Keywords

Introduction

A life-threatening or life-limiting illness in childhood is universally stressful for patients, caregivers, and their families. At some point throughout the course of treatment for such conditions, patients may require care in a pediatric intensive care unit (PICU). By its very definition, care provided in the PICU is intensive; sophisticated medical technology helps monitor and support basic bodily functions, and nursing is available at the bedside 24 hours a day. While many admissions to the PICU are acute and unplanned, others are planned postoperatively to allow for close monitoring following a major procedure. Regardless of the reason for a PICU admission, pediatric patients and their families are coping not only with the medical acuity of such an admission but also with the far-reaching impact of the experience.

This chapter will address the psychosocial aspects of intensive care, specifically for immunocompromised patients and their caregivers. It will highlight the emotional needs of patients and families in the PICU and consider how psychological interventions and pediatric palliative care (PPC) aim to bolster adaptive adjustment and coping and maximize well-being. It is important to recognize the inherent diversity of the population of immunocompromised patients receiving critical care. There is a wide range in terms of their medical diagnoses, the duration and intensity of medical treatments experienced, and most fundamentally, in terms of the individual differences that exist across patients and their family systems. As such, the experience of admission to an intensive care unit for this population should be considered within the larger context of a patient and family’s global experience of treatment.

Psychosocial Care

Patient Experience

PICU hospitalization results in significant distress and psychological sequelae for approximately 25% of youth admitted to the PICU, even up to a year after discharge [1,2,3]. Caregivers report many challenges for their children post-discharge, including a decrease in emotional well-being, confidence, and self-esteem; increased anxiety; sleep disturbance; social isolation; changes in memory, attention span, and cognitive functioning; and symptoms of posttraumatic stress disorder (PTSD) [2, 4, 5]. In an effort to mitigate some of the potential negative psychological effects of a PICU hospitalization, it is important to consider how children of different ages and developmental levels might make sense of their experiences.

Infants and Toddlers (Ages 0–2 Years)

Children in this age range are beginning to form strong bonds with their caregivers and are developing a sense of security within those relationships. Infants and toddlers experience separation from caregivers, pain, exposure to unfamiliar people, disruption of normal routines, and restriction of movement, as variables that may impede their development of security and trust. Although research points to the need for increased caregiver participation during a child’s hospital stay, in reality, this may not always be feasible [6]. Ideally, interventions would include caregiver presence and interaction in the form of holding, soothing, and play whenever possible. Maintaining a consistent daily routine (i.e., for waking, feeding, naptime, bathing, and bed time) can help very young children build a sense of security, comfort, and trust in an otherwise unpredictable environment.

Preschool Children (Ages 3–5 Years)

Preschoolers who have spent time in intensive care settings have largely been excluded from psychological outcome studies, likely due to the fact that they are more challenging to assess and validated instruments for this age group are lacking [7]. The cognitive development of preschoolers is marked by egocentricity, the emergence of symbolic thought, and magical thinking – the notion that one’s thoughts, desires, and wishes can have an effect on the external world. Providing simple, clear, and factual information about the child’s illness, required medical care, the potential impact on the child’s regular routine, and the anticipated expectations for caregiver presence are all important in facilitating adaptive adjustment and functioning. Pretend play, story books, music, videos, and drawing can be helpful ways to engage preschoolers around their illness and hospitalization experience and to encourage mastery of new routines and medical procedures.

School-Aged Children (Ages 6–12 Years)

Following a PICU hospitalization, a number of school-aged children report increased anxiety, fear of medical settings and interventions, delusional memories and hallucinations, and a negative impact on their friendships and sense of self [1]. This age group has the capacity for more logical thinking but continues to view the world in a fairly concrete way, with an emphasis on fairness and on cause and effect [8]. Some children may want to participate in discussions around their medical care, while others may shy away from medical discussions and decision-making opportunities. Encouraging communication between patients, caregivers, and medical providers is a critical step in determining how to best meet the unique needs of each patient [9].

Adolescents (Ages 13–18 Years)

This period marks the development of more complex thinking processes, including abstract thinking, the ability to consider multiple viewpoints, and the ability to reason and form new ideas. With this heightened capacity for abstract thought, adolescent patients are increasingly able to make informed decisions about their medical care. It is important to note that each child and adolescent’s cognitive development progresses at varying rates and that emotional distress can sometimes interfere with one’s ability to think with complexity about the intricacies of a situation. Providers should meet regularly with adolescent patients individually to provide them with opportunities for processing their emotions and talking about their decision-making processes without parental input.

Young Adults (Ages 19–25 Years)

Young adults are able to fully understand abstract concepts, consider the consequences of their decisions, and appreciate personal limitations. They have moved forward with the process of securing their autonomy and are likely more skilled in independent decision-making than they were in adolescence. This age group may also rely more on support from a romantic partner than on their parents. When in a medically compromised state, however, the role of parents may suddenly become more prominent again. This shift in independence may lead to emotional distress and regression. Identifying a healthcare proxy when appropriate and discussing preferences for care, communication, and decision-making will contribute to adaptive experiences for young adults in the PICU.

