© Springer International Publishing Switzerland 2017
Lawrence Berk (ed.)Dying and Death in Oncology10.1007/978-3-319-41861-2_1111. Pediatric Cancer and End-of-Life
(1)
Pediatric Palliative Care and Ethics, University of Kansas Medical Center, 3901 Rainbow Blvd., Kansas City, MO MS4004, USA
11.1 Introduction
Children continue to die from cancer. Despite the decline in mortality, pediatric cancer remains the leading cause of death from disease in children ages 1–14, exceeded only by accidental death. Childhood deaths due to cancer represent 58 % of disease-related deaths, outnumbering all other causes of death by disease combined (Curesearch 2015). An estimated 1,250 cancer deaths were expected to occur among children 0–14 years of age in 2015 and an additional 610 among adolescents 15–19 years of age, totaling almost 2,000 annual cancer-related deaths in children and adolescents between birth and 18 years of age (American Cancer Society 2015). For the remainder of this chapter, the term child or children will be used to describe individuals from birth to age 19.
The population referred to as Adolescents and Young Adults (AYAs) with cancer overlaps the adolescent population included in pediatric cancer statistics. The AYA population includes individuals with pediatric forms of cancer, between the ages of 15 and 40, treated on pediatric protocols. The treatment may occur in either a pediatric or adult program, and patients between 15 and 19 who are treated in an adult program will typically be counted in the adult statistics. As a result, it is difficult to accurately identify the number of 15–19 year olds diagnosed with or who have died with cancer. In 2011, an estimated 69,212 AYAs were diagnosed with cancer, which represented the leading cause of disease-related death in this age group (National Cancer Institute 2014).
Each day in the United States, approximately 43 children receive a new diagnosis of cancer (American Cancer Society 2015). In 2014, an estimated 15,780 new cases of pediatric cancer were diagnosed among children birth to 19 years in the United States. Overall, childhood cancer incidence rates increased by 0.6 % per year from 2007 to 2011. However, the 5-year relative survival rate increased from an average of 58 % in the mid-1970s to 83 % from 2004 to 2010 (Ward et al. 2014).
Despite improvements in diagnosis and treatment of pediatric cancer, it remains impossible to predict the outcome for a specific child. Thus, many children and families continue to struggle with the diagnosis, treatment, and unknown prognosis of pediatric cancer. Pediatric cancers may be cured, become a long-standing chronic condition, or lead to death. Regardless of the trajectory of a child’s cancer, all children with cancer benefit from support and assistance in maintaining or improving quality of life while on cancer treatment. In caring for children with cancer, therefore, it is prudent to follow the words of Benjamin Disraeli, “I am prepared for the worst, but hope for the best” (Disraeli 1833).
Whole-patient cancer care includes not only medical therapies, but also treatment directed at the psychosocial well-being of patients and their families (Adler and Page 2008). Whole-patient care enables continued cognitive, emotional, and social development in children living with or dying with cancer. Recent literature has called for the integration of psychosocial care into standard medical oncology practice including routine screening for psychological distress and identification of those in need of additional support (Carlson et al. 2012; Jacobsen and Wagner 2012). There is inconsistency of available psychosocial resources among pediatric cancer treatment sites. In the past, psychosocial care for children with cancer was initiated when problems such as depression, anxiety, or school avoidance became evident. Today, it is widely recognized that psychosocial care should be provided early in the trajectory of the child’s cancer experience. Children with cancer, and their family members, have multiple psychosocial issues and require a unique set of supports to help them conceptualize and operationalize the issues they will confront during treatment.
Coping strategies will vary from child to child, disease to disease, and age to age. The strategy may change as the child moves through developmental stages. Children undergoing cancer treatment need healthcare professionals, family, friends, teachers, coaches, spiritual leaders, and others in their natural environment to recognize their needs and that they differ from those of adults. Only then can young patients develop the necessary trust to allow those same individuals in to provide support when they are dying. The psychosocial state of the dying child is affected by developmental stage, the effectiveness of the child’s support system, symptom burden, relapse of cancer, pain and symptom management, treatment modalities and a wide range of other factors. The child’s developmental level will inform how well he is able to process the reality that some diseases are incurable and result in death. How the child transitions out of life will also be affected by the family’s level of distress. Many variables will impact the child’s final days and hours. The period leading up to the child’s death requires a great deal of sensitivity, honesty, and courage on the part of the healthcare team as they help the parent, child, siblings, and others assign meaning to the process and prepare for the child’s death (Lee et al. 2006).
