The Ethics of Disclosing and Discussing SUDEP with Families of Children Newly Diagnosed with Epilepsy

Clinical Scenario:

Introduction

Analysis

Epilepsy is defined as the predisposition to have unprovoked seizures and occurs in approximately 1% of children. Epilepsy is considered a heterogeneous condition, with many causes, outcomes, and co- morbidities, and is often now referred to as the epilepsies (England et al., 2013). There is a wide range of developmental levels, from entirely typically functioning individuals to those with disabilities, which are predominantly related to the underlying cause of the epilepsy. Mortality is increased in persons with epilepsy, and SUDEP is the most common cause of premature mortality in this patient population.

SUDEP describes the syndrome of death in a person with epilepsy where the cause is unknown, unlike death due to trauma, drowning, and status epilepticus. The pathophysiology appears multifactorial, with potential cardiac and respiratory components and is often associated with the underlying disorder. For example, certain genetic epilepsies, e.g. sodium channelopathy in Dravet syndrome (severe myoclonic epilepsy of infancy), have particularly high rates of SUDEP. The risk of SUDEP in children with epilepsy is approximately 1 in 4500 per year, and in adults 1 in 1000 (Harden et al., 2017). These statistics are on the basis of large scale populations and do not necessarily apply to individual patients that fall into specific subgroups of epilepsy, from those essentially unaffected by SUDEP, e.g. typical childhood absence epilepsy, to the channelopathies, epileptic encephalopathies, or presence of escalating nocturnal convulsive seizures that are associated with very high risk. In observed cases, the terminal event is typically preceded by a seizure (Sweinsson et al., 2018). Other common circumstances are nocturnal timing with the patient found in the prone position and in unsupervised settings.

While a dispassionate discussion of ethical principles may be applied to this situation, the reality of this is ethically and emotionally charged in real practice. A survey of American and Canadian adult and child neurologists published in 2014 revealed that neurologists felt that patients and families responded to a conversation about SUDEP with anxiety or distress over 60% of the time (Friedman et al., 2014). Appreciation and relief were perceived far less often. Thus, neurologists have shared the impression that disclosing and discussing SUDEP can produce negative consequences in patients and families. This has been our experience as well.

The question arises whether it is possible for a patient or family to provide an informed decision concerning whether or not they would like to hear about SUDEP. This seems different as an a priori decision, in comparison to families reporting post facto that they would have preferred to know in advance about this possibility, although an ability to potentially prevent this problem would supersede concerns about disclosure. The objective of obtaining informed consent cannot be completed because theneurologist would first have to explain or discuss theconcept in order to achieve this. Some neurologists may attempt to circumvent the details of the topic and ask patients something along the lines of, “Would you like to discuss rare but severe potential complications associated with epilepsy?” However, a lack of details in asking the family questions would limit the family’s ability to provide truly informed consent.

Navigating the Conundrum

It appears there are at least three approaches to this situation:

1. Withhold discussion of SUDEP for the newly diagnosed patient (and, by implication, reserve the discussion for when it may become applicable based on the appearance of risk factors, e.g. escalating seizure frequency, presence of nocturnal convulsive seizures, diagnosis of high risk condition, etc.);
2. Selective disclosure of SUDEP in the newly diagnosed patient;
3. Disclosure of SUDEP to all newly diagnosed patients.

Each of these scenarios may have merit and has been applied by neurologists in our experience. Each presents its own challenges. There is the additional concern about whether the child should be informed, or whether to discuss this with the parents/guardians and allow them to decide how and when to include the child in this discussion. It is not uncommon for families to report grave concerns about the child hearing about various aspects of epilepsy care, from the possibility of having surgery to the phenomenon of SUDEP.

Approach 1: Withhold discussion of SUDEP for the newly diagnosed patient

Barriers that have reportedly prevented SUDEP disclosure are the notions that certain patients are not at much greater risk than the general population, there is no proven way to prevent it, and furthermore that knowledge of this could be detrimental to the patient’s mood or quality of life (Friedman et al., 2014). Other potential reasons are the rarity of the event, so that the risks of discussion outweigh the potential benefits, in addition to lack of time or resources for a proper discussion, and lack of an opportunity to form a trusting relationship at the point of the clinical interface in the newly diagnosed patient.

