LOGIE ESSAY * DISSERTATION LOGIE

Anencephalic infants as organ donors:

Anencephalic inans as organ, dnors:Beware the slippery slope

Ferhaan Ahmad, MD

The Dr. William Logie medicalethics essay contest is open to stu-dents studying at Canadian medi-cal schools. The contest, named inhonour of Canada's first medicalgraduate, is sponsored by theCMA. Here we present the first-prize winner.

\ ver the past few decades,organ transplantation hasprovided hope to many

patients suffering from chronic orfatal illnesses. However, its use,especially for infants, is limited bythe relatively small number of or-gans available. Although cadaverdonors must fulfil brain-basedcriteria for death, infants rarelydie of insults that destroy thebrain but leave their organs intactfor transplantation.'

Because of the shortage ofappropriate infant donors, the useof various other sources, such asother animal species, human fe-tuses and dying human infants,has been proposed. Newbornswith anencephaly have recentlyreceived a great deal of atten-tion.2-7 The harvesting of theirorgans has been reported duringthe past 25 years.'

The use of this source seemsto offer benefits to almost every-one involved: recipients are oftenrescued from a fatal illness, bring-ing joy and relief to their families,and parents of donors are able toderive some satisfaction fromknowing that their child's short,tragic life had some meaning. Aswell, society is spared the cost ofcaring for a terminally ill child,and the transplantation costs willbe recovered when the recipientgrows to become a productive,taxpaying adult. Finally, insteadof watching helplessly as two ba-

Ahmad: first-prize winner

bies die, health care professionalsare able to save one.8,9

However, anencephalic in-fants do not meet existing criteriafor brain death, and this raisesnumerous ethical and legal issues.These concerns extend beyondanencephalic newborns and in-volve questions concerning thedefinition of death, the rationalebehind brain-death criteria, thenature of personhood and the va-lidity of performing nontherapeut-ic procedures on nonconsentingpatients.

Some background knowledgeis needed to understand the ethi-cal issues raised by the use oforgans from anencephalic in-fants.1'0' Anencephaly is caused bya failure of cranial neurulationthat results in the congenital ab-sence of the scalp, skull and fore-brain. Although some telencephal-ic tissue may be present, theseinfants have no functional cortex.The cause of the defect is un-known, but it is presumed to ap-pear in the first 4 weeks of gesta-tion. During pregnancy the diag-nosis may be made with ultra-sound examinations or detectionof a high maternal serum a-feto-protein level. Generally, when ananencephalic fetus is detected thepregnancy is terminated. If thebirth does occur, the diagnosiswill be obvious: the neonate will

236 CAN MED ASSOC J 1992; 146 (2)

Ferhaan Ahmad, a 1991 graduate of Mc-Gill University medical school, is a residentin internal medicine at the Royal VictoriaHospital, Montreal.

LE 15 JANVIER 1992

Strategies have been proposed to allow anencephalicinfants to be used as donors without legal or ethical

challenge, but each suggestion raises questions that go

beyond anencephaly and touch, literally, on issues of lifeand death.

lack much of the cranial vault andwill have a hemorrhagic mass oftissue in place of the normal cere-bral cortex.

The clinical picture foranencephalic infants who reachterm varies: one-half to two-thirdsare stillborn, and those born liveusually die within 1 week in theabsence of extraordinary care.They often survive longer whenmajor support is provided andsome have been reported to livefor months. These infants havespontaneous respirations andmany also have positive oculoce-phalic, pupillary and startle reflex-es.

Abnormalities of the visualand auditory systems may compli-cate the diagnosis of brain death,which depends on an examinationof cranial nerve function. As well,systems other than the brain mayalso be abnormal. The hearts andkidneys tend to be smaller andsometimes demonstrate anoma-lies, making them unsuitable fortransplantation.

The incidence of anencephalyappears to be decreasing, partlybecause of prenatal-screening pro-grams and elective abortions. Cur-rent US estimates place the inci-dence at 0.3 per 1000 births, giv-ing a US total of 1000 such birthsper year.

In the US, several authorshave estimated the number of pa-tients needing heart, liver and kid-ney transplantation and the num-ber of organs that could potential-ly be harvested from anencephalicbabies to meet this demand.2,10,12"13

Every year fewer than 1000 pa-tients with hypoplastic left heartsyndrome or endocardial fibro-elastosis - the respective inci-dences are 0. 1 6 and 0. 1 7 per 1000live births - require neonatalheart transplantation. Five hun-dred to 600 infants with biliaryatresia - the incidence is 0.07 to0.09 per 1000 live births - andcongenital metabolic disordersneed liver transplants each year.Neonatal kidneys are not abso-lutely necessary for transplanta-tion because infants are able toreceive adult kidneys.

