The Intersection between Voluntary Assisted Dying, Suicide and Advance Care Planning in Huntington’s Disease

Huntington’s Disease (HD) is an incurable progressive disease, that will advance to a stage that may cause intolerable suffering that is unable to be relieved in a manner considered tolerable by the individual (all criteria for Voluntary Assisted Dying in Australia and New Zealand )’; however, by the time a person with HD reaches an advanced and terminal stage, their decision-making capacity will be so impaired as to make them ineligible for Voluntary Assisted Dying (VAD) under all current and proposed legislation in Australia and New Zealand. Juxtaposing this ineligibility for VAD is the widely reported high incidence of suicide and suicidal ideation amongst people affected by HD. While the strong association between depression and other psychiatric manifestations with suicide has been explored, this paper explores whether suicide can ever be deemed rational and suggests that ignoring this possibility may result in a paternalistic approach that fails to meet the needs of the patient. In addition, health professionals can be left inadequately prepared and morally compromised. Given that Advance Care Planning (ACP) can provide an opportunity to refuse all medical treatment, except palliative treatment, which could result in an earlier death in a context of intercurrent illness or accident, could early and robust ACP discussions provide an acceptable alternative to suicide and VAD? Suicidal ideation in HD can be viewed as arising from multifaceted matrices including the impact of genetic testing, knowledge of the expected disease progression, including future neurological deterioration, psychiatric history as well as personal and family histories. When viewed this way, rather than just in terms of rationality or pathology, we may be in a better position to provide the best care and support to people with HD, including through ACP. Nurses need to consider these ethical dilemmas and remain informed on the debates as they may be at the forefront of a moral conflict between a person proposing rational suicide or seeking to implement refusal of treatment in an advanced care directive (ACD), and they must be aware and consider how their professional obligations and personal opinions interact with this complex topic.

Huntington’s disease (HD) is an uncommon, autosomal dominant neurodegenerative disorder presenting with a triad of symptoms in the motor, cognitive and psychiatric domains (Nance et al., 2011). People with HD have a high symptom burden and are diagnosed at a younger age than people with Parkinson’s Disease (El-Sourady et al., 2022), with symptom onset commonly occurring in the 40–50-year age range (Nance et al., 2011). Progression of symptoms tends to be slow and insidious, with death typically occurring 10-20 years after symptom onset (Langbehn, 2022). Prevalence of HD is cited at 5.70 per 100,000 in Australia (Pringsheim et al., 2012) although it is believed that the actual prevalence may be much higher. As an autosomal dominant disorder, each child of an affected parent has a 50% chance of inheriting the faulty gene. The disease trajectory in HD is complex and can vary significantly even between affected people in the same family (Macleod, Jury, & Anderson, 2017). The clinical course is characterized by a high symptom burden, significant stress on individuals and caregivers and gradual loss of independence (El-Sourady et al., 2022) and decisionmaking capacity (Ekkel et al., 2021). There is an elevated risk of suicidal ideation, suicide attempts and completed suicide in people with HD (Kachian et al., 2019). By the late stage, a person with HD will be unable to walk, communicate, eat or drink on their own, will have a severe cognitive impairment and may experience severe chorea or dystonia (Dellefield & Ferrini, 2011), which, for some, is perceived to be a future of intolerable suffering (Regan et al, 2018).

In most states of Australia and in New Zealand legislation is in place, or is being introduced, to enable people who meet specific criteria to access Voluntary Assisted Dying (VAD).

In Australia and New Zealand criteria that must be met include:

being 18 years of age or over

While HD is a life limiting disease, with irreversible decline in function that some may view as intolerable; by the time a person with HD reaches an advanced and terminal stage, their decision-making capacity will be so impaired as to make them ineligible for VAD under all current and proposed legislation in Australia and New Zealand (White et al, 2022).