The goal of this chapter is to provide a foundation for the development or growth of pediatric psychosocial care in various settings by addressing the psychosocial needs of the child before and during the end-of-life (EoL) period, and the needs of family and community before, during, and after the child’s death. This chapter will discuss the need to assess and treat a child’s pain as a prerequisite to addressing the child’s psychosocial needs; elucidate the role of developmental stages in the child’s response to having cancer and to dying; examine the unique needs of family members of the child with cancer; discuss end-of-life decision making; identify resources for families in the development of posttraumatic growth and resilience; and synthesize this information alongside the role of pediatric palliative care in partnership with the pediatric psycho-oncology team.
11.2 The Child with Cancer
11.2.1 Child Development in the Child with Cancer
A child’s experiences while living with cancer may have a significant impact on the experience of the child dying with cancer. Prior to discussing ways to care for the dying child and his family, a brief discussion of child development is warranted. The development of the child continues throughout cancer treatment and affects the manner in which the child lives and dies. In parallel, the cancer experience has a separate impact on the child’s development.
The psychosocial impact of cancer is significant from toddlers to teens. Children are dynamic beings who develop as a result of their experiences. Cancer impacts growth and development as the child is limited in participation at school, isolated from friends, undergoing painful procedures, encountering physical changes, and suffering from the symptoms of the disease and treatment. It is no surprise that ongoing evaluation and treatment of the youngster’s psychosocial needs is a prerequisite to ensuring optimal comprehensive child-centered cancer care. It is recommended that the child with cancer and his family members receive routine systematic assessments of their psychosocial healthcare needs (Kazak et al. 2015) and the provision of psychosocial supports and interventions, including psychiatry as needed, indicated by the assessment (Steele et al. 2015).
Child development does not occur in a static environment but the cancer experience may create such an environment. The infant who is confined to a crib does not have the opportunity to explore his environment. Toddlers, preschoolers, and young school-aged children learn through play and experiences with objects and may experience isolation and disruption in their active play and learning opportunities during cancer treatment. Older school-age children and adolescents are engaged in developing relationships with their peers and gaining independence from their parents. Suddenly, dependence on parents returns right at a time when they yearn to branch out on their own. Amputation, limb-sparing surgery, hair loss, or weight changes may result in reluctance to engage with peers. As cancer treatment progresses, some children see friends less often and may feel that interactions with peers no longer afford the rich opportunities they once did. It becomes difficult to identify characteristics that inform who they will be as an adult. Social skills, academic achievement, and answering the question “Who am I?” are threatened by the cancer diagnosis. This is challenging for the child whose treatment lasts a few months but devastating for the child who spends months or years on treatment and who may be facing death. Children with cancer are often described as wise beyond their years but they may also be socially, emotionally, and spiritually delayed due to the interruptions and negative impact of cancer on development.
11.2.2 A Steep Learning Curve for Children
Despite similarities in diagnoses and treatment children are not little adults in their adaptations to cancer. When an adult is diagnosed with cancer, they have already defined many aspects of who they are. They recognize the uniqueness and qualities of various relationships, how they cope with adversity, who can assist them during the course of their disease, and how they will navigate their world of work. Adults can make decisions that will lighten the psychosocial, emotional, and spiritual challenges of the cancer experience, identify who will be instrumental in helping them through the diagnosis, request help in developing coping strategies, and ask family and friends for assistance during difficult times.
The cancer experience presents very different challenges for a child. Although most children have parents and other caring adults who provide significant support throughout the cancer journey, children sometimes have needs that those adults cannot identify or address. For example, young children may not yet have the vocabulary to articulate their fears, and older children may not yet be able to define their needs, or may be hesitant to verbalize their needs to parents for fear of adding to their parents’ burden. Children usually cannot ask friends to step in or to be with them, even during critical stages of development when peer friendships are the most important relationships. Approaching the EoL as a child is the ultimate assault of feeling different and intensifies the child’s awareness that they are not “normal.” If the dying child has not continued his relationships with friends throughout his illness, he may feel very abandoned at the EoL and his friends may have more difficulty with grief.
