Several studies over the past decade have shown that there are discrepancies between physician and parent understanding of their child’s cancer prognosis, both at diagnosis and when the illness is more advanced. For example, Wolfe et al. showed that providers tend to recognize the child as having no realistic chance for cure approximately 100 days before families do and approximately 200 days prior to the patient’s death (Wolfe et al. 2000b). While this may be unsurprising (health-care personnel have prior training and experience recognizing incurable cancer), the discrepancy in recognition remains a critical issue in providing appropriate psychosocial care for patients and families. It is important that providers communicate to families when cure is unlikely as it influences when discussions regarding advance care goals and “do not resuscitate” (DNR) plans occur, with earlier communication leading to earlier discussions. Likewise, patients and families are more likely to endorse goals of “lessening suffering.” Parents are then more prepared for their child’s ultimate death, and physicians perceive the child’s death as more peaceful (Wolfe et al. 2000b; Maurer et al. 2010).

In contrast, when parents are unaware of their child’s impending death, children are more likely to die in the ICU and to receive cure-directed therapy that may impair their overall quality of life (and quality of death) (Valdimarsdottir et al. 2007). Parents with less time to prepare for their child’s death are more likely to have long-term psychological morbidities (Valdimarsdottir et al. 2007; Lannen et al. 2008), poor physical health, and poor quality of life (Jalmsell et al. 2011). These findings suggest that effective parent-provider communication and promotion of concordance regarding prognosis and goals of care will improve patient and family experiences. Unfortunately, doing so is challenging.

Several strategies for effective communication have been described (Von Gunten et al. 2000; Baile et al. 2000; Beale et al. 2005; Makoul 2001), all of which include similar guiding principles and efforts to make a human connection with the child and family (Table 14.1). A simple but successful outcome of effective communication regarding prognosis or goals of care is when families are able to “expect the worst” (e.g., fully understand and anticipate potentially difficult experiences, including the child’s death) while also “hoping for the best” (e.g., maintaining realistic hopes and goals while also identifying with their own good parent roles). This dual reality may, in turn, promote better patient and family coping, adjustment, and quality of life (Hill et al. 2013).

At more advanced stages of cancer, delineation of goals of care becomes a particularly important element of decision-making. When goals are clear, decisions regarding therapy may fall into place. For example, a parent whose goal is to “extend time with my child, without hope of cure” may decline intensive inpatient chemotherapy in favor of oral medications that can be administered at home. This alignment is especially true when clinicians apply models of shared decision-making, where exploring goals of care with patients and families may lead to discussions of how those goals may be best met (Waldman and Wolfe 2013).

Advance care planning (ACP) refers to the process of thinking about, talking about, and planning for future health care and end-of-life care. Engaging in ACP may be challenging for the same reasons it is difficult for patients, parents, and providers to discuss poor prognosis. In essence, preparing for a child’s death may feel inherently unnatural and be plagued by emotional distress, avoidance, and uncertainty. Determining if, when, and to what extent pediatric and adolescent patients are included in ACP discussions may be an additional challenge. Parents and providers may want to protect their children and therefore deliberately shield them from difficult conversations (de Vries et al. 2010). However, children ages 14 years and older often have adult-level comprehension of their situation and the consequences of their decisions. Patients between 10 and 20 years old report that “thinking about their relationships with others” is the single most important factor in their choices regarding continued cancer treatment and goals of care (Hinds et al. 2005). Nearly all adolescents and young adults living with life-threatening illness report wanting to be able to choose and record the kind of treatment they do (and do not) want, how they want to be cared for, what they want to share with others, and how they want to be remembered (Wiener et al. 2012).

Evidence suggests patients as young as 3 years old are aware of their prognosis without being told (Bluebond-Langner 1978). Patients of all ages who are unaware or shielded may paradoxically feel greater distress or anxiety by “not knowing;” providing age-appropriate honest information may alleviate some patients’ fears. When patients are present for initial discussions about diagnosis and prognosis, parent distress is significantly decreased (Mack et al. 2006). Likewise, among over 400 bereaved parents, none of those who talked to their child about death regretted having done so, but 27 % of those who avoided the topic regretted the lost opportunity (Kreicbergs et al. 2004b). The corollary is also true; nearly three quarters of parents did not experience regret. Most important, therefore, is to uncover and respect the family communication style.