Caregiver Experience in the PICU

In the context of a PICU admission, particularly when such admissions are characterized by unexpected or particularly complicated medical circumstances, it is understandable and adaptive for patients, as well as their caregivers and loved ones to experience very strong emotions. The experience of caring for, bearing witness to, and recovering from the treatment of a critically ill child can be psychologically, socially, and financially overwhelming for family members. Perhaps not surprisingly, parents of critically ill patients are particularly vulnerable population who have been found to be at risk for anxiety, depression, and PTSD symptoms [10, 11]. In practical terms, it is important to understand that the manner in which such feelings are experienced, processed, and expressed are highly variable and dependent on a number of factors, including the specific medical circumstances, one’s temperament, and one’s ability to reliably access effective coping strategies and utilize social supports. Some caregivers may become more withdrawn or tearful, while others may become more irritable or angry. Some may feel most comfortable with a constant presence at the bedside, others may need more time away from the hospital setting.

The literature has increasingly recognized the impact of a child’s critical illness on family functioning, and clinical practice has been expanding its focus from “patient”-centered care to “family”-centered care. This perspective highlights the significant role that the family plays in patient care, as well as the potential impact of family functioning on patient outcomes.

Clinical practice guidelines for the support of the family in critical care were originally published in 2007 by the American College of Critical Care Medicine and subsequently revised in 2017 [12]. An international, multidisciplinary team outlined 23 recommendations that focused on communication with family members, family presence, family support, consultations and ICU team members, and operational and environmental issues. Recommendations relevant for the pediatric immunocompromised patient are included in Table 17.1. Consistent with information provided throughout this chapter, the recommendations emphasize the importance of early and consistent access to psychosocial and palliative care as well as the importance of ongoing clear communication with patients and family members. Such interventions aim to maximize the effectiveness of team-family collaboration, maximize patient and family coping, and minimize patient and family distress.

Table 17.1 ICU family-centered care recommendations for pediatric patients
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Staff Experience

Providing care to seriously ill children and their families can be emotionally demanding. The intensity of the relationship that is often formed between providers and patients and their families in the PICU can be incredibly rewarding, but also stressful, physically demanding, and interpersonally challenging. The experience of observing very ill children at end of life can call into question accepted beliefs about life and death and contribute to feelings of helplessness, guilt, and failure. Repeated stress of this nature can lead to burnout, a syndrome characterized by emotional exhaustion, decreased work productivity and accomplishment, and a sense of detachment from one’s job [13].

To prevent chronic distress and burnout, it is crucial for PICU staff to develop individualized coping strategies for managing stress, grief, and loss. These coping strategies can take many forms – maintaining professional boundaries, delegating tasks when appropriate, finding support through a peer consultation or supervision group, developing ways to process and grieve patient deaths, attending professional trainings, engaging in self-care activities, and carving out time in the workday to rest and recharge. Support staff, including psychologists, psychiatrists, social workers, and chaplains may have a role in providing guidance to multidisciplinary team members and devising interventions that may be implemented on a larger scale for staff. Staff members should never feel that they are navigating their experiences alone; consulting with colleagues and seeking support are important components to successfully managing the complex emotional experiences involved in caring for critically ill patients and their families.

An Integrated Model of Care

As highlighted above, psychosocial and palliative care supports can be helpful in supporting patients, their family members, and PICU staff providing care for critically ill children. The following sections of this chapter will describe an integrated model of care that incorporates psychosocial support as a core component of an interdisciplinary pediatric palliative care service. It should be noted though that it would not be possible for any one psychosocial provider or consulting service to adequately address the emotional, psychological, or spiritual needs of patients and families during this phase of medical care. Ideally, the support provided to patients and family members admitted on an PICU would afford them access to experts in all aspects of psychosocial care, as is clinically indicated. In addition to a PPC service, this may include the involvement of a child life specialist, resource specialist, chaplain, social worker, psychologist, and/or psychiatrist. While all members of the team work to minimize patient and family distress and maximize adaptive adjustment, each brings a unique, discipline-specific perspective and skill set. PICU providers should be aware of the supportive services available at their institutions and understand how to recruit such supports whenever necessary.

Palliative Care

According to the World Health Organization, “Palliative care for children is the active total care of the child’s body, mind and spirit, and involves giving support to the family…Optimally, this care begins when a life-threatening illness or condition is diagnosed and continues regardless of whether or not a child receives treatment directed at the underlying illness.” Rather than applying to a particular phase of care or life, pediatric palliative care (PPC) is a philosophy of care predicated on goal-concordant care, effective and compassionate communication, meticulous symptom management, and a focus on the well-being of the child and the child’s family. There is nothing about PPC that is inherently at odds with intensive treatment aimed at sustaining or prolonging life. As such PPC should not be reserved for instances in which cure is no longer possible. And, while it is often mistakenly equated with end-of-life (EOL) care, it is highly relevant both before and at EOL and applies to seriously ill children throughout their illness trajectory. Thus, children with significant immunocompromise, including those who are critically ill, stand to benefit from pediatric palliative care, regardless of treatment goals or outcomes.

