernat2013;
(Cases 3–6), highlighting the integral nature of Robertson 1999). At first the rule seems
thesetwoprocesses. uncontroversial, for “the intentional killing of an
innocenthuman,evenforthegoodcauseofsav-
ingthelivesofothers,hasbeenalmostuniversally
Case#1:PediatricOrganDonation viewedasbothunethicalandillegal”(Veatchand
andtheDeadDonorRule
Ross2015).Yet,thechallengeindeterminingthe
Janie, an 8-year-old girl presents with a
timing of death raises ethical concerns(de Groot
severe traumatic brain injury following an
et al. 2012; Munshi et al. 2015; Rady and
automobile accident. After aggressive
Verheijide 2012; Sheth et al. 2012; Shore et al.
resuscitation, physicians discover she has
2012;Windetal.2012).
lostallneurologicalfunctionexceptforthe
Myriad philosophical, medical, religious, and
drive to breathe. Her parents choose to cultural interpretations influence the determina-
withdraw the ventilator based on her tion of death (President’s Commission 1981;
Shemieetal.2014).IntheUnitedStates,despite
4 J.D.ClarkandD.M.Dudzinski
alackofconsensus,currentmedicalpracticeand According tothe DDR and theUniform Ana-
thelegal systemrely ontheUniform Determina- tomicalGiftAct(UAGA),anactthatallowsper-
tionofDeathActof1981.Thisactstatesthat“an sons to express their interest in donating their
individualisdeadifthereisirreversiblecessation organs as a gift through donor registries, organ
ofcirculatoryandrespiratoryfunctions,orifthere recovery is permitted either following the deter-
is irreversible cessation of all functions of the mination of circulatory death (DCDD) or neuro-
entire brain, including the brain stem” (Grenvik logical death (DNDD) (Bernat 2013; Workman
1988;President’sCommission1981).Ifdyingisa et al. 2013). Historically, organ recovery has
progressive process of cellular demise, and the shifted between DCDD and DNDD. In the
declarationofdeathisdeterminedbyphysicians, 1950s and early 1960s, organ recovery occurred
therewillalwaysbeambiguityaboutwhensome- exclusivelyfollowingDCDD.In1968,following
oneistrulydead.Inmanyways,deathisasmuch advancements in mechanical ventilation, a Har-
a moral as a biological progression. In donation vardcommitteedevelopedclinicalcriteriaforthe
afterdeterminationofcirculatorydeath(DCDD), diagnosis of brain death (Beecher 1968), and
the“notouch”waittimesbetweendeclarationof organ recovery primarily shifted to DNDD
deathandprocurement(rangingfrom90sto5or (Bernat 2013; Workman et al. 2013). In the last
more minutes) are designed to honor the Dead two decades, based on growing disparities
Donor Rule, but some have argued that the bio- between the supply and demand of organs for
logical determination should matter less than the transplantation, DCDD is now an increasingly
moral one (Bernat 2010; Rady and Verheijide common practice, supported by multiple profes-
2012; Sheth et al. 2012; Breierley and Larcher sional societies and organizations (American
2011). Truog and Robinson shift the question AcademyofPediatrics 2013;Bernat2013;Gries
from “is the patient dead” to “are the harms of et al. 2013; Breierley and Larcher 2011). While
removinglife-sustainingorganssufficientlysmall DCDD remains controversial at the institutional
that patients or surrogates should be allowed to level, particularly in pediatrics, many hospitals
consent to donation?” (Miller and Truog 2008; have adopted policies allowing organ recovery
Truog and Miller 2008; Truog and Robinson based on circulatory determination of death
2003; Truog et al. 2013). With proper analgesia (Workman et al. 2011; Consolo and Wigmore
andcare,Janie’sorganscouldberecoveredifthe 2014; Martin et al. 2015; Matheny et al. 2009;
latter question prevailed, but not the former. Naimetal.2008;Sarnaiketal.2013;Stiersetal.
