Handbook of Clinical Neurology, Vol. 118 (3rd series) Ethical and Legal Issues in Neurology J.L. Bernat and R. Beresford, Editors © 2013 Elsevier B.V. All rights reserved

Chapter 18

Ethical issues in the care of the neurologically devastated infant TRACY K. KOOGLER* Department of Pediatrics, University of Chicago Medical Center, Chicago, IL, USA

INTRODUCTION As medical technology advances, the therapies available to save the lives of newborn infants born with complex congenital defects has increased, so that defects determined to be “lethal” are, in fact, no longer truly lethal (Koogler et al., 2003). With medical technology and at times complex surgery, children who would have died in the newborn period or within the first year of life can survive, although some will remain forever dependent on medical technology and others will never develop neurologically beyond the abilities of a newborn or young infant. These advancements, while increasing survival rates of young infants, have also brought forth numerous arguments surrounding the ethics of what should be done for some of these newborns. The technology and medical services exist to save their lives, but some say, “just because we can, does not mean that we should.” In this chapter, I examine the historic background of this debate and present an argument for thoughtful care in the decision-making process by examining the ethics of care and the best interests of the child.

HISTORY In 1973, Raymond Duff and A.G.M. Campbell described the cases of 299 consecutive deaths in Yale’s Special Care Nursery. They found life support was withdrawn in the newborn nursery of 43 (14%) children who had various congenital malformations, including congenital heart disease, myelomeningocele, and trisomy 13, 18, and 21. These children were perceived to have poor quality of life because of their disabilities. Some were expected to die in the neonatal period because of their anomalies, such that ongoing critical therapies were seen as only prolonging the dying process. Others might survive but because of their disabilities were seen to have a very poor quality

of life and the strain on the family to care for these children with few public resources was seen as too great. In the conclusion, Duff and Campbell stated: We believe the burdens of decision making must be borne by families and their professional advisers because they are most familiar with the respective situations. Since families primarily must live with and are most affected by the decision, it therefore appears that society and the health professions should provide only general guidelines for decision making. Moreover, since variations between situations are so great, and the situations themselves so complex, it follows that much latitude in decision making should be expected and tolerated. Otherwise, the rules of society or the policies most convenient for medical technologists may become cruel masters of human beings instead of their servants (Duff and Campbell, 1973). Today, some of these children, especially those with isolated congenital heart disease routinely receive cardiac surgery and grow into adulthood, attend school, and fully participate in family life. For other children, the decision remains mixed. Some countries and institutions routinely treat these newborns and encourage therapy, while others continue to encourage families to withdraw and withhold therapies because of the severity of their neurologic impairment. Family support groups for trisomy 13 and 18 have arisen, reciting testimonials of how these children have enriched families’ lives, to encourage families to pursue aggressive therapy (Janvier et al., 2012). Despite the developmental delay and complex daily care needs of these children, families integrate these special needs children into their daily lives, and shun the chronic care facilities where most of these surviving children were placed prior to the late 1970s.

*Correspondence to: Tracy K. Koogler, M.D., Associate Professor of Pediatrics, University of Chicago Medical Center, 5841 South Maryland Avenue, Chicago, IL 60637, USA. E-mail: [email protected]

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LEGAL GUIDELINES In the United States, the Baby Doe Laws led to more aggressive therapies for some of these children while leaving direction for other impaired infants in question. In 1982, an Indiana infant was born with trisomy 21 and a correctable gastrointestinal defect but was allowed to die in a neonatal intensive care unit (NICU) from lack of fluids and nutrition (In re Infant Doe, 1982). The U.S. national interest in this case and others led to the amendments to the 1984 Child Abuse Prevention and Treatment Act (often known as the Child Abuse Amendments or the CAA) (Table 18.1). These laws mandated states to develop a way to respond to cases of nontreatment of infants. They first described what later became hospital ethics committees to address complex ethical dilemmas regarding critically ill infants. They established that fluids and nutrition were obligatory for infants. Otherwise, they left medical therapies to the discretion of the physician, as long as they were not withheld solely for quality-of-life reasons. These guidelines expressly stated that children with anencephaly were included in the exceptions, but did not explicitly describe other common congenital defects such as trisomy 13 and 18 (Child Abuse Amendments, 1984).