When George Huntington first described Huntington’s disease in 1872, he remarked on the suicide rate. ‘The tendency to insanity, and sometimes that form of insanity which leads to suicide, is marked’ (Huntington, 1872). Most literature on HD refers to an increased rate of suicide and historically there has been considerable research on the topic. Honrath et al. (2018) report suicide as the leading cause of death in HD, after pneumonia. Early retrospective studies examining cause of death in people affected by HD demonstrated a higher rate of suicide amongst people with HD compared to the general community and suggested if anything, the suicide rates reported by Di Maio, Squitieri & Napolitano (1993), of 7.3% and Farrer, Opitz & Reynolds (1986) of 5.7% were an underestimate. Another retrospective study examining the deaths of people with HD in Hungary over a 77 year period revealed a suicide rate of 10.1% (Baliko, Csala & Czopf, 2004). In the most recent study, Alothman et al. (2022) examined 18 years of linked electronic medical records in the UK and concluded that people with HD were nine times more likely to die from suicide than people who do not have HD. They also described people with HD dying from suicide were much younger than those with HD who died of other causes. No relationship has been established between age of symptom onset and suicide or between stage of HD and suicide risk (Farrer, Opitz & Reynolds, 1986). In a systemic review in 2019, Kachian et al reported that 7-10% of people with HD attempt suicide at some stage in their life compared to a 1-3% rate of suicide attempts in the global population. The suicide rate in HD is also higher when compared to other progressive neurological conditions including motor neurone disease, Parkinson’s disease and Alzheimer’s disease (Kachian et al., 2019).

Suicidal ideation is reported by 20–30% of people with HD during their lifetime, including in pre-manifest individuals (Kachian et al., 2019), with rates varying between stages (Honrath et al, 2018), but present throughout the course of the disease (Kachian et al., 2019). Evidence for elevated risk of suicidal ideation in specific stages of HD is mixed (Kachian et al., 2019).

A higher incidence of suicidal ideation has been reported at the first appearance of HD symptoms, immediately prior to diagnosis (Di Maio, Squitieri & Napolitano, 1993; Paulsen et al, 2005) and again in stage two of HD, when independence is diminishing (Paulsen et al., 2005); however, both historical and current suicidal ideation rates reported are higher amongst people with symptomatic HD compared to those in the pre-manifest stage (Honrath et al., 2018). It is not clear the extent to which suicidal ideation is in itself a risk factor for suicide attempts or completed suicide (Hubers et al., 2013). Regular assessments of suicidal ideation are an essential part of good practice and are routinely conducted throughout the course of the disease. Suicide questionnaires are included as part of almost all research in HD and, of note, the frequently used Problem Behaviours Assessment rates suicidal ideation as ‘questionable’ if there is a plan for suicide at a later date when the disease is more severe (Kingma et al., 2008).

While a higher rate of suicidal ideation and actual suicide amongst the HD community has been established, the contributing factors are less clear. As Farrer et al. (1986, p. 310) put it, ‘it is unclear whether it represents a rational but extreme response to an intolerable situation or a manifestation of dementia’. Psychiatric symptoms are a core feature of HD and are a robust risk factor for suicide (Alothman, 2022). Depression is identified as the major predictor for suicide both in the wider community and in HD (Kachian, 2018). Depression occurs in people with HD at twice the rate of the general population, with 50.3% having sought treatment and 40% identified as currently depressed (Paulsen et al, 2005). Further study into the underlying mechanisms of depression is required, given that the nature of depression and suicidality is poorly understood and it remains unclear whether depression is due to biological changes in the basal ganglia or due to anticipation of the pending disease (Paulsen et al., 2005) or other factors. A depressed mood is identified as predictive of suicidal attempts and completed suicide (Hubers et al., 2012) while a small relationship between suicidal ideation and depression has been noted (Hubers et al., 2013) which increases with higher alcohol use (Wettzel et al., 2011). Although depression appears strongly associated with HD suicidality, it does not appear to explain all suicidal ideation, which can still be detected in cohorts of non-depressed individuals with HD (Halpin, 2012). A relationship has been identified between suicidal ideation and aggression and irritability (Kachian, 2018), along with anxiety and psychosis in people with symptomatic HD (Honrath et al., 2018), whereas higher rates of apathy are associated with lower rates of suicidal ideation (Honrath et al., 2018). More rapid disease progression is correlated with increased suicidal ideation (Hubers et al., 2013). Sociodemographic variables do not appear to predict suicidal ideation in people with symptomatic HD, however older age and being single or unemployed were associated with a presence of suicidal ideation in premanifest individuals (Honrath et al., 2018).

