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Pediatric heart, heart-lung, and lung transplantation: Ethics
Pediatric Heart, Heart-Lung, and Lung Transplantation: Ethics JOEL E. FRADER, M.D. Department of Pediatrics University of Pittsburgh School of Medicine, and Center for Medical Ethics University of Pittsburgh Pittsburgh, Pennsylvania
Generally speaking, the ethical problems that arise in pediatric transplantation and their solutions do not differ from the moral questions attendant to other medical situations. The concerns outlined in now-familiar texts of bioethics 1-3 or even pocket-sized"handbooks" or clinical ethics 4cannot, of course, receive iteration here. This article considers some special instances of major themes in medical ethics that have particular relevance to pediatric heart transplantation. Moskop 5 has discussed similar concerns, though primarily regarding liver and kidney transplantation. Others have also published discussions of the moral concerns of pediatric heart transplantation.6,7
BEST INTERESTS OF THE CHILD
Broadly accepted principles of medical ethics teach us that a main goal of medicine involves doing good (acting beneficently) for patients. In heart and lung transplantation, we naturally assume that provision of a "replacement" heart or lung for a child will benefit the patient by extending survival a n d / or improving the quality of life. However, we cannot use the mere capacity to keep patients alive as a warrant for transplantation. We now have wideAddress correspondence to Joel E. Frader, M.D., Children's Hospital of Pittsburgh, 3705 Fifth Avenue at DeSoto Street, Pittsburgh, PA 15213.
spread consensus that continued biological existence, in and of itself, may not constitute a benefit. Patients who cannot formulate or express their preferences because of age or neurologic impairment may not be good candidates for transplantation simply because no technical contraindication to surgery exists. We should not assume that heart and lung transplantation will benefit children if it merely keeps them alive without permitting greater comfort, function, a n d / o r a chance to grow and develop. Death is not always the worst possible alternative for the sick. Most problematic here is the fact of clinical uncertainty. Transplantation for children remains an emerging field. Although short-term (for these purposes, 1 year) survival after heart transplantation has improved dramatically in the last several years, 8 longer term results have been more troubling, 9,1~though they appear to be improving. 11'12 Pediatric heart and heart-lung transplant patients (perhaps excluding those undergoing transplantation in early infancy13) have faced problems with acute, chronic, or recurrent rejection resulting in morbidity and mortality, including death from coronary artery disease and rejection-related arrhythmias or myocardial failure. 8,14-18 Some patients have had to contend with lymphoproliferative disorders (LPD). 19 We do not yet know much about the extended outlook, including the cumulative risks of LPD or (other) malignancy from required
Prog Pediatr Cardiol 1994; 3(1):20-25 Copyright Q 1994 by Butterworth-Heinernann
Ethics
immunosuppression. Cyclosporine-treated patients have suffered renal compromise and hypertension. 1~176 The problems of rejection, atherosclerosis, renal and other end-organ injury, and life-style compromise may lessen with newer immunosuppressive regimens, such as FK 506, as short-term reports indicate, zl'z2However, we have no intermediate (5-year) or longer term data about FK 506treated patients. All of this is to say that both the medical and ethical benefits of heart, heart-lung and lung transplantation remain a matter of some uncertainty. We have not yet gotten to the point where we should view transplantation as the "standard of care" that physicians or parents have an obligation to attempt to provide to every technically acceptable candidate.
CONSENT FOR HEART TRANSPLANTATION IN A CHILD
Many decisions requiring explicit consent in pediatric heart or heart-lung transplantation involve children with chronic illnesses: dilated cardiomyopathies, congenital heart disease with previous palliation, cystic fibrosis with pulmonary hypertension and cardiac failure, etc. In these situations, patients and families usually come to appreciate the child's decline and may take their time in considering the alternatives available to them, including the possibility of forgoing further intervention. Over months or years, transplant professionals should avail themselves of the opportunity to evaluate the parents' (or other legal guardians') competency to make decisions and provide consent. Relationships developing over long periods should underscore the importance of recognizing that giving informed consent involves an extended process and has little to do with signatures on a form in the hospital chart, z3 Besides assessing patient and parental decision-making capacity, pediatric cardiologists, surgeons, and other members of the health care team have an obligation to address the other elements of informed consent, z4 First, they should provide families with comprehensive and thoroughly understandable information about the various options open to the child. Second, the professionals should evaluate the patient's and family's understanding of the child's situation and alternative courses open to them.