One approach to gauge readiness for advance care planning is to ask patients and families: (1) whether talking about what would happen if treatment was no longer effective would be helpful, (2) whether talking about medical care plans ahead of time would be upsetting, and (3) whether they would be comfortable writing down/discussing what would happen if treatments were no longer effective (Wiener et al. 2008). Other more specific and age-appropriate instruments have also been developed and can help guide conversations and address critical elements of patient and family values, beliefs, goals, hopes, and worries (Table 14.2) (Zadeh et al. 2014; agingwithdignity.org).

Other types of anticipatory guidance should include discussions about the child’s resuscitation status, preferred location of death, organ and/or tissue donation and autopsy, and funeral planning. Of note, many children who die from advanced cancer will not be candidates for organ donation due to concerns for disseminated cancer in body tissues. Other children only have isolated eligible tissues (e.g., their corneas). It is important to communicate to families who wish to donate that their preference is heard and honored, even if it cannot be met. Autopsy should be offered to every family. In a study of 30 bereaved parents, only 37 % recalled a conversation about autopsy, and 90 % of those who did not recall such conversations suggested they would have considered the procedure. The majority of parents indicated that discussions about autopsy would be appropriate when it is clear that cure is no longer possible, rather than at the time of their child’s death. Furthermore, educating parents about the potential research, emotional, and clinical benefits of autopsy would enable their informed decision-making (Wiener et al. 2014). Parents who choose autopsy for their children often do so to advance medical knowledge and/or respect their child’s wishes. Among 33 bereaved parents of children with brain tumors, none who requested autopsy regretted it, and 91 % stated they would make the same choice again (Baker et al. 2013). We have found that some parents may also find peace in knowing the definitive cause of their child’s death, while others may decline because they feel that they “know” the cause. Either choice is appropriate.

PPC care team involvement can help facilitate ACP. If PPC providers were involved early in the cancer experience, they may have joined the primary psychosocial oncology team in helping the family with initial adjustments to cancer and to challenges that develop over time. Later PPC involvement may then feel more seamless in advanced stages of the illness (Waldman and Wolfe 2013). Studies have shown that changes in terminology (e.g., “advanced care team” or “supportive care team”) may also lead to increased referrals, particularly later in the cancer experience (Dalal et al. 2011). Better description of palliative care as ‘an added layer of support’ may also enable its involvement. PPC expertise can help guide communication, enabling medical personnel to effectively and compassionately relay difficult concepts, thereby facilitating family understanding and, in turn, realistic expectations. Furthermore, PPC can assist with very difficult decision-making for parents, providers, and patients alike. Combined, PPC involvement may alleviate patient, parent, and provider suffering.

In advanced pediatric cancer, it is often unclear to what extent cancer-directed treatment will be effective. And as described above, it is natural for parents (and providers) to hold out hope for a cure and leave no stone unturned. This continued search for treatment options often includes consideration of enrollment in phase I clinical trials. Few studies, however, have described how parents or providers make decisions about experimental therapies. The goal of phase I research is to identify appropriate dosage and safety data for new drugs, not to demonstrate efficacy or to impact curability. It is unclear how many patients or parents are aware of this fact (Agrawal and Emanuel 2003). For example, the most common reason parents enroll in clinical research studies is to receive some direct medical benefit for their child; only 20 % report wanting to help future patients (Truong et al. 2011). Most adolescent patients hope enrolling in phase I studies will extend their lives (Miller et al. 2013), but many also endorse wanting to help other patients (Wendler et al. 2012). It is the responsibility of the interdisciplinary team to explore patients’ and families’ understanding of phase I research, their reasons for enrolling, and their hopes and other goals of care. Often, these conversations can be easily integrated into other decision-making and ACP discussions.

Patients with advanced cancer may also request continued, nonexperimental cancer-directed therapy, even when its efficacy is limited. This request may align with goals of care and parenting roles, as well as alleviate patient suffering and prolong time with family. Sometimes, however, cancer-directed therapy can directly impede quality of life, due to side effects, the need to be proximal to the hospital, restrictions in activities, and other clinical (or investigational study) requirements. Understanding patients’ and/or families’ specific values and goals of cancer-directed therapy therefore becomes more important over time. Eventually, quality of life may become a major factor in patient, parent, and provider decision-making, and providers must enable families to support the child’s quality of life, perhaps by giving them permission to reframe their goals and responsibilities.

The integration of palliative care has never necessitated forgoing other forms of treatment; however, because families and providers may link palliative care and hospice with the cessation of cancer-directed therapy, referrals to palliative care may occur late. In the United States, this bias may extend from legislation passed in the 1980s, defining hospice as a Medicare benefit that required patients to have a prognosis of 6 months or less and because of limited reimbursement mechanisms also required patients to forgo continued cancer-directed therapy. Outside of the United States, hospice is a term used to describe a philosophy of care that focuses on alleviating the suffering of patients with advanced illness. It may also be used in association with a specific location for end-of-life services.