In the United States, these guidelines led to more aggressive therapy being provided to infants born with trisomy 21, myelomeningocele, and other birth defects, who may have previously been untreated and allowed to die. Abnormalities, leading to severe neurologic devastation, such as trisomy 13 and 18, continued to be allowed to die and many families were informed that these children had lethal anomalies, i.e., they would die within the first year of life. Neonatologists resented these new guidelines because they felt the regulations placed too many requirements on physicians and families about how to care for critically ill newborns. Further, they felt the regulations endangered the best interests of the child and interfered with parental preferences (Kopelman et al., 1988). At the same time that the debate over very premature and neurologically challenged children was waging in the United States, an English court case was dealing with similar interests. Re B (1982) addressed the issue of whether parents had the right to refuse life-saving surgery for their disabled child. Alexandra had trisomy 21 (Down syndrome) and was born with an intestinal blockage that required emergency surgery. Her parents refused. The Court of Appeal agreed that she would have significant mental and physical disabilities but

Table 18.1 Amendments to the 1984 Child Abuse Prevention and Treatment Act (Child Abuse Amendments of 1984) Key guidelines 1. States must have programs or procedures or both in place within the state’s Child Protective Services system for the purpose of responding to reports of medical neglect, including instances of the withholding of medically indicated treatment (including appropriate nutrition, hydration, and medication) from disabled infants with life-threatening conditions 2. All disabled infants with life-threatening conditions must be given medically indicated treatment, defined in terms of action to respond to the infant’s life-threatening conditions by providing treatment (including appropriate nutrition, hydration, or medication) which, in the treating physician’s (or physicians’) reasonable medical judgment, will be most likely to be effective in ameliorating or correcting all such conditions 3. There are three circumstances under which treatment is not considered “medically indicated.” These are when, in the treating physician’s (or physicians’) reasonable medical judgment: ● The infant is chronically and irreversibly comatose ● The provision of such treatment would merely prolong dying, not be effective in ameliorating or correcting all of the infant’s life-threatening conditions, or otherwise be futile in terms of survival of the infant ● The provision of such treatment would be virtually futile in terms of survival of the infant and the treatment itself under such circumstances would be inhumane 4. Even when one of these three circumstances is present, and thus the failure to provide treatment is not a “withholding of medically indicated treatment,” the infant must nonetheless be provided with appropriate nutrition, hydration, and medication 5. The definition’s focus on the potential effectiveness of treatment in ameliorating or correcting life-threatening conditions makes clear that it does not sanction decisions based on subjective opinions about the future “quality of life” of a retarded or disabled person 6. The Department of Health and Human Services must publish interim model guidelines to encourage hospitals to establish committees to educate hospital personnel and families of disabled infants with life-threatening conditions, recommend institutional policies and guidelines concerning withholding of medically indicated treatment from such infants, and offer counsel and review in cases involving such disabled infants. Reproduced from Federal Register (1985).

ETHICAL ISSUES IN THE CARE OF THE NEUROLOGICALLY DEVASTATED INFANT did not believe her life would be intolerable so, therefore, it was in her best interests to have the surgery. In 1991, the case Re J further developed English courts’ thoughts on these difficult pediatric cases. The infant was a premature infant with severe neurologic injury resulting in seizures, blindness, deafness, and spastic quadriplegia. The courts ruled that decisionmaking in these children is a balancing act between the desire to save these children and the level of pain and suffering that they suffer and their quality of life. Further, this judgment stated these decisions should ideally be made between the physician and the parents and not by the courts. However, the courts were not in a position to mandate therapy felt unnecessary or unwarranted by a physician’s professional opinion (Morris, 2009). Most other countries left decision-making about imperiled newborns to physicians and parents at the bedside. No family or physician was successfully brought up on charges related to the CAA in the United States. Individual cases continued to be brought up intermittently throughout the world. The California case of Baby T was an example in which the Child Protective Agency was comfortable with decisions made by the hospital and its ethics committee. Baby T had been in the NICU for 4 weeks when the family requested withdrawal of life support after their child was diagnosed with nonketotic hyperglycinemia, a genetic disease with severe neurologic devastation and a severely shortened lifespan of less than 10 years. The NICU agreed to withdraw the ventilator. The child continued to survive for several days, at which time the family requested the removal of fluids and nutrition through a feeding tube. The hospital ethics committee met to discuss this decision. After much discussion, they agreed to withdrawal of fluids and nutrition as being the course of action in the best interests of the child and ethically appropriate. An anonymous caller informed child protective services of the decision. The case was investigated and the agency felt no additional action necessary after learning of the detailed review by the hospital ethics committee (Paris and O’Connell, 1991). The pendulum then began to swing in the United States. Parents of neurologically devastated children such as anencephaly and trisomy 13 and 18 began to demand intensive medical therapy that physicians deemed futile and not in the child’s best interest. In the case of Baby L, physicians refused to offer this child additional intubation, mechanical ventilation, and other intensive therapies after 23 months of repeated hospitalizations for respiratory failure and sepsis. The child suffered severe neurologic injury as a result of thick meconium and severe bradycardia during the delivery. The child was blind, deaf, and quadriplegic and only appeared to respond to painful stimuli. She lived at a chronic care facility because of her complex medical

