Children and organ donation in the United kingdom: A literature review

INTERNATIONAL PEDIATRIC NURSING Column Editor: Bonnie Holaday, DNS, RaN, FAAN

Children and Organ Donation in the United Kingdom: A Literature Review Rosario C.S. Baxter, BSc, MEd, MPhil, RNs, RNT, RCN, Cert HCIAE

T gan failure is a relatively recent development in the United Kingdom because the imma-

RANSPLANTATION for children with or-

this speciality, and creating satisfactory ways of treating previously intractable or fatal conditions.

turity and scarcity of organs for donation and the technical difficulties of transplantation (particularly in the younger child) have forced medical treatment in the past to consist of keeping the child alive until he or she, by virtue of size, would become a candidate for transplantation. For instance, surgical procedures may alleviate the effects of severe malformations of the heart, which may "buy time" for the infant. Treatments such as total parental nutrition can maintain the child's nutritional status for an indefinite period of time; however, despite strenuous measures taken to minimize the risks of contamination, potentially life-threatening infections are a constant hazard. Unfortunately, children may still die in the intervening period, either as a result of the illness or perhaps as a result of the interventions necessary to contain the progress of the condition. Professionals and society favor transplantation in children for specific reasons. It can offer a substantial improvement in the quality of life experienced by the child and family and reduce complicated maintenance therapies and the associated risks. In successful grafts, the cost of the transplant can be offset against the high recurring costs of treatments, such as dialysis. Increasing experience with performing grafts in children can have the added bonus of advancing scientific knowledge, constantly pushing back the frontiers in

EPIDEMIOLOGY AND TREATMENT OF ORGAN FAILURE IN CHILDREN

From the Department of Nursing, University of Ulster, Jordanstown, Northern Ireland. Address reprint requests to Rosario C.S. Baxter, MEd, MPhil, RN, RNT, RSCN, Cert HCIAE, University of Ulster, Newtonabbey, CountyAntrim, BT37 OQB, Northern lreland. Copyright 9 1996 by W.B. Saunders Company 0882-5963/96/1102-001253.00/0 136

Conditions such as cystic fibrosis, which were previously categorized as requiring long-term treatment that would probably end in terminal care, are now being added to the list of diseases for which transplantation is becoming an attractive and often cost-effective option. Management of conditions such as severe congenital heart defects represent some of the most difficult challenges in pediatrics, and this group of infants are among those who stand to benefit most yet are least likely to receive a donor organ because of the very small numbers of organs that become available in this age group. Transplantation between live relatives in the province of Northern Ireland is limited by medical, ethical, and psychosocial constraints and by the Human Tissue Act (1961), the Human Organs Transplant Act (1989), and the Human Organs Transplant (Northern Ireland) Order (1989). The benefits of transplantation for the child recipient include the ability to grow and develop normally; rehabilitation after receiving a new compatible organ has been described as "remarkable" (Ettenger, Korsch, Main, and Fine, 1979). Kantrowitz, Hailer, and Joos (1968) recorded one of the first heart transplants from an infant (anencephalic) donor in 1968; 5 years earlier Goodwin (1963) was responsible for the first reported attempt at renal transplantation from an anencephalic donor.

Corneal

Transplantation

Although the Hoffenberg Report (1988) estimates the demand to be small, corneal transplants are not considered to cause ethical probJournal of Pediatric Nursing, Vol 11, No 2 (April), 1996

CHILDREN AND ORGAN DONATION

lems because they can be removed from persons who have died.

Renal Transplantation In North America and regions covered by the European Dialysis and Transplant Association Registry (1986), more than 2,000 successful kidney transplantations were done in children, with at least 20% of organs coming from living related donors. The survival rate is more than 95% at 1 to 3 years (Broyer, Otte, Kachaner, & Goulet, 1989). In suitable cases, children weighing 10 to 12 kg can receive an adult kidney.

Liver Transplantation Children born with biliary atresia, metabolic disease, or liver tumours account for most of the candidates for whom liver transplantation would be the best or only option. In one European center no lower age limit is imposed on infants' eligibility for liver transplantation because many of these babies present with serious problems at a very early stage. The lower age limit for donors in the St.-Luc University Hospital in Brussels is the end of the neonatal period. By 1989, at least eight centers for pediatric liver transplantation were established worldwide (five in the United States and three in Europe). Recent success in the management of liver failure in infants has been found by using hepatic segments from living related donors, which in one program included 12 mothers, 7 fathers, and 1 grandmother (Broelsch et al., 1991).