Hospice care for pediatric cancer patients remains largely underutilized in the United States, perhaps because it is associated with similar barriers to palliative care services (Fowler et al. 2006). One approach to promoting early and ongoing PPC involvement is the relatively new provision of palliative and hospice care concurrently with cancer-related therapies. In 2010, the United States implemented the Affordable Care Act (ACA) in an effort to expand population access to multiple medical services. Included in this program was a provision, Section 2303, termed the “Concurrent Care for Children” Requirement (CCCR), allowing publicly insured children with life-limiting illness to receive “curative” treatments like chemotherapy or dialysis concurrently with hospice care. The program has several notable limitations, including a stipulation that children receiving this benefit have a life expectancy of fewer than 6 months. While the program has the potential to allow children with high needs to receive complete end-of-life care at home, few states have successfully implemented models of concurrent care for pediatric cancer patients to date. The long-term effect of this critical change has yet to be measured (Bona et al. 2014).

As research and clinical efforts have focused on improving quality of palliative and end-of-life care for children with cancer, the location of their deaths has also received attention. In the United States, most children and families may choose between death at home (with or without hospice) and death in the hospital. Very few states offer free-standing hospice facilities. In contrast, the latter are more standard in the United Kingdom and Canada. In Canada, parents of children with cancer and their health-care providers tend to prefer home as their first choice for end-of-life care and for the location of their child’s death (Kassam et al. 2014). Most importantly, however, is that families have the opportunity to plan the location of their child’s death, because in doing so, they experience less decisional regret (Dussel et al. 2009).

One challenge in enabling children to die at home is concurrently meeting their pain and symptom management needs. Children may often return to the hospital for intractable symptom control or because parents become overwhelmed or exhausted while caring for them. Children who receive PPC services, however, are more likely to die in their or their parents’ preferred location, providing another testament to the value of palliative care team integration in pediatric cancer care (Postier et al. 2014; Friedrichsdorf et al. 2015). Comprehensive end-of-life care should therefore address these preferences early, in order to plan and accommodate patient and/or family wishes.

Pain and symptom management is often the initial or primary reason for a palliative care referral. Parents of children with cancer report that pain, emotional distress, fatigue, and nutrition “cause the most problems” during cancer treatment, and these problems are associated with parent and survivor distress (Heden et al. 2013; Poder et al. 2010; Rosenberg et al. 2013). The most commonly reported symptoms are fatigue, reduced mobility, pain, anorexia, nausea, vomiting, and disordered mood, and these are often intertwined (Jalmsell et al. 2006). Many physical symptoms have psychosocial components and vice versa (Wolfe et al. 2015). For example, 96 % of children experience fatigue in the last month of life, and suffering from fatigue is associated with additional suffering from pain, dyspnea, anxiety, sadness, fear, diarrhea, nausea, vomiting, and anorexia (Ullrich et al. 2010b).

Certain cancer types, treatment regimens, and patient-ages may be related to symptom complaints and their management. For example, patients with central nervous system (CNS) tumors have higher prevalence of neurocognitive deficits, impaired speech, or other motor dysfunctions (Jalmsell et al. 2006). Some studies suggest patients with leukemia or lymphoma report worse physical and psychosocial quality of life compared to those with solid non-CNS and CNS tumors, and recent HSCT is associated with worse psychosocial health (Tomlinson et al. 2011). Other studies suggest that patients with bone sarcoma have greater disability and inferior quality of life (Nagarajan et al. 2004; Barrera et al. 2012). Finally, patients diagnosed as adolescents and young adults may be at higher risk of depression, anxiety, and fatigue (Poder et al. 2010).

At the end of life, the prevalence of troubling symptoms is particularly high; 89 % of bereaved parents believe their children suffered “a lot” or “a great deal” in the last month of life (Wolfe et al. 2000b). Unfortunately, treatment of specific symptoms is frequently inadequate. For example, bereaved parents report their child received treatment for pain (80 %), dyspnea (70 %), or fatigue (20 %), but these treatments were successful in only 27, 16, and 5 % of cases, respectively (Wolfe et al. 2000a). Additionally, the symptoms that become the most distressing to parents are those which are persistently unrelieved (Pritchard et al. 2010), and parents are more likely to consider hastening their child’s death if they believe their child is suffering (Dussel et al. 2010).