Primary and Subspecialty PPC

Palliative care is in effect oftentimes delivered by the primary (medical and psychosocial) team or critical care team, or both. This may be because a PPC team is not available, or because care for the seriously ill child does not require subspecialist PPC involvement. In this scenario, the primary team and critical care teams ensure the child’s comfort, communicate with the child/family regarding prognosis and goals of care, and attend to the other aspects of palliative care while also overseeing ongoing therapy directed at the child’s underlying medical condition. In such situations, they should possess basic palliative care skills, knowledge, and behaviors.

In other situations, such as challenging symptom management, complex communication or prognostication, goals of care that are challenging to navigate, or simply the need for added support for a child or family, the involvement of a subspecialty PPC team may be beneficial. Helpful strategies for introducing PPC to families are described in Box 17.1. In 2006, Hospice and Palliative Medicine (HPM) became a formal physician subspecialty, and board eligible physicians must now have completed a 1-year HPM fellowship.

The subspecialty practice of PPC is rooted in an interdisciplinary approach, in which nurses, physicians, social workers, and members of other disciplines see patients and provide care synchronously, as opposed to asynchronously (which would be a multidisciplinary approach). Such an interdisciplinary approach to patient care allows physical, emotional, and spiritual needs of the child and their family to be simultaneously addressed and permits a synergistic approach to the multidimensional needs of critically ill children and their families. While still a nascent subspecialty, PPC teams are increasingly available, especially in the inpatient setting. A 2013 survey of Children’s Hospitals found that 69% had a PPC program [14]. When a PPC team is involved, it is often helpful to delineate the roles of each team and how they will work together collaboratively, thus minimizing confusion and duplication of roles.

Box 17.1 Strategies for Introducing Palliative Care to Families

  1. 1.

    Present PPC as a group of physicians, nurses, and social workers that partners with the child’s current team(s) to maximize support for the child and family by

    • Focusing on quality of life for the child and family (“helping your child feel as well as possible”)

    • Supporting the family’s goals of care (“helping you to think about what is most important”)

    • Helping them to think through options or the types of care that might make the most sense for them (“helping you to plan for now or the future”)

    • Serving as an added layer of support

  2. 2.

    Refrain from equating PPC with there being “nothing left we can do,” or a “redirection of care,” as this framework does not accurately reflect PPC and is unlikely to resonate well with families.

Integration of Pediatric Palliative Care in the PICU

Early, integrated access to palliative care concepts (e.g., symptom assessment and intervention, effective communication) for children with cancer and their families is recognized as a standard of care in pediatric oncology [15]. Recognition that palliative care is an essential component of comprehensive care for critically ill adult and pediatric patients is also mounting [16, 17]. Moreover, there is increasing agreement that integration of palliative care during acute and chronic critical illness may benefit patients and families facing challenges post-discharge from the PICU [17]. While use of specific criteria prompting palliative care consultation may well increase access to palliative care for patients and families who would benefit from it [18], such criteria have not been delineated for the PICU population. Levine et al. have described strategies, including prompts, or “triggers,” for early PPC consultation in the hematopoietic cell transplant (HCT) population [19], where palliative care is associated with less intensive care at end of life (e.g., intubation, receipt of CPR). Whatever the setting, strategies promoting early integration of PPC are determined by the specific population and its needs, available data, and the resources and support available.

Symptom Management

Intensive symptom management is a cornerstone of high-quality PPC. Critically ill, immunocompromised children may experience a range of pain and non-pain symptoms. For example, children with cancer experience multiple symptoms during the last month of life, with fatigue, pain, and dyspnea resulting in significant suffering. Palliative care services are associated with less child suffering from symptoms [20]. For all children, and especially those with advanced illness, intensive management of symptoms should be a top priority. Some of the most relevant symptoms are discussed below.

In all instances, integrative therapies should be part of the symptom management plan. Depending on the symptom and patient preferences, such integrative and mind/body therapies might include guided imagery, relaxation, hypnosis, art/pet/play therapy, massage, heat/cold, distraction, Reiki, acupuncture, clinical aromatherapy, prayer, nutritional supplements, and cannabidiol oil. Many families utilize such strategies though they do not share this information with their care team. It is therefore recommended that clinicians inquire about their use, and when permissible, support their use in the PICU.

Pain

Though triggered by tissue damage, pain is often greatly influenced by cognitive, behavioral, emotional, social, and cultural factors. Critically ill children experience pain that stems from a range of sources, including the physiology of their underlying condition, procedures, and other aspects of medical care. Effective pain relief is necessary to mitigate suffering and to prevent central sensitization, a central hyperexcitation response leading to escalating pain.