Recoverywouldbepermittedviaahighlycontro- 2015;Workmanetal.2013).Inpediatrics,society
versial practice called imminent death donation, givesparentsa“limitedauthoritytodecidewhatis
in which Janie’s kidneys would be recovered in bestfortheirchildrenandtheextenttowhichtheir
the operating room prior to withdrawal of the childrenshouldcontributetoothers,evenifdoing
ventilator. Following organ recovery, she would soisnotnecessarilyintheirmedicalbestinterest”
bereturnedtoherparents atwhichtimetheven- (Veatch andRoss2015).Hence, oursociety pro-
tilator would be discontinued to allow her to die vides parents with the authority to consent to
(Morrissey2012).Whilethereisacertainconsis- deceased organ donation from a minor either
tencyandscientifichonestyaboutacknowledging through DCDD or DNDD, even if there is no
theambiguityofdeterminationsofdeath,thereis directbenefittothechild(Martinetal.2015).
a grave societal risk to abandoning the DDR. The controversies around DCDD and immi-
Organ recovery becomes the direct cause the nent death donation cannot be adequately
patient’sdeath(regardlessofunderlyingterminal addressedhere,buttheyreflectanimplicitmoral
illness), a violation of a deeply held ethical obli- commitment to increase the donor pool. The
gation notto kill patients (Rodriguez-Arias etal. underlyingethicalquestionremains:doesincreas-
2011; Veatch 2015). For this reason, imminent ingthedonorpoolpromisesuchbenefittorecip-
death donation remains highly controversial and ients to justify recovering organs from the dying
iscurrentlynotpracticed. even when the recovery itself is the cause of
EthicalConsiderations 5
death? Some say “yes” (Morrissey 2012; Truog
and Miller 2008); others “no” (Bernat 2013; Case#2:ParentsandMinorsasLivingOrgan
Veatch 2015). Fundamental to the practice of Donors(continued)
medicine is the ethical principle of non- deceased donor liver, which would poten-
maleficence, the professional duty to minimize tiallydelaythelivertransplantandincrease
the harm of medical care itself and permit only the child’s risk of death, or should his
harmsthatarelikelytoleadtogreaterbenefitfor mother serve as a living related donor
the patient. Emphasis on innovation, including withalltheassociatedriskstoher?
alternativetreatmentsfororganfailureaswellas
split liver donation and domino donation, are
mechanisms designed to increase the donor pool Livingorgandonationisanothermechanismto
without significantly increasing risk of harm to increasethesupplyoforgansfor transplantation.
potential organ donors (Careddu et al. 2015; Since living organ donation provides no health
Feier et al. 2014; Marin-Gomez et al. 2014; benefits (only risk) to the donor, numerous safe-
Matsunamietal.2015;PopescuandDima2012; guardshavebeendevelopedthroughfederalreg-
van Dijk et al. 2014). The responsibility for ulations to ensure that living donors make
balancingharmsandbenefitsindonorsandrecip- informed, coercion-free, donation decisions. The
ientsfallstohealthcareproviders,transplantpro- donormustbe:(1)medicallyandpsychologically
fessionals, and policy makers. In the case suitable for donation, (2) deemed competent to
scenario,Janie’sorganscouldberecoveredpost- make the decision, (3) willing to donate without
humously through DCDD, yet her parents’ wish pressure or manipulation, and (4) fully informed
to hold her at the time of death needs to be hon- of the risks, benefits, and alternatives to both
ored.Furtherdiscussionwithherparentsandthe donor and recipient (Consolo and Wigmore
organ procurement organization, in addition to 2014; Veatch and Ross 2015; Ventura 2010). In
coordination with a palliative care team, may order to adequately protect living organ donors,
lead to a plan that respects her parents’ wishes this process requires that the potential donor has
andallows fororganrecoverywithinthecontext an independent donor advocate team to provide
oftheDDR. unbiased support with special attention to donor
needs(Ventura2010).