21
Perhaps somewhat more controversially, many feel that the medical team caring for the child should make recommendations about which course of action seems most appropriate for each patient. A danger of favoring a particular option involves paternalism, where the professional substitutes his or her own judgment about what is best for the patient. Doctors and their health care colleagues need to remember that we all have personal beliefs (such as religious outlooks or personal moral codes) and interests, and we need to avoid insensitivity to the perspectives and values of the patient and family. The remaining element of informed consent involves guarding against coercion and promoting voluntariness in decision making. We sometimes forget the substantial gaps in real or perceived power and authority between professionals and patients or parents. Family members may not understand that doctors' recommendations are just that, and that parents or mature minors may freely reject the suggestions. Families may feel especially impotent when they wish to forgo recommended intervention and accept the death of the patient while providing only comfort measures. Professionals must not coerce families by threatening to withdraw medical attention: health care providers should find ways to support decisions they do not like, even if it means arranging for alternative care givers for the patient and family. Can we really permit parents to refuse transplantation7 Would any parent voluntarily decline the offer'?.Doesn't the very thought of saying no to a potentially life-saving therapy create such strain for conscientious parents that, by forgoing a transplant, they create the impression they are violating their responsibilities to their child? Although some may react to parental refusals with shock, we can still make moral distinctions between heart transplantation and the provision of antibiotics for systemic infection. Given risks of permanent morbidity while awaiting a new organ, risks associated with surgery itself, and for the longer term, risks of chronic immunosuppression, we should not create the impression that parents have a duty to agree to transplantation. Naturally, pediatric cardiologists, cardiothoracic surgeons, and others caring for the patient often have clear and strong feelings favoring transplantation. If so, they may have a hard time maintaining a supportive posture if parents
22
Progress in Pediatric Cardiology
refuse transplantation. This suggests that neutral parties, such as patient representatives who do not work directly with the transplant team, might aid in providing information, answering questions, and ensuring parental understanding and voluntary decision making. Note above the references to mature minors and other allusions to involving child patients. Law and ethics seem on the slow path to acknowledging that the capacity for decision making for medical treatment does not correspond magically to the arrival of one's 18th birthday. Maturity sufficient to permit participating in or taking full responsibility for one's medical care develops at varying rates among children. What little study has been done indicates that children have often acquired the understanding and skills necessary to decide about their own medical treatment by - 1 4 years of age. 25-28 At least one state in the United States, Alabama, fixes the age of consent at 14 years; another, Oregon, permits medical consent at age 15, and in South Carolina, 16-year-old patients can consent to medical care with restrictions regarding surgery. 29 Schowalter and co-authors 3~ noted years ago that dying children with chronic illness frequently develop knowledge and maturity well in advance of their chronologic age. Medical professionals have a clear ethical responsibility to assess the ability of child patients to understand and participate in decisions- including those about life or d e a t h - a n d permit minors' involvement appropriate to their abilities. This m a y at times involve complex negotiation with parents who maintain the myth that they can and should protect the child from emotional trauma. Again, what data exist suggest that most such efforts to shield children fail. 31-32 The attempts at protectionism leave many seriously ill children bitter and bewildered about why parents, other family members, o r otherwise caring medical professionals do not permit the youngsters to share important fears and worries and to make their farewells, when appropriate. Consent regarding emergencies may be more complex. However, relatively few occasions exist where professionals and parents have to make urgent decisions to accept or reject an available organ without substantial prior opportunity for reflection. Many people regard the birth of a baby with lethal congenital heart disease, such as hypoplastic left heart syndrome, as an emergency. But several
factors belie this notion. First, such infants increasingly have the diagnosis made or suspected by prenatal ultrasonography. Second, treatment with sustaining measures, in particular prostaglandin El, usually provides days or weeks for careful deliberation. Third, and most definitively, donor hearts rarely become available as soon as they could be used. Pediatricians, surgeons, and the other members of the team caring for the potential transplant patient may mistakenly see the need for urgent decisions before the p a r e n t s - or even the care provide r s - have had a sufficient chance to assess the situation and reflect on the wisdom of attempting heart transplantation. The pediatrician, pediatric cardiologist, and transplant team members should remember that a patient may be placed on the waiting list and later have the name withdrawn or a heart declined if one becomes available. Listing a patient does not constitute a commitment to use an offered heart and may reduce undue psychological pressure.