Routine assessment is a central component of effective pain relief. A range of behavioral and physiologic assessment and self-report tools exist. While self-report is considered the gold standard in symptom management, tools based on pain behaviors (e.g., grimacing, positioning) may be of particular utility in the PICU, when children may be unable to verbally self-report.

The WHO Pain guidelines were recently revised to contain two steps: step one for mild pain and step two for moderate/severe pain. For mild pain, acetaminophen ibuprofen, choline magnesium trisalicylate or celecoxib may be sufficient. Celecoxib, which has low antiplatelet activity, may be a good choice for children with thrombocytopenia. In the end, opioids are commonly needed to control pain in critically ill children.

A previously used intermediary step, the WHO pain guidelines proposed weak opioids, such as codeine, for moderate pain. However, codeine should be avoided when at all possible due to its potentially dangerous side effect profile and its lack of superiority over non-opioids. Its most dangerous side effects, oversedation and respiratory depression, are due to polymorphisms in the CYP2D6 gene in “ultrametabolizers” which rapidly convert codeine to its active form, morphine. At the other end of the spectrum are 10–40% of individuals with CYP2D6 polymorphisms that cause them to be “poor metabolizers” who cannot convert codeine to morphine and are therefore more likely to achieve inadequate analgesia. In addition, medications that are CYP2D6 inhibitors (e.g., bupropion, fluoxetine, paroxetine) can lessen the efficacy of codeine.

Many families hold misconceptions about opioids and worries about respiratory suppression, addiction, dependence, and the potential for opioids to hasten death. It is therefore important to explore concerns that they may hold. While opioids that are not carefully titrated against symptoms could theoretically cause life-threatening respiratory depression, no association between administration or escalation of opioids and length of survival has been found [21].

Non-pain Symptoms

Children also often experience a multitude of non-pain symptoms, including GI, hematologic, respiratory, and skin symptoms.

GI symptoms include nausea and vomiting , which may be common and may arise from a range of causes, including dysmotility, irritation or obstruction of the GI tract, medications, toxins, motion (i.e., motion sickness), and emotions (e.g., anxiety). Nausea is in fact frequently a result of several factors in this population (e.g., medications and dysmotility). Whenever possible, it is far preferable to prevent nausea than it is to treat it once started. A number of neurotransmitters are also associated with nausea, including dopamine, substance P, serotonin, acetylcholine, and histamine. Depending on the suspected underlying pathophysiology and neurotransmitters at play, drugs such as metoclopramide, olanzapine, aprepitant, 5-hydroxytryptamine antagonists, scopolamine, and meclizine may be used. Additional agents that relieve chemotherapy-associated nausea and vomiting include steroids and lorazepam. Nausea accompanied by retching is usually the most distressing form of nausea and should be prevented or treated intensively when present. Vomiting may also occur in the absence of nausea, such as with increased intracranial pressure.

Constipation is another common GI symptom, as many children receive medications that slow GI motility (vincristine, opioids). An assessment for potential constipation should take into account the child’s usual bowel patterns. Treatment of constipation should address both stool consistency (“mush”) and colonic motility (“push”) by including a stool softener such as docusate in combination with a laxative such as Senna. Children on standing opioids should always be started on a bowel regimen, which can be titrated as needed. For children in need of treatment of opioid-induced constipation, parenteral methylnaltrexone is usually very effective. For diarrhea, loperamide (an opioid that does not cross the blood-brain barrier) may be helpful. For severe diarrhea, such as is seen in graft versus host disease, octreotide may be indicated.

Some children, such as those with solid tumors in the abdomen, may develop bowel obstruction. Symptoms of bowel obstruction include nausea, vomiting and abdominal pain (which may be continuous or colicky in nature). The primary treatment for malignant bowel obstruction is interventional – whether it be surgical correction, placement of a stent across the obstruction, or placement of a venting gastrostomy tube. Medical management to relieve symptoms may be most appropriate in the setting of advanced illness. Such management usually consists of a combination of analgesic (an opioid), antiemetic (haloperidol or ondansetron), and antisecretory agents. Antimuscarinic/anticholinergic drugs (e.g., atropine, glycopyrrolate) relieve colicky pain from smooth muscle spasm and bowel wall distention. Octreotide, a somatostatin analog, is also sometimes used. It exerts its effects of decreased secretions and peristalsis and a decrease in splanchnic blood flow, through several pathways.

Hematologic issues include thrombocytopenia (or bleeding) and anemia . For symptoms of bleeding, platelet transfusions may be considered if the platelet count is low or if platelet dysfunction is suspected. Fresh frozen plasma or cryoprecipitate may also be an option, if coagulation abnormalities are present. If life-threatening hemorrhage is a possibility, dark sheets and a fast-acting sedative should be at the ready, as such an event can be disturbing for all. Many children with hematologic/oncologic conditions encounter anemia. For those with symptoms (shortness of breath, fatigue, tachycardia, dizziness), red blood cells may be a consideration.