In pediatrics, usually a parent or adult family
Case#2:ParentsandMinorsasLivingOrgan
memberdonateseitherakidneyoraportionofa
Donors
liver to the child. Parent-to-child living donor
Frank,a12y/oboy,presentscriticallyillto transplants may reduce the child’s requirement
theintensivecareunitwithacutefulminant
for immunosuppressive therapies and reduce
hepatic failure of unclear etiology. Due to graft failure rates, clear recipient benefits (Feng
rapidprogressionandworseningencepha-
et al. 2012; Nijagal et al. 2012). However, is a
lopathy, a liver transplant is emergently
parent,whoisincrisismodewiththeiracutelyor
necessary. Transplant evaluation reveals
chronically ill child, capable of making an
that his motherqualifies as a potential liv-
informed donation decision? (Ventura 2010).
ing donor, and she strongly desires togive
Doesaparenttrulyhaveachoice,givenherrole
the “gift of life” to her son. However, her
astheprotectorforherchild?
anatomy is slightly abnormal, making
Empirical data reveal that many parents and
organrecoverypotentiallydifficult,increas- closefamilymembersmakeextremesacrificesto
ingtheriskstothemother.Despiteknowing
save the lives of children, even for children with
these risks, the child’s mother pleads to
renal failure, when dialysis is an alternative
donate her liver to save her child’s life.
(Crowley-Makota et al. 2004; Forsberg et al.
Should the child instead be listed fora
2004,Spital2005).Onequalitativestudyrevealed
that the majority of parental liver donors “never
6 J.D.ClarkandD.M.Dudzinski
reallymadeadecisiontodonate;rather,agreeing underthisagemaynothavethecapacitytomakea
to donate was an automatic leap” (Crowley- fully informed decision. The donor advocate
Makotaet al.2004). Another study revealed that shouldhave: (1) “training and educationin child
there was a “total lack of choice” in parental development and child psychology, (2) skills in
donation. Parental liver donors felt a moral obli- communicating with children and understand
gationandcouldnotaccepttheguiltofsayingno children’s verbal and nonverbal communication,
(Forsberg et al. 2004). In reality, while parental and(3)workingknowledgeoftransplantationand
donors are not typically coerced by health care organ donation” (Ross et al. 2008). Finally, the
providers, the intimacy of the parent-child rela- AAP includes an additional criterion that
tionship and obligatory nature of the donation “requires the emotional and psychological risks
mayunderminethevoluntarynatureofthechoice to the child donors be minimized” (Ross et al.
(Crowley-Makota et al. 2004; Spital 2005). The 2008).Priortoconsideringanyminorasapoten-
processofparenttochilddonationmustbecare- tial living donor, transplant centers should care-
fully evaluated by transplant teams. Rather than fully review these guidelines and incorporate
prohibitingparentsfromdonating,transplantcen- themintohospitalproceduresandpolicies.Child
ters have an obligation to use a systematic living organ donation should continue to be
approach in providing medical, psychological, viewedasalastresort.
social, and spiritual support to parental donors,
particularly when donation decisions need to be
Case#3:TheImpactofNationalOrgan
made urgently (Crowley-Makota et al. 2004;
AllocationonIndividualPatients
Forsbergetal.2004;Reding2008;Thomasetal.
Twocriticallyillinfantsarehospitalizedin
2014; Toker and Salzar 2012; Spital 2005). Fur-
the same cardiac intensive care unit, both
thermore, if the transplant is not successful, the
requiring life prolonging interventions for
donating parent needs help from the transplant
endstageheartfailure.Pedroisa9-month-
team to cope with their crushing loss and guilt
old boy with single ventricle physiology
(Erimetal.2012;Forsbergetal.2004).
who suffers from progressively worsening
Althoughthemajorityofpediatriclivingdona-
heart failure and is not a candidate for
tioninvolvesanadultdonatingtoachild,children
further palliation. He requires inotropic
have also served as living donors (Ross et al.
support and mechanical ventilation. He
2008; Veatch and Ross 2015; Breierley and
hasbeenlistedforadeceaseddonorheart
Larcher 2011). While extremely rare, theAmeri-
transplant for approximately 2 months.
