Goals of care conversations and advance care planning for paediatric patients living with serious illness

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Introduction

Medical advances have led to higher rates of survival of children with chronic and sometimes complex health conditions, many of whom would not have survived previously^[1][2]. Children living with medical complexity often require multiple interventions to maintain their health, including surgical procedures, hospitalizations, and home technological support. Their families frequently face difficult decisions about which interventions are in their child’s best interests. The potential for burden and other harms mandates health care providers (HCPs) to carefully counsel families (and legal guardians), always acknowledging the delicate balance between promoting survival and compromising quality of life. The aim of any paediatric HCP involved with complex care cases should be “to add life to the child’s years, not simply years to the child’s life”^[3].

There continues to be a relative dearth of information regarding advance care planning in paediatric compared to adult populations. The emphasis on beneficence in paediatric substitute decision-making may lead to a bias toward curative or life-prolonging options. The concept is also consistent with parental obligations to protect children and society’s predisposition to favour life for its own sake. In general, children are expected to outlive their parents. Furthermore, the overriding instinct to protect children from harm can lead to avoidance of difficult topics, even to the extent that some children, despite having adequate capacity to understand and cope with their condition, are excluded from discussions about the gravity of their illness. Lastly, concerns about the legal status of advance care plans for minors may contribute to the lack of evidence and guidance on this issue.

What is advance care planning?

Advance care planning is an extension of usual discussions about the future, typically for patients with a serious illness, that focus on possible medical needs as things change. Advance care planning is rarely a single event. Rather, it is a process that should occur over time as more becomes known about an individual’s condition and prognosis. The purpose of advance care planning is to give patients and families an opportunity to identify what matters most concerning medical care, based on their personal beliefs, values, and wishes in the context of their medical situation. Planning can encompass both short and longer term health goals and treatments. While plans may include drafting formal advance directives specifying what treatments should (or should not) be provided under specific circumstances, the benefits of advance care planning are as much procedural as they are substantive. Engaging patients and families in open dialogue helps ensure that the treatment provided aligns with shared goals of care, whether life-prolonging or palliative.

Advance care planning should start with discussions about ‘goals of care’, to capture what is hoped for from medical interventions. Goals of care should be identified using a shared decision-making framework^[4][5]. The patient and/or the legal guardians, together with members of the health care team, should work collaboratively to determine realistic goals within the context of the illness^[6].

Goals of care represent the culmination of three sequential activities (Table 1). While goals can be readily identified in most clinical situations, a physician’s ability to confidently recommend the best treatment to achieve them is sometimes hampered by degree of uncertainty. When uncertainty is high, it can be tempting to describe options in ‘black and white’ terms, with the hope of helping families navigate the ‘shades of gray’. However, humility and the acknowledgement of uncertainty are often more appreciated by family and other caregivers^[8][9]. When possible, time-limited trials to assess an uncertain treatment’s effectiveness in meeting goals of care can be useful^[10].

Table 1. Sequential activities to establish goals of care
1. Ensure understanding	The first step is to ensure there is an accurate understanding of the current medical situation and prognosis. Without this step, goals of care may be based on incorrect assumptions. It is the responsibility of the health care team to share this information with patients and families in a compassionate but clear manner. Take time to answer questions, clarify misconceptions and, when uncertainty exists, provide professional opinions based on the best available evidence and clinical experience.
2. What matters most?	Once an accurate appreciation of the clinical situation is established, including the capabilities and limitations of medical interventions in the present context, the patient-family decision-makers can consider what matters most to them. These are personal considerations based on values, hopes, beliefs, and wishes. What matters most may be informed by positive desires and motivations (e.g., “to keep fighting” or “to be at home as much as possible”) or by yearning to minimize fears and worries (e.g., “I just don’t want to see her suffer” or “I’m not ready to die yet”), or some combination of both.
3. Recommend treatment	Only the family can decide what is most important to them, but it is the responsibility of medical providers to take that information and ‘operationalize’ it into goals of care. This process requires active listening and understanding of preferences shared, then using that information to make treatment recommendations that align with them. Treatment plans developed in this manner are more likely to result in goal-concordant care[7]. When clinicians are unsure of what is ‘hoped for’, recommending a plan of treatment becomes a challenge. This scenario often results in listing possible treatments for a decision-maker who lacks the medical knowledge and experience to judge or apply options in context. In contrast, when overarching preferences of the patient and family are known, physicians are ideally suited to make treatment recommendations to meet those goals. Indeed, it is incumbent upon them to do so.