Cardiac Transplantation It is suggested that approximately 150 infants/yr born in the United Kingdom with cardiac defects might be considered suitable for transplantation. Since 1984, several European institutions have started programs for cardiac transplantation in children. The Enfants Malades hospital in Paris has now conducted four out of five successful heart transplants in children 1 month to 10 years old. Bailey et al. (1986) reported successful cardiac transplantation in the newborn. Broyer et al (1989) contend that cadaver donor transplantation may be an option in the newborn subject to the availability of the organ.

Other Transplantable Organs Conditions peculiar to childhood, such as combined cardiac and pulmonary anomalies or

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pulmonary fibrosis, along with congenital malformations of the intestine, until lately have been managed with varying success. The technical complexities of transplanting these organs in children means that many such transplant procedures are still in the experimental stages. Approximately 30 combined heart and lung transplants have been performed according to Broyer et al. (1989), and two small intestine grafts in children have been performed in France.

SOURCES AND RESOURCES IN ORGAN TRANSPLANTATION As the success rate of organ transplantation grows, there is a correspondent increase in demand. In Europe, Australasia, and North America, legal, emotional, organizational, and ethical barriers have resulted in approximately half of the available organs being successfully transplanted. China has been accused of supplying the organs of executed prisoners for transplantation within and outside of its own country (Dorozynski, 1993). Countries, such as Japan, that forbid the use of organs from brain stemdead patients rely more heavily on the use of live related donors. Gore, Taylor, and Wallwork (1991) conducted an extensive review to estimate figures for sourcing organs from from brain stem-dead donors in intensive care facilities in the United Kingdom. A protocol exists in the Royal Devon and Exeter Hospital to electively ventilate prospective donors, described by Collins (1992) as " . . . the management of an inevitable death in such a way that opportunities for donation are not missed." Although the British Medical Association has published guidelines endorsing the use of elective ventilation, others such as Routh (cited in Collins, 1992) call for further analysis of the implications of the procedure, citing possibilities such as misdiagnosis or having to prioritize for the acutely ill as potential pitfalls. He argues that the ethical justification for elective ventilation would be strengthened with previous knowledge of the donor's wishes in this respect. The supply of organs for children requiring transplantation, in common with the adult population, suffers from a chronic shortfall, and the problem will be exacerbated as public expectations grow that previously fatal conditions are amenable to treatment. The well-publicized case of the multiple organ transplant recipient Laura Davies illustrates the difficulty of balancing the ethical principles of beneficence and

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justice in allocating scarce resources. A theory of justice might say that resources should be directed to (1) those whose future contribution to society would be high; (2) those in greatest need or for whom the treatment would provide the greatest benefit; (3) those who can afford to pay the price; (4) those who would receive the highest dividend in improvement in quality of life; or (5) those who are lucky enough to win the lottery. Even those in society who might agree that one (or more) of the above options is a satisfactory way to distribute resources often have a different view when children are involved. Ward and Hetherington (cited in Castledine, 1993) stated that " . . . all kids should get the treatment they need . . . . " According to Caplan (1992), at least in the United States, there is no consistent set of standards for admission to transplant programs. Nonmedical criteria may include national interest or other publicity considerations, age, sex, geographical location, perseverance of patient and doctor, and whether the case is unusual enough to attract interest. Caplan adds that money remains the best guarantee of obtaining the required health service. In this way the patient (as in the Laura Davies case) can skip the "tunnel" that is normally negotiated to gain access to an organ, namely the stages of eligibility, admissibility, distribution, and selection. As donation becomes a more readily considered option for childhood disease, the distribution of resources will become an even more acute problem. Even if not every child gets an equal share of the resource, ensuring that the influence of the above-mentioned factors is diminished will become an even greater challenge in aspiring toward egalitarian distribution. The public relations officer of the United Kingdom Transplant Authority estimates that of 918 donors in 1993, only 3% were children younger than 5 years. These figures are categorized into donors who died from natural causes, trauma, suicide, and unknown causes. It is suggested that approximately 1% of donors will be younger than 1 year. According to this source, the anencephalic donor is not specifically profiled in the United Kingdom, although it was stated that approximately one anencephalic donor per year is utilized for heart and/or liver transplants only. This information is an estimation and is not verifiable through the United Kingdom Transplant Support Service Authority.