can Academy of Pediatrics (AAP) “holds that
Mariaisa3-month-oldgirlwithcongenital
minors can morally serve as living organ donors
cardiomyopathy who has been supported
but only in exceptional circumstances when spe-
with mechanical ventilation and inotropic
cificcriteriaarefulfilled”(Rossetal.2008).These
support for 2 weeks. She was listed for a
criteria are adapted from the US Live Organ
deceaseddonorhearttransplantinthelast
DonorConsensusGroupandinclude:(1)“poten-
week,asthereisconcernthatsheisrapidly
tialdonorandrecipient[must]behighlylikelyto
worseningandwillneedaventricularassist
benefit”; (2) “Surgical risk for donor must be
device,whichwillbetechnicallydifficult.A
extremely low”; (3) “all other opportunities for
heartbecomesavailablethatwouldbesuit-
transplantationhavebeenexhausted,nopotential
able for both Pedro and Maria. Based on
adultlivingdonorisavailable,andtimelyand/or
listingorderandobjectivemarkersofsever-
effective transplantation from a cadaver donor is
ityofillness,Pedroshouldreceivetheavail-
unlikely”;and(4)“theminor[must]freely[agree]
able heart; however, the cardiology and
to donate without coercion (established by the
critical care teams are concerned that
independentdonoradvocate)”(Rossetal.2008).
Inaddition,theAAPrecommendsanagecut-off (continued)
of at least 11 years, acknowledging that children
EthicalConsiderations 7
of distribution, meaning patients with similar
Case#3:TheImpactofNationalOrgan medicalconditionsshouldhaveequalopportunity
AllocationonIndividualPatients(continued) for transplant evaluation and should have equal
Maria’s clinical status is worsening at a access once listed. Factors such as race, gender,
muchfasterratethanPedro’s.Thecardiol- geographic location, and socioeconomic status
ogist and critical care team struggle with shouldnotforeclosethepossibility ofevaluation
theallocationsystem. (OPTN/UNOSEthicsCommittee2015).
Applyingtheseprinciplesraisechallengesand
controversies. Most ethicists argue that equity
While this scenario rarely occurs at a single should be prioritized above efficiency (Veatch
transplantcenter,organallocationaffectschildren andRoss2015).Systemsthataccountmerelyfor
at the regional and national levels every day. efficiencywouldleadtoinjustice,where“difficult
When one child receives a life-saving organ, to treat” patients would be excluded. Alterna-
another child goes without and faces with addi- tively,accountingmerelyforequityordistributive
tional risks and potential complications of justicewouldleadtoanincreaseinthenumberof
waiting,includingdeath.Inthisscenario,thephy- organs that are not successfully transplanted.
sicians at the bedside are direct witnesses and Within this context, prioritization of these two
arbiters of an imperfect allocation system. Who principles becomes challenging (Veatch and
and how should the clinicians decide? Should Ross 2015). Should a patient who is less sick
theydefertothenationalsystem,knowingthatit and lives closer receive the organ to minimize
was developed based on an explicit, transparent, warmischemictime,orshouldtheorganbetrans-
and expert guided system? Or knowing the two ported further away to a sicker patient, risking a
criticallyillchildreninfrontofthem,shouldthey longer ischemic time and delayed function? Is
decide?Cantheyreliablypredictwhichchildwill ranking distribution based on age unfair, espe-
havethebetteroutcome? cially consideringthatgraftsurvivalinteenagers
Organ allocation is regulated at the national tends tobeshorter? Should deceaseddonorsplit
level by the National Organ Transplant Act liver donation be performed, in which one lobe
(NOTA) and the Organ Procurement and Trans- goestoachildandonetoanadult?Thisprocess
plantation Network (OPTN) Final Rule (OPTN/ allows two people to benefit from one donor
UNOS Ethics Committee 2015). Based on organ, but could lead to worse outcomes for the
advances in surgical technique and medical ther- adultwhomaydobetterwithafullliver.
apies,theoutcomesfollowingtransplantcontinue Second, when considering efficiency, the
to rapidly evolve. Appropriately, these improve- assessment of benefits and burdens is subjective
mentscontinuetoleadtoongoingevaluationsand and based on the perceptions of the stakeholders
adjustments to the organ-specific allocation sys- involved. For example, factors that may be
tems.Foreachorgan,theallocationssystemshave included in the benefit to burden ratio include
undergonemanyrevisionsinthelastfewdecades. number of lives saved; reduction in graft failure;
Overall, the regulations are guided by three improvementinqualityoflife,usingcalculations
ethical principles: (1) equity, in which patients such as the quality-adjusted life years (QALY);
with similarconditions shouldhaveequal access andtheavailabilityofalternativetreatments,such
tosimilartreatments;(2)efficiency(orutility),in as dialysis (Veatch and Ross 2015). While all of
which organs should be distributed in a manner thesemeasuresshouldbeincludedinthecalcula-
thatoptimizeoutcomesforthegreatestnumberof tion, objectively comparing patients based on
people;and(3) respect forpersons,inwhichthe these factors is challenging (Keller et al. 2014).
