Cancer is the leading cause of disease-related death in children in the United States. Despite the numerous advances in pediatric oncology, some children are diagnosed with terminal malignancies and many others will become terminal later in their cancer trajectory. A child may die as a result of direct cancer progression or complications of treatment. For instance, uncontrolled invasion of cancer into major organs and bone marrow leads to a terminal prognosis. Additionally, the side effects of chemotherapy, including myelosuppression, cardiac toxicity, liver or renal toxicity, can also lead to death. At other times, the total body burden of cancer leads to cachexia, profound weakness, and eventual death. Reaching this stage in the cancer journey is a very difficult time in a child’s and family’s lives. Great care must be taken to provide compassionate care and support to families and children in this stage. This can begin by eliciting and then honoring their wishes and values.
Decision-Making in Pediatrics
Throughout the cancer journey parents and their child will have to make many decisions regarding their care, starting at diagnosis. Conversations about what is important to them early in their journey are necessary for developing a strong provider-parent relationship and will help establish goals of care.
Goals of Care
Ideally, conversations regarding goals of care start early in the cancer journey and are revisited periodically as the treatment and disease course progresses, and goals of care change. This is important all throughout the journey but is particularly crucial as the patient becomes terminal. These conversations must elicit the child’s/parents’ values, hopes, dreams, and fears, as well as assess their physical and emotional suffering. By carefully weighing the expected results of treatment and side effects against the child’s/parents’ values and goals, a picture begins to emerge that will help families make decisions regarding their care and help the physician to understand the family’s decisions and to align the treatment with their goals.
Goals of care can be different for different families and children, and they will also change over time, depending on prognosis. Initially, the goal is most often for cure and thus treatment will be disease-directed and cure-oriented. Treatment regimens may be intense with the potential for high toxicity. As the disease advances, the goal may change to slowing the cancer progression to prolong life and increase quality of life (QoL). At this point the treatment will still be disease-directed, but it may be limited to treatments that allow for a good QoL and do not cause significant toxicity. Once a child enters a terminal stage, the goal may shift to providing comfort and minimizing suffering. Treatments, if desired, would ideally have little to no toxicity and allow for maximum comfort and QoL.
There are a few common goals that arise as children approach the end of life: minimizing physical and emotional suffering, enhancing QoL, dying peacefully, and leaving a legacy. Palliative care physicians can help families achieve these goals, as well as align any remaining medical treatment with these goals. Table 50.1 shows a list of possible questions that may facilitate these difficult conversations.
|Is it ok to talk about some of the experiences of other families I have cared for?|
|Some children/families worry about what may happen if treatments don’t work, is that something you worry about?|
|Can you tell us your understanding of the situation?|
|What are your concerns at this time and for the future?|
|I worry that [child’s name] will not tolerate more chemotherapy. Can we talk about that?|
|I understand your primary hope is cure; what else do you hope for?|
|In light of what we have discussed, what is most important to you now?|
|How do you feel about your treatments?|
|What symptoms are the most bothersome?|
|What are the things that bring you joy/comfort?|
|What activities bring you peace or comfort?|
|How and where do you want to live the rest of your life?|
Unique to pediatrics is the concept of concurrent care; this type of care allows children to continue receiving cancer treatment while enrolled in a hospice near the end of life. This is not the case for adults; they must stop all disease-directed treatment and even stop seeing their primary oncologist once they enroll in a hospice. Concurrent care allows for a gentle transition between the oncology treatment team and the palliative care/hospice team. Palliative care, which is an integrated, interdisciplinary team-based, holistic approach to address symptoms in seriously ill children, can also help to facilitate complex medical decision-making with patients, their families, and their primary medical teams. Patients can continue to see their oncologist until they are no longer physically able. This may be very helpful to families, as there is often a strong bond with the physician who has been on this journey with them. Concurrent care allows time for the hospice/palliative care team to integrate themselves and build a rapport with families before having to discuss very difficult topics such as “do not resuscitate” (DNR) and end of life. Palliative care is ideally integrated early in the disease process so that the full benefits, including impeccable symptom control, truly partnered medical decision-making, advance care planning, and optimal end-of-life care. Furthermore, opportunities for legacy building and bereavement support are viewed as a thread of continuity from the time of initial contact to the present, rather than as the sudden introduction of a team of strangers at the end of an illness.