Respiratory symptoms : A subjective sensation of shortness of breath, or dyspnea , may arise from a range of sources, including respiratory causes (e.g., airway secretions, obstruction, infection) and cardiac causes, as well as psychological factors (e.g., anxiety). Dyspnea stems from a mismatch between afferent sensory input to the brain and the outgoing motor signal from the brain. The degree of dyspnea does not correlate reliably with respiratory parameters such as respiratory rate and oxygen saturation. It is for this reason that management of dyspnea in a child at end of life should be guided by self-report and clinical assessment, as opposed to vital sign values alone. Because opioids work directly on the brainstem to reduce the sensation of respiratory distress, they are the mainstay of dyspnea treatment. Contrary to popular belief, they do not work by causing sedation. The dose of opioid for dyspnea reduction is 25% of the amount that would be given for pain. Non-pharmacologic strategies can also alleviate dyspnea. They may include cool air flowing toward the face as well guided imagery or hypnosis to reduce anxiety can be effective. In contrast, oxygen is no more effective than blowing room air in reducing dyspnea. Noisy breathing at end of life is discussed below.

Some neurologic symptoms , such as increased intracranial pressure and spinal cord compression, are encountered in children with brain tumors or metastatic and solid tumors. The approach depends on the clinical situation and the goals of care and may involve radiation therapy, surgical interventions, and steroids. Seizures may also occur, particularly in children with brain tumors, and the incidence may increase at end of life. For children at risk of having a seizure at end of life, a plan for managing seizures should be made in advance, and anticonvulsants should be readily available. Children in the ICU are at especially increased risk for experiencing delirium , characterized by waxing and waning attention, confusion, and disorientation. While agitation and increased motor activity are often features of delirium, a hypomanic form occurs, which is particularly under-recognized. This brain disorder may have a range of causes, including medications (most notably anticholinergics and benzodiazepines), infection, electrolyte imbalances, and organ impairment. Environmental strategies (providing a calming environment) and orienting the child while addressing contributing factors are helpful. In some circumstances a neuroleptic medication may be indicated. For further discussion of delirium, refer to Chap. 18.

Skin care issues include prevention of problems by ongoing and timely assessment, frequent turning and repositioning, and alleviating pressure whenever possible. These measures are described in more detail in Chap. 16. Specific skin disorders include pruritus , which may be due to a systemic process or to medications. General approaches that may be helpful include using moisturizers and trimming fingernails. If the process is histamine-medicated, an antihistamine may be helpful. Other therapies such as topical or systemic steroids or specific therapies (e.g., cholestyramine or naloxone in biliary disease) may be indicated. Because most of the pruritus caused by opioids is not due to histamine release, antihistamines are rarely helpful for opioid-related itch. It is instead advisable to trial nalbuphine or alternatively a low dose of opioid antagonist or opioid rotation.

Psychological symptoms commonly experienced by children with advanced illness in the ICU include depression and anxiety. Diagnosing depression in either critical care setting or advanced illness may be a challenge because neurovegetative symptoms may not be reliable indicators. Relying on the expression of other emotions such as helplessness, hopelessness, worthlessness, and guilt may be of greater utility. Ongoing assessment and monitoring of depressive symptomatology is essential as one’s medical course evolves. The most helpful strategy for child psychological distress in this context is to create opportunities for them to share and explore their concerns, worries, and hopes in an open and supportive setting. Members of the healthcare team representing psychology, social work, chaplaincy, child life, and palliative care may be well positioned to support children – and their families – in this way. Antidepressants may reduce depressive symptoms, though the downside to their use in advanced illness is that their effect is often preceded by a lag phase. When there is insufficient time for a typical antidepressant to take effect, methylphenidate, which has an immediately positive effect on mood, may be considered.

Communication, Decision-Making, and Goal-Concordant Care

At the heart of palliative care – whether primary PC or subspecialty – is the patient (or family’s, depending on the situation) goals of care. Exploration of goals of care can occur in a variety of contexts, from intimate discussions at the bedside to larger family meetings and tends to occur over the course of several conversations. They may be indicated in a range of situations, such as consideration of tracheostomy, incorporation of new technology intended to sustain life outside the PICU (e.g., G-tube feedings or ventilatory support), initiation of new life-sustaining therapy (e.g., dialysis), or determination of resuscitation status.

When family goals are not clear and the next best step has not been defined, these conversations aim to elucidate patient/family preferences, values, and goals. Once these have been established, a plan of care can then be formulated. To explore a family’s goals of care, it is most helpful to ask open-ended questions such as “What is most important to you?” and “What are you hoping for?” and “What worries you?” These types of questions tend to be far more productive than a narrower question focused on medical care such as “What do you want us to do?” A particularly helpful strategy is to offer a recommendation to the patient/family, which is founded on the patient/family’s goals of care, takes the medical situation into account, and is formulated through clinician expertise. Such a recommendation is frequently appreciated by families, who commonly experience relief when the burden of a decision is lifted from their shoulders. Finally, the outcomes of goals of care communications should be documented in the medical record so that other members of the medical team who were not part of the conversation can understand the goals and rationale underlying a decision or the course of care predicated on these goals.