system is transparent and respectful of autono- Who decides who receives greater benefit? For
mousandnonautonomouspatientsalike(OPTN/ example,whenthecurrentlungallocationscoring
UNOSEthicsCommittee2015;VeatchandRoss system wasdeveloped,childrenundertheageof
2015). Equity emphasizes fairness in the pattern 11 were excluded because adult lungs cannot
8 J.D.ClarkandD.M.Dudzinski
successfullybetransplantedinchildren(anappeal
to efficiency). However, Sarah Murnaghan was Case#4:TransplantEligibilityforPediatric
10 years old in need of a lung transplant due to PatientswithComplexSocialCircumstances
pulmonary failure secondary to cystic fibrosis. orMedicalConditions(continued)
Because the allocation scoring system did not dependent, but has suffered from poor
includechildrenundertheageof11,Sarah’spar- growth over the past 6 months. Despite
ents believed the system unfairly discriminated nutrition consults and gastrostomy tube
against her. They filed a lawsuit against UNOS, placement, he continues to demonstrate
and the adult lung allocation system was tempo- poor growth. The nephrologist believes he
rarilymodifiedtoincludechildren.Althoughher would benefit from a transplant; however,
first lung transplant immediately failed, she was the child’s social situation is complex. He
successfullytransplantedwithinamonth(deSante haslivedwithhisauntsincetheageofthree
etal.2014).Asaresultofthiscase, UNOScon- becausehismotherlostcustodyduetosub-
tinuestograpplewiththeseissues,includingpri- stance abuse. His aunt is a single mother
oritization for pediatric patients (OPTN/UNOS withthreechildren.Despiteherbestefforts,
EthicsCommitteeandPediatricTransplantCom- shehasnotbeenabletobringhimtoallof
mittee 2014). Fortunately, for Sarah, her parents his appointments and occasionally has
had the will, means, and access to draw national failed to fill his prescriptions. The family’s
attention to Sarah’s ordeal, yet not all patients primary language is Spanish. When the
sharethisadvantage(deSanteetal.2014). childispresentedtothetransplantteamto
Finally, loopholes in the organ allocation sys- determineeligibility,thenephrologistslack
tems,suchastheuseofexceptionscoresinpedi- consensusastowhetherthechildshouldbe
atric liver transplantation, can lead to unfair listed.
practices. Exception scores are used to account
for disease factors beyond chronic liver failure
that are not reflected in pediatric end-stage liver Althoughorganallocationisanational,trans-
parent, comprehensive, and federally regulated
disease(PELD) scores andmay increasethe risk
process, institutional listing practices are largely
for morbidity and mortality. These exception
unregulated.Whiletransplantteamsattheinstitu-
scores ultimately prioritize these patients on the
tionallevelareheldaccountabletoUNOSandthe
transplant waiting list. In a retrospective cohort
OPTNfortheirtransplantoutcomes,thespecifics
study,Hsuetaldemonstratedregionalvariability
of their listing practices are not monitored or
andracialandsocioeconomicdisparityintheuse
federally regulated. Yet listing is necessary to
of exception scores. Those without exception
gain access to the national organ allocation sys-
scores had a decreased likelihood of transplanta-
tem.Ifapatientisneverreferredorevaluatedfor
tion (Hsu et al. 2015). These disparities are
transplant,shewillcertainlynotreceiveone.Are
concerning and raise important consideration for
listinginclusionandexclusioncriteriafair?What
ongoingreviewoftransplantratesatthelocaland
arethelistingcriteriabasedonandhowaretrans-
national levels to improve the creation of a fair
andefficientorgandistributionsystem. plantteamsheldaccountableforlistingpractices?
The same ethical principles of organ allocation
alsoapplytolistingpractices.