As children progress through the cancer experience, they may also move through new developmental stages that bring increased understanding and anxiety regarding their diagnosis. For example, the 5-year-old diagnosed with stage IV rhabdomyosarcoma may be unaware of the significance of his diagnosis but in a year or two he may question if he is dying from the disease. He may feel a lack of trust toward parents and healthcare providers if they have not previously communicated honestly with him. Or the adolescent with acute myelogenous leukemia, who previously allowed parents to assume all responsibility for decision making, may suddenly exert his autonomy and choose to make his own decisions about his impending death. Similarly, children may grieve anew and with more intensity as they pass through developmental stages that bring with them a new understanding of their losses or impending EoL. Healthcare providers and parents should anticipate that these changes will occur and attempt to understand upcoming developmental stages before the child needs intensified support.
11.3 First: Pain and Symptom Management
Pain in a dying child may prevent him from being able to identify how to express his needs or where to go for help. Healthcare professionals should assess the child’s pain frequently, seek to understand the child’s pain, and work until adequate pain control has been achieved. Simultaneously, psychosocial care team members should partner with the pediatric oncologist to identify physical, psychological, social, emotional, and spiritual needs that impact the experience of pain. Pain and symptom management enables the child to focus on the psychosocial and existential pain and suffering that intensify at the EoL.
Being a child dying of cancer-related causes is even more difficult than being a child living with cancer. It is a challenge for the child to be heard in the healthcare setting. Pediatric patient reports risk being overshadowed by the reports of parents, especially as the child approaches death. Healthcare providers must work to hear the voice of the dying child related to pain, symptoms, concerns, and health-related quality of life (HRQL). Patient-reported outcomes, or PROs, are not often found in pediatric research. Rather, many studies opt to report parent or healthcare professional responses or extract data from the medical record. About 85 % of studies on children with cancer at the EoL did not include PRO measurements. One reason cited was the lack of reliable and valid instruments for use in clinical care (Hinds et al. 2007). In addition, measuring patient-reported pain can vary based on the developmental stage of the child and can be impacted by other psychosocial variables present in the ill or dying child.
HRQL measures can provide insight into the experiences of children with cancer at EoL. Measuring HRQL in children with cancer is complicated by the unique symptoms and disease burdens that make comparison to peers with or without disease difficult. Thus, it is not surprising that parent reports of HRQL have been more frequently used in research (Hinds et al. 2007). Despite the challenges, it remains best practice to attempt to gather PROs to most effectively assess the child’s pain and symptoms. The recommendations from a joint workshop of the Institute of Medicine and the American Cancer Society suggest that researchers routinely collect and integrate a core group of PROs and parent reports, into the healthcare delivery system as a method to improve clinical care (Nass et al. 2015).
Children with cancer at EoL require close psychosocial surveillance in an attempt to provide intervention in remediable areas. Sadness, pain, nausea, lack of appetite, and feeling irritable were reported by one-third of children at EoL with the majority rating their pain as moderate to severe and half describing their pain as “highly distressing.” Children who had received chemotherapy within the past 4 months reported greater symptom burden (Collins et al. 2000). More research is needed to determine if children who are not on active treatment or who are at home may experience less significant symptoms at the EoL. Still, parent reporting may be the only available report of a dying child’s experience. Interviews of parents whose children died of cancer a mean of 3 years previously found that fatigue, pain, dyspnea, and poor appetite were the most frequent symptoms at their child’s EoL, with 89 % of parents reporting that their child suffered “a lot” or “a great deal” (Wolfe et al. 2000). A decade later, 84 % of parents at a mean of 4.5 years after their child died of cancer still reported that their child had suffered “a lot” or “a great deal” from the same symptoms of pain, fatigue, and poor appetite (Heath et al. 2010).