Of the approximately 1000anencephalic infants born in theUS every year, only a fraction arepotential donors. Donors musthave sufflcient birth weight andsurvival time to allow transplanta-tion, and their organs must be freeof major malformations. Botkinl2estimates that from 300 to 450anencephalic infants per year maybe considered potential donors inthe US. However, Shewmon andcolleagues' suggest that the annualnumber of successfully transplant-ed anencephalic kidneys, heartsand livers will at most be, respec-tively, 0, 9 and 2.

They cite those numbers be-cause of factors such as the avail-ability of alternative treatments,the unsuitability of some anence-phalic organs, the refusal of someparents to agree to donate suchorgans, the difflculties associatedwith matching donors and recipi-ents, and the relatively low long-term survival rate for recipients ofdonor hearts and livers. Thus,

anencephalic infants may not beas rich a source of organs as somehave predicted.

The advent of organ trans-plantation meant a novel defini-tion of death had to be formulat-ed, and guidelines had to be de-veloped on when it was ethicallyand legally defensible to harvestorgans. A committee at HarvardMedical School developed abrain-based definition of deathmore than 20 years ago. Death issaid to have occurred upon:

* the irreversible cessationof circulatory and respiratoryfunction, or

* the irreversible cessationof all functions of the entire brain,including the brainstem.'2,14

Canada and many other juris-dictions have adopted the "dead-donor rule," which specifies thatanatomic gifts can be made onlyafter the donor has been declareddead. Anencephalic infants cannotbe considered dead because theyretain cardiorespiratory andbrainstem function. This is thecrux of the matter: should wemodify our definition of death, orthe way we view anencephalic in-fants, so that these infants may beused as organ donors? Many au-thorities have argued that the po-tential benefits should not beabandoned merely because theseinfants do not fulfil existing crit-eria for brain death.2-7

Strategies have been proposedto allow anencephalic infants tobe used as donors without legal orethical challenge, but each sugges-tion raises questions that go be-

CAN MED ASSOC J 1992; 146 (2) 237JANUARY 15, 1992

yond anencephaly and touch, lit-erally, on issues of life and death.Proposals that have appeared maybe grouped in several categories:waiting for death to occur beforeharvesting organs, expanding thedefinition of death, creating a spe-cial legal category for anencephal-ic infants, and defining them asnonpersons.

The current criteria for deathhave gained some acceptance. Thedead-donor rule has allowed or-gans to be recovered from manynonanencephalic donors, and ide-ally one would want the samerules applied to anencephalic in-fants. It is possible to wait for thepotential donor to die of cardiore-spiratory failure, but the hypoxiapreceding death damages the liverand heart and in general makesthem useless for transplantation.

An alternative is to providethe infant with ventilatory assis-tance and intensive care to pre-serve the organs until death oc-curs. In these circumstances,death may be pronounced whenall brain function ceases. Howev-er, there is no reason for braindeath to occur spontaneously inthis infant because mechanicalventilation and intensive care willmaintain the brainstem at thesame time it is preserving the

transplantable organs. Moreover,brain death is difficult to ascertainin any infant, but especially in ananencephalic one.1'8'"

Instead of using the brain-death criteria, one may removethe infant from ventilatory sup-port for short periods at regularintervals until the baby dies spon-taneously from cardiorespiratorycauses. Of course, the definitionof death depends on an irrevers-ible cessation of function, and the-oretically the patient can be re-vived. Nevertheless, there are con-vincing arguments that resuscita-tion is not ethically needed, oreven desirable, in this anencephal-ic patient. The sole reason forgiving the infant intensive supportin the first place is to preserve theorgans for donation. In otherwords, a nontherapeutic proce-dure is being performed on a pa-tient who cannot derive any bene-fit from it and who is in noposition to give consent.

