We present the case of a 2-year-old boy with epidermolysis bullosa and supraglottic stenosis whose parents refuse an elective tracheostomy because of the significant care the tracheostomy would require. The patient’s family lives in a rural area with few health care resources and his parents are already handling hours of daily skin care for his epidermolysis bullosa. In an attempt to convince the parents to pursue the intervention, the medical team recommends that the family move to an area with additional resources to assist in the child’s care. The parents refuse to move, citing the many benefits their home environment provides for their son. The medical team calls an ethics consultation, questioning whether this decision constitutes medical neglect. This case raises important questions about medical decision-making in pediatrics. First, is a parent’s refusal of a recommended medical intervention because it would require moving their family to a new environment a reasonable decision? Second, how broadly can parents define their child’s best interest? Should only physical interests be included when making medical decisions? Is there a limit to what can be considered a relevant interest? Third, can parents only consider the interests of the individual child, or can they consider the interests of other members of the family? Finally, what is the threshold for overruling a parental decision? Is it whenever the parent’s definition of a patient’s best interest is different from the medical team’s, or do other criteria have to be met?

The most prevalent standard for evaluating pediatric medical decisions is the best interest standard (BIS). By this stand, surrogate decision-makers are expected to choose the option that is in the child’s best interest. Clinicians tend to define best interests narrowly, focusing on medical harms and benefits. This sometimes leads them to discount or dismiss other interests, including social relationships or family burdens. In this ethics round, we present a case in which the parents place a greater emphasis on their child’s nonmedical interests, and perhaps family interests, than they do on pursuing a treatment that would increase his chance to live a longer life. Specifically, the parents feel that moving their child from their rural environment to receive better home nursing care after a tracheostomy is too great a cost to justify the projected benefit of an elective tracheostomy.

The central question of this case is whether the parents’ decision reaches a certain threshold at which the medical team should report the case to Child Protective Services and seek state intervention to order the tracheostomy.

JZ is a 2-year-old boy with recessive dystrophic epidermolysis bullosa (RDEB). JZ and his parents live in a rural community several hours from the hospital where he receives interdisciplinary care for his RDEB. JZ requires hours of daily skin care from his parents. Both JZ’s parents and the hospital social worker have exhaustively sought home nursing resources in JZ’s community, but few exist. JZ only receives 2 hours of early intervention per week.

JZ also has supraglottic stenosis, which causes noisy breathing and frequent “choking episodes” when he gets a viral respiratory infection. During some of these episodes, his parents sought care at a local emergency department. During these episodes, he is able to maintain his oxygen saturations, but his dyspnea and stridor are significant enough that the local providers have recommended intubation. His parents have always refused intubation because of concern that this would lead to scarring and worsening of his stenosis.

On the basis of the recurrent stridor, the otolaryngology attending recommends an elective tracheostomy to provide JZ with a safe airway. The attending estimates that JZ has a 10% to 20% chance of death due to an acute respiratory failure without a tracheostomy compared with 5% with a tracheostomy. A tracheostomy would require additional hours of daily care from his parents, which they do not feel they have the capacity to provide given the lack of resources in their community. JZ’s parents decline an elective tracheostomy.

After the parents’ decision to decline a tracheostomy, JZ’s dermatologist recommends that the family move to an area with more resources. When asked about moving to an urban setting, JZ’s parents state that they feel it is better for JZ to live in their rural community even if this means his life will be shorter. His family has a strong network of family and friends in the community, and JZ loves being outdoors. They fear that moving him to an unfamiliar urban environment would cause him significant distress.

The medical team requests an ethics consult to discuss whether the parents’ refusal of an elective tracheostomy constitutes medical neglect.

This case is a classic example of an ethical conflict; both the medical team and the parents have strong convictions about what is best for JZ, they just do not agree on what that is. When reasonable people have different assessments of what is best for a child, how do we evaluate what is the right option? The BIS states that the surrogate should choose the option that maximizes the benefits and minimizes the harms to the child.1  The BIS is relatively clear in its criteria for decisions. It is less clear how, exactly, it should be used. It can be used as an ideal, but that is not always attainable. It can be used as a standard of reasonableness to judge what factors surrogates can consider in coming to a decision. Or it can be used operationally, as a threshold for intervention.1  In this case, are JZ’s parents being reasonable? Is refusing a medical intervention that would require uprooting a child from his home environment a reasonable interpretation of his best interest?