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needs. Her mother rejected the claim that the physicians could limit life-saving therapies and went to court to seek relief. The judge leaned in favor of the mother, although the lawyer for the hospital stated physicians were not inclined to change their mind even under court order because they felt such therapies were unethical for the patient. A guardian ad litem was appointed for the child; this guardian found a pediatric neurologist at an outside institution who agreed with the assessment of her ability to feel pain and critical illness, but he was willing to accept the child in transfer to his hospital. The child survived for several more years (Paris et al., 1990). A final example is the case of Baby K. Baby K was born with anencephaly, the one genetic defect noted in the CAA amendments not to require intensive therapies. Baby K survived the newborn period and resided in a nursing home. While there, she was transferred back to the hospital on multiple occasions with respiratory failure requiring mechanical ventilation, which her mother demanded. Physicians went to court stating further mechanical ventilation was futile; however, the courts stated that it had to be offered because of the Emergency Medical Treatment and Active Labor Act, which requires hospitals and, notably, emergency departments to treat anyone who presents to them in extremis (In the Matter of Baby K, 1994). By 2000, a few states began to develop futility laws that allowed physicians to stop therapies on any patient that they deemed futile. One of the first of these futility laws was a Texas statute, which allowed physicians to withdraw life-sustaining therapies unilaterally if the physicians felt such therapies were futile, and were only prolonging the dying process. In order to do this, a series of steps had to be fulfilled, including a hospital ethics consultation and an attempt by the hospital to transfer the patient to another institution willing to continue the medical therapies. In 2004, Sun Hudson was born with thanatropic dysplasia, a severe form of dwarfism, which results in early death from respiratory failure. This child had respiratory distress at birth necessitating mechanical ventilation. Once his diagnosis and prognosis were determined, the hospital recommended withdrawal of this life support because the child had a genetic condition incompatible with prolonged life. The single mother, who appeared to have a psychiatric illness of her own, refused withdrawal of life support. Texas Children’s Hospital contacted 40 additional hospitals, all of which refused transfer. At that point, the hospital provided the mother with a lawyer to represent Sun’s interests and went to court. Both the probate judge and the court of appeals ruled in favor of the hospital and Sun was removed from life support on March 15, 2005 (Paris et al., 2006).

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England had a similar case in 2006, which brought much news media attention and public angst. Charlotte Wyatt was born very prematurely and suffered a severe brain injury. She had been sustained on mechanical ventilation for the first year of her life. Physicians went to court to permit withdrawal of life-sustaining therapy against parental wishes. In Portsmouth NHS Trust v. Wyatt, Justice Hedley stated: I can only offer three answers: first, that she can be given as much comfort and as little pain as possible; secondly, that she can be given as much time as possible to spend physically in the presence of and in contact with her parents; thirdly, that she can meet her end, whenever that may be, in what Mr. Wyatt called the TLC [tender loving care] of those who love her most. Although I believe and find that further invasive and aggressive treatment would be intolerable to Charlotte, I prefer to determine her best interest on the basis of finding what is the best that can be done for her (Isaacs et al., 2006). France established a new law, known as Leonetti’s law, which discussed patients’ rights concerning endof-life decision-making (Table 18.2). This law required physicians and parents to have more frank discussions

about a child’s best interest in the newborn nursery. Previously, many of these decisions concerning neurologically devastated infants were held behind closed doors with just physicians and nurses. The medical team, citing the infant’s best interest and paternalism, then unilaterally carried out the decisions. Additionally, this law increased the use of pain medication and palliative care at the time of withdrawal of life-sustaining measures, although euthanasia was expressly forbidden. Although this law gave parents some say in the management of their child, it still allowed the physician to stop lifesustaining therapy against parental wishes if the physician in consultation with another physician felt that ongoing medical therapies were not in the best interest of the child (Garel et al., 2011). Many other countries moved towards an acceptance of withdrawal of life support for some infants with severe neurologic impairment and other life-limiting diseases. However, other countries enacted express prohibitions on limiting therapies. One example is the Italian Ministry of Health, which passed regulations stating infants should receive all necessary medical care starting at birth, regardless of their gestational age or other congenital or genetic defects. Most Italian physicians believe these regulations are too stringent and do not serve the best interests of the child (Simeoni, 2012).

Table 18.2 Decree 2006-120 of February 6, 2006 concerning the statutory collegial procedure 2005-370 of April 22, 2005 relative to the rights of patients and at the end of life Article 1: the article R. 4127-37 of the Code of public health is replaced by the following amendments: Article R. 4127-37, I: In all circumstances, the doctor must endeavor to alleviate the patient’s suffering by means adapted to his/her state of health and assist him/her morally. He/she must refrain from any unreasonable obstinacy in his/her investigations or therapies and may stop undertaking or pursuing treatments which appear pointless, disproportionate, or which have no other aim or effect than the artificial maintenance of life. Article R. 4127-37, II: When the patient is unable to express his/her will, the doctor shall not decide to limit or stop ongoing treatments without having previously implemented a collegial procedure in the following manner:

The decision shall be reached by the doctor treating the patient, after consultation with the care team if there is one and in concordance with the opinion of at least one other doctor, called in as a consultant. The motives for the opinion shall be made explicit. There must be no hierarchical tie between the doctor treating the patient and the consultant. The opinion of a second consultant shall be requested by these doctors if this is considered useful by one of them. The decision shall take into account: the desires which the patient may have expressed previously, in particular in his/her advance directives, if he/she has written any; the opinion of the trusted person he/she may have appointed; as well as that of the family or, failing that, of one of his/her close relations. When the decision concerns a minor or a person of full age under protective supervision, the doctor shall also ask for, depending on cases, the opinion of persons having parental authority or of the guardian, except in situations where emergency might make this consultation impossible. The motives for the decision shall be made explicit. The views provided, the nature and meaning of the consultations that took place in the care team, and the reasons for the decision shall be noted in the patient’s record. Reproduced from Baumann et al. (2009). These translations are by the authors and do not constitute an official translation and have not been validated by the French authorities.

ETHICAL ISSUES IN THE CARE OF THE NEUROLOGICALLY DEVASTATED INFANT

THE GRONINGEN PROTOCOL The most controversial published guideline in this area is the Groningen protocol from the Netherlands. This protocol provides for active euthanasia of severely ill children who are seen as having intractable pain and a very poor quality of life. With the publication of this protocol in the New England Journal of Medicine (Verhagen and Sauer, 2005a), and Pediatrics (Verhagen and Sauer, 2005b), neonatologists and ethicists internationally questioned the practice and concluded that children who likely would be treated in other countries, such as those with severe spina bifida, were being euthanized in the Netherlands. A review of the reports of this protocol reveals that numerous meetings, court cases, and decisions led to its development. Beginning in the 1980s, a working group on the ethics in neonatology of the Perinatology Division of the Nederlandse Vereniging Voor Kindergeneeskunde (NVK, Dutch Association of Paediatrics) began to examine end-of-life decision-making in neonatology. The group stated that neonatologists, as the providers of life-saving therapy, also had the responsibility to stop these therapies if they were merely prolonging the dying process or the child’s perceived outcome was considered unacceptable. The working group outlined criteria that, taken together, describe a quality of life (Table 18.3) in which continuing medical therapy was considered “futile.” The report stated that, in reviewing these criteria, physicians should emphasize the pain and suffering of the infant and the perceived future life. If this quality of life was found to be unacceptable, then medical therapy should be considered futile and medical therapies should be stopped after consultation with the parents. This report was approved by the NVK in 1992 and is called “Doen of laten? Grenzen van het medisch handelen in de neonatologie” or “To act or to abstain? The limits of medical practice in neonatology.” Table 18.3 Working Group, Ethics in Neonatology, Dutch Association of Paediatrics Criteria for Quality of Life 1. Expected extent of suffering (in terms of permanent functional impairments, pain, anxiety, prospects for the future and room for hope) 2. Life expectancy 3. Expected possibilities of communication (both verbal and nonverbal) and interpersonal contact 4. Expected possibilities of self-sufficiency (ability to read, write, and work) 5. Extent of dependency on medical care and impact of further treatment on the patient (for example, frequency of hospitalizations) Reproduced from Moratti (2010b). With permission from Issues in Law and Medicine.

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This paper also describes two situations in which deliberate ending of life through a lethal injection might be acceptable. The first is a severely neurologically injured infant, not requiring life-sustaining therapy, who is experiencing great pain and suffering if it is concluded that the child might survive for several weeks or longer. The second case is an infant in whom life support had been removed but the child does not die quickly and humanely. The working group did not reach consensus as to whether lethal injection was appropriate in these cases, although they did clearly state that appropriate pain medication should be administered to prevent pain. In 1995, two court cases clarified the Dutch legal system’s thoughts on these types of case. The first case involved a gynecologist, Prins, who administered a lethal injection to a 3-day-old infant girl with severe spina bifida. Her congenital defect was so severe the physicians and parents decided to withhold advanced medical therapy and provide comfort and pain management only. The family and physicians felt her suffering and pain were not well controlled with pain medication and the decision was made to administer a lethal injection. Prins reported himself to the local coroner, and the case was reviewed at both district court and appeals court level, both of which acquitted him. The second case involved a general practitioner, Kadijk, who was caring for a 24-day-old infant with trisomy 13, a genetic disorder with multiple congenital malformations, severe developmental disability, and a shortened lifespan of usually less than a year. The child’s parents had taken their infant home with comfort care and expected death shortly. At 24 days of life, the parents and physician felt that the life was producing suffering and her prolonged death was unacceptable so the decision was made to give her a lethal injection. Kadijk reported the incident and again, both the district and appellate courts found him not guilty. Despite the fact that these two cases preceded the legalization of adult euthanasia in the Netherlands, they outlined an acceptable way to perform euthanasia in infants in certain rare circumstances. Lethal injection should only be used to end extreme suffering in a severely disabled infant, whose parents agree with the decision. Further, the physician must consult an independent physician about the case for confirmation of the diagnosis, prognosis, and plan. Finally, if the child is hospitalized, the medical team, including nurses caring for the infant, must agree on the euthanasia decision. After the death, the physician must list the cause of death as unnatural, mandating review by the coroner and subsequent review by the legal system (Moratti, 2010a). Reports of physician-assisted suicide and euthanasia in adults were well documented in the Netherlands in the 1990s (van der Maas et al., 1996). It was not until April

