Ethical and Legal Considerations Related to Disorders of Consciousness

Abstract

Purpose of Review:

The purpose of this review is to describe ethical and legal issues that arise in the management of patients with disorders of consciousness ranging from the minimally conscious state to the coma state, as well as brain death.

Recent Findings:

The recent literature highlights dilemmas created by diagnostic and prognostic uncertainties in patients with disorders of consciousness. The discussion also reveals the challenges experienced by the disability community which includes individuals with severe brain injury who are classified as having a disorder of consciousness. We review current guidelines for management of patients with disorders of consciousness including discussions around diagnosis, prognosis, consideration of neuropalliation, and decisions around life sustaining medical treatment.

Summary:

In the setting of uncertainty, this review describes the utility of applying a disability rights perspective and shared decision-making process to approach medical decision making for patients with disorders of consciousness. We outline approaches to identifying surrogate decision makers, standards for decision making and decision-making processes, specifically addressing the concept of futility as a less useful framework for making decisions. We also highlight special considerations for research, innovative and controversial care, brain death, organ donation, and child abuse and neglect.

INTRODUCTION

Disorders of consciousness (DoC) comprise a spectrum of states of arousal and awareness less than full consciousness, including the locked-in state. [1,2] These include the minimally conscious state (MCS), vegetative state (VS), and coma; while strictly not a DoC, we include a brief discussion of brain death (BD) [3–8, •• 7]. DoC have important implications for at least two reasons. First, different states relate to the individual’s ability to interact with the environment, express preferences, and ultimately, may impact whether the individual has claims to certain moral goods. Second, these states may impact how individuals or surrogates weigh the utility of those goods. This article reviews recent literature relevant to DoC, including diagnostic and prognostic uncertainties and the numerous ethical and legal issues that arise in decision-making amidst uncertainty [•• 9,10]. We provide the perspective of a disability rights framework. Finally, we discuss special considerations in research, innovative and controversial care, brain death, organ donation, and child abuse and neglect.

UNCERTAINTIES RELATED TO DoC

Diagnostic uncertainties in DoC include disagreements over the appropriate contributions of clinical and other modalities in defining the patient’s state [•• 9]. Although recommendations to utilize validated and standardized neurobehavioral assessments increase consistency and minimize diagnostic uncertainty, there remain equivocal states of awareness [•• 4,11]. Both overappreciation and underappreciation of brain function lead to value-laden management considerations. Overappreciation does not necessarily represent meaningful capacity for interaction with the environment [12,13]. Underappreciation can lead to misdiagnosing MCS as VS, increasing likelihood of withdrawal of life-sustaining medical therapies (LSMT) [14–17], where more accurate diagnostics could lead to improvements in functional outcomes [18,19].

Prognostication in patients with DoC also remains ethically challenging for clinicians. Although prognostic information can help facilitate decision-making, guidelines for managing DoC discourage clinicians from offering universal statements of poor prognosis early following injury, with newer evidence suggesting that up to 20% of patients with DoC have improved functional outcomes at 6–12 months post-injury [•• 4,18,19]. Additionally, while recommendations encourage use of serial standardized behavioral evaluations, the tools prove inconsistent, within and between patients [•• 9, •• 20]. Inability to accurately and reliably prognosticate has important implications for decision-making.

Limitations in accurately predicting one’s capacity for pain and suffering challenge attempts to provide adequate pain management, even with the use of validated pain scales [11,21,22]. Potential benefits of drugs for dyskinesia, aggression and increased functional brain activation must be balanced against potentially disruptive side effects [23–25]. Finally, while it is critical to avoid premature termination of LSMT and to optimize outcomes for individuals who may benefit from rehabilitation, it is difficult to identify which patients will benefit and can create unrealistic expectations for those who may not [•• 4, 18,19, •• 26].