The medical team believes that a tracheostomy will increase JZ’s chance at a longer life. Prolonging life is clearly one of JZ’s interests. But that interest needs to be weighed, somehow, against his interest in having a better quality of life. Malek2  attempted to define the range of interests a child might have. Her account suggests that we must consider their physical wellbeing, mental development, relationship with others, and sense of self.2  She describes 13 specific interests of children, only 4 of which are aimed at their physical wellbeing. The others include emotional development, play, cognitive development, communication, interaction, relationships with others, identity, sense of self, and autonomy. In this case, JZ’s parents may be weighing some of these interests more heavily than his interest in a prolonged life. For instance, JZ loves being outdoors, so his interest in playing outside might seem most important to his parents. Perhaps they believe that his cognitive development will be better in an area where he can engage directly with nature rather than in an urban environment with, perhaps, limited outdoor exposure. Or it may be important to them that JZ continues to develop relationships with the community of friends and family in his hometown. It is also possible that his parents are considering interests outside of those Malek2  describes. They may even be considering how a move would affect other aspects of their lives that would indirectly promote or counteract his interests, such as the family’s financial stability if they lose their employment by moving.

Malek2  concludes, and I agree, that promoting one sole interest at the expense of others is not a reasonable way to promote a child’s best interest. Whatever the BIS demands, it should, at minimum, include an attempt to consider all of a child’s interests, especially when furthering one particular interest might interfere with serving other important interests. In this case, a tracheostomy would offer a better chance at a longer life, but it might come at the expense of JZ’s ability to play, learn, and develop relationships within his community. I would argue that it is reasonable for JZ’s parents to prioritize other interests ahead of his chance for a longer life.

JZ’s parents may or may not be right in their belief that it is better for JZ to live a shorter life in their rural community. But even if the medical team does not agree with their decision, this does not give them the right to overrule it. The American Academy of Pediatrics sets the threshold for state intervention as “parents who deny their children medical care [that is] likely to prevent death or substantial harm or suffering.”3  To determine if a choice crosses that threshold, we must first determine how likely a substantial harm might be, what constitutes substantial suffering, and how to quantify each.

Diekema4  proposed that, rather than using the BIS, we should instead base decisions about state intervention on what he calls “the harm principle.” By this view, parents are not obligated to do what is best for the child. They are only obligated not to do anything that is harmful. Eight conditions must be met to justify state intervention based on the harm principle.4  First, the child must be at significant risk of serious harm. Second, the harm must be imminent. Third, the medical intervention must be necessary to prevent the harm and, fourth, be proven efficacious. Fifth, the projected benefits of the intervention must outweigh the risks and burdens. Sixth, options that are less intrusive to parental autonomy should be considered. Seventh, it must be reasonable to generalize state intervention in all similar situations. And finally, most people should agree that the state intervention is reasonable.

One can argue that a 10% to 20% risk of death is a significant risk of serious harm. However, the harm is not imminent; JZ’s risk of death is only when he has a respiratory infection. A tracheostomy is an effective intervention, and there are no less intrusive alternatives. However, JZ’s parents feel that the benefit of a tracheostomy does not outweigh the risk of the intervention or the burden of caring for it. Finally, although JZ does have an increased risk of death without a tracheostomy, his overall risk of death due to supraglottic stenosis is only estimated to be between 10% and 20%. Would it be reasonable to overrule every parent who refuses an intervention that will decrease their child’s risk of death from 10% or 20% to 5%? I am not convinced that this is a good precedent to set because I do not think that most people would agree to generalize state intervention to all situations like this.

JZ’s parents have provided him with good medical care, particularly in light of the limited resources available to them. Because they will be the ones who would bear the burden of moving to a foreign environment and raising their child outside of their supportive community, they have the right, as his parents, to define his best interests on the basis of their values and priorities. If they choose to define his best interest broadly and include consideration of his mental, emotional, and social development in their decision, that is their prerogative. Furthermore, their assessment that remaining in their rural community outweighs the benefit of a potentially longer life in a foreign environment may be a reasonable assessment of what is best for JZ when accounting for all of his interests. Finally, regarding the question of medical neglect, their decision to refuse an elective tracheostomy does not reach the threshold of harm as defined by Diekema4  and, therefore, does not warrant state intervention.