ROSARIO C.S. BAXTER

BRAIN DEATH

Three sets of guidelines designed to provide standards for the diagnosis of brain death in children have been devised, all of them in the United States. These consist of (a) the Task Force on Brain Death in Children (1987), of representatives including several pediatric and neurological organizations, (b) An ad hoc committee of Boston Children's Hospital (1987), and (c) guidelines produced by Ashwal and Schneider (1987). Each of the guidelines appeared in 1987, and each is different in certain respects. Main variations are based on determination of irreversibility in infants, although all are criticized for basing criteria, such as the length of time between electroencephalographs (EEGs) on little empirical evidence. The President's Commission (1981) earlier defined brain death as "the total and irreversible cessation of all brain function." Tests used to determine brain death generally are considered to be reliable, except in the newborn (Mohanadas & Chou, 1971). The lack of agreement regarding brain stem death (BSD) determination in children has raised a number of sensitive medicoethical issues, including the appropriate use of intensive care resources and the effective utilization of organs. There is a dearth of research on BSD in children that is compounded by an absence of detailed clinical and laboratory analysis of the problem. Since Mollaret and Goulon (1959) first graded coma into four levels, the most profound being defined as coma de passe (a state beyond coma), i.e., a total loss of sensory, motor, and conscious brain function, various attempts have been made to draw up guidelines to determine BSD (Beecher, 1968; Conference, 1976). General criteria applied to assess the presence or absence of BSD in adults must first ensure that the patient has been free for 24 hours from agents that might influence the test results. These includes drugs that might depress the central nervous system and conditions that produce hypothermia. It examines (a) coma, i.e., unreceptive and unresponsive; (b) Apnea, i.e., absence of spontaneous respiration (excluding other causes, such as drug toxicity) for a period of time (3 [Beecher, 1968] to 10 [President's Commission 1982] minutes) once the apneic threshold (minimum Pco2 at which respirations are stimulated) is reached; (c) lack of spontaneous movement or response to deep

CHILDREN AND ORGAN DONATION

stimuli; and (d) absence of reflexes as elicited by corneal and pupillary stimulation, caloric irrigation to induce ocular response, and catheter introduction to induce pharyngeal yawning and swallowing responses. The Ad Hoc Committee of Boston Children's Hospital (1987) made it clear that the primary (and utilitarian) aim of their work was to "define irreversible coma as a new criterion for d e a t h . . , because obsolete criteria.., can lead to controversy in obtaining organs for transplantation." Criteria included the use of an EEG to confirm BSD. Guidelines produced by Ashwal and Schneider (1987b) at Loma Linda University recommend that for infants 12 months old or older, one isoelectric EEG is sufficient (in addition to the previously mentioned criteria) to declare brain death. They suggest that infants between 2 and 12 months with one isoelectric EEG have cerebral blood flow studies to confirm BSD. According to this criteria, babies younger than 8 weeks require confirmation of brain death by laboratory studies because some infants who have expressed no brain or EEG activity have shown such function 24 hours later. (Cerebral blood flow testing requires the use of contrast arteriography, a complex and expensive technique that is not available in most hospitals, making its use very limited.) Cerebral blood flow studies have also been challenged by Volpe (1987), who questions our lack of knowledge of the value of such tests and the possibility of such tests interfering with collection of other data. The Task Force on Brain Death in Children (1987) recommends two EEGs at least 48 hours apart; again, this has provoked criticism, this time from Freeman and Ferry (1988). The Task Force on Brain Death in Children did not deal specifically with the anencephalic infant. After the 1976 criteria were published, in 1979 the Conference of Medical Royal Colleges and Their Faculties in the United Kingdom (1976) issued criteria calling brain death "permanent functional death of the brainstem." The 1979 conference identified "brainstem death as death itself." Scant attention has been devoted in each set of guidelines to BSD in children; indeed, the President's Commission (1981, 1982) demanded " . . . caution in applying neurologic criteria to the determination of brain death in children." The President's Commission was among the first to recognize that children present particular difficulties in attempting to reach