CONFLICTS OF INTEREST Professionals and institutions often have an interest in pursuing aggressive therapies. Most obviously, in the system of medical care in the United States, economic rewards go to those who intervene aggressively, regardless of outcome. In addition, professionals who develop treatment programs want their efforts, especially those they see as innovative or special, to succeed. Physicians may also have research careers or professional reputations that ride on the volume and success of interventions they undertake. These considerations apply to pediatric heart and lung transplantation just as in other medical fields. These interests may bias treatment recommendations and require serious efforts to avoid real or apparent conflicts with the interests of patients. Parental conflict of interest regarding treatment decisions has received a great deal of public attention, especially regarding decisions for seriously ill or "handicapped" newborns. Congress structured the 1984 Amendments to the Child Abuse Prevention and Treatment A c t - t h e so-called Baby Doe l a w - to exclude parents from medical decisions for infants with life-threatening disorders. Presumably, Congress believed that parents would too often refuse therapy based on "selfish" concerns, such
Ethics
as the psychological and financial well-being of their entire family. During and after the Baby Doe controversy, authors asked, What about the family? 3a-36The commentators argued that parents have legitimate worries about the impact of focusing so much of their time, attention, and economic resources on a single child, especially when there are other children in the family. Our society has not settled this matter. It does seem clear that if we insist that parental conflicts of interest disqualify mothers and fathers from participating in decisions about their sick children, we should not then demand that parents fully shoulder the consequences of others' choices. Perhaps we should not, for example, tell parents they must accept heart transplantation for a newborn with a hypoplastic left heart and then require the family to pay for the care and subsequently provide all the time and attention necessary to maximize the outcome for the baby. Without much greater investment in the social resources needed to support transplant programs (medical insurance, home care services, day care for immunocompromised children, subsidies for parents who must reduce or quit work to provide care for ill children, etc.), expropriation of parental decision-making authority seems inappropriate.
JUSTICE Recent studies have documented inequalities in the application of tertiary medical care, including heart and heart-lung transplantation, to women, ethnic minorities, those in rural areas, and, of course, the uninsured. 37-4ITransplantation is available to those who qualify for various state and federal programs to assist the poor. However, millions do not qualify for these government programs and cannot afford private insurance. Many more are underinsured and cannot meet the high costs of a transplant without supplemental funding. Although many transplantation-related professionals who bill independently have generously waived or reduced their fees for economically stressed families, hospitals that provide the essential services may not be willing or able to do this. Those institutions who offer "free" care - especially for particular geographic areas or special populat i o n s - must guard their resources to provide more
23
routine services for a large number of patients with lower costs of care per illness. The need to secure independent, up-front financing for transplantation services has turned many parents into fund-drive managers and publicity agents for their children. Although they are trying to help their child, the time and energy parents devote to such efforts drain their psychological resources and may deprive the sick child or other family members of more direct support. The economic realities of transplantation, like the cost of some sophisticated cancer therapies or other treatments, should challenge the medical community to re-examine our approach to health care resource allocation. We need to ask if providing rescue-oriented medicine, such as transplantation, is the best way to husband our medical resources. Does our devotion to this form of macroallocation unnecessarily divert resources from simpler and less expensive acute care and from perhaps more cost-effective preventive measures, such as immunization of children in the United States? If we determine that the costs of transplantation deserve our continued support, it seems incumbent on us to seek payment reforms that do not exclude patients or excessively strain families on the basis of economics or other medically irrelevant criteria. We also have to consider microallocation of transplant resources beyond the current major determinant (ability to pay). How should we choose among competing candidates for the same organ? Of course, some medical factors, such as patient and organ size, blood type, antibody status, etc., may trump other considerations. Other so-called clinical issues such as likelihood of success or urgency of need seem less clear-cut. Besides the notorious difficulty physicians have in predicting outcomes, no obvious medical or value criteria settle the perpetual disputes about whether patients who are more likely to survive should receive priority over those who will probably die quickly without immediate transplantation (but also have a lower probability of survival even with rapid treatment). Physicians should remember that such decisions ultimately express values and preferences that we can not readily reduce to "objective" schemes for making choices. Transplant teams nevertheless want clear guidelines about how to pick just one recipient from among a handful of medically equivalent patients.