Living within an HD family may be itself a risk factor. It is possible that watching family members progress through an incurable illness, while recognising this represents your own future, might instigate suicidal thoughts (Alothman, 2022). A familial disposition to depression and suicide may be a factor (Epping et al., 2011), with an increased risk of suicide reported in families where one member has committed suicide (Di Maio et al., 1993; Farrer et al., 1986). An alternative perspective presented in a qualitative study of suicidality, concluded that the HD community did not connect suicide to depression or mental illness but instead saw it as a response to the realities of a deteriorating disease and concerns over future care (Halpin, 2012). Whilst the sample was small, it is worthy of consideration given its personal narratives and the themes that arose.

A clear link has been established between HD and suicide and between suicide and depression and key periods of suicide risk have been suggested. The focus of this paper now moves to perspectives regarding the act of suicide. Attitudes to suicide vary within the HD literature; however, the majority advance an argument of reversibility, with most studies recommending a need for increased vigilance and monitoring of suicidal ideation and risk, for example, ‘ suicide is one of the few potentially preventable causes of premature death in this disorder’ (Wetzel et al., 2011 p. 2). They did however conclude that the absence of psychiatric symptoms does not indicate lack of suicidal ideation, so it may not be fully preventable.

Paulsen et al. (2005 p. 729) took this further suggesting the need to ‘separate suicide associated with major depressive disorder from rational suicide in persons with a known terminal illness’. Amongst the HD specific literature Halpin (2012) presents the most compelling arguments for accepting the possibility or concept of rational suicide, stating that to ignore it may result in paternalism, in inadequately prepared and morally compromised health professionals and pessimism and hopelessness amongst people with HD. Furfari et al. (2015) present a case study of a woman with HD treated in hospital with intubation and intensive care after a suicide attempt, who clearly demonstrated lack of coercion and no mental illness and who sought discharge to complete her suicide and end her suffering. She had previously documented an advance directive requesting no active treatment. The authors report the moral distress for all concerned and suggest that in this example the principle of beneficence might in fact be allowing relief of suffering (through death) in a situation where the woman had already identified and documented her condition and quality of life to be unbearable.

The strongest view against rational suicide is put forward by an eminent psychiatrist, in the HD field, Adam Rosenblatt,.

It cannot be overstressed that suicide in HD is a preventable manifestation of disease. The concept of a “rational” suicide in someone with HD, a person who dispassionately “chooses” to “take his own life” before symptoms become too advanced, is a myth. Suicide is devastating to the people left behind and increases the risk of suicide in the next generation. It must not be rationalised, romanticized, or accepted (Nance et al., 2011 p.68).

Physicians generally hold the most negative view of suicide and VAD because it opposes the norms and ethics of their practice that focuses on treating and curing (Halpin, 2012). By contrast a 70-80% acceptability rate for rational suicide amongst health professionals involved in end of life (EoL) care has been reported (Werth & Holdwick, 2000).

If it is acknowledged that suicide and depression are not synonymous and that some people with suicidal ideation do not have a mental illness, then it can be considered that suicide is not always an irrational decision (Cutcliffe & Links, 2008; Ho, 2014). The prevailing libertarian view in most Western cultures is that the individual owns their body and therefore, with an emphasis on autonomy, the individual can choose where and when to die (Cutcliffe & Links, 2008; Ho, 2014). This perspective was a significant driver for development of VAD legislation. Where access to VAD is not an option, suicide may be seen as the only alternative way to control the circumstances of one’s death (Kresin et al., 2021).

The most widely cited author on rational suicide is a psychologist, James Werth. He developed criteria for rational suicide (see Table 2), a version of which is almost always cited in the literature on this topic and which provides a framework for evaluating whether a suicide is rational or not. Rational suicide can be defined as the taking of one’s own life that is characterised by reason or by making sense to others. Essential characteristics of rational suicide include being free from psychological distress, having a realistic assessment of their life situation, contemplating the decision for some time and ideally involving the individual’s significant others (Werth & Holdwick, 2000).