Some of these concerns are overlapping. Are the risks of awareness outweighed by the potential benefit of knowing the information in the patient at very low or essentially no increased risk? Logistical issues are challenging, despite their seeming banality. Neurologists are faced with greater patients to see, more patients in less time, and there may be pressing issues of more relevance to that particular patient, e.g. safety rules for children living with epilepsy, rescue therapy indications and procedures, compliance with medication and follow-up, diagnostic testing, etc. A lack of evidence, to date, that SUDEP is actually preventable leads to the question of whether the patient or family benefit from knowing about it. 

Approach 2: Selective Discussion of SUDEP

In the case of a newly diagnosed patient, it is rarely possible to have identified whether that patient is at a high-risk category, much less have had the opportunity to develop a trusting relationship with the neurologist. On the other hand, a highly mobile population as well as rapid and sometimes unanticipated changes in insurance plans lead to the realization that there may be only a few opportunities, much less a single one, for the neurologist to interact with the patient and family. Neurologists have expressed the decision to invoke SUDEP in the case of a patient showing poor medication adherence in an effort to provide a rationale if not motivation to improve compliance. 

In this scenario, the initial potential psychological harm of creating fear and sense of loss of control would potentially be superseded by the potential long-term benefit of improved seizure control, and hence decreased risk of SUDEP. Here each patient is analyzed on a case-by-case basis with the ultimate decision based on a combination of patient and parent factors and the clinical course of the patient, where there is a perception of more benefit than harm from the conversation. Yet, this invites the paternalism of the neurologist in gauging the response of the particular patient or family. Alternatively, in the case of newly diagnosed childhood absence epilepsy, with the presence of only absence/petit mal seizures and without convulsive events, it would be irrelevant to raise the prospect of SUDEP. It is more likely that positive change would be effected by devoting time discussing seat belt use, pedestrian safety, avoiding smoking, or even healthy dietary habits than discussing SUDEP. A counterargument could be made, however, that children with absence epilepsy are at risk for development of convulsive seizures, in which case there is at least some elevated risk of SUDEP. Then there is the problem of whether to disclose SUDEP in the patients with the benign focal epilepsy syndromes of childhood, such as benign Rolandic epilepsy. In such cases, the risk of SUDEP is considered infinitesimally small, yet there have been reported cases in children with these diagnoses (Doumlele et al., 2017). Thus, it appears to be uncertain whether any epilepsy patients are actually immune from SUDEP. 

Approach 3: Disclosure of SUDEP to All Newly Diagnosed Patients

SUDEP disclosure is recommended as a practice guideline for patients with epilepsy (Harden et al., 2017). The issue at hand is disclosure in the newly diagnosed patient and family, who are grappling with a new diagnosis and acceptance of a condition that is generally treated with daily medication on a long-term basis and a sense of uncertainty given the inherent unpredictability of the course of epilepsy. Patient survey data indicate that parents of affected children, both high and low risk, prefer to have had the discussion despite an increase in initial anxiety (Ramachandrannair et al., 2013). It is possible that neurologists are projecting their own opinions when deciding that this disclosure creates excessive harm. A qualitative study of young adults with epilepsy reported that > 80% supported uniform disclosure (Tonberg et al., 2015), and that this may lead to behavioral changes including improved medication adherence. 

In the case of the new onset pediatric epilepsy patient, the patient lacks the ethical maturity and legal status to make decisions independently and must yield to his or her parents or guardians as surrogate decision-makers. The physician is well served by the traits of humility, honesty, and compassion to balance and contrast the autonomy of the patient and parents versus a paternalistic assessment of beneficence and nonmaleficence. While it may appear overly paternalistic to enact a unilateral decision to avoid SUDEP disclosure to “protect” families, there are many items that are not disclosed to patients during the course of a clinic visit, especially a first time visit. It is logistically impossible to cover every possible diagnostic, therapeutic, and outcome scenario in every clinical setting. This includes rare events in particular, e.g. esoteric, rarely reported reactions to medications that are being prescribed. It is conceivable that clinicians are concerned about such current matters as the now ubiquitous patient ratings, with the proliferation of patient satisfaction surveys both by the Internet and hospitals. It certainly seems plausible that such rankings may suffer by raising such an emotionally laden and possibly unexpected topic.