MAKING ETHICAL DECISIONS FOR PATIENTS WITH DoC

Surrogate Decision Makers:

In the setting of uncertainty, the principal ethical consideration for patients with a DoC is how their pre-morbid moral preferences will be recognized, generally through surrogate decision-makers. For pediatric patients, parents are default surrogate decision-makers. When parents are unavailable, there is not consistent guidance on the best alternative surrogate decision-makers [27]. For patients above the age of majority, decision-makers may be identified based on preferences if the individual has capacity, which is unlikely, or appointed based on state surrogate decision-making laws [28]. Caregivers may also apply for guardianship of adults, which grants varying degrees of authority based on type, and may or may not include authority to terminate LSMT [29–32].

Standards for Decision Making:

In pediatrics generally, decisions are expected to reflect patient best interests, respecting ethical principles of beneficence (maximizing benefit) and non-maleficence (minimizing harm). As patients reach the age of majority, their developing autonomy should lead to incorporation of their values and preferences [33]. Once patients reach majority, if they have capacity, they make decisions autonomously. If capacity is lost, decisions should be made based on advanced directives or otherwise expressed prior preferences, using a substituted judgment standard, attempting to as closely reflect what the individual would want if they had capacity.

In patients with DoC, capacity for decision-making is typically absent. For children and adults with a DoC who never gained capacity, decisions should be grounded in best interests. Parents or caregivers are usually presumed to be best situated to identify a child’s best interests, although the recent case of Charlie Gard reignited discussion about the limits of the best interests standard, and who should have the authority to determine best interests [34]. Gard was an infant in the United Kingdom with progressive neurocognitive decline that led to his inability to interact with his environment. Despite parental wishes to pursue experimental therapy, the court determined such treatment was not in his best interests and that he should be allowed to die. In response to this case, debates focused on whether best interests can be objectively defined, how to determine best interests, the potential for parental interests to be masked as child interests, the need for a range of parental discretion in making decisions, and the potential overreach of the state in overriding parental authority [34–40]. Some critics of the best interests approach propose an alternative harm threshold for overriding parental decisions, where parents are granted wide decision-making latitude and state intervention is only justified when decisions reach a threshold level of harm [31, 36]. Others appeal to the perception of “relational potential” between parents and children to argue that parents should be permitted to make decisions about LSMT provided that suffering is minimized, even when these run counter to assessments about risks and benefits made by those outside of the relationship that the family is interested in preserving [42].

Decision-making and Disability:

A unique aspect of decision-making for patients with DoC is the overlay of perceptions about disability in the medical and social contexts. Much of the discussion of decision-making for this population focuses on decisions about withholding or withdrawing LSMT. However, surrogate decision-makers for individuals with DoC may choose to pursue LSMT. These decisions can generate moral distress among clinical teams whose perspectives may differ. Understanding decision-making within a disability framework may help to alleviate this tension.

It is impossible to define a singular disability perspective for a heterogenous population; however, important themes within a disability framework emerge as relevant to ethical decision-making for individuals with DoC. Healthcare providers may place less value on the quality of life of individuals with disability and these views often diverge from those living with disability [43]. Additionally, perspectives about quality of life may evolve for those living with disability. As well, those living with disability can experience exclusion and inability to function at full capacity due to misperceptions about awareness or ability. While those living with disability have diverse perspectives and viewpoints, they often describe a shared experience of exclusion, isolation, being devalued, and dismissed in dominant physical, social, emotional and cultural contexts. Ethical decision-making for individuals living with DoC requires attention to promoting inclusivity and optimizing rehabilitation to promote capabilities.

A number of legal protections address these concerns. Individuals with disabilities have rights to due process and equal protection under the Constitution, although constitutional claims can be difficult to enforce. The Americans with Disabilities Act prohibits discrimination in the setting of disability and requires accommodation to promote inclusivity. The Affordable Care Act prohibits disability-based denial of coverage and denial based on pre-existing conditions, providing access to individuals with DoC. Of particular importance for children, the Individuals with Disabilities Education Act provides services to facilitate a “free and appropriate” public education in the least restrictive environment possible [44]. When managing patients with DoC, clinicians should be mindful to counsel families regarding the long-term potential for a degree of functional recovery and should be aware of issues within the disability community that may impact short- and long-term decision making.