JZ and his parents face extensive medical challenges, which are magnified by living in a rural health care setting. Rural settings tend to have fewer providers and other health care professionals compared with urban areas. But there are advantages to living in rural areas, too. People in rural areas tend to be more interconnected; they help each other out, making communities self-reliant. People living in rural areas have shared experiences and values with regular interactions at work, in the community, and in houses of worship, thus providing support for one another in ways not often seen in urban settings.5,6  Just as many urban inhabitants could not see themselves living in small rural settings, rural residents find it difficult to leave the deeply shared values and support found in their community.7  JZ’s parents believe that these strengths are paramount in providing him a rich but possibly shorter life if they remain in a rural setting.

Twenty percent of the US population lives in rural America, including >12 million children. The health care disparities experienced by rural pediatric patients include increased rates of obesity and death from unintentional injury.8  Children with special health care needs experience additional disparities. They often live hours from the nearest tertiary-care center or subspecialty care. Any visit to a subspecialist requires significant travel accompanied by the loss of work, wages, and school instruction. Specialized community- or home-based medical care teams, including emergency medical staff or home nursing, may be limited or nonexistent in rural communities.

JZ has a complex disease, RDEB, that requires hours of daily care for skin protection and alleviation of pain. Children with RDEB may have additional needs for feeding and respiratory support.9  The placement of a tracheostomy places additional burdens on the family. The current standard for home placement with a tracheostomy requires an awake adult present at all times as well as 2 family members who are trained in all facets of tracheostomy care.10  In their rural setting, with limited home health care resources, this burden will fall on the parents. This could limit their ability to have a stable income or participate in the care of other children.11 

On the basis of consideration of all these factors, the parents of JZ have made the difficult decision to refuse elective placement of tracheostomy, which would lessen (but not eliminate) his risk of death from acute respiratory failure. The heath care team disagrees.

The ethics question focuses whether the clinicians should accept the family’s decision of nonintervention. Addressing the ethics question requires us to reflect on the BIS for JZ. This case highlights the challenges of using a traditional BIS, which focuses on the medical needs of the child in isolation from a more holistic view of the child as an integral part of the family.

Multiple models have been proposed to attempt to balance the interest of a child with the interest of the family. The direct interest model put forth by Groll12  would recognize and hold valuable the interests of other family members and indeed the family itself. Additionally, it allows parents to consider their own subjugation of interests as it relates to the care of their child.

As a society, we give wide latitude for parents to make health care decisions for their children as long as no undue burden or significant harm exists.4  For JZ, his enjoyment of the outdoors and his parents’ strong network of family and friends in the rural community must be respected by the health care team as compelling interests that are integral to his overall wellbeing. So, in the case of JZ, if moving to an urban area removes all the community support that allows JZ and his parents to thrive, then it is justifiable to allow the parents to choose a path of nonintervention, although that path has a slightly higher rate of morbidity. We base this decision on our assessment of that. The potential benefits to JZ of the elective tracheostomy cannot be viewed in isolation but must be balanced with the quality of the life that impacts JZ and his family.

The case of JZ in many ways exemplifies the moral tension many clinicians experience between what appears to be in the best medical interest of a vulnerable child and the preferences of that child’s parents. Here, that tension pulls between the physicians’ recommendation of an elective tracheostomy to establish a safer airway for JZ and his parents’ declination of this intervention, citing caregiving concerns for tracheostomy maintenance. Before we proceed with an analysis of whether the parents’ rejection of the recommended tracheostomy constitutes sufficient reason to invoke state intervention, it is important to consider what state intervention would actually look like in JZ’s case and what impact that intervention could have on the child and family. In this case, there is no clear pattern of neglect or abuse. JZ’s parents have done a good job of providing the extensive care his condition requires. This says a lot about their desire for his wellbeing and their ability to follow through on that desire. Thus, the state intervention would likely involve a court order compelling surgical placement of the tracheostomy but avoid the more intrusive intervention of removing JZ from his parents’ care. However, if the parents’ primary concern is the caregiving burden the tracheostomy would require of them, we should consider the possibility that this additional burden will either cause the parents to voluntarily enter JZ into medical foster care or perhaps even result in such poor caregiving that JZ could be removed from their care involuntarily.