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2002 that euthanasia was officially legalized through the Termination of Life on Request and Assisted Suicide (Review Procedures) Act. This act permitted physicianassisted suicide or euthanasia when patients were cognitively competent, aware of their condition, had unbearable suffering, and requested it consistently over time. Additionally, the patient’s physician must obtain a second opinion from another physician and the physician must be present for either euthanasia or physicianassisted suicide. Parents’ permission is necessary for teenagers 12–16 years of age, and it is not permitted in children under 12. Finally, physicians must contact the coroner after the patient’s death, at which point a regional review committee will examine the case to ensure it complies with the Act (de Haan, 2002). These new guidelines, however, offered no guidance to physicians caring for infants and young children, despite the request of neonatologists for official legal guidance on infant euthanasia since the outcome of the two cases in 1995. In 2001, the NICU at the University Medical Center in Groningen had an infant girl born with epidermolysis bullosa, type Hallopeau–Siemens, which is an extremely severe skin disorder in which anything that touches the skin can cause severe peeling, equivalent to a severe burn. In most cases, the child has the majority of his skin affected such that he has an equivalent of an 80–90% burn and there is no known cure or effective therapy. The child has significant pain and suffering and usually dies of infection. The parents of the child in question requested euthanasia because of the baby’s pain and suffering. The neonatologists refused and transferred the child back to the community hospital where the child died several months later. The pain and suffering described by the parents in this child’s last months led the neonatologists at Groningen to review their practice and propose the Groningen protocol to allow for euthanasia of newborns with intractable pain and suffering (Moratti, 2010a). First, the physicians divided the patients who died in their unit into three different groups. Group 1 infants were bound to die even with maximal intensive care therapies. These included children with anencephaly, and severe lung hypoplasia. Group 2 infants could likely be kept alive with intensive therapies but they had a very poor prognosis for neurologic outcome. This group included children with significant hypoxic-ischemic brain injury and those born extremely prematurely. Group 3 infants do not currently require intensive care therapies; however, they have unbearable, chronic, untreatable suffering with no prospect of improvement. This group is described as including the most severe cases of spina bifida. The neonatologists established that groups 1 and 2 commonly have life-sustaining therapies withheld or withdrawn throughout the world. For infants in group

1, critical care therapies only prolong the dying process and comfort measures are indicated. Group 2 children may benefit from medical therapies, but their overall quality of life, as outlined previously in Table 18.3, meant that withdrawal of life support was ethically permissible. The third group was the most problematic because parents and physicians perceived great suffering and pain; however, these children did not currently require life-sustaining therapies so withholding or withdrawing therapies was not imminently possible. Families of these infants were requesting lethal injections or high doses of pain medication to end the infant’s life. The Groningen protocol was developed with consultation from a local prosecutor to allow for euthanasia in this small group of infants with hopeless prognosis and severe suffering. These guidelines required that the diagnosis and prognosis must be certain using appropriate medical data to ascertain that the child’s diagnosis is hopeless, that his or her life includes unbearable suffering, and that he or she has a shortened life expectancy. Further, all alternative therapies must have been considered. A second opinion from a physician who is independent of the treating physician must agree with the diagnosis and prognosis. Both parents must provide informed consent. The euthanasia procedure must then follow accepted medical standards. After the child’s death, the coroner and prosecuting authority must be notified. Finally, the parents must receive grief counseling, support, and followup case review and genetic counseling (Verhagen and Sauer, 2005a). Previous publications had estimated that physicians deliberately ended the lives of between 10 and 15 infants annually in the Netherlands who had severely debilitating disease and suffering. The Groningen NICU had four cases meeting these criteria in which the protocol was followed, and the child was euthanized. Each case was reported to legal authorities and no charges were brought against the physicians. The physicians also reviewed 22 cases of infant euthanasia reported to district attorneys’ offices in the Netherlands between 1997 and 2004 (Verhagen and Sauer, 2005a). In July 2005, the Association of Paediatrics in the Netherlands adopted the Groningen protocol for the entire country. A year later, the Minister of Health and the Minister of Justice established a Central Expert Committee to review euthanasia in neonates. This committee was convened on November 1, 2006, but the first case reviewed by the committee was in 2009 and concerned another child with epidermolysis bullosa. The committee found that all requirements had been fulfilled (Moratti, 2010a). The authors of the Groningen protocol also reviewed all neonatal deaths within the first 2 months of life in the 10 major Dutch NICUs from October 2005 to