24
Progress in Pediatric Cardiology
Ethicists and physicians have proposed various methods that promote choice on the basis of a patient's potential to contribute to society or as a reward for some past efforts. 1 Most agree that none of these plans fully satisfies our sense of fairness. Economics aside, it seems we have settled on waiting time as the final arbiter in these contests. 42 Unfortunately, waiting time does not accurately reflect the underlying, more arbitrary, time of onset of illness. M a n y factors limit or slow the referral to appropriate transplant centers, and hence issues of access to care once again loom large. A recent experience in our institution p r o m p t e d us to reevaluate the fairness of using waiting times to determine transplant priority, at least for newborn heart transplantation. A fetus whose heart disorder was spotten b y sonography early in pregnancy was placed on the candidate list at 30 weeks of gestation. When an appropriate heart later became available, the infant was delivered surgically and underwent transplantation. She had been born at about the same time as others in the region w h o also turned out to be heart transplant candidates but had not been diagnosed and listed prenatally. We realized on reflection that access to prenatal diagnosis in the United States is highly dependent on socioeconomic factors. 43 Thus, fetal identification and listing of a candidate for neonatal heart transplantation unwittingly involves, given current United Network for Organ Sharing rules, inequitable discrimination. Assuming the practice of transplantation continues to grow in the Unites States, the medical c o m m u n i t y and the public will need to scrutinize transplant policies and practices for their ethical implications. As noted here, transplantation poses various moral problems that deserve immediate attention and systematic change.
REFERENCES 1. Beauchamp TL, Childress JF. Principles of Biomedical Ethics, 3rd ed. New York: Oxford University Press, 1989. 2. Beauchamp TL, McCullough LB. Medical Ethics: The Moral Responsibilities of Physicians. Englewood Cliffs, New Jersey: Prentice-Hall; 1984. 3. Engelhardt HT. The Foundations of Bioethics. New York: Oxford University Press; 1986. 4. Jonsen AR, Siegler M, Winslade WJ. Clinical Ethics: A PracticalApproach to Ethical Decisions in Clinical
Medicine. New York: Macmillan Publishing Co.; 1982. 5. Moskop JC. Organ transplantation in children: ethical issues. J Pediatr. 1987;110:175-180. 6. Hugo-Hamman CT, Vosloo SM, De Moor MMA, Odell JA. Paediatric heart transplants-should we do them'?. S Air Med J. 1991;80:434-436. 7. PeabodyJL. SessionIX: social and ethical controversies in pediatric heart transplantation. J Heart Lung Transplant. 1991;5:860-876. 8. Fricker FJ, Trento A, Griffith BP. Pediatric cardiac transplantation. Cardiovasc Clin. 1990;20:223-235. 9. Addonizio LJ, Hsu DT, Smith CR, Gersony WM, Rose EA. Late complications in pediatric cardiac transplant recipients. Circulation. 1990;82(suppl 4): 295-301. 10. Taggart DP, Dark JH. Heart transplantation in children. Br Med J. 1991;302:1035-1036. 11. Michler RE, Rose EA. Pediatric heart and heart-lung transplantation. Ann Thorac Surg. 1991;52:708709. 12. Pennington DG, Noedel N, McBride LR, Naunheim KS, Ring WS. Heart transplantation in children: an international survey. Ann Thorac Surg. 1991;52: 710-715. 13. Bailey LL, Wood M, Razzouk A, Van Ardsdell G, Gundry S, Loma Linda University Pediatric Heart Transplant Group. Heart transplantation during the first 12 years of life. Arch 5urg. 1989;124:1221-1226. 14. Pahl E, Fricker FJ, Trento A, et al. Late follow-up of chiIdren after heart transplantation. Transplant Proc. 1988;20:743-746. 15. Heublein B, Haverich A, Borst HG. Long-term follow-up after orthotopic heart transplantation. Thorac Cardiovasc Surg. 1990;38:285-290. 16. Pahl E, Fricker FJ, Armitage J, et al. Coronary arteriosclerosis in pediatric heart transplant survivors: limitation of long-term survival. J Pediatr. 1990; 116:177-183. 17. WarnerJO. Heart-lungtransplantation: all thefacts. Arch Dis Child. 1991;66:1013-1017. 18. Whitehead B, Helms P, Goodwin M, et al. Heartlung transplantation for cystic fibrosis, 2: outcome. Arch Dis Child. 1991;66:1022-1026. 19. Baum D, Bernstein D, Starnes VA, et al. Pediatric heart transplantation at Stanford: results of a 15year experience. Pediatrics. 1991;88:203-214. 20. Fricker FJ, Griffith BP, Hardesty RL, et al. Experience with heart transplantation in children. Pediatrics. 1987;79:138-146. 21. Armitage JM, Fricker FJ, Del Nido P, Cipriani L, Starzl TE. The clinical trial of FK 506 as primary and rescue immunosuppression in pediatric cardiac transplantation. Transplant Proc. 1991;23:30583060.