The World Health Organization guidelines acknowledge the large proportion of children with cancer who experience pain and the “widespread inadequacy” of pain management efforts (WHO 1998). The data suggest that the majority of children diagnosed with cancer each year in the United States experience moderate-to-severe pain throughout their treatment (Mercandante 2004; Gordon et al. 2005). Painful procedures, disease- and treatment-related pain, and pain at the EoL continue to result in physical and psychosocial distress for the child with cancer. There may be a variety of reasons why pain and symptom management remains onerous. Palliative and hospice models of care are assumed to afford an increased focus on pain management. However, pediatric oncologists acknowledge that they do not always refer patients to pediatric palliative care. They would be more likely to refer patients if hospice facilities would admit a child who was still receiving chemotherapy, if palliative care was renamed “supportive care” or if palliative care was not perceived negatively by their patients (Wentlandt et al. 2014). Children who receive Medicaid are eligible for concurrent care under the Affordable Care Act of 2010. They may continue aggressive, life-prolonging, or curative treatment along with hospice care.
There is increasing responsibility for parents to manage their child’s pain at home both during treatment and possibly at the EoL. Parental attitude about children’s pain may play a significant role in the administration of pain medication. Parents are identified as most likely to undermedicate a dying child’s pain due to misconceptions regarding appropriate analgesic use and how children express pain (Fortier et al. 2012). Parents and children may be concerned about addiction, the side effects of medications, or may believe that cancer pain at the EoL is unavoidable (Ameringer et al. 2006). Adolescents may hesitate to report pain if they feel that their opinions will be ignored, that their social activities will be restricted (Wild and Espie 2004), or that they may have to undergo undesirable diagnostic tests (Ameringer 2009). Assessing a child’s cancer pain and its impact on function and quality of life requires conscientious and continuous communication, ideally with a variety of individuals, always including the child and, whenever possible, the parents, bedside nurse, child life specialist, and others who have the opportunity to observe the child in various settings.
A multimodal approach to pain treatment maximizes function by providing children with a degree of control over their pain. Utilizing complementary pain management strategies to supplement pharmacologic methods may best improve function as the child’s pain decreases. Complementary strategies including distraction, breathing for relaxation, progressive muscle relaxation, and guided imagery are among the techniques that psychologists, psychiatrists, social workers, and other healthcare professionals may employ with children with cancer and their parents. Hypnosis has been suggested to have efficacy in the reduction of pain and anxiety during venipuncture, bone marrow aspirate, and lumbar puncture. Additional research is needed that incorporates adequate power, appropriate control populations, objective measurement of results, and long-term follow-up to further legitimize hypnosis as a care modality for children living and dying with cancer (Wild and Espie 2004). It is recommended that parents learn complementary strategies so they may act as their child’s coach and have the opportunity to directly contribute to their child’s pain management. In addition, parents may opt to employ the strategies for personal use in coping with stress and anxiety.
The developing child brings changing physical, psychological, social, emotional, and spiritual needs to the EoL. Achieving adequate pain and symptom management is the first step in developing a healthy psychosocial foundation from which the child can move forward in their personal experience. The existence of pain may disable the dying child as he attempts to address the psychological, social, emotional, and spiritual issues that intensify near the EoL.
11.4 The Family
Cancer in childhood can impact HRQL, mental and physical health, activities of daily living, family dynamics, identity formation, parental and sibling roles, school success, a sense of meaning, and spirituality (Jones 2012). Pediatric cancer is a family illness and in order to “die well” from cancer, a child and his family must first learn to navigate all of those issues to first live well with cancer. When a child has cancer, the roles of each family member change, and the family’s balance may be threatened or lost entirely. As the needs of each family member come into focus it is hoped that resources will more fully address those needs.
The challenges begin at the time of diagnosis, and follow each family member for the rest their lives. When a child dies, it is not unusual for family members to have very different experiences during the dying process and in bereavement. Some individuals may experience posttraumatic growth, or the concept that difficult life struggles can lead to an individual’s growth in positive life-changing directions (Calhoun and Tedeschi 2004), and resilience will be realized. For others, struggling with anxiety, depression, guilt, and other negative emotions may become a lifelong reality. Healthcare professionals within the field of psycho-oncology assess family members for psychosocial needs and treat those issues. The recent development of psychosocial standards for children with cancer and their families, from diagnosis through bereavement, may begin to bridge the gap and ensure that all children dying with cancer and each family member will receive optimal psychosocial assessment and treatment (Wiener et al. 2015).