This approach can give rise toethical difficulties that are seriousbut resolvable. It does seem toviolate the Kantian ethical man-date, which proscribes the use of aperson as a means to an end. Theprinciple of autonomy places greatvalue on respect for the personand the right to self-determina-

tion.'5 From this perspective, per-forming a nontherapeutic proce-dure on one nonconsenting indi-vidual solely to benefit another isimmoraL"6

On the other hand, the princi-ple of autonomy is balanced bythe principle of beneficence inmany clinical situations, particu-larly when incompetent patientsare involved. Although theanencephalic newborn does notbenefit from the prolongation ofits life, at least the infant does notappear to be harmed by it. If otherlives may be saved, it is probablynot a great indignity to give theinfant intensive support for ashort period. After all, brain-deadadults are also given similar sup-port until their organs can beharvested. The difference is thatthe adult is dead, whereas theanencephalic child is still aliveaccording to all criteria. From anethical point of view, briefly pro-longing life for organ-harvestingpurposes is probably less objec-tionable than some other propos-als.

The strategy described abovemay be ethically acceptable, butgives rise to many practical diffi-culties. In 1987, that protocol wasimplemented at the Loma LindaUniversity Medical Center in Ca-

INTRODUCING AN ARTH

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lifornia.'7 Twelve anencephalic in-fants were recruited. The first sixwere resuscitated immediately atbirth and aggressively maintained.Only one child met the criteria forbrain death in the 1-week limitimposed by the protocol. The lastsix were entered into a modifiedprotocol in which resuscitationwas not performed at birth, butonly when the patient was aboutto die spontaneously. Once again,only one child met the criteria forbrain death within 1 week ofbirth. The other babies continuedto survive until supportive mea-sures were discontinued after 1week.

Not 1 of the 12 subjects, in-cluding the 2 who met brain-deathcriteria in the 1-week limit, wasfound suitable for organ donation.Therefore, providing intensivecare while awaiting brain deathdoes not seem worth while.

Since it does not seem possi-ble to work within current legaland ethical constraints, more in-novative means to allow harvest-ing of anencephalic organs havebeen suggested. One proposal in-volves redefining death as the ab-sence of cortical function insteadof whole-brain function. Whereasthe brainstem controls only "vege-tative" functions, it is the higher

Anencephalic infantsdo many of the samethings that normalinfants do, and theparents of one mayhave great difflcultyaccepting that their

child is dead.

brain that is responsible for en-dowing human qualities - per-sonality, consciousness and socialinteraction. In other words, it isthe higher brain that is responsi-ble for personhood. If its function-ing has been irreversibly disrupt-ed, the individual ceases to be aperson and is dead. The anence-phalic infant, lacking a functionalcortex, could therefore be regard-ed as dead and as a suitable organdonor.

This redefinition of death hassome philosophical merit, but its

adoption may have serious socialand legal consequences. One diffi-culty is that we would abandonbrain-death criteria that have,over time, been accepted by mostsegments of society. Anencephalicinfants do many of the samethings that normal infants do, andthe parents of one may have greatdifficulty accepting the idea thattheir child is dead. Public confi-dence in the procedures used todetermine death, in health careprofessionals and in organ trans-plantation programs may be seri-ously eroded. Death is seen bymost people as an all-or-nothingtruth. They may feel that authori-ties are artificially redefiningdeath every once in a while toaccommodate organ donation, sothe social difficulties involved inexpanding the definition of deathare formidable.

The cortical-death definitionalso appears to be rather ambigu-ous. If cortical-function criteriaare to be used, then the degree ofimpairment to pronounce deathmust be determined. People suf-fering from severe mental retarda-tion or various dementias, for ex-ample, might also fall within anexpanded definition of death. In-deed, the desire to find organdonors may encourage progres-

RITIS THERAPY THAT OFFERS EFFICACY AND

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sively looser criteria for death.This phenomenon, the "slipperyslope," is well known.

The use of cortical-death crit-eria, then, is undesirable becauseit may cause public confidence toerode and may be applied inap-propriately in clinical situationsnot originally envisaged.

Other authors have recog-nized that anencephalic infantsare living persons. They proposeleaving the definition of deathintact, and simply exemptinganencephalic infants from thedead-donor rule. This rule, meantto protect dying patients, cannotbe meaningfully applied toanencephalic infants, who lackany interests. A variation of thisargument is that these infantsshould be placed in a special cate-gory that is legally equivalent tobrain death so that organs may betaken from them. This specialtreatment is justified on thegrounds that anencephalic infantsare unconscious ("brain absent"),are facing imminent death, andhave no hope of improving.2,7' 6Thus, we can avoid modifying thebrain-death criteria and at thesame time can harvest organsfrom this one special group ofpatients.