Clinician Language: More than Mere Words

Similar to scalpels for surgeons, words are the palliative care clinician’s greatest tools. Surgeons learn to use their tools with extreme precision, because any error can be devastating. So too should clinicians who rely on words. (Eric Cassell)

Our communications with patients and families about goals of care and medical treatment may have many aims. At the same time, we always hope to convey a message of compassion, respect, willingness to explore the patient/family’s goals, hopes and fears, truthful expression of the medical situation and the care that can be provided, a commitment to continuing to take the very best care of them/their child, and a recognition that there may be multiple forms of caring. Use of words without full consideration of their impact can negatively impact how we think about our patients, the care we have to offer, and the decisions that are made. Careless communication is not without consequence; even unintentional messages can reinforce unspoken biases and assumptions. See Table 17.2 for concrete examples of unintended messages in the ICU environment, as well as recommended alternative communication strategies.

Table 17.2 Considerations for clinician communications
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Advance Care Planning

In the setting of advanced illness, advance care planning allows the child’s voice to be heard, even when they lose the capacity to express their own wishes. This is, in a sense, a way of preserving the child’s autonomy. Consideration should be given to allowing the child to participate in such discussion if they have the developmental and cognitive capacity to participate. It has been shown that children with cancer as young as 10 years are able to understand and weigh risks, benefits, and burdens in end-of-life discussions such as those regarding phase I trial participation or resuscitation status [22].

A range of guides to facilitate advance care planning with children and adolescents are available (available at www.agingwithdignity.org). My Wishes, for children, helps them express how they would wish to be cared for in case they become seriously ill. Voicing My Choices is designed for adolescents and young adults and allows them to express how they wish to be comforted, supported, treated, and remembered. Both are available in English and Spanish and can be worked through with the child/adolescent, or alternatively as a guide to parents or other caregivers wishing to begin a conversation. Five Wishes guides adults through important considerations about their care and allows them to document them. Once completed and witnessed, it meets the legal requirements for an advance directive in 42 US states and the District of Columbia. It is available in 28 languages.

Considerations at the End of Life

Conversations at End of Life

In the last phase of a child’s life, end-of-life discussions such as those regarding resuscitation status or life-sustaining treatment (LST) are of great importance and are a commonly encountered issue in the PICU. However, for children in the PICU, for those with cancer, and for pediatric HCT recipients, these conversations frequently occur late in the child’s illness and sometimes do not occur at all. In a study of PICU and oncology physicians and nurses, 71% of all clinicians believed that such discussions happen too late in the child’s clinical course [23]. In this study, the top three barriers were unrealistic parent expectations, differences between clinician and patient/parent understanding of prognosis, and perceived lack of parent readiness to have the discussion. It is likely that clinician factors also play a role, since in this same study, less than half felt prepared to discuss resuscitation status with school-aged and adolescent patients and to conduct a family conference. The inherent nature of these high-stake conversations, frequently fraught with emotion and prognostic uncertainty, also adds to the challenge. These challenges suggest opportunities for collaboration between PICU and palliative care clinicians. An additional challenge is clinician desire to protect families from distress or loss of hope. However, most families can tolerate talking about their child’s end of life and find relief when the care team opens to door to conversation about it. Moreover, parents value honesty, and honest, compassionate conversations can promote parent hopefulness, as well as trust and connection with the care team.

Despite the above challenges, clinicians should proactively create opportunities to have these conversations with patients/families, rather than deferring them until the last phase of life. Through earlier discussions that do not occur in the midst of a crisis, patients/families are much better positioned to consider these decisions. Earlier discussions also give them the opportunity to think and plan before a crisis arises.

Cancer-Directed Therapy

Provision of cancer-directed therapy in the absence of an agreed upon goal of cure warrants careful consideration, weighing the potential benefits with impact on child well-being. Even when there is no realistic hope for cure, it is not uncommon for families to choose continued cancer-directed treatment [24]. In fact, many families will seek out such treatment if it is not offered [25]. Reasons for this may include provision of hope and perceived increased attention and care from the medical team associated with receipt of treatment. Ongoing hope for cure/disease control is also prevalent. The majority of parents maintained a primary goal of extending life, in the last moments of a child’s life [26, 27]. From the perspective of oncologists, parent preference for such continued treatment is a major contributing factor for its prescription [28].

Conversations regarding cancer-directed therapy at a child’s end of life are in many ways similar to goals of care conversations about other treatments. Clinicians should be mindful of the fact that families often hold blended goals, and pursuit of cancer-directed therapy does not in and of itself mean that a family is unaware of the child’s limited prognosis. Clinicians should also maintain nonjudgmental attitude if a family seeks to pursue cancer-directed therapy in the face illness deemed incurable by the medical team. While such therapy can present the risk of toxicity, there may be some benefits to the child and family beyond life extension. For example, Wolfe and colleagues found that in the child’s last 12 weeks of life, receipt of mild cancer-directed therapy was associated with improved psychological quality of life [29]. Finally, it is helpful to avoid labeling cancer-directed therapy as either “curative” or “palliative.” Such distinctions are usually artificial, and by equating “palliative” with “no hope for cure” undermine the term “palliative.”

