Pediatric neurology is a complicated specialty area where extremely rare conditions are seen with relative frequency. Unfortunately, the underlying cause of a given condition may be impossible to identify due to the immature state of the science. Without this information, it is extremely difficult to discuss a child's prognosis with any degree of certainty. In these situations, it is often necessary to rely on past experiences, realizing that many of these children will survive only to experience a neurological sequelae ranging from mild learning disabilities to in some cases a persistent vegetative state. The problem then becomes how healthcare decisions should be made in a case where serious impairment is likely, although the state of the science is such that we cannot know the extent of the impairment. This article discusses decision-making practices and offers ways in which nurses can facilitate and argue for the importance of parental involvement in decision making for their child with neurological impairment.
Status of Decision Making
Currently there are no universally accepted guidelines for decision making that address those cases in which a child's medical prognosis is uncertain. The following serves as an example of the problem.
C.P. was a full-term newborn admitted to the inpatient neurology service after he began having seizures shortly after delivery. C.P. had no difficulty feeding and had normal muscle tone and cried when he needed to be changed. However, the neurology team had a tremendous amount of difficulty controlling his seizure activity. A leading expert in neonatal neurology was consulted and determined that the most likely diagnosis was a type of epilepsy called Ohtahara syndrome. Ohtahara syndrome is a rare neurological disorder of infancy, which consists of frequent generalized seizures and continuous abnormalities on electroencephalogram. This disorder is most often associated with mental retardation and a severe psychomotor prognosis (Ohtahara & Yamatogi, 2006). Children with this rare disorder are faced with a lifetime of severe neurological impairment, if they are even able to survive into childhood. After the diagnosis had been made, the neurology team, nurses, and social worker met with this family to discuss what it meant to live with this disorder and what treatment options were available. At no time during this meeting did any of the healthcare professionals offer withdrawal of treatment. It was the parents who requested a do-not-resuscitate status for C.P. (It was difficult to assess the parents thoughts during this time. The parents were both withdrawn, neither appeared bonded to the child, and they did not visit often. There was very little interaction with the members of the healthcare team.) During this meeting, the parents also asked the team if it would be possible to withhold nutrition and allow C.P. to die. The medical team suggested that the parents spend more time thinking about the decision to withhold nutrition and reassured the family that the team would be available to discuss that option again at a later date. The parents went home for the night. Members of the healthcare team later expressed shock and disbelief that this family was willing to stop feeding this child-the option had never been considered by the team. Some thought that although C.P. may live with significant disability, it would be wrong to end his life. The same night as that meeting, C.P. suddenly died, and the parents never needed to make the decision to withdraw feedings.
Members of the healthcare team struggled with this case for some time after C.P. died. One reason for the continued distress surrounding this case may have been due to the fact that there were members of the healthcare team whose desired goals were not the same as those of the parents. For some, the goal was simply to reduce the number of seizures the child was experiencing, which could have been successfully accomplished with medications. Others believed the goal should be to preserve life but only if the family could be assured that there would be some quality to it. In this case, successfully treating the seizures would not necessarily impact the degree of neurological impairment suffered by this child. Although this child's extent of neurological injury could not be precisely predicted, past experience led us to believe that the child would probably have to live with a severe degree of cognitive and physical impairment. Aggressively treating his seizures could have prolonged his life. Should the parents be supported in their wish to stop the treatment for their newborn son when we have available to us medications that could have been useful in extending his life? Is a life with a handicap not a life worth living? Should a decision to withdraw care be supported because a child must live with mental retardation and a physical handicap? Let us briefly examine guidelines that exist to address this issue.
The Baby Doe rules were first composed in the early 1980s in response to a decision made by parents of an infant who had Down syndrome and a tracheal esophageal fistula. These parents decided to forgo life-saving surgery for their infant who otherwise had no major physical defects, which ultimately prompted the Department of Health and Human Services to extend the Rehabilitation Act of 1973 to protect handicapped infants. These new rules stated that treatment could not be withheld if an infant was handicapped or if the intervention was not contraindicated because of the handicap (Devettere, 2000). Although these rules were initially intended to protect the rights of handicapped infants, their continued use today is somewhat controversial. Kopelman (2005) argued that these rules do not consider the wishes of the family and essentially are in opposition to the use of individualized plans of care that are based on context. The Baby Doe rules cannot be applied to every situation in which an infant may survive only to experience a severe handicap. What happens in a situation where ongoing medical treatment is considered to be futile or becomes a burden to the child?