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22. Dew MA, Harris RC, Simmons RG, Roth LH, Armitage JM, Griffith BP. Quality-of-life advantages of FK 506 vs. conventional immunosuppressive drug therapy in cardiac transplantation. Transplant Proc. 1991;23:3061-3064. 23. Lidz CW, Applebaum PS, Meisel A. Two models of implementing informed consent. Arch Intern Med. 1988;148:1385-1389. 24. Meisel A, Roth LH. What we do and do not know about informed consent. JAMA. 1981;246:24732477. 25. Weithorn LA, Campbell SB. The competency of children and adolescents to make informed treatment decisions. Child Dev. 1982;53:1589-1598. 26. Melton GB. Children's competence to consent: a problem in law and social science. In: Melton GB, Koocher GP, Saks MJ, eds. Children's Competence to Consent. New York: Plenum; 1983:1-18. 27. Morrissey JM, Hofmann AD, Thrope JC. Consent and Confidentiality in the Health Care of Children and Adolescents: A Legal Guide. New York: Free Press; 1986. 28. Holder AR. Legal lssues in Pediatrics and Adolescent Medicine, 2nd rev. ed. New Haven, CT: Yale University Press, 1985. 29. Capron AM. The competence of children as selfdeciders in biomedical interventions. In: Gaylin W, Macklin R, eds. Who Speaks for the Child: The Problems of Proxy Consent. New York: Plenum Press; 1982:57-114. 30. Schowalter JE, Ferholt JB, Mann NM. The adolescent patient's decision to die. Pediatrics. 1973;51: 97-103. 31. Anthony EJ, Koupernik C, eds. The Child in his Family: The Impact of Disease and Death. New York: John Wiley and Sons; 1973. 32. Bluebond-Langner M. The Private Worlds of Dying
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33. 34. 35.
36.
37.
38.
39.
40.
41.
42.
43.
Children. Princeton, NJ: Princeton University Press; 1978. Strong C: The neonatologist's duty to patient and parents. Hastings Cent Rep. 1984;14:10-16. Hardwig J: What about the family? Hastings Cent Rep. 1990;20:5-10. Frader J: Forgoing life-sustaining food and water: newborns. In: Lynn J, ed. By No Extraordinary Means: The Choice to Forgo Life-Sustaining Food and Water. Bloomington, IN: Indiana University Press, 1986:180-185. Frader J: Ethics in pediatric intensive care. In Fuhrman BP, Zimmerman JJ, eds. Pediatric Critical Care. St. Louis, MO. Mosby-Year Book; 1992:7-15. Engelhardt HT. Allocating scarce medical resources and the availability of organ transplantation: some moral presuppositions. N Engl J Med. 1984;311: 66-71. Kjellstrand CM: Age, sex, and race inequality in renal transplantation. Arch Intern Med. 1988;148: 1305-1309. Held PJ, Pauly MV, Bovbjerg RR, Newmann J, Salvatierra O. Access to kidney transplantation: has the United States eliminated income and racial differences? Arch Intern Med. 1988;148:2594-2600. Kilner JF. Selecting patients when resources are limited: a study of U.S. medical directors of kidney dialysis and transplantation facilities. A m J Public Health. 1988;78:144-147. Evans RW. Money matters: should ability to pay ever be a consideration in gaining access to transplantation? Transplant Proc. 1989;21:3419-3423. Halasz NA. Medicine and ethics: how to allocate transplantable organs. Transplantation. 1991;52: 43-46. Wertz DC, Fletcher JC. Ethical issues in prenatal diagnosis. Pediatr Ann. 1989;18:739-749.
Generally speaking, the ethical problems that arise in pediatric transplantation and their solutions do not differ from the moral questions attendant to other medical situations. The concerns outlined in now-familiar texts of bioethics 1-3 or even pocket-sized"handbooks" or clinical ethics 4cannot, of course, receive iteration here. This article considers some special instances of major themes in medical ethics that have particular relevance to pediatric heart transplantation. Moskop 5 has discussed similar concerns, though primarily regarding liver and kidney transplantation. Others have also published discussions of the moral concerns of pediatric heart transplantation.6,7
BEST INTERESTS OF THE CHILD
Broadly accepted principles of medical ethics teach us that a main goal of medicine involves doing good (acting beneficently) for patients. In heart and lung transplantation, we naturally assume that provision of a "replacement" heart or lung for a child will benefit the patient by extending survival a n d / or improving the quality of life. However, we cannot use the mere capacity to keep patients alive as a warrant for transplantation. We now have wideAddress correspondence to Joel E. Frader, M.D., Children's Hospital of Pittsburgh, 3705 Fifth Avenue at DeSoto Street, Pittsburgh, PA 15213.