There should be a certain threshold level of risk to warrant such discussion in a clinical setting. Patient or family autonomy is difficult to exercise in this setting because the neurologist must make the decision about SUDEP disclosure and discussion. The principle of nonmaleficence suggests refraining from disclosure due to the discomfort, anxiety, or fear it could produce. Yet, there is the contrasting matter of whether there are indeed actionable items that patients and families could undertake to mitigate their own level of risk. While not proven, there is a rational basis to believe that patients at high risk can mitigate this by avoiding when possible a prone sleep position, use a lattice pillow that allows rebreathing through expandable material, or wear a seizure detection device (Liebenthal et al., 2014). Furthermore, it seems plausible that achieving improved seizure control, whether with increased medication adherence or even earlier use of epilepsy surgery when applicable, could reduce the risk of SUDEP and serve as a rationale for early SUDEP disclosure.

Preservation of patient/family autonomy presents unique challenges in this situation. Families cannot be easily given sufficient information to decide without essentially providing the disclosure information of this highly charged topic. Yet, it is considered preferable for families to learn of this information from their physician as compared to online browsing. Analysis using focus groups and detailed interviews to explore parental views led to the parents’ preference for disclosure by pediatric neurologists as a face to face exposure, mostly at the time of epilepsy diagnosis, and with the parent deciding whether the child is present (Ramachandrannair et al., 2013). These factors were cited despite the emotions reported of feeling overwhelmed and anxious following the discussion. Whether this Canadian study is generalizable warrants further study, as there may be other factors depending upon patient heterogeneity, including region, urban vs. rural location, parental education and background, socioeconomic status, family history of epilepsy, type of epilepsy, and patient’s risk categorization of SUDEP.

The Argument to Withhold Discussion of SUDEP

Logistical and emotional barriers exist that prevent a productive discussion of SUDEP. In a survey of American and Canadian adult and child neurologists, respondents mentioned several barriers, including the patient being at low risk, no proven way to prevent SUDEP, concern that it would negatively affect patients, not yet established a trusting relationship with the patient, in sufficient time in clinic, and lack of adequate, high-yield information (Friedman et al., 2014). 

The inability to prevent SUDEP, especially in the new onset patient with minimal if any risk of the occurrence, leaves neurologists wondering why or how the patient or family may benefit from knowing about it. An additional counter-incentive is that the discussion may not be feasible given the strict amounts of time allowed for visits in some clinics. There is also a knowledge and resource gap. Survey data demonstrates that neurologists who treat more patients with epilepsy per year are more likely to discuss SUDEP compared to neurologists who treated fewer epilepsy patients (Friedman et al., 2014). Actual realization of more consistent SUDEP disclosure will likely require a culture shift that ensures adequate knowledge of SUDEP and its risk factors among neurologists as well as clinical management support to facilitate and encourage such discussions. This should arguably include research demonstrating that truly no harm, or the most minimal amount, is committed in the process of the SUDEP discussion.

The Argument to Discuss SUDEP Selectively

It would seem reasonable to initiate the SUDEP discussion in patients showing several risk factors for SUDEP, in contrast to the clinical scenario provided at the outset of this discussion. An example of the benefit of the discussion would be the circumstance of escalating seizures associated with poor medication compliance. Thus, disclosure would depend on assessment of risk factors as well as the level of family, and potentially patient, ability to cope with the information. Research is needed to create a list of variables to identify patients and families when exercising a selective approach to the SUDEP discussion.

The Argument to Discuss SUDEP with All Patients and Families with Epilepsy

In the tragic instances of families having gone through a SUDEP experience, the argument has been compellingly made that it is far better to have understood this is a possibility as compared to learning about it in the aftermath of a devastating event (Stevenson and Stanton, 2014; Gayatri et al., 2010). The issue arises whether this applies a priori to the new onset patient and family. Yet, neurologists may be exhibiting overly paternalistic bias when deciding to withhold the discussion for fear of malfeasance, and thus the argument exists to discuss this universally at the time of diagnosis. In that case, it becomes the province of the clinician to gauge the risk of that particular patient and couch the discussion accordingly. 