The previous case exemplar helps to highlight some of the issues surrounding medical conditions and what can be classified as medically futile. The idea of medical futility has been widely addressed within healthcare literature. A futile treatment can be defined as "an action, intervention, or procedure that might be physiologically effective in a given case, but cannot benefit the patient, no matter how often it is repeated. A futile treatment is not necessarily ineffective, but it is worthless, either because the medical action itself is futile, or the condition of the patient makes it futile" (Clark, 2001, p. 181). Nelson (1992) referred to treatment as futile if "it will not serve any useful purpose for the patient, may cause the patient needless pain and suffering, and does not achieve the goals of curing and restoring the patient to an acceptable quality of life" (p. 429). The problem in the case of a child is who determines what level of burden is acceptable for the child to live with? The goal of treatment helps to determine whether or not an action is considered futile. If a child could continue to live for many years with something as simple as medications, do we as healthcare professionals have an obligation to protect that child's right to life? What counts as a life of quality? If we cannot determine if a case is futile, then how can we make decisions regarding how aggressive treatment should be? In these situations, some would argue that decisions should be made primarily with the child's best interest in mind.
Many healthcare professionals would support the argument that the best-interest standard should be applied when making decisions for a child, but how is this idea incorporated into practice? As Kopelman (2005) noted, some have proposed using the best-interest standard to make decisions when a child's future is uncertain:
The best-interests standard is a moral and legal standard for guiding decisions for persons lacking decision-making capacity and who have not left advance directives; it should be used by guardians, judges, clinicians, or other responsible persons for making decisions for the incompetent person by assessing the net benefits and burdens and selecting the best available options for the incompetent person. (p. 798)
However, determining what is in the best interest of the child is a difficult task. Some may approach the dilemma by considering the ethical principal of beneficence. The idea of beneficence, or doing good, underpins all healthcare practice and is clearly articulated in the Hippocratic Oath, which states that physicians will intervene "for the benefit of the sick according to (his/her) ability and judgment" (Devettere, 2000, p. 67). The oath does infer a degree of medical paternalism where the physician is the authority on what actions are best. This idea of paternalism can be seen when interventions are often based on what the physician believes is best for the patient as opposed to considering the situation from the patient's perspective (Devettere, 2000). Frequently, when deciding what can be done for a child, the medical team considers all possible treatment modalities and will use certain interventions even when there is no evidence to support their potential effectiveness. The physician's treatment decisions sometimes seem to be geared toward prolonging life regardless of the quality of that life. The focus is on the idea that any life is worth preserving. "It is inconsistent with a child's best interest to impose a burden disproportionate to the benefit achieved or to demonstrate disrespect for a child's dignity and worth as a person" (Nelson, 1992, p. 428). How can anyone evaluate the life worth of a child with a neurological impairment? Because we cannot predict the future with any accuracy, is it acceptable to withhold treatment? In the case of a child with a severe neurological disorder, many physicians make this decision by themselves by not offering the family an option to withhold treatment as long as something exists, which may provide the patient with some degree of benefit, where what counts as benefit may be ill defined. Mims and Crisham (1996) stated, "lacking sufficient evidence to stop treatment, the health care professional must continue, in order not to violate his or her moral obligation" (p. 238). Healthcare providers are challenged with the need to protect the best interest of the infants they are caring for. Although these treatment decisions may be made with only good intentions, they may be ill considered without family input. This is because decisions should not be made without an understanding of the family. The family's needs and concerns should be part of a decision-making process focused on the needs of the other. In determining the best treatment for a child, how should the family's perspective be made a part of the decision-making process? "Our social system generally grants patients and families wide discretion in making their own decisions about health care and in continuing, limiting, declining, or discontinuing treatment, whether life-sustaining or otherwise" (American Academy of Pediatrics [AAP], 1994, p. 533). Instead of independently making decisions for our pediatric patients, should we consider the wants or needs of the family? (Table 1).