The opposing view on rational suicide is presented as a largely philosophical debate, tending to use examples of healthy people choosing suicide, in an endeavour to counter the viewpoints of the rational suicide proponents (Pilpel & Amsel, 2011). They suggest that, ‘The ‘rational suicide’ proponents are in a dilemma: either accept there is nothing wrong with this suicide, or they must make certain substantive value judgments...’ (Pilpel & Amsel 2011). However, exponents of rational suicide contend that it needs to be assessed on the merits of each individual case and may in fact be judged differently by different practitioners (Cutcliffe & Links, 2008; Ho, 2014).

From a Catholic and some other Christian perspectives, belief in the sanctity of human life means that neither VAD nor suicide are seen as acceptable means of ending life. This belief system sees life as a gift from God that is precious and should always be protected and can only be ended by God. Therefore, to prematurely and unnaturally end life is contrary to love of self and love for God. Some Catholic teachings also encourage embracing suffering, as a means of being better able to identify with the suffering of Christ.

People affected by HD and their families, often express an understanding and acceptance of their family member’s suicide, relating it to prevention of further suffering and degeneration. Members of the HD community, while recognising that depression can be a related factor, generally see suicide for themselves or their family members as rational (Halpin, 2012). This is a stark contrast to the psychiatrist’s view quoted earlier (Nance et al, 2011 p.68). Kresin et al (2021) postulate that access to VAD may reduce the incidence of suicide by giving people incentive to live longer, past the point of incapacity, secure in the knowledge that autonomy in death will be possible, even when they can no longer carry out the act themselves. They identify the perspective that VAD provides a safe, regulated medical procedure as opposed to the often violent and uncertain outcome of a suicide attempt and may therefore be seen as more acceptable. This argument has only recently appeared in the discourse on VAD in Australia and may have emerged due to a national focus on suicide prevention (Kresin et al, 2021).

In any discussion about rational suicide, VAD is perhaps the ‘elephant in the room’, with opponents viewing VAD as sanctioned suicide. Euthanasia laws in the Netherlands, which allow for preparation of VAD wishes in advance, have similar criteria to Werth’s for rational suicide, in that any request must be voluntary and carefully thought through and that the person’s condition must have no prospect of improvement, there are no logical treatment alternatives and that their suffering is unbearable (Booij et al., 2013). Figures from the Netherlands describe the granting of six to ten euthanasia requests a year for people with HD in a total of 50-60 deaths per year of people with HD (Booij et al., 2013). This computes to approximately 14% of all HD deaths compared to a rate of approximately 2% of all deaths in the Netherlands, occurring as a result of euthanasia. This figure is much lower than the suicidal ideation rates discussed earlier and prompts the question as to whether legalising euthanasia has any effect on the incidence of suicide in the HD population.

Because many people with HD clearly understand their future as HD progresses, it follows that many of them think about and plan for end of life (EoL) (Booij et al., 2013, Booij et al., 2014).

People with HD’s views on VAD are strongly shaped by their clear understanding and experience of what their future with the disease will be like, and fear of loss of physical function and self-identity (Regan, Preston, Eccles, & Simpson, 2018). They therefore have a tendency to view VAD as desirable or necessary to alleviate suffering and where it is not legally available, suicide by other means might be considered a rational option (Regan et al., 2018) and may occur earlier in the disease trajectory, due to fear of being physically unable to complete this when they most desire it. People with HD, when considering EoL, tend to focus more on what they would perceive as unbearable, rather than on what could be done to best support them (Ekkel et al., 2021). Regan et al. (2018) found that people with HD emphasised their right to autonomy and perceived VAD as ‘an act of kindness’. Given all of this, a focus on early and robust ACP may provide an alternative to contemplating suicide as the only other option and may also provide reassurance and psycho-education to meet the needs of people with HD who want to discuss EoL issues but are unsure how to (Regan et al., 2018).

Two separate studies (Ekkel et al., 2021; Regan et al., 2018) showed that people with HD, similarly to the wider community (Bernard et al 2020), have varying approaches to the future, with some wanting to make plans and arrangements and others avoiding thinking about the future or not worrying about it yet. However, it appears that the majority of people with HD do have thoughts about EoL (Booij et al., 2014) and would like to discuss EoL care, but don’t know how, when or with whom to have these conversations, or even what options are available (Regan et al., 2018). Familiarity with their own anticipated future, from witnessing other family members with HD, results in people with HD having more detailed and specific thoughts about EoL, but not necessarily thinking any more about EoL than other people (Booij et al., 2014). Health professionals have a legal, moral and professional obligation to discuss EoL issues with people with HD; however, in a scoping review of the literature only about a third of people with HD were found to have an advance care plan (El-Sourady, 2022).