This strategy is attractive in

some ways, but there are pitfalls.If the anencephalic infant is recog-nized as a living human beingwithout interests, a great burdenwill be placed on the shoulders ofparents who are asked to donatetheir child's organs. By allowingremoval of the heart, liver andkidneys they will be acceding tothe active killing of their baby.Even if this newborn has no legalrights, the guilt associated withthe decision may outweigh thesatisfaction the parents feel afterhelping save another infant's life.

As well, technical errors maybe made in categorizing infants asanencephalic - the definition ofanencephaly is not precise.8 More-over, even with a clear definitionthis condition cannot always bediagnosed precisely. It has beenquestioned whether anencephalicchildren lack all capacity for con-sciousness. "The difference be-tween decerebrate and normalnewborns lies not so much in theiractual functional abilities as intheir potential for future cognitivedevelopment," Shewmon and col-leagues' assert. "Therefore, bothprudence and logical consistencydemand that we attribute toanencephalic children at least asmuch consciousness and capacityfor suffering as we attribute to

laboratory animals with evensmaller brains, which everyoneseems to feel obliged to treat 'hu-manely.' "

It appears, therefore, that thebasis for ascribing a special statusto anencephalic infants may beinvalid. Moreover, this proposalmay not be practical in the realworld.

As with the proposals to rede-fine death, attempts to reclassifyanencephaly may put society on aslippery slope. This concept sug-gests that a "good" practice, ifallowed, will lead to an "evil"practice. This link between"good" and "evil" may take twoforms. The conceptual slope ispresent when there is a conceptualsimilarity between the two prac-tices and there are no clear dis-tinctions separating them. Theempirical slope may be observedwhen there are clear distinctionsbetween the two practices, butuncontrollable societal forces willlead us inexorably from a "good"practice to an "evil" one."

Here, the conceptual slipperyslope applies in this way: if onetakes organs from these infants,there is no reason to prohibitsimilar harvesting from other in-fants with severe brain abnormali-ties and terminal illnesses, and

TOLERABILITY WITH LITTLE IN VITRO EFFECTI

indeed from adults in a perma-nently vegetative state.

The empirical slippery slopemay lead us to apply the sameprinciples to other people whocurrently have legal rights. Thosepeople who are going to die soonanyway - patients in the endstage of a terminal illness, forinstance, or prisoners on deathrow'l'3 -may be killed at anopportune time when an organrecipient has been found. Once aspecial exception has been madefor one group of human beings,there is no reason why exceptionsshould not be made for othergroups. Because anencephalic in-fants may not be able to satisfymuch of the demand for organs,sociey may in fact be encouragedto find additional exceptions.

This slippery slope has al-ready been observed. Among thebabies referred to Loma LindaUniversity as potential donorswere infants "born with an abnor-mal amount of fluid around thebrain or those born without kid-neys but with a normal brain."'The referring physicians were un-able to make a distinction be-tween patients with anencephalyand those with milder disorders.Consequences of creating a specialcategory for anencephalic infants,

Because anencephalicinfants may not be

able to satisfy much ofthe demand for

organs, society may infact be encouraged to

flind additionalexemptions.

then, can range far beyond theintended scope.

The final approach to thequestion of anencephalic organdonation is based on the conclu-sion that anencephalic infants arenot persons. Some philosophersargue that all newborns, whethernormal or otherwise, are nonper-sons and acquire personhoodsome time after birth. Many oth-ers are less extreme, but maintainthat personhood is incompatiblewith severe neurologic deficits: "Ifwe compare a severely defective

human infant with a nonhumananimal, a dog or a pig, for exam-ple, we will often find the nonhu-man to have superior capacities,both actual and potential, for ra-tionality, self-consciousness, com-munication, and anything elsethat can plausibly be consideredmorally significant. Only the factthat the defective infant is a mem-ber of the species Homo sapiensleads it to be treated differentlyfrom the dog or pig. Species mem-bership alone, however, is notmorally relevant."'8

Many of the concerns raisedover the expansion of the defini-tion of death and the creation of aspecial category for anencephalicinfants also apply to their classifi-cation as nonpersons - distin-guishing between degrees of neu-rologic deficit, errors in diagnosis,erosion of public confidence inthe system, and a slippery slopeleading to an ever-increasingnumber of people being consid-ered nonpersons. In addition, re-garding an anencephalic infant asa nonperson would deprive him ofall rights, including protectionagainst human experimentation.It is unlikely that a mother wouldeasily accept the contention thatshe has given birth to a nonper-son.

ON CARTILAGE PROTEOGLYCAN SYNTHESIS.