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a diagnosis of BSD. Developmental factors, a greater perceived resistance to asphyxia, and the potential for significant recovery even after prolonged coma are some of the variables that make the application of adult criteria to children less valid (although even the above claims, according to Ferry and Freeman [1988], are invalidated). Moshe and Alvarez (1986) went on to suggest that 5 years and younger was the stage at which the aforementioned "caution" should be exercised. Ashwal and Schneider (1987a) argue that this was an arbitrary age limit that reflects the little available empirical evidence on BSD in children. In a review of compiled research, the same authors calculated the age frequency of children reported as brain stem dead. The youngest infants who were brain stem dead were between 28 and 34 weeks gestation (n = 164). The most common age at diagnosis was 0 to 4 months (n = 19). One of the difficulties with retrospective studies (and currently with reporting procedures of organ donors in the United Kingdom) is the lack of specificity leading to a diagnosis of BSD, especially in children younger than 5 years. This makes analysis of the data difficult because of problems in obtaining centrally held information. Neurological examination has been suggested by Moshe and Alvarez (1986) as an accurate predictor of outcome, although the findings were based on a very small sample (n = 15). The complexity of symptoms in children presenting with suspected brain stem failure makes the value of diagnostic tools such as neurological function in isolation hard to estimate. The diagnosis of BSD would appear to become less accurate with the decreasing age of the child, and thus Litaka (Martin and Cox, 1978) suggest that the criteria are invalid for application in infants. Litaka et al. recorded the deviation from normal standards that exists in children, which makes the diagnosis of BSD particularly open to interpretation. Although pupil reflexes are absent before 29 weeks gestation, they are well evident at 32 weeks. Caloric testing (irrigation of the auditory canal with iced water), a basic of BSD testing in the adult, is technically very awkward in a young or preterm infant. Combined reflex tests, such as those identified by Allen, Burkholder, and Comiscioni (1978) although known to have an error rate, have not been tested on children to assess the degree, if any, of error. The apnea threshold in children

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ROSARIO C.S. BAXTER

has yet to be determined with confidence. Studies by Rowland, Donnelly, and Jackson (1984) and Outwater and Rockoff (1984) concluded that various preoxygenation periods of 5 or 10 minutes before testing, and testing times for apnea of either 5 or 10 minutes, were most useful. Individual differences in children's metabolism and control of carbon dioxide production make guidelines difficult to compile with a high level of accuracy. This finding is endorsed by Freeman and Ferry (1988), who challenge the guidelines compiled by Ashwal and Schneider (1987b), the Ad Hoc Committee of Boston Children's Hospital (1987), and the Special Task Force on the grounds that they cause further confusion in determination of brain death in the child. Lawson, Bennett, Campbell, Pirofsky, and Hodges (1973) confirm the physician's lack of clarity regarding the child's status as organ donor. BRAIN ABSENT In an attempt to overcome the many ethical and legal problems associated with trying to diagnose BSD in infants and to specifically address the anencephalic infant, a diagnosis that defies a declaration of death based on accepted criteria, Harrison (1986a) proposes the term brain absent. Creating special provisions would allow harvesting of organs from such infants. Those who support this approach may argue that infants with anencephaly are persons who deserve respect, but because they are brain absent, this should be viewed as equivalent to brain death, thus organ retrieval would be permissable (Capron, 1987). Because the present definition of brain death asks for "complete and irreversible cessation of all brain functions, including those of the brain stem," it would not be possible to diagnose infants with anencephaly as whole brain dead or even brain stem dead. Although anencephalics lack a cerebral cortex, they have a brain stem that regulates a wide variety of bodily functions, including spontaneous respiration. It would appear