ETHICAL ISSUES IN THE CARE OF THE NEUROLOGICALLY DEVASTATED INFANT September 2006 to see what impact, if any, the approval of the protocol by the Association of Paediatrics would have on practice. The infants’ charts were reviewed and their cases were placed into one of the three categories. The charts of 367 infants were reviewed and 359 had adequate documentation to categorize. Additionally, face-toface interviews with the physicians were completed for 192 cases. Of these cases, 340 (95%) had withdrawal of life support. Category 1 children accounted for 208 (58%) of the cases and category 2 accounted for 150 (42%) of all cases. Only one case was classified as category 3. There was only one case in which it appeared that morphine was intentionally used to shorten the lifespan of the infant. The infant suffered from type II osteogenesis imperfecta, a genetic disease of the bones in which the bones are very brittle and daily routine care of the infant leads to multiple fractures of all the bones. The physicians felt that they were ameliorating the pain and suffering of the infant with the pain medication and, therefore, felt that they had to shorten the life to alleviate the pain and suffering. Initially, the attending physician listed the death as a natural one, but after review by the entire medical team weeks later, it was determined that in fact this child had his life deliberately ended (Verhagen et al., 2009). These data showed that, for the most part, infants in the Netherlands are permitted to die but are not being given lethal injections. When the injections are considered lethal, they appear to be pain medications in large doses after it has become apparent to the medical staff and parents that the child is suffering and in pain that is not alleviated by the regular doses of analgesics. Many neonatologists think that proper pain management practices dictate that analgesic doses could be titrated up until the pain was controlled without resorting to lethal doses. Palliative care programs and pain services routinely state this goal is possible so that children die painfree. The practice of active euthanasia by lethal injection is problematic and thankfully not warranted in most cases, even in the Netherlands. The Groningen protocol is a Netherlands’ practice, which appears to be appropriately monitored and controlled. However, it is not a practice that is widely accepted as ethically appropriate outside the Netherlands.

PROFESSIONAL CODES AND BIOETHICS REVIEWS After the publication of the Groningen protocol, several countries re-examined their own policies and made recommendations. The American Academy of Pediatrics published a policy statement on the Noninitiation or Withdrawal of Intensive Care for High-Risk Newborns. This policy divides newborns into three categories based on prognosis. The first group of infants has a high

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likelihood of early death and those infants who survive have a high risk of suffering from unacceptable morbidity. For these infants intensive care is not indicated. The second group of infants has a high likelihood of survival and the risk of unacceptable morbidity is very low. For these infants intensive care is indicated. The third group of infants has an uncertain prognosis, but it is likely to be very poor and survival is likely to be associated with a diminished quality of life. For these infants, parental decision-making should determine treatment choices. This statement goes on to state that all decisions should be made in the context of open and honest communication between the physician and the parents. Parents need to play an active role in decision-making. Comfort measures must be given to all infants. Finally, therapies must provide benefit to the infant. It is inappropriate to continue therapies on infants with conditions incompatible with life, or when treatment is deemed to be harmful, of no benefit, or futile (Committee on Fetus and Newborn, 2007). At about the same time, the Nuffield Council on Bioethics in England also established a working group to examine the ethically appropriate treatment of newborns. This group concluded that the infant’s best interests must take absolute precedence. Therapy should benefit the child and not just prolong death or produce pain and suffering. The child’s predicted ability to survive and the degree of disability he or she will experience are to be taken into account when making decisions about intensive care therapies. Parents should be involved in decision-making. No life should be actively ended, regardless of the severity of the disease process. Further, oral nutrition and hydration should only be withheld when it clearly causes pain and discomfort to the infant. However, the baby’s best interests should prevail, so that when an infant has severe pain and suffering, pain-relieving therapies may be initiated even if these therapies potentially shorten the child’s life. Finally, if the parents and medical team cannot come to an agreement concerning an infant’s best interest, then a clinical ethics committee should be convened to provide consultation (Nuffield Council on Bioethics, 2006).