In a world where material resources are limited, we mustmake difficult choices: what is possible is not always

appropriate.

Although the use of anence-phalic infants as organ donors ini-tially seemed to be of great bene-fit, offering hope and solace toalmost everyone involved, a morecomprehensive examination re-veals great cause for concern. TheBioethics Committee of the Cana-dian Paediatric Society has ad-vised caution: "An infant withanencephaly is a human being,albeit severely malformed, andtherefore must be treated in thesame way as any other humanbeing. As with all newborns thecriteria and ethical principles thatapply in organ transplantation in-volving other children and adultsalso apply if an infant with anen-cephaly is the potential donor.Physicians who accept the respon-sibility of caring for such an in-fant must have the interests ofthat patient as their primary con-sideration in decision-making andbe bound by the same moral andethical judgements as those usedin caring for any other patient....Neonatal intensive care units areheavily used, expensive and fre-quently overcrowded. It is clearlyunethical to transfer the resourcesneeded for neonatal intensive care(a well-established system of prov-en benefit) to an experimental,unproven project such as themaintenance of infants with anen-cephaly for the purposes. of organdonation."'9

In a world where materialresources are limited, we must

make difficult choices: what ispossible is not always appropriate.Often, economic costs must beentered into the equation. Simi-larly, moral costs must be consid-ered. The transplantation of or-gans from anencephalic newbornshas moral costs that may outweighthe moral good that it offers.While further research is still war-ranted, it must fall within theconfines of current ethical andlegal standards.

The issues discussed do notapply solely to anencephalic chil-dren. Because many are related tosocietal views of personhood, lifeand death, we will be confrontingthem repeatedly in different con-texts.

References

1. Shewmon DA, Capron AM, PeacockWJ et al: The use of anencephalicinfants as organ sources: a critique.JAMA 1989; 261: 1773-1781

2. Truog RD, Fletcher JC: Anencephalicnewborns: can organs be transplantedbefore brain death? N Engl J Med1989; 321: 388-390

3. Caplan AL: Ethical issues in the use ofanencephalic infants as a source oforgans and tissues for transplantation.Transplant Proc 1988; 20 (suppl 5):42-49

4. Idem: Should fetuses or infants beutilized as organ donors? Bioethics1987; 1:119-140

5. Helzgreve W, Beller FK, Bucholz B etal: Kidney transplantation fromanencephalic donors. N Engl J Med1987; 316: 169-170

6. Fletcher JC, Robertson JA, HarrisonMR: Primates and anencephalics assources for pediatric organ transplant:medical, legal and ethical issues. FetalTher 1986; 1: 150-164

7. Harrison MR: Organ procurement forchildren: the anencephalic fetus as do-nor. Lancet 1986; 2: 1383-1385

8. Fost N: Removing organs fromanencephalic infants: ethical and legalconsiderations. Clin Perinatol 1989;16: 331-337

9. Rothenberg LS: The anencephalic neo-nate and brain death: an internationalreview of medical, ethical, and legalissues. Transplant Proc 1990; 22:1037- 1039

10. Shewmon DA: Anencephaly: selectedmedical aspects. Hastings Cent Rep1988; 18: 11-18

11. Shinnar S, Arras J: Ethical issues inthe use of anencephalic infants asorgan donors. Neurol Clin 1989; 7:729-743

12. Botkin JR: Anencephalic infants asorgan donors. Pediatrics 1988; 82:250-256

13. Medearis DN, Holmes LB: On the useof anencephalic infants as organ do-nors. N Engl J Med 1989; 321: 391-393

14. Leggans T: Anencephalic infants asorgan donors: legal and ethical per-spectives. J Leg Med 1988; 9: 449-465

15. Landwirth J: Should anencephalic in-fants be used as organ donors? Pediat-rics 1988; 2: 257-259

16. Harrison MR, Meilaender G: Theanencephalic newborn as organ donor.Hastings Cent Rep 1986; 16: 21-23

17. Peabody JL, Emery JR, Ashwal S:Experience with anencephalic infantsas prospective organ donors. N Engl JMed 1989; 321: 344-350

18. Singer P: Sanctity of life or quality oflife? Pediatrics 1983; 72: 128-129

19. Bioethics Committee, Canadian Pa-ediatric Society: Transplantation oforgans from newborns with anenceph-aly. Can Med Assoc J 1990; 142: 715-717

244 CAN MED ASSOC J 1992; 146 (2) LE 15 JANVIER 1992