The field of pediatric palliative medicine has undergone significant growth in the last decade. Palliative care services were once only considered appropriate for children who were imminently dying, but are now recognized as vital to the care of those with complex potentially life-threatening conditions. Hospitals that care for the approximately 55,000 children who die each year in the United States have an obligation to provide excellent pediatric palliative care.^1-3 Although palliative care is becoming standard of care in many hospitals, on average only 10% of pediatric patients who are eligible receive palliative care.¹ A recent survey by the Center to Advance Palliative Care found that although 69% of hospitals caring for children had a pediatric palliative care program, the staffing and scope of programs varied widely.⁴ Healthcare professionals find this domain of practice extremely difficult and referral for services is sometimes delayed or omitted. This chapter addresses the core challenges of providing palliative care, supplying practical and, to the extent possible, evidence-based answers to these challenges.

Palliative care seeks to maximize quality of life for patients and families through an interdisciplinary approach to minimize distressing or uncomfortable symptoms that patients experience and maximizing the quality of their remaining lives, while providing support for decision making concentrating on aligning treatments with family and patient goals of care. These services are provided across the care continuum and across care locations in conjunction with disease-directed therapies. Collaboration and communication among patients, families, and healthcare professionals is essential.

Figuring out which patients and families would benefit from palliative care, and when, is challenging for two main reasons. First, the prevailing medical model presents palliative care as a mutually exclusive alternative to curative care. This is a false dichotomy: patients can simultaneously receive care that seeks to cure disease or extend life while also receiving complementary care that seeks to minimize bothersome symptoms and maximize the quality of life (Figure 10-1). Second, among the approximately 55,000 children who die each year in the United States,⁵ several trajectories of dying exist, including⁶:

1. 
   

   Children who die suddenly, before any diagnosis is made (e.g. due to trauma or conditions such as sudden infant death syndrome or occult cardiac arrhythmias). These families warrant bereavement care.

   
   
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   Children whose conditions are inevitably fatal. This group can be subdivided into the following groups:

   
   
   
   
   1. 
      

      Patients who will inevitably die relatively quickly after diagnosis (e.g. nonviable prematurity, inoperable brain tumor). All of these patients warrant palliative care immediately.

      
      
   2. 
      

      Patients who will inevitably die, but years to decades after diagnosis (e.g. many neurodegenerative disorders). These patients warrant palliative care, but when to institute such care is debatable. We believe that, along with life-extending care, complementary palliative care and advanced-care planning should be initiated at the time of diagnosis.

   
   
3. 
   

   Children who have conditions that make them extremely frail and vulnerable (e.g. severe spastic quadriplegia with swallowing dysfunction and risk of aspiration pneumonia; cancer that requires debilitating chemotherapy; sepsis that makes the child critically ill). Because of their fragile health, such children have an increased risk of dying on any given day; however, the risk is still low enough that although death is likely at some point, it is not inevitable in the next year or even the next decade. For these patients, complementary palliative care and advanced-care planning are warranted as early as possible, with a transition toward a more exclusive focus on palliative care if the condition progresses to the point where the benefits of life-extending care are outweighed by the suffering such care imposes.

A study evaluated demographic and clinical features of 24,342 children who died >=5 days after admission in a sample of children’s hospitals, comparing those who did and those who did not receive a palliative care consultation. About 4% of children received palliative care. Palliative care was more common in older patients and was associated with fewer hospitalization days prior to death. Children who received palliative care in this study were less likely to have invasive interventions, received fewer medications, and were less likely to die in an ICU.⁷

Another study provides a snapshot of 515 children receiving palliative care by six hospital-based pediatric palliative care teams in the United States and Canada. The two most common principal underlying diagnoses in this cohort of children were genetic or congenital disorders (40.8%) and neuromuscular disorders (39.2%). The study further highlighted that palliative care for children is principally about how to help children and families live with serious illnesses, as 69.7% of the patients were still alive at the end of the 12-month follow-up.⁸

Talking about pediatric palliative care with families is one of the most demanding and difficult tasks of clinical medicine. Dividing the task into two major components—what the patient and family are up against, and what can and will be done—and having a distinct plan for each aspect of the conversation (which will likely occur over several interactions) can be helpful.