more accurate to describe this infant as higher brain absent. According to the President's Commission (1981) no one with spontaneous respiration meets current criteria for brain death. SUMMARY The Hoffenberg Report (1988) points to the technical difficulties of attempting to transplant small livers or kidneys into larger, older recipients and the inappropriateness of transplantation for neonatal renal or hepatic failure. This is in contrast to findings from other regions that are achieving success in transplantation in the neonate. Chronic rejection of any organ means that the younger the recipient, the greater is the likelihood that he or she will eventually be in need of a second, third, or fourth organ replacement, leading to increased future demand on organ resources. This article has shown the evolution of transplantation in childhood organ failure. Using available data from the United States and Europe, it has illustrated the ever-expanding variety of conditions that are rapidly increasing the demand for organs in this section of the population. The elimination of other methods of obtaining organs for infants and the older child has been examined in the context of the difficulties inherent in matching principles of justice and beneficence. The complexities of addressing BSD, particularly in its application to children, highlights the problems that emerge in trying to find new sources to address an escalating need. It also points toward some of the deficiencies in devising a coherent policy and the measures sometimes taken to circumvent conventional practice (Zaner, 1989) to facilitate organ retrieval. Freeman and Ferry (1988) advise against too heavy a reliance on invalidated "guidelines" that may become the accepted dogma (e.g., the Task Force on Brain Death in Children, 1987) and gain eventual acceptance as the "gold standard" for many lay and professional individuals and groups.

REFERENCES Ad Hoc Committee of Boston Children's Hospital (1987). Boston: Author. Allen, H., Burkholder, J., & Comiscioni, J. (1978). Clinical criteria of brain death. Annals of the New York Academy of Science, 315, 70-96. Ashwal, S.A., & Schneider, S. (1987a). Brain death in children: Part I. Journal of Perinatology, 3(1), 5-11. Ashwal, S.A., & Schneider, S. (1987b). Brain death in children: Part II. Journal of Perinatology, 3(2), 69-77.

Bailey, L.L., Nehlsen, R., Cannarelly, S.L., Doroshow, R.W., Jacobson, J.G., Martin, R.D., Allard, M.W., Hyde, M.R., Dangbui, R.H., & Petri, E.L. (1986). Cardiac allotransplantation in newborns as therapy for hypoplastic left heart syndrome. New England Journal of Medicine, 315, 949. Beecher, H.K. (1968). A definition of irreversible coma. Report of the Ad Hoc Committee of the Harvard Medical School to Examine the Definition of Brain Death. Journal of the American MedicalAssociation, 205, 337-340.

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Broelsch, C., Whitington, P.F., Edmond, J.C., Heffron, T.G., Thistlethwaite, J.R., Stevens, L., Piper, J., Whitington, S., & Lichtor, J.L. (1991). Liver transplantation in children from living related donors.Annals of Surgery, 214(4), 428-439. Broyer, M., Otte, J-B., Kachaner, J. & Goulet, O. (1989). Organ transplantation in children. Intensive Care Medicine, 15, 576-579. Caplan, A.L. (1992). Should fetuses or infants be utilized as organ donors? Bioethics, 1, 119-140. Capron, A.M. (1987). Anencephalic donors: Separate the dead from the dying. Hastings Center Report, 17, 5-9. Castledine, G. (1993). Laura Davis: Should children always get the treatment they need? British Journal of Nursing, 2(21), 1077-1078. Collins, C. (1992). Elective ventilation for organ donat i o n - T h e Case in Favour. Care of the Critically Ill, 8(2), 57-59. Conference of Medical Royal Colleges and Their Faculties in the UK: Diagnosis of death. British Medical Journal, 2, 1187-1188. Department of Health (1990). The Human Organ Transplants (Northern Ireland) Order 1989: Regulations. Circular HSS (General Hospital Services). Belfast: Her Majesty's Stationary Office. Dorozynski, A. (1993). Europe condemns sale of organs. British Medical Journal, 307, 756. Ettenger, R.B., Korsch, B.N., Main, M.E. & Fine R.M. (1979). Renal rehabilitation of children and adolescents with end stage renal disease. In Chyatte, S.B. (Ed.), Rehabilitation in Chronic Renal Failure (p 115). Baltimore: Williams and Wilkins. European Dialysis and Transplant Association Registry (1986). Demography of dialysis and transplantation in children in Europe 1984. Nephrology, Dialysis, Transplanta-

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The Hoffenberg Report: Report of a Working Party on Organ Transplantation in Neonates. (1988). London: Department of Health and Social Services.

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