11.4.1 Parents
Although research regarding parents’ experience at the EoL of their child is increasing, there are still more questions than answers. Culture, ethnicity, and religion should be considered to ensure that healthcare providers are sensitive to family needs and beliefs. However, the broad overview of cultural beliefs should be used only to identify the most cultural sensitive and effective way to start the conversation about EoL with the family. Despite religion or ethnicity, every child and parent is an individual and each will express unique beliefs and needs that will guide the EoL discussion. The needs of dying children and their parents become evident when trusting relationships with HCPs develop. Ongoing, thoughtful, effective, and compassionate communication can open doors that inform healthcare providers about the best care for that individual child, parent, or family member.
Parents’ needs are most critical at three stages surrounding their child’s EoL: when making decisions related to their child’s impending death, immediately preceding and at the time of death, and during bereavement. Parents have extremely difficult decisions to make when their child is dying with cancer and it is important for healthcare professionals to work with parents and support them in decision making. The personal preferences or beliefs of the healthcare professional must be removed from the decision making process unless there is concern that the child is suffering or being harmed in some way by parental decisions. Some parents want their child to receive aggressive life-sustaining treatment (LST) until the child’s body stops functioning. Other parents request that all life support be removed for fear that their child will suffer or to ensure that the child dies with dignity. It is agonizing for parents to make decisions that will have some impact on either their child’s quality of quantity of life. Parents may state that they cannot possibly make a decision and may ask the physician to make the decision regarding when LST should cease. It is widely accepted that parents should make these decisions in the best interest of their child. Regardless of what decision a parent makes, they will carry the decision with them for the rest of their life. Compassionate, gentle support and guidance from the healthcare team will enable parents to make the choice that they believe is in the best interest of their child.
The current orthodoxy is that most people would prefer to die at home. However, freedom from distress and pain and being surrounded by loved ones tends to trump location of death as EoL approaches (Waghorn et al. 2011). Dying at home does not constitute a “good death” for all individuals. Parents should feel supported in whichever location is chosen for their child’s death. Some children and parents prefer to continue to receive care and support in the hospital, especially when strong relationships have developed with the cancer care team. Children who are permanently ventilator dependent may have to live in a ventilator care center or a nursing home. Healthcare professionals should support the family with their choice regarding location of death and focus on identifying ways to improve quality of care in all places children with cancer die, including hospitals, nursing homes, and the child’s own home. When a child is dying in the hospital parental needs increase. These needs include a place to sleep; the ability to participate in their child’s care; the opportunity to be with their child and to be able to talk to, touch, hold, and protect their child; to know that their child’s pain is adequately controlled; to receive complete information about their child in a way that they can understand; to feel free to ask questions; and to have their authority as a parent respected. When these needs are not adequately met parents report higher levels of complicated grief symptoms 6 months after the child’s death (Meert et al. 2012).
For some children with cancer, a consequence of extended medical treatment is significant changes in their physical appearance. Parents vividly remember these changes and may feel frustrated and hurt that their child had to suffer. Many parents find it helpful to share in their child’s daily care during this process. Parents report sadness when they cannot hold their dying child and that they feel comforted when they are physically close to the dying child, lying with the child in one bed, holding the child in the hour of or after death, and washing the child after death (Falkenburg et al. 2016). Parents’ transition from providing active care for their child in the hospital to the days after the child’s death is a stark change. Offering tangible memories of the child may aid in reducing the contrast of having a child one day and not having their child the next. Blankets, pillowcases, stuffed animals, or articles of clothing that carry the scent of the child may be treasured by parents. Other memories may be created by nurses or child life specialists and placed in a box to be given to the parents. Locks of hair, molds of hands and feet or photographs of the child and family may provide additional comfort in coming days. Advance care planning should include an understanding of what will bring comfort to the individual family during the days, hours, and minutes prior to the child’s death.
Retrospective studies reveal common themes of what is important to parents when a child is dying with cancer. In a meta-ethnography of parental decision making at the EoL of their child, three major themes were identified by parents as being of the greatest importance as their child was dying. First, a multifaceted concept of communication emerged to include comprehensive discussions regarding all aspects of their child’s diagnosis, prognosis, positive and negative effects that might alter treatment decisions, and emotional communication including comforting the parent. Next, parents expressed wanting more time with their child as a key factor in considerations and decision making. Parents shared a desire to have both palliative care and cancer-directed therapy concurrently. Some parents, in hindsight, would have chosen to stop medical care sooner. Finally, a desire to understand their child’s prognosis was cited as a factor when making EoL decisions for their child. Many parents acknowledged that their goals approximated those of their child’s oncologist when the doctor communicated effectively regarding prognosis. Differences between the oncologist’s prognosis and that of others, such as the intensivist or palliative care specialist, for example, were difficult for parents to understand (Heinze and Nolan 2012). These themes may serve to provide a point of reference for healthcare professionals as they work to communicate effectively with the child’s family as the child is dying.