Permissible Action
Oftentimes, although a child may survive with severe neurological impairment, we may not provide families with the choice to withhold treatment and instead focus on keeping the child alive, emphasizing the potential the child may have for some quality of life. One question that arises in these difficult situations relates to the definition of quality of life. We often justify withdrawal of treatment when the person's prognosis involves what we consider to be a poor quality of life, but each individual may have a very different view of what constitutes a good quality of life. One person may refuse treatment in a situation where he would be left without the ability to feed or care for himself. Another person may feel that as long as he retains the ability to experience pleasure from seeing his family members that his life has quality and is still worth living. It is extremely difficult to put aside our own personal feelings of how we measure quality of life and not let our own thoughts affect the types of medical treatment options that we offer to families. This is difficult even when we are treating an adult patient who is able to clearly articulate his or her own wishes. It becomes even more difficult when a parent is deciding for a child. How can we as medical professionals be sure that the parent is making a decision that adequately reflects what is in the child's best interest? Do we have the obligation to intervene on behalf of the child if we think the parent is making the wrong choice? How can we ever claim to know a child better than his or her parent?
Some may argue that the principle of preserving quality of life would support aggressively treating a seizure disorder with multiple antiepileptic medications. The argument to support this notion would include the idea that decreasing the number of seizures a child experiences in any given day will provide him with more periods of alertness, which would provide the opportunity to learn new skills and have more meaningful interactions with their family. Probably no one would believe anyone would argue that these goals are not in the child's best interest. However, by aggressive treatment with multiple antiepileptic medications, we are exposing children to a variety of side effects that may include symptoms ranging from weight gain and lethargy to liver dysfunction and life-threatening rashes (Stevens-Johnson syndrome). Not only that, but some may argue that we are helping to prolong a life for a child who may have a limited or nonexistent ability to interact with the environment around them. We may believe that these children probably do not experience this suffering, but are we correct in assuming this? Do we also need to consider the amount of suffering experienced by the family? Should we consider the effects on the family when a child will survive with severe but not life-threatening neurological deficits? If we are supposed to be treating the family, this should be raised as a concern and the family should be involved in all aspects of decision making for their child. It is the family who will care for this child day in and day out for the rest of their lives. Aggressively treating a seizure disorder has pros and cons, and the family should be able to weigh the risks and benefits for their child. How can we decide that someone should be forced to take on such an overwhelming responsibility? When parents have to make decisions regarding the care of their child they are affected by "profound uncertainty about the baby's future, deep concern for the baby's current suffering and future health, and fear of how the unfolding events are going to affect and be affected by their lives outside of the hospital" (Hardart, 2000, p. 165). Although the medical team has the luxury of only having to consider what treatment options are available for this patient, the family also needs to incorporate how their lives will be forever altered by their decisions. Who has the ultimate responsibility of deciding what is best for the child?
Decisional Authority
By strictly adhering to the Baby Doe regulations, the child's best interest is not taken into consideration, and the treatment may be considered burdensome (Devettere, 2000). Are there not situations in which it would be considered acceptable to withdraw life-sustaining treatment? Life-sustaining medical treatments include "the dramatic measures of contemporary practice such as organ transplantation, respirators, kidney (dialysis) machines, and vasoactive drugs, it also includes less technically demanding measures such as antibiotics, insulin, chemotherapy, and nutrition and hydration provided intravenously or by tube" (AAP, 1994, p. 532). Applying this idea to the case presented earlier, nutrition and antiepileptic medication would be considered life-sustaining treatments. The question then becomes, would the parent's decision to withhold nutrition have been in the best interest of their child?
A combination of subjective and objective measures that examine the effectiveness, benefits, and burdens associated with treatment has been proposed by some as a means to facilitate decision making (Clark, 2001). AAP (1994) has outlined benefits and burdens for the pediatric patient. The AAP considers benefits to include the ability to experience pleasure, intellectual satisfaction, and an increased quality of life. Burdens are pain, physical disability, and emotional suffering (AAP, 1994). Healthcare providers should take into consideration these issues when examining possible treatment options and the effect those decisions may have on the child's quality of life.