spread consensus that continued biological existence, in and of itself, may not constitute a benefit. Patients who cannot formulate or express their preferences because of age or neurologic impairment may not be good candidates for transplantation simply because no technical contraindication to surgery exists. We should not assume that heart and lung transplantation will benefit children if it merely keeps them alive without permitting greater comfort, function, a n d / o r a chance to grow and develop. Death is not always the worst possible alternative for the sick. Most problematic here is the fact of clinical uncertainty. Transplantation for children remains an emerging field. Although short-term (for these purposes, 1 year) survival after heart transplantation has improved dramatically in the last several years, 8 longer term results have been more troubling, 9,1~though they appear to be improving. 11'12 Pediatric heart and heart-lung transplant patients (perhaps excluding those undergoing transplantation in early infancy13) have faced problems with acute, chronic, or recurrent rejection resulting in morbidity and mortality, including death from coronary artery disease and rejection-related arrhythmias or myocardial failure. 8,14-18 Some patients have had to contend with lymphoproliferative disorders (LPD). 19 We do not yet know much about the extended outlook, including the cumulative risks of LPD or (other) malignancy from required
Prog Pediatr Cardiol 1994; 3(1):20-25 Copyright Q 1994 by Butterworth-Heinernann
Ethics
immunosuppression. Cyclosporine-treated patients have suffered renal compromise and hypertension. 1~176 The problems of rejection, atherosclerosis, renal and other end-organ injury, and life-style compromise may lessen with newer immunosuppressive regimens, such as FK 506, as short-term reports indicate, zl'z2However, we have no intermediate (5-year) or longer term data about FK 506treated patients. All of this is to say that both the medical and ethical benefits of heart, heart-lung and lung transplantation remain a matter of some uncertainty. We have not yet gotten to the point where we should view transplantation as the "standard of care" that physicians or parents have an obligation to attempt to provide to every technically acceptable candidate.
CONSENT FOR HEART TRANSPLANTATION IN A CHILD
Many decisions requiring explicit consent in pediatric heart or heart-lung transplantation involve children with chronic illnesses: dilated cardiomyopathies, congenital heart disease with previous palliation, cystic fibrosis with pulmonary hypertension and cardiac failure, etc. In these situations, patients and families usually come to appreciate the child's decline and may take their time in considering the alternatives available to them, including the possibility of forgoing further intervention. Over months or years, transplant professionals should avail themselves of the opportunity to evaluate the parents' (or other legal guardians') competency to make decisions and provide consent. Relationships developing over long periods should underscore the importance of recognizing that giving informed consent involves an extended process and has little to do with signatures on a form in the hospital chart, z3 Besides assessing patient and parental decision-making capacity, pediatric cardiologists, surgeons, and other members of the health care team have an obligation to address the other elements of informed consent, z4 First, they should provide families with comprehensive and thoroughly understandable information about the various options open to the child. Second, the professionals should evaluate the patient's and family's understanding of the child's situation and alternative courses open to them.