Although palliative care services are available in most academic children’s hospitals in the United States, primary (basic symptom management and communication skills) and secondary (advanced symptom management, facilitation of complex medical decision-making, and management of complicated family dynamics) services are available to only a fraction of the 21 million children worldwide with serious diseases. Thus it is important that all physicians develop primary palliative care skills to care for terminal children and their families.
Honest, clear, and compassionate communication is critical for shared decision-making and subsequently advanced care planning. Two categories of communication needs have been described in the medical literature: cognitive and affective. The cognitive needs generally include the need to know medical facts, such as the diagnosis, treatment options, side effects, and prognosis. The affective needs in communication encompass the need to express oneself and to be understood, validated, and supported. To meet both of these needs, the physician must have knowledge of the family and child and their preferences for receiving and delivering information, such as who will be present when important news is delivered and how much detail should be given. Eliciting preferences for information is an important step to take prior to family meetings.
Family meetings are an important procedure for giving and receiving sensitive information with families. They must be planned with great thought given as to who will be present, who will lead, and what will be discussed. The presence of the interdisciplinary team may be helpful to encompass all aspects of the child and family’s care but this must be weighed against the potential to overwhelm families with too many people in the room. Before the meeting, the medical team should meet first to discuss the medical aspects of the discussion, including prognosis, proposed treatment options, and palliative measures, to ensure everyone agrees and minimize opposing views in front of the family. The meeting should take place in a quiet, private place with care given to minimize distractions and interruptions. One person should be designated to lead. Someone from the team should review with the family their communication preferences, informational needs, whom they want present, and any other questions to be added to the agenda. Often parents and providers are unsure whether to include the child in these meetings. The answer to this will depend on the parents’ wishes, the developmental age of the child, and sometimes the child’s wishes. While honesty is always recommended, communication with children must be carried out delicately and in a developmentally appropriate way. When parents choose not to include the child in the team discussion, they can be encouraged to share the information later either by themselves or with the help of a trusted member of the care team. This allows time for parents to process the information first before sharing it with their child. Last, these difficult conversations will be performed all at once, albeit rarely. In many cases the conversation will be spread over several sessions to allow time for grieving and processing.
Tools for the Providers
Medical decision-making has become more complex as medicine has evolved, particularly since the introduction of life-sustaining technology in the 1950s. Difficult conversations can be challenging for providers. Training in such specialized communication skills has not traditionally been part of the medical training curriculum although this has changed in the last few years. Many tools exist to help medical providers as they navigate these conversations with families. One tool is NURSE, which is a helpful mnemonic that stands for Name, Understand, Respect, Support, and Explore and can help providers respond to the patient’s or family’s emotions. These words refer to types of statements that can be used to respond to emotions. For example, the medical provider can name the emotion they are witnessing that demonstrates that the provider is paying attention to the emotion. Another option is for the provider to make a statement relating to how difficult, scary, or frustrating a situation is. Statements of respect refer to acknowledgments of the excellent care that a parent has provided for his or her child. Support statements can help foster trust in the patient/family-physician relationship and reassure families that they will not be abandoned. Last, the medical provider can simply explore the family’s/patient’s concerns or goals especially as disease or treatment progresses. Reflective listening and offering a silent ear are additional valuable communication tools that help families to feel heard, listened to, and supported. Other helpful resources include Vital Talk ( www.vitaltalk.org ), a communication skills course for medical providers for delivering serious news and the accompanying application for smartphones (Vital Tips), and the Courageous Parents Network ( www.courageousparentsnetwork.org ), an organization founded by parents to empower and equip parents and providers caring for children with serious illness.
Advanced Care Planning
Advanced care planning is now commonplace in the adult health care system; however, it is not standard practice in pediatrics. Data in the adult literature support advanced care planning, as it helps avoid aggressive yet futile interventions, and therefore minimizes suffering at the end of life. Advanced care planning improves concordance between care delivered and the patient’s preferences for care. This same benefit should be extended to children and adolescents approaching end of life. In fact, some literature already supports the use of advanced care planning in pediatrics, as well as the need for further research in this area. , Many tools for advanced care planning have been developed for adults but probably the most widely used tool is a legal advanced directive called Five Wishes , which takes into account medical, spiritual, emotional, and personal concerns. There are also pediatric versions, such as “My Wishes” or “Voicing My Choices,” which take into account the developmental age of this special population. Although these documents are not legally binding for pediatric patients they can help initiate these difficult conversations, as well as allow the child and parents to express their wishes. There are different aspects of advanced care planning but commonly it includes assigning a proxy medical decision-maker, preferences for end-of-life care, whether to attempt cardiopulmonary resuscitation (CPR) at end of life, the use of life-sustaining medical therapies, preferred location of death, and legacy planning.