To facilitate decision making in uncertain medical situations, many factors should be taken into consideration. These factors include determining the patient's goal, whether or not the treatment will be burdensome, and the wishes of the parent (Nelson, 1992). What about persons who are incapacitated or lack the ability to make decisions for themselves? Who should be responsible for protecting the rights of the child? This is an issue we often deal with when caring for children with neurological impairment. It may be necessary to first address the issue of what constitutes a "person." Philosophers have identified characteristics necessary to be considered a person, which is important because persons are believed to have certain rights. These characteristics include having interests, having cognitive awareness, being capable of relationships, and having a sense of futurity (Edge & Groves, 2006). The idea of personhood is important to consider in a situation where a child may be neurologically devastated. In the case study presented earlier, some may argue that it is difficult to determine personhood in an infant when we do not have the capability of measuring to what extent the infant will be able to develop both cognitively and physically. However, we can often assess the degree to which an infant or child can maintain relationships, and we can observe the degree to which the infant responds to its parents or its environment. In an older child, these criteria become more easily observable. If we do consider an infant to have personhood, then they also have a right to self-determination. Because infants are unable to speak for themselves, it is the parents who are left with the burden of deciding for the child. Parents have no way of knowing what their child would want because they cannot express their wishes. However, the parents often considers their own personal or family values to decide what they think would be in the best interest for their child. The healthcare provider should be available to aid the family in this difficult process.
Professional Responsibilities
The medical team often makes decisions on behalf of the child by not offering all available treatment options to the family. Physicians may decide when a child should and should not continue to receive aggressive treatment and only present to the family what they think would be the best course of action. For example, in the case presented earlier, no member of the healthcare team thought of offering the family the possibility of withholding treatment. However, after a child is discharged home with their family, we may never see them again. The healthcare team teaches the family how to administer medications, how to do range-of-motion exercise, and how to set up tube feedings. What obligation do we have to ensure that these parents have the capability to carry out tasks which are vital to the care of the infant? Does our obligation to the child and their family end after discharge? Without providing parents with the option to not treat, we are deciding that the family will essentially need to drastically change their lives as they know it. We are deciding that this family will need to be responsible for everything this child will require for the remainder of their lives. Is it necessary to consider the impact on the family as a unit, or is it enough to believe that our primary responsibility is to our patient alone? What about the burden placed on our healthcare system?
Healthcare providers often do not think about cost as a factor when considering the needs of a child with epilepsy. However, it is imperative to consider the societal effects of allocating already-scare resources to prolong the life of a child who may not realistically have a future. The annual costs, both direct and indirect, of treating one person with epilepsy have been estimated at approximately $60,000 (Begley et al., 2000). Children with epilepsy may use more healthcare resources than children with other chronic illnesses because epilepsy is associated with several comorbidities (Bazil, 2004). Children with epilepsy often require additional services, including physical therapy, early intervention, psychosocial counseling and treatment, and nutrition supplementation. It has also been shown that adults with epilepsy are less likely to obtain college degrees and are more likely to be unemployed (Bazil, 2004). If a child with epilepsy survives into adulthood, should we consider the contributions they will make to society when discussing treatment options? Currently, healthcare coverage is a concern for most Americans. According to the Federal Interagency Forum on Child and Family Statistics (2005), in 2005, there were 8.1 million children living in the United States without health insurance. These children lack the basic necessities to maintain health including well-child care visits and immunizations. Is it ethically responsible for us to spend so many rare healthcare dollars to treat a child because his or her parent has asked us to do everything possible? Do we not also have a responsibility to the child who does not have the means to address his or her basic health needs? As a society, we need to reevaluate how healthcare dollars are spent to ensure that all of our citizens may have access to high-quality care.