Generally, goals of care for children and youth living with serious illness fit into 1 of 3 categories (Table 2). While treatment objectives may be shared by the family and health care team, not all are achievable. Goals of care are limited, ultimately, by the limits of medicine. When goals are focused on prolonging life but the clinical situation leaves no reasonable chance to do so, it can be useful to acknowledge this: “I know it’s important that we do everything possible to prolong life, but I’m worried more chemotherapy/additional procedures/CPR won’t help us accomplish that goal” (if appropriate add: “and may in fact shorten life”).

Table 2. Goals of care categories
1. Prolonging life	Patients should have full access to medical, procedural, and surgical treatments, including resuscitative measures (i.e., CPR), provided these options are believed to be in the child’s best interests.
2. As much good time as possible	Patients should receive focused attention on achieving or maintaining a quality of life (QOL) defined by the patient and/or family. Care that prolongs life should be considered for patients experiencing an acceptable QOL or whose treatment offers realistic hope of ‘return to baseline’ QOL. For some patients, this goal may include a decision not to attempt resuscitation. Such is often the case when a patient or family believes that resuscitative measures would be unlikely to help  restore the patient’s  QOL.
3. Focus on comfort	While maximizing comfort should always be a goal of care, it is paramount for patients in this category, and should supersede goals 1 and 2. As patients near the end of life or experience a QOL that does not meet personal threshold (self-defined or determined by their substitute decision-maker(s)), and are believed to be suffering, treatments that prolong this state without meaningfully adding to comfort may be discontinued, provided the medical team agrees.

Goals of care direct current treatment in the present, while advance care planning involves thinking about or applying goals of care to potential future states. Both align the values, goals, and wishes of a patient and family with the care being received. While clarity regarding goals of care should guide current treatment, some patients and caregivers are reluctant to think too far ahead, preferring instead to “cross that bridge when we get there”^[11]. If the patient and family do not appear ready to engage in advance care planning, particularly when death is not believed to be imminent, respect for autonomy and parental authority includes supporting them as they defer decision-making.

Why should care planning discussions happen?

Parents may be more optimistic than health care team members about a child’s prognosis^[12]. The reverse can also occur^[13]. Health care professionals may also underestimate parental understanding and receptiveness to discussions about foregoing life-sustaining interventions^[14]. A retrospective survey of parents whose children died in paediatric intensive care units showed that although the discussion was initiated by physicians 90% of the time, almost one-half of parents thus engaged reported they had considered withdrawal of life-prolonging treatment before this option was raised by staff^[15].

Although the possibility of premature death is widely known for many conditions (e.g., congenital anomalies, paediatric cancers, neuromuscular conditions), preferences regarding treatment may not be solicited if members of the health care team assume that the patient and/or family are in favour of life-prolonging treatment. One international survey showed that 45% of paediatric oncologists do not initiate this discussion^[16]. And despite recent increased attention to the benefits of early integration of palliative care, the evidence suggests that the rapid emergence of novel treatments and technologies may be compounding clinician hesitancy to talk about situations where cure is not possible^[17].

These discussions may occur even less commonly for paediatric patients with chronic conditions, for whom the timing of death cannot be easily predicted (e.g., severe cerebral palsy, neurodegenerative disorders, complex congenital heart disease, and short gut syndrome). Because children with these conditions and their parents may have varied understanding of the potential for dying until discussion is initiated, the health care practitioner should share valuable information and provide the opportunity for reflection.

While honest discussions about ‘what if?’ scenarios, including the possibility of a shortened life, are often emotionally laden, they are still generally appreciated. In a small exploratory study involving adolescents and young adults with metastatic cancer and HIV, there was an overwhelming endorsement of the use of an advance care planning document (‘Five Wishes’)^[18]. Respondents believed that completing the document would be helpful both in their own situation (19/20; 95%) and for others living with serious illness (18/20; 90%). None of the participants described the experience as stressful. In another study of adolescents followed in a tertiary hospital outpatient cardiac clinic, 92% wanted a physician to inform them if their condition was terminal and 91% wished to be involved in discussions about their end-of-life care^[19]. By comparison, 71% of their parents endorsed open discussions with the adolescent, despite hesitancy expressed by some. Indeed, parents of children with cancer^[20][21], serious neurologic impairment^[22], and a variety of other serious illnesses^[11] value the opportunity to discuss their children’s prognosis and engage in advance care planning. In one study involving parents of children with cancer, 87% desired as much prognostic information as possible^[21]. Moreover, while 36% found the prognostic details shared “very” or “extremely” upsetting, these parents were just as likely to report that receiving the information was important and helped with decision-making.