21
Perhaps somewhat more controversially, many feel that the medical team caring for the child should make recommendations about which course of action seems most appropriate for each patient. A danger of favoring a particular option involves paternalism, where the professional substitutes his or her own judgment about what is best for the patient. Doctors and their health care colleagues need to remember that we all have personal beliefs (such as religious outlooks or personal moral codes) and interests, and we need to avoid insensitivity to the perspectives and values of the patient and family. The remaining element of informed consent involves guarding against coercion and promoting voluntariness in decision making. We sometimes forget the substantial gaps in real or perceived power and authority between professionals and patients or parents. Family members may not understand that doctors' recommendations are just that, and that parents or mature minors may freely reject the suggestions. Families may feel especially impotent when they wish to forgo recommended intervention and accept the death of the patient while providing only comfort measures. Professionals must not coerce families by threatening to withdraw medical attention: health care providers should find ways to support decisions they do not like, even if it means arranging for alternative care givers for the patient and family. Can we really permit parents to refuse transplantation7 Would any parent voluntarily decline the offer'?.Doesn't the very thought of saying no to a potentially life-saving therapy create such strain for conscientious parents that, by forgoing a transplant, they create the impression they are violating their responsibilities to their child? Although some may react to parental refusals with shock, we can still make moral distinctions between heart transplantation and the provision of antibiotics for systemic infection. Given risks of permanent morbidity while awaiting a new organ, risks associated with surgery itself, and for the longer term, risks of chronic immunosuppression, we should not create the impression that parents have a duty to agree to transplantation. Naturally, pediatric cardiologists, cardiothoracic surgeons, and others caring for the patient often have clear and strong feelings favoring transplantation. If so, they may have a hard time maintaining a supportive posture if parents
22
Progress in Pediatric Cardiology
refuse transplantation. This suggests that neutral parties, such as patient representatives who do not work directly with the transplant team, might aid in providing information, answering questions, and ensuring parental understanding and voluntary decision making. Note above the references to mature minors and other allusions to involving child patients. Law and ethics seem on the slow path to acknowledging that the capacity for decision making for medical treatment does not correspond magically to the arrival of one's 18th birthday. Maturity sufficient to permit participating in or taking full responsibility for one's medical care develops at varying rates among children. What little study has been done indicates that children have often acquired the understanding and skills necessary to decide about their own medical treatment by - 1 4 years of age. 25-28 At least one state in the United States, Alabama, fixes the age of consent at 14 years; another, Oregon, permits medical consent at age 15, and in South Carolina, 16-year-old patients can consent to medical care with restrictions regarding surgery. 29 Schowalter and co-authors 3~ noted years ago that dying children with chronic illness frequently develop knowledge and maturity well in advance of their chronologic age. Medical professionals have a clear ethical responsibility to assess the ability of child patients to understand and participate in decisions- including those about life or d e a t h - a n d permit minors' involvement appropriate to their abilities. This m a y at times involve complex negotiation with parents who maintain the myth that they can and should protect the child from emotional trauma. Again, what data exist suggest that most such efforts to shield children fail. 31-32 The attempts at protectionism leave many seriously ill children bitter and bewildered about why parents, other family members, o r otherwise caring medical professionals do not permit the youngsters to share important fears and worries and to make their farewells, when appropriate. Consent regarding emergencies may be more complex. However, relatively few occasions exist where professionals and parents have to make urgent decisions to accept or reject an available organ without substantial prior opportunity for reflection. Many people regard the birth of a baby with lethal congenital heart disease, such as hypoplastic left heart syndrome, as an emergency. But several
factors belie this notion. First, such infants increasingly have the diagnosis made or suspected by prenatal ultrasonography. Second, treatment with sustaining measures, in particular prostaglandin El, usually provides days or weeks for careful deliberation. Third, and most definitively, donor hearts rarely become available as soon as they could be used. Pediatricians, surgeons, and the other members of the team caring for the potential transplant patient may mistakenly see the need for urgent decisions before the p a r e n t s - or even the care provide r s - have had a sufficient chance to assess the situation and reflect on the wisdom of attempting heart transplantation. The pediatrician, pediatric cardiologist, and transplant team members should remember that a patient may be placed on the waiting list and later have the name withdrawn or a heart declined if one becomes available. Listing a patient does not constitute a commitment to use an offered heart and may reduce undue psychological pressure.
CONFLICTS OF INTEREST Professionals and institutions often have an interest in pursuing aggressive therapies. Most obviously, in the system of medical care in the United States, economic rewards go to those who intervene aggressively, regardless of outcome. In addition, professionals who develop treatment programs want their efforts, especially those they see as innovative or special, to succeed. Physicians may also have research careers or professional reputations that ride on the volume and success of interventions they undertake. These considerations apply to pediatric heart and lung transplantation just as in other medical fields. These interests may bias treatment recommendations and require serious efforts to avoid real or apparent conflicts with the interests of patients. Parental conflict of interest regarding treatment decisions has received a great deal of public attention, especially regarding decisions for seriously ill or "handicapped" newborns. Congress structured the 1984 Amendments to the Child Abuse Prevention and Treatment A c t - t h e so-called Baby Doe l a w - to exclude parents from medical decisions for infants with life-threatening disorders. Presumably, Congress believed that parents would too often refuse therapy based on "selfish" concerns, such
Ethics
as the psychological and financial well-being of their entire family. During and after the Baby Doe controversy, authors asked, What about the family? 3a-36The commentators argued that parents have legitimate worries about the impact of focusing so much of their time, attention, and economic resources on a single child, especially when there are other children in the family. Our society has not settled this matter. It does seem clear that if we insist that parental conflicts of interest disqualify mothers and fathers from participating in decisions about their sick children, we should not then demand that parents fully shoulder the consequences of others' choices. Perhaps we should not, for example, tell parents they must accept heart transplantation for a newborn with a hypoplastic left heart and then require the family to pay for the care and subsequently provide all the time and attention necessary to maximize the outcome for the baby. Without much greater investment in the social resources needed to support transplant programs (medical insurance, home care services, day care for immunocompromised children, subsidies for parents who must reduce or quit work to provide care for ill children, etc.), expropriation of parental decision-making authority seems inappropriate.