Nursing's Role
The Nursing Code of Ethics states that, "the nurse strives to provide patients with the opportunities to participate in planning care, assures that patients find the plans acceptable and supports the implementation of the plan" (ANA, 2001, p. 9). According to this statement, nurses should not be solely concerned with doing whatever they can to treat the patient. Rather, the focus is on developing a plan of care that is acceptable to the family. How can we do this if we do not provide patients and their families with all of the information and available treatment options? Most would agree that the decision to treat a child with an uncertain prognosis is not an easy one to make. The dilemma in and of itself has several implications for nursing practice. If we believe that one of our professional duties is to advocate for our patients, then we have a responsibility to ensure that their wishes are being met. We are in a position to be able to spend time with our patients and help them decide what they feel would be best for their child. For the parents to be able to make an informed decision, they need to be provided with all of the available information. AAP (1994) stated that parents have the "ethical and legal right to adequate information about reasonably available diagnostic and therapeutic options (including risks, benefits, nature, and purpose of the options)" (p. 533). Nurses should assume the responsibility of educating their patients and their families on different treatment options, including the option not to treat. Our experience provides us with the ability to discuss with parents both the joys and sorrows associated with caring for a child with neurological impairment. Nurses can facilitate discussion among parents experiencing similar issues so that they can learn and receive support from one another. Then, when parents' wishes seem to contrast that of the medical team, we can help assure that the child's voice is heard by making sure that the parents' wishes are expressed to the medical team.
Proposed Nursing Guidelines
Nurses are in a unique position to be an integral part in the development of guidelines to help parents make decisions for their children. These guidelines should incorporate several techniques that can be used to address some of the issues presented in this article. The first suggestion should be to always keep the families informed of any available information. Nurses need to ensure that the information shared with these families is clearly articulated and understood and that the families involved have the capability to make an informed decision. Nurses need to familiarize themselves with the latest research to ensure that the information they are providing to families is up-to-date and accurate. It is also important to offer families an opportunity to speak with other families raising children with similar chronic illnesses. Those families are the only people who can truly understand the quality-of-life issues for the child and the rest of the family members.
When working with families, the use of nondirective counseling should be considered an appropriate strategy. Carl Rogers originally described this type of counseling more than 50 years ago. Using a nondirective counseling approach includes recognizing the feelings of another person and allowing them to lead the direction of the conversation. This is done successfully by reflecting on what the parent says, clarifying statements as needed, and summarizing for the parent what you believe are their major concerns or fears. Questions are asked only to encourage the parents' participation in the subject and to keep them focused on the topic (Rogers, 1951). Using nondirective counseling, the nurse does not give advice or an opinion on the topic at hand. This technique allows the nurse to gain a better understanding of the families' needs and concerns without introducing their own personal bias into the situation.
A social worker, child life specialist or psychologist should be introduced to the family as soon as possible. This will not only provide the family with emotional support, but it may also be helpful in guiding the family through the decision-making process. Wolfe et al. (2000) found that, "when a psychosocial clinician (psychologist or social worker) was involved in the child's care there was greater concordance in the timing of physicians' and parents' understanding that the child had no realistic chance for cure" (p. 2474).
The use of an ethics committee or palliative care team can be useful to help members of the healthcare team address issues surrounding the treatment of a child with neurological impairment. Many hospitals now have ethics committees that are available for consultation. In the case presented earlier, the institution involved does have both an ethics committee and palliative care team, which may be consulted by any member of the healthcare team at any point in time. Unfortunately, these consultations were not utilized at the time because the child died before the discussion could occur. An ethics committee can be useful for guiding clinicians in deciding what is best for the patient and who should make that decision. Palliative care teams can offer advice on how to ensure that the child is maintaining comfort regardless of what treatment decision has been made. These teams are often a crucial element to ensuring that the best interests of the child are maintained.
Although we often think of the suffering endured by these families during these difficult situations, the emotional distress of the healthcare provider also needs to be addressed. It is necessary to provide adequate support for all healthcare providers caring for these patients and their families. Caring for a child with neurological impairment is never easy, but it is the responsibility of the nurse to ensure that steps are followed to guarantee that in each case parents have the knowledge and support they need to make the appropriate decision for their own child and family.
Summary
When a child's prognosis is uncertain, there is often disagreement among members of the healthcare team as to what the treatment goals should be. The nurse in these situations should work with the family and the physician so that goals are recognized and incorporated into the plan of care. In most situations, the family and the healthcare providers are all working toward the best interest of the child. The problem is that there are no clear guidelines for determining the best interest of the child. Our own personal values often influence what we think is an acceptable quality of life. Therefore, it is important to understand that people may have different perspectives, and we need to explore those before treatment decisions can be made. Nurses can be instrumental in encouraging difficult conversations between the family and healthcare team so that all perspectives may be considered and the best decision for the child can be made.
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