Overall, universal discussion of SUDEP may be beneficial because some evidence suggests that families and young adult patients want to hear about it (even if they do initially feel stress or apprehension), may ”discover” it on the Internet (or elsewhere), and deserve a full understanding of the disease process. Some potentially modifiable risk factors for SUDEP may be important for patients and families to know to help reduce mortality (medication compliance, prone sleeping position), especially once the rationale is understood.

Reconciling the Different Arguments

Differing viewpoints have been published regarding the universal disclosure of SUDEP in neurological practice (Brodie and Holmes, 2008). At least in Australian law, neurologists are not found negligent for avoiding a discussion of SUDEP (Beran, 2014). Yet, the ethical dilemma is in a state of flux, and new information is emerging about risk factors, potential interventions, and family reactions. It is plausible that the discussion could lead to more harm than benefit, but it is also recognized that patients have ready access to information on SUDEP, and discovering its existence on the Internet seems less preferable than a guided discussion. The burden of initiating the discussion falls on the physician, who is more likely to raise this than families, even for those that are interested if not gravely concerned about it. There has been an increasing call for physicians to educate about risks in low-incidence adverse outcomes (Palmboom et al., 2007).

More studies and information on physician and patient/family communication about SUDEP is likely to occur over the next decade, and it would behoove the practicing neurologist to keep several ethical questions in mind. Scientific research may be highly focused on answering specific research questions, but the practitioner must decide on the applicability of those questions on the situation at hand. Does the study population match the patient and family in question? Is the discussion feasible in the current clinical environment, or is it better suited for a follow-up visit, the time of which to be determined based on issues such as the clinical course, diagnostic findings, and compliance factors? If research demonstrates that a plurality of patients and families would prefer to hear the information, does the clinician apply a rule of disclosure to the newly diagnosed patient universally without concern for other factors, from risk stratification to a family’s level of tolerance, adaptability, and coping? The practicing neurologist can keep several ethical principles in mind to assimilate the ever-increasing new information and decide how best to approach this:

Beneficence vs. Nonmaleficence

Trying to maximize the patient’s benefit and minimize harm comes naturally to most physicians. It may be helpful to remember that patients are heterogeneous, and keeping the individual patient’s personal and social setting in context could guide the most appropriate action in a particular situation.

Autonomy vs. Paternalism

For children, parents act as the surrogate decision makers. As children age, they begin to gain comfort in the process of assent and then should be able to consent upon reaching the legal age of majority. Parents and doctors may both have their own personal biases separate from the patient. The physician must acknowledge and respect the autonomy of the parents to make those decisions for the patient, along with the patient’s input if the patient is cognitively mature enough to participate in assent. However, the physician can also exercise judgment about what is necessarily helpful or harmful for families and tailor the discussion to suit the patient’s individual needs in the appropriate sociocultural context.

Case Resolution

In the case posed at the outset of this discussion, the mother has initiated a question relevant to mortality in a very low risk setting for SUDEP. It would be most appropriate to guide a calm and rational discussion defining SUDEP and couching it in terms of the risk for this child, which would at most be 1 in 4500 of occurrence, or in contrast, 1 in at least 4499 of not occurring. It is sometimes helpful to contextualize the answer, even stating the risk of SUDEP occurring is less than the risk of death from a car accident, yet we make the decision to drive as a way of life without dwelling on this decision to the point of distress. Following a clear discussion where all questions are addressed appropriately, Mia’s parents report less anxiety now that they have better perspective.

Conclusion

The frequency of this situation belies its ethical complexity and difficulty in actual clinical practice. The SUDEP discussion is challenging, especially in newly diagnosed patients who have had neither a chance to adapt to the diagnosis nor develop a sustained relationship with the neurologist. Neurologists should keep an open mind about the desires of patients and families, remembering that patients and families may make different choices than neurologists would and be respectful of their decisions. A balanced discussion of risk, while informing families about SUDEP, places the information in context and allows for the palatable disclosure that is an intrinsic component of clinical medicine.

The authors have disclosed no conflicts of interest.

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Affiliations

James J. Reese, Jr., MD, MPH
Department of Neurology
University of New Mexico
Albuquerque, NM

Phillip L. Pearl, MD
Boston Children’s Hospital
Department of Neurology 
Harvard Medical School
Boston, MA

Correspondence

James J. Reese, Jr., MD, MPH
Department of Neurology
University of New Mexico
Albuquerque, NM
[email protected]

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