JUSTICE Recent studies have documented inequalities in the application of tertiary medical care, including heart and heart-lung transplantation, to women, ethnic minorities, those in rural areas, and, of course, the uninsured. 37-4ITransplantation is available to those who qualify for various state and federal programs to assist the poor. However, millions do not qualify for these government programs and cannot afford private insurance. Many more are underinsured and cannot meet the high costs of a transplant without supplemental funding. Although many transplantation-related professionals who bill independently have generously waived or reduced their fees for economically stressed families, hospitals that provide the essential services may not be willing or able to do this. Those institutions who offer "free" care - especially for particular geographic areas or special populat i o n s - must guard their resources to provide more
23
routine services for a large number of patients with lower costs of care per illness. The need to secure independent, up-front financing for transplantation services has turned many parents into fund-drive managers and publicity agents for their children. Although they are trying to help their child, the time and energy parents devote to such efforts drain their psychological resources and may deprive the sick child or other family members of more direct support. The economic realities of transplantation, like the cost of some sophisticated cancer therapies or other treatments, should challenge the medical community to re-examine our approach to health care resource allocation. We need to ask if providing rescue-oriented medicine, such as transplantation, is the best way to husband our medical resources. Does our devotion to this form of macroallocation unnecessarily divert resources from simpler and less expensive acute care and from perhaps more cost-effective preventive measures, such as immunization of children in the United States? If we determine that the costs of transplantation deserve our continued support, it seems incumbent on us to seek payment reforms that do not exclude patients or excessively strain families on the basis of economics or other medically irrelevant criteria. We also have to consider microallocation of transplant resources beyond the current major determinant (ability to pay). How should we choose among competing candidates for the same organ? Of course, some medical factors, such as patient and organ size, blood type, antibody status, etc., may trump other considerations. Other so-called clinical issues such as likelihood of success or urgency of need seem less clear-cut. Besides the notorious difficulty physicians have in predicting outcomes, no obvious medical or value criteria settle the perpetual disputes about whether patients who are more likely to survive should receive priority over those who will probably die quickly without immediate transplantation (but also have a lower probability of survival even with rapid treatment). Physicians should remember that such decisions ultimately express values and preferences that we can not readily reduce to "objective" schemes for making choices. Transplant teams nevertheless want clear guidelines about how to pick just one recipient from among a handful of medically equivalent patients.
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Progress in Pediatric Cardiology
Ethicists and physicians have proposed various methods that promote choice on the basis of a patient's potential to contribute to society or as a reward for some past efforts. 1 Most agree that none of these plans fully satisfies our sense of fairness. Economics aside, it seems we have settled on waiting time as the final arbiter in these contests. 42 Unfortunately, waiting time does not accurately reflect the underlying, more arbitrary, time of onset of illness. M a n y factors limit or slow the referral to appropriate transplant centers, and hence issues of access to care once again loom large. A recent experience in our institution p r o m p t e d us to reevaluate the fairness of using waiting times to determine transplant priority, at least for newborn heart transplantation. A fetus whose heart disorder was spotten b y sonography early in pregnancy was placed on the candidate list at 30 weeks of gestation. When an appropriate heart later became available, the infant was delivered surgically and underwent transplantation. She had been born at about the same time as others in the region w h o also turned out to be heart transplant candidates but had not been diagnosed and listed prenatally. We realized on reflection that access to prenatal diagnosis in the United States is highly dependent on socioeconomic factors. 43 Thus, fetal identification and listing of a candidate for neonatal heart transplantation unwittingly involves, given current United Network for Organ Sharing rules, inequitable discrimination. Assuming the practice of transplantation continues to grow in the Unites States, the medical c o m m u n i t y and the public will need to scrutinize transplant policies and practices for their ethical implications. As noted here, transplantation poses various moral problems that deserve immediate attention and systematic change.
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