Case#4:TransplantEligibilityforPediatric Childrenwhoarewardsofthestate;havepar-
PatientswithComplexSocialCircumstances ents who are “nonadherent”; are from families
orMedicalConditions with limited English proficiency; or who have
A 5-year-old boy, Henry, has stage V geographic or financial limitations are at risk for
chronic polyuric kidney disease due to
both explicit and implicit judgments regarding
obstructive uropathy. He is not dialysis
transplanteligibility.Thesechildrenmaybeinap-
propriately excluded from consideration. While
EthicalConsiderations 9
access to long-term medical care is essential for pediatric institutions should build support net-
optimal transplant outcomes, social inequities works designed account for and address dispar-
also mean that these children will be excluded ities so that equity guides pediatric transplant
unless resources are devoted to helping them evaluation (Bilhartz et al. 2015; Connelly et al.
strengthensocialsupportandoptimizelongterm 2015; Griffin and Elkin 2001; Moseley and
medicalcare.Justicerequirestransplantteamsto Kershaw2012).
ensurethatthesociallyoreconomicallydisadvan- In addition to the social vulnerabilities
taged or marginalized are not excluded simply described above, children with genetic syn-
because of these factors. If we know resources dromes,cognitivedysfunction,psychiatricdisor-
are limited for many in our society, failure to ders, and physical disabilities are also at risk for
provide provisions for patients living in those discriminationwithregardtotransplanteligibility.
circumstances simply perpetuates the societal In 2013, the parents of Amelia Rivera, a 3-year-
injustice. Multidisciplinary teams help to ensure old girl with Wolf-Hirschorn disease, publicly
these patients are not lost. Such teams should suspected discrimination based on Amelia’s
include social workers, child development and “mental disabilities.” Within a few months,
education specialists, child life specialists, cul- Ameliasuccessfullyreceivedakidney transplant
tural navigators, language interpreters, palliative from her mother (USAToday 2013). While this
care specialists, medical specialists, and trans- case was unique in the publicity it gained,
plant surgeons (Fowler et al. 2015; Lefkowitz Richards et al found that there are inconsistent
etal.2014).Inaddition,apatientorfamilyadvo- practices among national transplant centers
cate is especially important for children who are regarding the process for neurodevelopmental
vulnerable,broadlyspeaking. assessment and how this assessment influences
If social circumstances are challenging, trans- transplant eligibility (Richards et al. 2009).
plantteamshaveethicalobligationstomarshalall While some may argue that transplant outcomes
themedicalandpsychosocialsupporttheycanfor are worse for children with disabilities, a retro-
thefamily.Transplantteamsshoulddevelopsup- spectivecohortanalysisfromalargeUNOSdata
portnetworksandsystemstohelpcaregiverspro- set of children who received their first kidney
vide the medical care required for successful transplants demonstrated no difference in graft
transplant.Becausecircumstancesmightalsopre- or patient survival in children with intellectual
clude“optimal”medicalcareforHenry,thetrans- disabilities compared to other pediatric patients
plantteamshouldshiftfocusandaskifadequate (Wightman et al. 2014). While further data are
support is feasible and likely. It is unfair and needed, precluding transplant eligibility solely
unreasonabletoexpectHenry’sfamilytodemon- on the basis of intellectual disability is not
strate the kind of adherence transplant teams supportedby empiricalevidence andis discrimi-
expect from well-funded, well-insured families natory (Panocchia et al. 2010). According to the
with a broad social network, reliable transporta- Convention on the Rights of Persons with Dis-
tion, and more flexibility in caregivers’ employ- abilities, “State Parties shall take all necessary
ment. The disadvantages already posed by the measurestoensurethefullenjoymentbychildren
children’ssocialcircumstancesshouldnotunduly with disabilities of all human rights and funda-
precludethemfrombeneficialmedicaltreatments, mental freedoms on an equal basis with other
but instead qualify them for additional support children” and “prevent discriminatory denial of
and benefits, whenever possible. Furthermore, health care or health services on the basis of
forpatientswhoarewardsofthestate,thetrans- disability” (United Nations General Assembly
plantteamshouldworkcloselywithchildprotec- 2006). Therefore, transplant teams should
tive services to ensure that medical foster care is developaformalevaluationprocessthatincludes
available (Kelley et al. 2012). In general, socio- neurodevelopmental and genetic specialists to
economic status, ethnicity, and gender should assess transplant eligibility in a transparent,
never preclude transplant evaluation, and informed, and inclusive manner (Richards et al.