However, thus far, it seems if we look only at the isolated physical interests of the child, there is good evidence that the tracheostomy would be the “best” decision: a well-maintained tracheostomy would provide JZ a safer airway, decreasing the risk of death from 10% or 20% to 5%. However, within an intimate family, as JZ is, a child’s interests exist in a deeply interconnected and interdependent web of a multitude of other interests, and medical decision-making must acknowledge, if not embrace, that.13  JZ has a clear physical interest in maintaining a safe airway. He also probably has relational and emotional interests in maintaining a close caregiving relationship with his parents (which a tracheostomy might threaten). Others’ interests are at play here as well. JZ’s parents are citing the additional time and effort required by a tracheostomy as their reason for rejecting it. Perhaps they already feel their ability to care well for JZ’s siblings or other members of the family is being compromised by the time and energy burdens required for JZ’s care. Or perhaps they feel their own interests or the interests of their marriage would be threatened by the additional caregiving burden. Any of these explanations would be reasonable and also could (and likely do) coexist alongside a deep and committed love for their son and his wellbeing.

These realities relate to the secondary clinician concern that the family’s rural home is too removed from the medical resources of a city. Although well intended, I am sure, the dermatologist’s recommendation that the family move to the city seems tone deaf to the realities and complexities of family decision-making, as if one type of benefit to one child would necessarily compel the uprooting of an entire family from their home, their jobs, their community, their schools, their friends, and potentially much more. Family decision-making is necessarily full of interfamilial tradeoffs and compromises. Each family is different. Parents make decisions all the time to compromise one family member’s interests to serve the interests of others (eg, changing the diet of the whole family to accommodate a food sensitivity, leaving the children in the care of a sitter for a week so the parents can go on an adults-only getaway, etc). It is unlikely that the parents have not considered the possibility that JZ would have access to more resources in the city; it is much more likely that they have considered this possibility and still have decided that, on the whole, the family (including but not limited to JZ) is better off in their rural community. Medical decisions are no different than all the other complex decisions families must make, and to insist that they be so would impose artificial, and at times harmful, conditions on the natural decision-making process of an intimate family.

However, the case description has not provided much clarity on the underlying values motivating the parents’ rejection of the tracheostomy. So, my first recommendation would be to have deeper and more targeted conversations about their reasons for refusal. What interests are on the parents’ minds as they make this decision? Perhaps their reasons are informed by a misunderstanding of what tracheostomy care actually looks like or requires: Is it as burdensome as they expect? Might it actually decrease their stress and worry and thereby improve their ability to care well for themselves and their family?

However, if conversation with the parents fails to produce any movement toward an agreement, the parents’ decision to refuse the tracheostomy must ultimately be respected. Although the decrease in chance of death by 5 to 15 percentage points does represent a clear medical benefit, it does not represent such a dramatic benefit as to outweigh the possible harms to the child and family. The decision in JZ’s case, therefore, lives in the zone of parental discretion,14  where we as clinicians and ethicists can say, “I might make a different decision for my own child, but as a parent, I should have the right to make the decision.” These parents deserve that right, too.

Ethical dilemmas occur when all the choices are bad. In this case, there are 2 options that are each bad but for different reasons. It is bad to send a child home with a 20% chance of dying when a treatment could lower that risk by 75%. Ordinarily, we would consider such a treatment obligatory. But, in this case, the treatment imposes significant burdens on the patient and his family. The treatment does not involve the parents merely giving their child medication or consenting for a curative operation. They would have to dramatically change their lives, either by the intensity of the monitoring that they would have to provide or by uprooting their family and moving to a new town. Not undertaking the treatment is likely to have serious consequences as well. The child will likely have continued (although intermittent) exacerbations of his respiratory distress. The balance of harms and benefits might shift if the child had an isolated supraglottic stenosis, but he also has a separate and serious complex chronic disease. His parents have, apparently, been providing excellent care for that disease. This is a case in which the real question is not about the child’s best interest. Instead, it is a question about the appropriate limits of the sacrifices that we can reasonably expect parents to make. These parents have already made more sacrifices for their child than most do. That gives them enhanced authority to make the difficult choice that they now face. And the medical team should respect their decision.

Dr deSante-Bertkau conceptualized and designed the article and drafted the initial manuscript; Drs Shubkin, Nelson, Salter, and Lantos drafted the initial manuscript; and all authors critically reviewed and revised the manuscript, approved the final manuscript as submitted, and agree to be accountable for all aspects of the work.

FUNDING: No external funding.

BIS

best interest standard

RDEB

recessive dystrophic epidermolysis bullosa

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Competing Interests

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.