RELIGIOUS BELIEFS Although laws and professional codes and guidelines provide some direction to physicians, parents rely on their cultural background and religion to help make decisions concerning their children. The world’s major religions have many similarities concerning end-of-life and medical therapies, but they also have some differences. Fundamentally, they all believe that God makes life and death decisions. A physician may possess healing powers because of God, but only God decides when someone dies. This leads to “miracle” language by families who hold out hope of a miracle that their child will

222 T.K. KOOGLER survive, or be less disabled than the medical team prephysicians’ perceptions can be markedly different. The dict. As a result, they may question prognostication issue then becomes, who decides and how do they decide? and resist limitation of therapy based on a physician’s A physician is obligated to determine the medically prediction of death or poor neurologic prognosis. appropriate options for therapy for a child. Medical therThe reality is that physicians’ predictions can be inacapies should always benefit the child and in most cases curate, and often someone says the child is going to die the potential benefits should outweigh the burdens of and therapies should be withdrawn, when in fact the phythe therapies. Clearly, all children should receive approsician thinks the quality of life is going to be exceedingly priate pain relief and if infants can take food by bottle or poor by medical standards and that the child should be by nursing then they should be offered nutrition, unless allowed to die. The child then survives and a “miracle” such feeds just cause them pain and suffering. Other occurs and thus physicians’ predictions become less therapies need to be based on the child’s diagnosis, progbelievable. Miracle language makes discussions about nosis, and potential future. Comorbidities should be limitations on therapies very difficult but physicians examined for each therapy. must learn not to discount the miracle and thus remove As an example, a child with trisomy 13, central apnea, any hope, while at the same time presenting reasonable and complex heart disease might not be a good candidate next steps in the child’s care. for heart surgery because the risks and burdens outReligions also have tenets or laws concerning intenweigh the potential benefits. This child, because of the tional killing, which may interpret withdrawal of lifecentral apnea, is unlikely to be able to survive without sustaining therapies as equivalent to murder. The Qur’an lifelong mechanical ventilation. Adding complex heart contains the maxim: “Whosoever takes a human life . . . it surgery to chronic mechanical ventilation and other medis as if he has taken the life of whole Mankind” (Qur’an ical conditions from trisomy 13 only increases the mor5:32). Most scholars interpret this as intentional taking of bidity and mortality of the surgery and subsequent life, but some Muslims, especially families, may see withtherapies. However, the infant with trisomy 18 who goes drawal of life support as also taking a life, especially home with tube feeds, learns to take a bottle, and then when it is their child (Gatrad et al., 2008). develops congestive heart failure unresponsive to medJewish law can also be interpreted to prevent withications from a ventricular septal defect (VSD) might drawal of life-sustaining measures, however, it is perbe a candidate for a cardiac catheterization procedure missible to not increase existing therapies and to with closure of the VSD by a device. This would be a withhold additional therapies. Jewish law describes a much lower-risk procedure than complex heart surgery, “gosess” as a person, child, or adult, whom physicians with less morbidity and mortality, in a child who is thrivbelieve will die within 2–3 days. In Jewish law, medical ing overall at home despite his neurologic disabilities. teams should not do anything to prevent death from Physicians are not obligated to offer therapies that will occurring so, therefore, additional therapies should only prolong the dying process and not provide comfort or not be added, as these therapies will just impede the improvement in lifespan or quality of life. While extracordeparture of the dying soul. At the same time, nothing poreal membrane oxygenation is available at many tertiary should be done to intentionally hasten the death. Withcare facilities as a temporizing measure for severe heart drawal of existing therapies is sometimes seen as hastenfailure, it is not an obligation of physicians to offer it to ing the death (Shinwell and Shinwell, 2008). every dying child. In fact, best interest standard would state that the physician must evaluate its effectiveness and only offer such heroic measures when there is some realistic thought that it will reverse the severely debilitated COMMENTARY state in which the child currently exists. Physicians must The physician often is placed in a difficult position: balremember that just because the technology is available ancing the best interests of the child, the applicability of does not mean that it is appropriate therapy for every child. local laws or culture dictating how to care for the child, Withdrawal of therapies that are only prolonging the and the parents’ wishes or demands. What is the approdying process is also ethical. However, as several of priate ethical response to the difficult dilemma of balanthe newer laws and codes state, parents should be cing these frequently conflicting directives and involved in the decision-making to withdraw lifepressures? Is there a single right answer for treating neusustaining therapies. In some countries, it is typical for rologically devastated newborns? physicians to be quite directive to families about withThe simple answer is no. Each child is an individual, drawal decisions and essentially to state, “This is in your and therefore, each decision must be made individually child’s best interest. We are sorry, but we are going to by carefully examining the best interests of that child. stop these interventions because they are not benefiting As many of the controversial cases above show, however, your child and are merely prolonging the dying process.” the parents’ perceptions of best interests and the Other countries believe that parents need to be given all