Beyond the value placed on open communication about serious illness, prognosis, and goals of care by paediatric patients and their families, available evidence suggests additional important benefits. In a recent multicentre study^[20], adolescents with cancer and their families were randomized to receive three weekly sessions of an advance care planning intervention versus routine care. Family members engaged in the advance care planning intervention reported increased positive appraisals of themselves as caregivers at 3 months compared with those in the control group. The researchers hypothesized that advance care planning helped participants become more informed about their child’s medical condition, putting them in a better position to advocate for their child. In a retrospective survey completed by bereaved parents of children who died from a variety of chronic complex conditions, the 61% who reported advance care planning discussions prior to their child’s death reported feeling more prepared at end of life, perceived their child to have had ‘good’ to ‘excellent’ quality of life, and planned for the location of death^[23]. Ensuring that patients and families have accurate information about prognosis and opportunities to identify goals of care can also result in meaningful alterations in care at the end of life. Adjustments might include integrating palliative and home care services, reduced attention on unhelpful or potentially harmful treatments, earlier formulation of ‘do not resuscitate’ orders, and increased attention to reducing suffering^[24].

When should these discussions take place and who should participate?

Goals of care and advance care planning discussions for children and youth are most frequently triggered by admission to intensive care^[25], where the stimulus for discussion is often the anticipation of impending death. The transition to a palliative focus of care may be abrupt, with the intents to avoid prolonging the time to death and limiting nonbeneficial or even harmful interventions. While it is understandable that the need for critical care might prompt these discussions, evidence suggests that they should start earlier, sometimes even shortly after diagnosis and, occasionally, even before a child is born^[25][26].

Medicine has always sought to help patients live well for as long as possible, and it is most often reasonable to assume that children and parents desire both quantity and quality of life. But for young people living with a serious illness, there may be a point where preference for quantity or quality becomes relevant to treatment decisions. When discussions regarding goals of care occur amid a life-threatening event, the objective is often to set limits or review the merits of potentially life-sustaining treatments. While such conversations are necessary, capable paediatric patients and substitute decision-makers may be reluctant to engage. Some may dismiss concerns expressed by the medical team, calling attention to the fact that they have persevered through similar situations before^[27]. Others may take offence because clinicians guiding these decisions are personally unfamiliar with their child and are basing recommendations on the very sick patient in front of them (e.g., when a child is ‘at their worst’), rather than the child at baseline (e.g., ‘at their best’)^[28][29]. Even parents who acknowledge the limits of what medical interventions can accomplish may still be inclined to ‘keep fighting’ as an expression of faith or belief that a ‘good’ parent never gives up^[30].

By initiating conversations with patients and their families about values, beliefs, and wishes early in the disease course, health care providers introduce the notion that treatment decisions should be based on more than just what will result in the longest possible lifespan. While earlier conversations will frequently elicit the goal of cure (or prolonging life), their outcome may be less important than a physician and family deliberating together. Earlier conversations have the added benefit of inviting patients and families to reflect on difficult subjects from a safe distance – when the prospect of death feels remote or unlikely – rather than mid-crisis.

Goals of care should be periodically revisited, especially following significant changes in health status or other major life events. As varying degrees of health and quality of life are experienced over time, it is not uncommon for goals to change. Nothing has more influence on goals of care than the life experiences of the child and family. Even individuals who previously held firm to prolonging life at all costs often reconsider goals of care in the face of persistent suffering without meaningful chance of recovery. Families must be given the opportunity to reflect on how their child’s condition and quality of life have changed over time and what matters most to them moving forward. Never assume that goals expressed strongly early in the course of a serious illness will stay the same. When reviewing goals of care, explain that the purpose of discussion is to ensure that optimal care is being provided in a changing situation. This starting point is likely to be supported by everyone involved. If requests to review goals of care are perceived by families as attempts to change their minds about care, they will be less receptive to engaging in conversation or sharing perspectives.