10 J.D.ClarkandD.M.Dudzinski
2009). In certain circumstances, special accom- of best interest as well as different goals and
modations comparable to those made for Henry values. Typically, the transplant is driven to pro-
maybenecessaryforchildrenwithdisabilities. longthelifeofthechild;dependingonthetypeof
organ, the child may survive without significant
morbidities.Ontheotherhand,amongthediverse
Case#5:BestInterestandParentalRefusal
reasons why parents might refuse, parents may
ofTransplant
choose to avoid the short and long-term harms
Michael is an interactive and playful
and risks of the transplant itself. Parents may
9-month-old child with biliary atresia who
perceivetransplantasanexchangeofonechronic
suffersfromendstageliverdisease.Despite
illness(endstageorganfailure)foranother(long-
a 90 % survival rate at 5 years following
term immunosuppression, risk for graft rejection
livertransplant,thechild’sparentsrefuseto
andfailure);theymaychoosetoallowtheirchild
consent to transplant, stating that the
todieby“natural”cause.Forparents,uncertainty
child’s life is in “God’s hands” and their
inoutcomemayalsoplayarole.Whiletheriskfor
child must remain “whole.” The
significant morbidity may be reported as low,
hepatologist strongly recommends a trans-
predicting actual outcomes for an individual
plant,sinceMichaelwilllikelydowelland
child is nearly impossible. Parents of a child
will certainly die without it. Should child
with end stage organ failure may feel that their
protective services be enlisted to compel
child has “been through enough” and may not
listing? Or should he respect the parent’s
wish to pursue additional painful and harmful
wishes knowing there are many otherchil-
interventions thatcarry additional riskfor signif-
drenonthelistawaitingascarcecadaveric
icant morbidity. Under these circumstances, who
organ?
decides? Should the physicians refer the case to
child protective services in attempt to compel
listing for transplant? Should the parent’s deci-
Health care providers have the professional
sions be respected? Since organs are scarce,
andethical duty torecommend therapiesthat are
shouldtheybepreservedforchildrenwhosepar-
inthebestinterestsoftheirpatients.Pediatricians
entsareinagreementwithtransplant?
not only provide the best care for the child, but
Parentalrefusalofmedicalinterventionsisnot
also respect parental authority to make treatment
a new phenomenon in pediatrics, including
decisionstheymakefortheirchild.Thisauthority
refusal of organ transplants (Brahams 1996;
isconstrainedifthebasicneedsofthechildarenot
metorifthereissignificantriskofdirectharmto Coates 2000; Cronin et al. 2013; Downie 1994;
Dyer 1996; Shapiro 2005; Superina et al. 1999).
the child (Ross 1998; Diekema 2004). Although
there is significant risk for both short- and long- Ethicalframeworks,includingtheHarmPrinciple
and Constrained Parental Autonomy, have been
term morbidities following transplant, transplant
developedinattempttoaddresscomplexcasesof
teamsmaythinktransplantationhassuchhopeof
benefit that they want to compel treatment by parentalrefusalofmedicalinterventionsorthera-
pies (Diekema 2004; McDougall and Notini
enlisting child protective services. As the short-
2013; Ross 1998). Determining whether or not
andlong-termoutcomesfollowingtransplantcon-
an intervention or therapy should be compelled
tinue to improve, pediatric transplant teams may
feel more responsibility to “advocate” for their is not difficult at the far ends of the spectrum.
Medical interventions or therapies that are life-
patientsinthisway.
savingwithahighprobabilityofsuccessandare
In these complex situations, balancing the
associated with low morbidity and risk are typi-
advocacyroleofthetransplantteamandthepar-
callycompelled,includingemergentbloodtrans-
entsischallenging.Invirtuallyallcircumstances,
fusions, insulin for diabetes, and appendectomy
boththepatientandtheprovidersareadvocating
forappendicitis.Experimentaltherapies,orinter-
forthepatient,butareguidedbydifferentnotions
ventions that have a low probability of success,