Futility;

The concept of futility justified withdrawal of LSMT in landmark DoC cases including Karen Quinlan, Nancy Cruzan, and Terri Schiavo, but the concept of futility is now disfavored because it is difficult to define [14–16,45]. Where LSMT is withdrawn based on permanence and futility, the patient’s death is essentially self-fulfilling, as it cannot be known whether the patient may have improved [•• 20]. This challenge highlights the difficulty with terminology, as something is futile for a permanent/persistent state only in conjunction with failure to meet a goal and the potential goal of rehabilitation is not practically possible when LSMT is withdrawn. Rather than discussing decisions in terms of futility, language of shared decision-making may be more appropriate.

Shared Decision-Making:

Shared decision-making (SDM) offers a framework for decision-making for patients with DoC despite uncertainties and variable risk/benefit considerations. [6, •• 9,46–48, •• 47]. SDM balances physician paternalism with patient/surrogate autonomy and parental authority. The process is highly preference sensitive, incorporating goals and values of decision- makers; lack of standardization, however, can make SDM challenging [• 38]. Active listening to elicit goals and values is key to SDM. Some have recommended that clinical teams first evaluate the risks, benefits, and feasibility of medical options, prior to offering options to decision-makers; others criticize this approach as vulnerable to clinician biases [49–51]. Clinicians must consider and mitigate their biases as they often help families navigate unsatisfactory options and historically express preferences that death is superior to living with a DoC [52,53]. Clinicians should also have open communication about uncertainties [•• 54,55]. Recognizing that patient and family goals may evolve, re-evaluation of expectations and goals of care for surrogates, early introduction of palliative care, and promoting longitudinal relationships may prove useful [33, •• 54]. In some circumstances, increased physician directiveness may be helpful, using “mutually supportive” or “collaborative” decision-making [33, •• 54, •• 56].

Offering and Terminating LSMT:

Decisions to offer, withdraw or withhold LSMT commonly evoke ethical considerations, as it is difficult to identify whether LSMT further individual best interests. Some have argued that there is not an obligation to offer all LSMT, even if desired by surrogates [57]. When LSMT are offered, surrogates may refuse them as not aligned with patient best interests [14–16,45]. Although, legally and ethically, there is no distinction between withdrawing and withholding LSMT, decisions to withdraw can be perceived differently than withholding, and this should be explored with families in considering initiation of LSMT. Time-limited trials of LSMT with re-evaluation of potential for recovery may also be helpful in some cases.

SPECIAL CONSIDERATIONS

Research, Innovative and Controversial Therapies:

Several areas raise particular concern in surrogate decision-making for patients with DoC. First, while there is a need for high quality research in DoC, many studies focus on diagnostics but do not evaluate meaningful outcomes [3, •• 9,12, •• 20,58,59]. Determining whether it is in a patient’s best interests to participate in research raises a challenge, particularly because individuals with DoC are often unable to assent. Moreover, once patients reach the age of majority, state laws may not permit guardians to make decisions about research participation [• 44]. These challenges are exacerbated with newer therapies that are unproven but for which parents may be motivated to try, such as the parents of Charlie Gard. There is debate over whether experimental therapies are ethical to try in end-of-life situations, as well as whether individuals should have access to such therapies outside of traditional expanded-access processes [60,61]. Additionally, advances in neuroscience technology have led to the use of functional imaging and brain computer interfaces to potentially ascertain preferences from individuals in vegetative states with regards to their healthcare [62–64]. While these advances may have the potential for meaningful contributions, they should be considered innovative, requiring careful scrutiny before relying upon them. Finally, decisions about controversial therapies, such as growth attenuation for children with DoC, challenge traditional decision-making frameworks. Parents should be offered guidance in navigating such situations and ethics consultation should be considered [65,66].