CPR was originally developed in the 1960s to reverse unexpected cessation of cardiopulmonary function in previously healthy individuals undergoing operations using anesthesia and with the expectation of recovery. However, blanket application of this intervention is not appropriate for all patients. By the mid-1970s, concerned clinicians created the first institutional policies seeking to protect patients who asserted their right to die without CPR in situations where it was known to be ineffective. It is well known that chronically ill children tend to do poorly with CPR, even when witnessed arrest occurs in hospitals in well-resourced countries. Although cardiorespiratory activity may return with CPR, functional recovery is typically very poor; these children rarely return to their already-compromised baseline.
CPR is also, unfortunately, sometimes attempted for patients whose disease trajectory is well known to the clinical team and who are simply in the process of dying. Patients and families may be unable to accept the medical facts and continue to hold unrealistic expectations. Some clinicians may have formed strong bonds with patients and their families over the years and feel compelled to offer CPR as the child deteriorates, even when death is the expected outcome.
Palliative care providers can help patients and families reach decisions regarding whether or not their child should undergo CPR when arrest ensues. Most patients and families are able to agree with recommendations to forgo potentially harmful interventions when given the historical context of CPR, the dismal outcomes in debilitated patients, and an understanding of the beneficial interventions that can be implemented at end of life to ensure comfort. Families can usually understand the real risks of pain and prolonged suffering CPR may cause if implemented.
Presently, fully informed, joint decisions can be made by the patient or surrogate medical decision-maker and the attending physician to accept or forgo CPR, and this is documented as the “code status.” A patient’s code status is an important designation within the health care system. It can range from “full code” (all resuscitative and life-sustaining measures are provided) to “do not resuscitate” or DNR (no CPR is provided). At end of life, treatment can be personalized based on the patient’s or family’s wishes. The components of the code status encompass chest compressions, intubation, mechanical ventilation, defibrillation, and medications used during resuscitations.
The decision to withhold life-prolonging medical therapies and CPR at the end of life (DNR) is an important decision for families. Every attempt to honor this decision should be made and there may be legal consequences if it is not. Without these interventions, the disease course will progress and the child will reach the end of their natural life. Comfort measures should be in place for the child to ensure that the child does not suffer.
Indications for surgical and other invasive procedures may arise in children with severe underlying medical conditions who have advanced directives, including a DNR already in place. A child may develop a problem that requires a surgical or interventional procedure under anesthesia that may be completely unrelated to the child’s underlying process. One example would be a child with metastatic osteosarcoma who develops acute appendicitis. Other children may develop complications of treatment for their underlying condition; a fairly common example would be that of a child with refractory leukemia who develops neutropenic enterocolitis with perforation and septic shock, and in whom surgical intervention is considered. Some children may experience worsening of a symptom as their disease progresses that may be improved with an invasive intervention. Common examples are malignant effusions causing pain and respiratory distress, malignant ascites, and malignant bowel obstruction unresponsive to medical therapy.
The decision to pursue an invasive procedure is always very difficult, especially as risks increase and significant moral distress can occur in providers trying to discern the best course of action. Multidisciplinary consultation may facilitate truly informed and empowered medical decision-making. Ethics committees can help individual clinicians, the entire health care team, and the patient and family members to comprehend the overall clinical situation, properly weigh the relative risks and benefits of specific proposed procedures, and may reassure clinicians, most often surgeons and interventional radiologists, who may be wary of performing the procedures. In some cases surgical intervention is not the right choice and the family may choose to spend the child’s last days with an integrated palliative approach, which includes appropriate pharmacologic therapy (inclusive of palliative sedation, if indicated), nonpharmacologic adjuncts, and comprehensive psychosocial support, which certainly minimizes the existential pain even very young children may experience (see Table 50.2 ). Such an approach may help minimize decisional regret in parents, families, and clinicians, and may improve the overall family satisfaction with care, reduce costs, and mitigate the bereavement process after the child dies.