There is emerging consensus that the responsibility for initiating these discussions rests with health care providers, not the patient or family^{[22][25][26][29][31]}. Parents describe the need to be ‘ready’ for advance care planning discussions (Table 3), but many acknowledge that there is never a ‘good’ time to have them. Some prefer to have these emotionally difficult conversations when a child is stable; others during or immediately after acute deterioration^[23]. Most parents of children with a serious medical condition acknowledge thinking about ‘life and death’ issues, but sometimes their attempts to initiate conversations with their child’s health care provider are unheeded^[31]. Clinicians seeking additional guidance on how to start these conversations are encouraged to explore the Serious Illness Conversations Guide-PEDS^[6].

Table 3. Initiating advance care planning conversations

Signs that a patient or family might be ready: Thinking out loud about the future or expressing worry: How will I take care of her as she gets bigger? I’m scared that if my lungs get worse, I won’t be able to breathe  ‘What if’?’ questions: What if this new treatment doesn’t work? What if these pneumonias keep occurring? Concerns about quality of life or suffering: We never see him smile any more. I just feel like he’s unhappy. I don’t want any more chemo! I’m tired of feeling sick all the time; I’m tired of coming to the hospital for nothing. Probe using open-ended questions: How do you feel about your child’s quality of life? Are there aspects of your/your child’s health that worry you? What’s most important to you as things start to change? Have you been thinking about what will happen if things get worse?

Expressions of hope or faith are often misinterpreted as signs of not being ready to engage in advance care planning or to an assumption that goals of care are firmly in the ‘prolong life’ category. However, families facing critical situations can maintain hope while simultaneously grasping the reality of their child’s prognosis^[32]. The ‘wish, worry, wonder framework’ described in the Serious Illness Conversations Guide-PEDS is an effective way to continue conversations while acknowledging and validating expressions of hope^[6]. Instead of trying to destroy hope, the framework recommends aligning with it (“I wish…”), then acknowledging persistent concerns (“I worry…”). These steps can then be followed by a request to discuss the best way to care for a child should those undesired concerns come to fruition (“I wonder…”).

Families prefer to engage in advance care planning with a trusted health care provider who is familiar with the child and their underlying illness and who will lead these conversations sensitively and compassionately^[25]. While the provider with primary responsibility for the child’s most significant medical issues should be involved, their leadership should not be presumed. Numerous concerns and barriers have been identified by clinicians that delay or even prevent advance care planning with paediatric patients and families ^[33]. When clinicians feel uncomfortable leading such conversations, consulting with colleagues or specialists who are more experienced with their facilitation (e.g., in palliative or critical care) can be helpful.

Paediatric patients should be invited to participate in serious illness discussions, even when they lack the capacity to make decisions regarding their goals of care. Parents and other substitute decision-makers should be encouraged to watch for and listen to their child’s voice, behaviours, and body language, particularly in young or non-verbal children. Along with showing respect for the child^[34], parents that describe feeling as though their child ‘made the decision for them’ may feel less burdened by the task^[35]. Parents and capable youth can sometimes disagree on goals of care. Clinicians confronted by this difficult situation are encouraged to consult the CPS position statement Medical decision-making in paediatrics: Infancy to adolescence for guidance. While a serious illness discussion often begins with one family member, it is important to involve all relevant decision-makers in the evolving conversation to achieve consensus. For some families, bringing extended family members, a respected spiritual leader, or other community members into the conversation is vital. Language interpreters can be invaluable.

Barriers to implementing advance care plans

While the main purpose of advance care planning is thinking about and discussing future health states, it is not uncommon for decisions regarding treatment to emerge from the process. Such decisions should be documented in the patient’s chart and shared appropriately with care providers less familiar with the patient or uninvolved with discussions. Accessible documentation increases the likelihood that carefully considered goals and wishes are honoured^[36][37].

Legal documents that outline care preferences and recognize specific substitute decision-makers are commonly referred to as ‘advanced directives’ (in Canada, the more accurate term is ‘Power of Attorney for Personal Care’). However, most jurisdictions in Canada do not have legislation granting legal validity to advance directives made by capable mature minors. The stipulated legal age for advance directives (or ‘expressed capable wishes’) is 16 years in New Brunswick, Newfoundland and Labrador, Ontario, Prince Edward Island, and Saskatchewan. (See Table 1 in Medical decision-making in paediatrics: Infancy to adolescence for a summary of Canadian law regarding age of consent and advance directives.) Furthermore, advance directives as legally binding documents to guide future care can only be made by and for oneself. Health care providers must honour the valid advance directives of a previously capable adult. By contrast, when substitute decision-makers (e.g., parents) create documents together with health care providers to guide an incapable patient’s future care, consent for the plan of care is still required in the moment.