Brain Death:

Brain death is described as a state where there is apneic irreversible unconsciousness [• 67]. The Uniform Determination of Death Act establishes legal criteria for defining death, requiring, “irreversible cessation of all functions of the entire brain, including the brain stem,” [68]. Since its establishment, legal and ethical challenges to brain death have emerged [69]. Key among them are discrepancies between the legal definition of death and the medical standards used to determine brain death, which highlight that brain death raises both biological and social considerations and has led to differences in whether one may be considered brain dead in different states [70–• 72]. Clinical conflicts emerge between care teams and families when the concept of brain death is challenged, which may be accompanied by requests to transfer patients for accommodation of their beliefs [73,74]. Caring for patients in such challenging cases creates questions about the obligations of clinicians to provide critical care interventions that they may believe not to be in the patient’s interests. Legal cases challenge whether the clinical determination of death meet the “acceptable medical standards” requirement under the UDDA, whether loss of neuroendocrine function is required under “all functions of the entire brain,” whether consent is necessary to perform the exam, and whether individuals should be permitted to refuse the determination of death, as allowed under some state laws that provide accommodation for such beliefs [• 67,71, • 72,75–77]. Some have called to revise the UDDA in response to such challenges; others challenge whether brain death should continue to exist [• 72,78–80, • 79].

Organ Donation:

Many decisions for patients with DoC invoke considerations of end-of-life, raising the possibility for additional ethical concerns related to ensuring that individuals are not exploited for the purposes of organ donation. The case of Jahi McMath raised concerns about how implicit or explicit biases could impact communication and trust in declaring brain death [•• 81]. These challenges exist in the setting of already charged questions related to organ donation following cardiac death (DCD) in patients for whom LSMT are withdrawn and for patients who may be organ donors based on brain death. For DCD donors, a key question includes whether circulation could be restored, making organ donation unethical [82,83]. For potential organ donors based on brain death, some questions include whether brain dead patients are actually dead, and whether organ preservation processes are justified [84,85].

Child Abuse and Neglect:

Abusive head trauma can lead to a DoC. Decision-making for patients with abusive head trauma is complicated by the fact that parents, the default decision makers, may be involved in causing or failing to prevent injury. Clinicians may question parents’ ability to act within the child’s best interests [86]. The situation raises the potential for conflict of interest in decision-making as decisions to withdraw LSMT may lead to criminal consequences, although at least one state has questioned criminal charges on these grounds [87,88]. Clinical teams may struggle to include parents in decision-making, which risks loss of input about important values and goals [33]. Additionally, parental exclusion may lead to decision-making influenced by clinical team biases, which can disproportionately impact African American and Latinx populations from the point of referral to child welfare through resolution of cases [89]. Incorporation of a guardian ad litem to advocate for the child may assist teams in representing the child’s interests while permitting parental involvement to the extent possible [86,90].

CONCLUSION

Uncertainties related to diagnosis and prognosis in patients with DoC generate a number of legal and ethical considerations for decision-making. The distinction between different states of consciousness invokes differential considerations for claims to moral goods. Disability ethics and shared decision making provide important frameworks for decision-making. Pediatric clinicians should be aware of special considerations related to research, innovative therapies, brain death, organ donation and child abuse.

KEY POINTS:

1. Uncertainty in diagnosis and prognosis in disorders of consciousness complicates understanding of how to weigh claims to moral goods by individuals in various states of consciousness.

2. Utilizing shared decision-making frameworks that elicit patient and family values and reflect upon clinical team biases can facilitate discussions about medical decision-making for individuals with disorders of consciousness.

3. Understanding the disability rights perspective may help to explain different approaches to decision-making for patients with disorders of consciousness.