The term Advance Care Plan or Advanced Care Directive is used to cover a variety of documents that people may use to express their values and preferences for care and treatment. ACP involves reflection, discussion, determination and documentation of an individual’s values, goals and preferences in relation to their future medical treatment and care, to guide clinical decision making (Bernard et al., 2020). It usually also involves the appointment of a substitute decision maker in case they are unable to make decisions for themselves in the future (Ekkel et al., 2021). Ideally ACP is undertaken directly with the person with HD themselves, along with their family and future substitute decision makers, while they are still able to articulate their preferences (Keijzer-van Laarhoven et al., 2020). ACP can enhance quality of life in advanced HD and provide reassurance to relatives (Ekkel et al, 2021) while reducing potential misunderstandings between health professionals and families (Lennard, 2018). A clearly written Advance Care Plan is likely to result in less aggressive interventions (Boersma, Miyasaki, Kutner, & Kluger, 2014; Gadoud & Johnson, 2015; The Victorian Department of Health, 2014) which could otherwise prolong dying and diminish quality of life.

ACP is a dynamic process and should be embedded as part of routine clinical practice, with consideration given to clinic visits dedicated just to ACP in order to overcome the most common barrier of insufficient time (Cooper, 2022). While some people may not want to undertake ACP, there is no way to predict those who will and those who will not, so health professionals must initiate these discussions (El-Sourady, 2022), encouraging people with HD to discuss and review their preferences at identified points along their illness trajectory, perhaps annually or in conjunction with functional changes (Vaughan & Kluger, 2018). People with HD report that they are generally able to return to living their regular daily lives once they have discussed ACP, without this causing lingering distress (Ekkel et al, 2021). Follow up ACP discussions and treatment options, in relation to emerging symptoms, are generally viewed as part of expected guidance from treating health professionals throughout the disease trajectory (Seeber et al, 2019).

ACP should include developing a values directive, which describes a person’s views, values and preferences to guide alternative decision makers and doctors when needing to make medical decisions for them. An instructional directive is an optional component, is legally binding, and should unambiguously outline specific treatments that a person consents to or refuses and the specific circumstances under which they would want or not want those treatments (The Victorian Department of Health, 2014). The full implication of stated preferences and potential scenarios where these might be applied, should be discussed with the individual as part of the process. ACP for a person with HD should include but is not limited to : appointing substitute decision makers and powers of attorney, enduring guardian, decisions regarding resuscitation, types of treatment desired or refusal of treatment (such as antibiotics, ventilation, intensive care interventions), decisions regarding hospitalisation and preferred place of death and tube feeding (Hosken, 2019). ACP discussions and documentation should include treatments both now and into the future, with clearly identified stages a person may like to refuse specific treatments (Hosken, 2019). In the author’s clinical experience, people with HD are often not aware that they can refuse medical treatments, such as antibiotics in the event of pneumonia for example, which may result in their death in the setting of an intercurrent illness. Within the author’s service, a survey of people with people with HD and their caregivers, regarding their EoL fears and wishes, found that participants over-whelmingly wanted HD specific advance care planning information to help support them in this process.

An argument is sometimes made that ACP is flawed in that it requires individuals to anticipate what their future may hold, a state they have not previously experienced, without knowing how they may adapt to their condition in the future or if their values might change (Lennard, 2018). However, most people with HD have lived experience of the disease, having witnessed often multiple family members EoL experiences and are therefore in a unique position to understand their likely future and predict their future self (Regan et al 2018). ACP discussions often consist of people with HD seeking reassurance that they will not have to endure the disease until the very end (Ekkel et al, 2021), like they have witnessed in other family members.