Resuscitation Status and Life-Sustaining Treatment

While resuscitation status and use of LST at EOL are highlighted as distinct topics, the overall approach to discussing them is the same as for other medical decisions. These conversations should be focused on the goals of care, as opposed to limitations of care. Instead of specifically discussing “withdrawing support” or a do-not-resuscitate (DNR) order, a discussion centered on goals of care will naturally lead to considering which interventions are in the child’s best interests. And from there, clinicians can make recommendations based on these goals and medical knowledge of potential benefit and/or harm of these interventions. During these discussions with patients/families, what will be done to continue to care for them, such as continuing to be present and ensuring their comfort, should be reinforced. This is particularly important because families are often hesitant to limit resuscitation or LST as they fear it will also trigger limitation of other aspects of care. Clinicians should similarly not assume that having a DNR order in place or foregoing LST indicates that other types of care not specified have changed.

With regard to resuscitation status, many patients/families do not understand the legal mandate that resuscitation for cardiorespiratory arrest be attempted unless a DNR order is in place. This concept is important to explain particularly if discharge from the hospital is under consideration. In this case, most states have out-of-hospital DNR verification forms or physician orders for life treatment (POLST) forms. POLST forms (www.polst.org) are medical orders communicating patient preferences regarding resuscitation efforts and life-sustaining treatment across care settings. Both types of forms affirm that emergency response teams provide symptom management with comfort and relief of suffering when called to the scene, rather than initiating resuscitative efforts.

Feeding and Hydration

For children who can no longer feed by mouth, medical nutrition, either nasogastric or gastrostomy feedings, or intravenous nutrition or hydration may be options. Whether to proceed with these interventions for children approaching end-of-life requires careful evaluation of the risks and benefits, taking into account the family’s goals, the medical circumstances, and the child’s prognosis. Conversations around this topic must be approached with great sensitivity as it often evokes intense emotions in both family members and members of the care team alike.

Families often hold the misconception that medical nutrition and hydration are needed to prevent their child from experiencing hunger and thirst. However, clinical experience with patients able to articulate their experience tells us that they experience little hunger and that thirst may be alleviated with attentive mouth care and moistening. An honest and sensitive discussion about the potential harms of medical nutrition should be made with the family. Such potential harms include increased secretions and fluid overload (e.g., pulmonary edema), both of which can contribute to respiratory distress.

If possible, the optimal approach is to provide anticipatory guidance to families, describing how appetite and thirst wane, and the body’s ability to handle medical nutrition/hydration decrease at end of life as well. Exploring the meaning that provision of nutrition and hydration that they may hold is helpful, as is identifying other ways in which they can continue to love and care for their child.

Other Considerations

Location of Care

For many children with advanced illness in the PICU, their final location of care may be the PICU. While much emphasis is placed on location of care (and location of death), with home deaths assumed to be the preferred location of death, the opportunity to plan for end of life may be what is most important. In a study of 140 parents who lost a child to cancer, the opportunity to plan location of death was associated with outcomes consistent with high-quality palliative care, even among nonhome deaths. It may therefore represent a more relevant outcome than actual location of death [30]. For children who do die in the PICU setting, technology may place distance between the parent and child. This highlights the importance of reconsidering and potentially discontinuing medical technology that is not of benefit to the child. Parents often also benefit from encouragement and assistance in touching, holding, or even sleeping alongside their child despite the surrounding tubes, lines, and technology.

For those families who express a desire for their child to spend their last moments in a non-ICU environment, transfer to the hematology-oncology unit is sometimes possible. Such a transfer may take some planning, to ensure adequate staffing (e.g., additional nursing, respiratory therapy). Palliative care teams may be helpful in coordinating such efforts and in providing additional support for the hematology-oncology in caring for the child. In some institutions, palliative transport and discontinuation of LST (e.g., extubation) is possible, logistical and clinical circumstances permitting. Families view this opportunity as positive and meaningful, with memories of the experience bringing them comfort and a sense of fulfillment [31].

Autopsy and Organ Donation

Postmortem examinations (autopsies ) may yield important information furthering clinical and research efforts. For example, Sirkia found that of 40 children who died of progressive cancer, autopsy afforded new information in 20% of cases and important additional information in 55% [32].

Families also value new information that may be discovered about their child, often viewing the contribution to medical knowledge as part of their child’s ongoing legacy. Return of autopsy results may also provide an opportunity for families to return for follow-up discussions, which in and of themselves are often helpful during bereavement [33]. Many families appreciate the opportunity to consider autopsy in advance of the child’s death, when possible. Some have in fact been considering it, are unsure how to bring it up with the medical team, and are relieved when the door is opened to conversation about it. In support of this, Wiener found that most bereaved parents feel that autopsy should be introduced when it becomes clear that cure is no longer possible [34].