ETHICAL ISSUES IN THE CARE OF THE NEUROLOGICALLY DEVASTATED INFANT 223 the information and make the decision themselves. be preferable for parents to determine a child’s best Placing the decision on the parents is very difficult. As interest in isolation and not consider family dynamics; noted above, some families interpret their religious edicts however that is unrealistic as everyone is a member of not to withdraw therapies; other parents simply do not a family. Every member of a family makes some sacriwant the responsibility. They would prefer the weight fices for others in the family, such that no one’s best of such a decision to rest on the physician. A physician interests are always served. Further, in some countries, may need to own the burden of withdrawal of life support resources are simply not available to care for a complex in order to relieve a grieving family of the responsibility. child appropriately. Families are then required to care for A physician’s role is complex because she truly needs their child with inadequate resources or, in rare cases, to limit the best interest standard to medical best interests parents seek out resources abroad. and not necessarily bring her personal cultural or reliThe way to navigate this best interest decision-making gious beliefs into the decision-making. Also, it is probaand minimize disputes and mistrust is through open conbly best to avoid quality-of-life language and thoughts versation between the family and physician, as contained because physicians often demand higher quality-of-life in several of the policies presented above. The physician standards than individual families. At times, because must first ascertain the family’s desires and wishes for of local medical resources or laws, outside influences their child. Allowing the family to voice their wishes must also dictate what the physician can offer to families and fears will help establish trust and will also hopefully in both therapies and limitations of therapy. determine how the physician should proceed with the Parental roles are also complex because they must conversation. A good listener will recognize the fears provide all the other areas of a child’s best interest. They and hopes the family has for their child. One might also bring the cultural, religious, and individual family belief be able to determine if the family truly wants to make the system to the decision-making table. At times parents decision, or whether they want the physician to own the may be as distraught over conflicts within these systems decision. The conversation can take shape to include as they are with medical decisions that they dislike. Some those elements the family finds important and further families might recognize their child is suffering and wish discussion of other items that are medically necessary to allow it to end, but fear that God will punish them if or appropriate for the best interest of the child. they relieve their child’s suffering. In other circumThe physician must respect the family’s choices stances, parents may have strong convictions about the within the limits of the medical system. Clearly some sysappropriate care of their child, but these convictions tems will prevent withdrawal of some therapies and most go against beliefs of the grandparents and they fear retsystems will prevent a physician from giving a lethal ribution from within the family. Both situations can be injection to an imperiled infant, even when the pain quite difficult for families. and suffering appear intractable. However, physicians Parents also determine the quality of life that is are not obligated to offer heroic measures to prolong acceptable for their child in most cases. For some, a a child’s dying process. Basic measures to improve the severely disabled child with little ability to interact with child’s quality of life are appropriate and should be done the world will not be an acceptable life and they will with the parents’ approval; however, critical therapies desire withdrawal of therapies. These families might which are thought to have more burdens than potential even want some form of intentional killing if the child benefits do not have to be offered to families. continues to live for days, weeks, and even months withCritical therapies, such as mechanical ventilation, that out medical intervention. In most cases, withdrawal of are thought to provide a temporary bridge while a child therapies is permitted, but for most, the practice of recovers from a viral bronchiolitis or a single episode of intentional lethal injections is illegal, and believed to aspiration pneumonia, may be offered and might even be unethical by most physicians. For other parents, the be obligatory if the family wishes to pursue aggressive most important value is life itself; the quality of that life therapy. Just the child’s neurologic state or quality of life is not important in a person’s best interest. For these famis not sufficient for a physician to limit therapies, espeilies withdrawing therapies that are maintaining a baby in cially unilaterally. The quality-of-life measure must be his or her current state can be very difficult, if not imposexamined in the context of the other medical issues of sible. They see the smallest amount of time alive as prethe child and potential risks and benefits of the proposed cious, even if it is a couple of hours or days. therapy. Families’ voices must be heard and respected At other times, a family might look at a child’s best whenever possible as long as the proposed management interests in the context of a family’s best interest. A does not cause the child undue pain or suffering or developmentally delayed, technology-dependent child merely prolong the dying process. The best interests of will add many burdens to any family. Some absorb this a child are multifactorial, and therefore input from mulextra stress, work, and financial burden willingly and tiple sources is necessary to determine the best interests others feel it will be too costly for the family. It would for any given child.

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CONCLUSIONS I have described similarities and differences amongst cultures in approaching the neurologically devastated child. The most important feature of each approach, however, is that the best interest standard is used to determine the best care for the child. Second, most processes insist on including the parents, and having the parents agree to limitations of therapy prior to implementing them. Third, all protocols insist that a child who is dying be pain free, even if the administration of pain medications might shorten the lifespan. Minimizing pain and suffering and providing comfort care to children in all aspects of their life is necessary but absolutely essential in the dying infant. Finally, only the Netherlands considers active euthanasia to be morally permissible and a close review of their literature reveals that it is actually quite rare. This practice remains controversial, as it should. Pain and suffering should be able to be alleviated in almost all cases without the consideration of a lethal injection. The central issue in the care of any infant, including one with significant neurologic impairment, is to examine the child’s best interests from all areas, not just medical. This requires teamwork between the physician, medical team, and family.

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