Autonomy is promoted through ACP and individuals may feel reassured by the knowledge that their preferences for EoL care will be followed and that the burden for decision making will not be placed on their families (Booij, Engberts, Rödig, Tibben, & Roos, 2013; Huntington Society Canada, 2016; Regan, Preston, Eccles, & Simpson, 2018). However, an ethical dilemma may arise, if an individual’s condition has progressed so that they are no longer able to express their viewpoint and others around them consider they are no longer aware of or committed to their previous beliefs expressed in an advance care plan and appear to be currently able to derive enjoyment from life (Keijzer-van Laarhoven et al, 2020; Lennard 2018). In this situation questions may arise as to whether a previous autonomous decision should take precedence over protecting their current presumed wishes (Keijzer-van Laarhoven et al, 2020, Lennard 2018) and will require the treating team and substitute decision maker to interpret the values documented in the ACP. In relation to HD, there is a unique future risk associated with not adhering to the ACP wishes and directives. Given that the nurse-patient relationship is based on a foundation of trust and honesty (Snir et al, 2022), where a family member may be also a person with HD themselves, lack of adherence to, or respect for, a previous ACP decision may undermine the very nature of that relationship and take away their hope for their own future EoL care. While no link has been found in the literature, the question must be asked as to whether disregard for the Advance Care Plan could then increase the risk of suicide in other family members with HD, as a means to assert some control over their own future.

The clearly established links between HD and depression, and between depression and suicide compel health professionals to undertake regular assessments of depression and suicidal ideation with their patients with HD, and ensure that they are offered medication and therapeutic interventions, such as counselling to treat depression (Nance et al, 2011). Nurses are closely involved with people with HD and their families, so they may be the first one to whom suicidal ideation is expressed or the question of VAD is raised (Snir et al, 2022). The nurse should engage in empathic but exploratory discussion with the individual to gain an understanding of the factors contributing to this thinking and questioning, as well as passing this information on to other members of the team (Snir, 2022). This may be the trigger for initiation of ACP discussions. Routinely discussing end of life wishes and assisting patients in ACP is an important part of providing care to people with HD, to enable them to maintain autonomy and quality of life, given they will inevitably have considered EoL at some stage in the disease trajectory (Booij et al, 2014).

Identifying how to respond to rational suicide is a greater challenge, given that most health guidelines, practices and policies are written on a premise of suicide being driven by a psychiatric disorder and as such health professionals are charged with identifying potential risks and a duty of care to try to prevent suicide (Cutliffe & Links, 2008; Ho, 2014). Nurses working with a person with HD who expresses their intent to take their own life are faced with an ethical dilemma, potentially causing moral distress, and need to manage the discomfort and dissonance arising from this. The nurse may hold a belief that the individual is rational and accepts their right to autonomy, yet they may be working within a policy and an organisation that requires them to keep that person physically safe and do all in their scope to prevent their suicide (Cutliffe & Links, 2008, Rich & Butts, 2004). ‘Moral pro gress in nursing necessitates that nurses ponder these ethical uncertainties in supporting or opposing intervention with patients who are contemplating rational suicide’ (Rich & Butts, 2004 p.277), otherwise they may be unprepared psychologically and professionally when faced with this situation (Rich & Butts, 2004). The literature draws out this dilemma however it offers no solutions, with the one exception a proposed model for ongoing research, training and lobbying for policy change on a larger scale (Werth & Holdwick, 2000).

A higher incidence of suicide amongst people affected by HD and the strong association between depression and suicide has been demonstrated and this knowledge is at the forefront of good practice in working with people with HD. Given that VAD is not available for people with HD, awareness of the possibility of rational suicide is important. Whilst there is a significant body of writing on the topic of rational suicide, this remains controversial, posing unresolved ethical dilemmas for practitioners working with people with HD who are contemplating suicide. Given the close relationship that may exist between nurses and people with HD and their families, they may be the first person with whom these suicidal thoughts or questions of VAD are raised. This provides an opportunity and perhaps an imperative to introduce the concept of ACP, to enable the individual to assert some autonomy over their future, which may provide an acceptable alternative to suicide and VAD. If suicide is viewed as arising from multifaceted matrices including the impact of genetic testing, knowledge of the expected disease progression, neurological deterioration, psychiatric history and personal and family histories rather than just in terms of rationality or pathology we may be in a better position to provide the best care and support to a person with HD (Halpin, 2012), including through ACP. Awareness of these issues, and the ethical dilemmas that may arise from them, is essential for nurses, to reduce the moral distress they may otherwise experience when faced with a conflict between their professional obligations and their own personal views and beliefs.