Even without legal authority, the clinical value of written documents that clearly articulate wishes surrounding end-of-life care for paediatric patients cannot be understated. Paediatric patients and their families are entitled to the benefits of advance care planning and specifically, the opportunities to reflect on goals of care in the context of a serious illness and how those goals might apply to future health states. Decisions documented as part of this process, including not to attempt resuscitation, should not be viewed by paediatric health care providers as ‘final’ but rather as a prelude to necessary discussion when a health crisis occurs. The conversation might begin with: “I understand that you’ve had conversations about your preferences in the event of…”.

The role of palliative care

Palliative care aims to provide comfort, reduce suffering, and improve quality of life ^[38]. Though originally conceived as a model of care at the end of life, contemporary paediatric palliative care is focused on living more than dying. Calls for earlier discussion of goals of care and advance care planning echo advocacy to integrate palliative care early in the disease course for children with a serious or life-limiting condition^[39]-[41]. When introduced early, palliative care is often provided as a consultative service, with involvement ebbing and flowing based on clinical need. One analogy that has been presented is that of a ‘dimmer switch’ instead of an ‘on-off switch’ model of palliative care, such that palliative measures are sometimes introduced alongside potentially curative treatments.

Paediatric palliative care teams are widely available at tertiary care centres across Canada and have expertise in leading goals of care and advance care planning discussions^[42]. Teams can facilitate these conversations virtually, enabling families who live far from the hospital to access palliative care expertise. Referrals are welcomed from community providers practising throughout the regions they serve. Paediatric palliative care providers can also help ensure that decisions arising from advance care planning are honoured, particularly when they involve a desire to focus on comfort and avoid intensive interventions as much as possible near the end of life. Moreover, paediatric palliative care teams can often help access enhanced supports (e.g., increased nursing, visiting hospice care, equipment and medications to optimize comfort at home) and assist with documentation (e.g., a ‘DNR’ or ‘no CPR’ order) for different care settings.

Recommendations:

1. Health care providers should initiate conversations about goals of care and advance care planning.
2. Conversations should start early in the disease course for children living with serious illnesses, especially for those who may have a shortened lifespan.
3. Health care providers should assess patient and/or parent readiness to engage in advance care planning. Signs of readiness may be verbal (e.g., “I don’t ever want to go back to the ICU again”; “Something’s different … he’s lost that spark”) or non-verbal (e.g., the patient can no longer do the things that they loved to do).
4. Unless urgent decisions are needed, health care providers should respect a family’s wish to delay conversations about the future, but with an agreement to check in again at a later time.
5. A health care provider who has a trusting relationship with the child and family should be continuously involved in these discussions, whenever possible.
6. Health care providers should emphasize that the primary goal of these discussions is to engage patients and families in thoughtful conversations about what matters most to them. Decisions regarding future care or the drafting of advanced directives may or may not be an outcome.
7. Specialist paediatric palliative care teams are available and should be accessed to facilitate goals of care discussions and advance care planning for children within their jurisdiction, when needed. These conversations can be conducted using virtual care technologies.

Acknowledgements

This position statement was reviewed by the Complex Care and Hospital Paediatrics Section Executives of the Canadian Paediatric Society. It has also been reviewed by the CPS Acute Care, Adolescent Health, Community Paediatrics, and Fetus and Newborn Committees. The statement builds on a 2008 statement entitled “Advance care planning for paediatric patients”, written by Dr. Ellen Tsai.

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CANADIAN PAEDIATRIC SOCIETY, BIOETHICS COMMITTEE (June 2023)

Members: Julie Emberley MD (Chair), Kelly Cox MD (Board Representative), Amaryllis Ferrand MD, Michael van Manen FRCPC MD PhD, Adam Rapoport MD FRCPC MHSc, Rebecca Greenberg PhD RN

Author: Adam Rapoport MD FRCPC MHSc

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References

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Disclaimer: The recommendations in this position statement do not indicate an exclusive course of treatment or procedure to be followed. Variations, taking into account individual circumstances, may be appropriate. Internet addresses are current at time of publication.