11.4.2 Siblings
Siblings of children with cancer have long been a population whose needs are recognized but not always addressed. While the body of research regarding bereaved parents’ psychosocial outcomes increases, there continues to be a paucity of literature regarding the needs of siblings when a brother or sister dies (Rosenberg et al. 2012). Siblings of children dying with cancer are a psychosocially at-risk group and should be provided with appropriate supportive services. Parents and professionals should be advised about ways to anticipate and meet siblings’ needs, especially when siblings are unable to visit the hospital regularly (Gerhardt et al. 2015).
Increased rates of anxiety, depression, and illicit substance use during the year after their loss was reported in a study of siblings who had lost a brother or sister about 12 years previously. The participants cited lack of communication, poor preparation for their sibling’s death, not getting to say goodbye and a perception that the cancer experience had a negative impact on relationships in their family as the reasons for their challenges, which returned to baseline after the first year. It was agreed among nearly all siblings that, despite those improvements, they still were affected by the loss 12 years later and about half reported that the experience affected their career and educational goals (Rosenberg et al. 2015). Other investigations identified siblings with a higher risk of anxiety when their social support needs were unmet the last month of their brother or sister’s life (Eilertsen et al. 2013) and lower self-esteem, difficulties falling asleep, and lower maturity levels 2–9 years post the death of a brother or sister (Eilegård et al. 2013). Of note is that siblings may also experience posttraumatic growth after their brother or sister’s death. Nearly all siblings report changes in perspectives, interests, and relationships in the year after their brother or sister has died. These changes include positive alterations, which include personal growth, new meaning, or purpose (Foster et al. 2012). Severe psychopathology is rare but there are several reviews that identify anxiety, depression, posttraumatic stress, lower quality of life, decreased healthcare utilization, and disruption to academic and social functioning as long-term problems for bereaved siblings of children who died with cancer (Alderfer and Hodges 2010; Alderfer et al. 2004; Sveen et al. 2014; Kacel et al. 2011). Bereaved siblings also frequently exhibit greater challenges than peers who are not bereaved (Wilkins and Woodgate 2005).
The field of psycho-oncology may have the chance to learn from missed opportunities. Of note is The International Society for Pediatric Oncology guidelines for the support of siblings of children with cancer published almost 20 years ago (Spinetta et al. 1999). The guidelines called for healthcare professionals and parents to involve siblings immediately after the child’s diagnosis with cancer and to keep them informed throughout the cancer trajectory, emphasizing the positive and optimistic aspects of treatment whenever possible. Despite these recommendations, supports for siblings continue to be sparse in many programs. Access constitutes a significant challenge as most healthcare professionals involved in sibling support are not at the hospital or treatment center when siblings typically visit on nights and weekends. Some siblings may have limited or no opportunities to visit if the family lives a distance away from the cancer treatment center.
A new opportunity to identify ways to support siblings presents in a more recent set of guidelines (Gerhardt et al. 2015). The guidelines describe broad categories that should be considered in the standard of care for siblings of children with cancer including sibling stressors, communication needs, psychosocial impact, bereaved siblings, supportive care and barriers. The findings related to bereaved siblings describe siblings’ desire to be involved and informed when their brother or sister is dying. In addition, the guidelines emphasize the potential for bereaved siblings to have increased incidence of internalizing problems, difficulties in peer relationships, and long-term outcomes affecting educational and career goals. It is recommended that ongoing support is provided to families, especially following the death of a child.
Siblings usually want to be where their brother or sister and parents are at the end of the child’s life. Parents’ desire to protect their healthy child(ren) may result in denying visits so the sibling does not have to see his brother or sister as they are dying. Parents should receive counseling and support in how to address this issue with their other child(ren). Siblings should be offered the opportunity, with age-appropriate guidance and preparation, to see their brother or sister who is dying to be able to say their good-byes (Giovanola 2005) and to participate as much as they desire in funeral planning and attendance.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