Like autopsy, families often appreciate the opportunity to discuss in advance of their child’s death. While some families find the option of organ donation meaningful, the array of factors influencing their decision is complex. Representatives from organ procurement organizations are available to speak with families about the possibility of organ donation, which frequently facilitates a decision to pursue organ donation. A shared approach in which medical teams and organ procurement organizations’ representatives partner to provide families with the support and information needed when considering this topic may be particularly helpful to families.

Palliative Sedation

Many children in the PICU are sedated at end of life. Among those who are not, some experience symptoms or suffering that is refractory to the most intensive efforts. For these children, palliative sedation may relieve their suffering by reducing their level of consciousness. Prior to embarking on such sedation, its indication, goals, endpoints, and potential effects on the child should be discussed with the family and members of the interdisciplinary care team. These issues should be revisited as sedation is ongoing as well. Open and ongoing communication is particularly important as moral distress may emerge. Agents such as midazolam, pentobarbital, dexmedetomidine, or propofol most commonly used for palliative sedation should be administered via continuous infusion with a bolus dose available and dose escalation undertaken as needed. Previously started opioids should be continued for the purposes of analgesia and withdrawal prevention, though opioids alone should never be used for palliative sedation per se.

Supporting the Child and Family at End of Life

Anticipatory Guidance

A critical aspect of supporting families in the last moments of the child’s life is provision of anticipatory guidance. If death is imminent, families should be informed of this, even if the time frame is uncertain. Even letting them know that it may range from hours to days (or longer) is often helpful to them. Families also appreciate anticipatory guidance with regard to physical changes that may become evident, such as decreases in child responsiveness, and changes in circulation and breathing pattern. For some children, breathing may become noisy, due to pooling of secretions. At the point when this occurs, children are not sufficiently conscious enough to be disturbed by this. For family members at the bedside, however, such noisy breathing may be distressing. Anticipatory guidance around this can oftentimes mitigate such distress. When this is not the case, an anticholinergic agent such as atropine ophthalmologic drops, scopolamine, l-hyoscyamine drops, or or glycopyrrolate can be helpful in drying secretions, as can gentle suctioning.

As medical management is increasingly guided by symptom control as opposed to physiologic derangements, monitoring of vital signs may be less relevant. Open conversation with families about ongoing intensive care of the child now guided by clinical cues from the child as opposed to monitoring of vital signs may help them to know that possible removal from monitoring does not indicate lessening of care.

Family Togetherness and Memory Making

Families may not know what is permissible or acceptable, particularly in the high-tech environment of the PICU. As above, they may benefit from encouragement and assistance in maintaining close physical contact. Bereaved parents also describe the importance of opportunities to care for their child, as well as privacy, and access to professional and personal support [35]. Attention should also be paid to spiritual needs, whether it be through visits from hospital chaplaincy or leader of their faith community, as well as prayer and ritual [36, 37].

The family should also be offered the opportunity for siblings to spend time with the child. Child Life Specialists are often invaluable in talking with siblings and preparing them for changes they may see. Such times of family togetherness are often opportunities for memory-making including taking photographs, making molds of family members’ hands together. Additional memory-making activities that might be offered include locks of the child’s hair and recording of the child’s heartbeat (sometimes done by music therapists). These memories and mementoes are an important way for families to maintain a sense of connection to their child in the moment and moving forward.

On some occasions, resuscitative efforts may be made at the end of a child’s life, either because they experience an unplanned cardiopulmonary decompensation or because such efforts were part of the plan of care. While witness resuscitative efforts are controversial, parents should have the opportunity to decide for themselves whether to be present for the resuscitation. If they do, the presence of a seasoned clinician dedicated to supporting the parents is essential. Parents describe it as distressing, but for many, it also provides benefits. One study found that the vast majority of bereaved parents who had witnessed resuscitative efforts for their child would do so again [38].

Bereavement

Families who have lost a child face enormous challenges. The support they received prior to their child’s death should continue into bereavement, as families often describe a double loss – the loss of their child and the loss of the care team whom they have known, trusted, and traversed a very traumatic time in their lives [39]. Clinicians can both commemorate the child and express support for the family in a number of ways, including attending the funeral or memorial service, sending a condolence note, or making a phone call. Family members place great importance on these expressions of support and commemoration, appreciate them when they occur, and may be disappointed if they do not occur [40]. Many families also appreciate an opportunity to review the child’s illness course and end of life. If an autopsy has been conducted, results can be shared during the visit. Families may hold difficult or confusing misconceptions or questions in need of answers. Addressing these allows them to undertake the difficult psychological work lying ahead. Staff, including trainees, may similarly hold lingering thoughts and unanswered questions about caring for the child. Debriefing sessions for staff may allow them to review the child’s story and remember remember him/her. They may be particularly helpful to hematology/oncology or PICU staff who cared for the child during part but not all of the illness course and wish to hear the story in its entirety.