The first component focuses on delivering the bad news itself, which may be a new diagnosis or the latest in a long series of illnesses and deteriorating health. This bad news threatens the goals, plans, and hopes of patients and parents, and the resulting emotional tumult can make even the most seasoned clinician feel overwhelmed and desperate. Preparation is therefore essential: have a precise plan of what information is to be conveyed to whom, allow ample time to hold this meeting, and have the discussion in a private environment with appropriate support persons (e.g. other family members, nursing staff, social workers) present. After briefly reviewing the clinical events, preface your remarks with a warning, such as, “I’m sad to say that the news I have is bad,” and then state the new information as simply as possible, such as, “The tests show that your child has cancer.” Then stop talking and let the family assimilate this information. Your quiet, compassionate presence will be perceived as supportive. Answer questions as they are posed, avoiding the temptation to downplay or sugar-coat the bad news. Given the turmoil that such news often creates in the minds of those who receive it, simply relaying the information and responding to initial questions may be all that can be accomplished at the first meeting. Before ending the meeting, however, propose some specific plans for follow-up conversations or actions. For example, say, “I will be back to check on you and your child in 2 hours, and I can answer any additional questions that may have occurred to you and start to discuss plans at that point.”

The second component of palliative conversations focuses on possible therapeutic actions and deciding on which ones to pursue.^9,10 After the family has had adequate time to absorb the bad news (which, depending on its severity and unexpectedness, can take from minutes to weeks or even longer), the objective of this conversation is to help formulate new goals, plans, and hopes in light of the constraints imposed by the diagnosis. Instead of focusing on dying and death per se, this conversation focuses on what the therapeutic alliance among patient, family, and care team can strive to accomplish, despite the fact that death is likely to occur in the near future. A useful way to proceed is along the following lines: “Learning that your child has an incurable condition that will shorten her [or his] life has been incredibly upsetting and difficult. Given the problems that the condition creates, I am wondering how we can best care for your child. What are your major hopes for her?” At this juncture, family members often both mourn the loss of hope for a normal life and mention the desire to keep pain or other forms of suffering to a minimum or quality-of-life goals, including social or spiritual concerns. These hopes provide the basic goals of all subsequent palliative and end-of-life care plans, and they are the compass by which the rest of clinical care is oriented.

One of the most challenging aspects of providing palliative care to children and families has been overcoming the stigma that palliative care entails giving up; that to receive palliative care services, parents must forego disease-directed treatments. Historically this has been the case when hospice services are engaged. Since palliative care and hospice care are often linked, the bias that families feel toward hospice has extended to palliative care teams. However, this provision changed in 2010 for patients under the age of 21 with the passing of the Patient Protection and Affordable Health Care Act (PPACA). This act includes a provision entitled “Concurrent Care for Children” which requires that programs for children in state Medicaid or Children’s Health Insurance Programs (CHIP) allow children under the age of 21 to receive hospice care while still receiving curative treatments, as discussed in further detail later in this chapter.¹¹

The evidence is clear that pediatric patient care has become more complex and an increasing proportion of hospitalizations are attributable to pediatric patients with complex chronic conditions (CCC). Many of these patients receive palliative care consults to help with symptom management, quality-of-life issues, or end-of-life care. Despite the overlap of palliative care and the care for pediatric patients with CCC, these patients still require robust, dedicated primary and specialty care services and care coordination. Palliative care can be an important adjunct to the treatment team; however, the patients require ongoing care coordination sometimes out of the scope and staffing abilities of a palliative care team.^12-14 Many hospitals have developed specialized pediatric medical teams to help manage and coordinate care for this population.¹⁵

More pediatric patients are able to pursue parallel disease-directed care and palliative care at the same time due to the “Concurrent Care” provision of new federal regulations. Palliative care can start at diagnosis of a life-limiting illness and patients can benefit from the depth of services throughout the disease process. In the future, we hope that pediatric patients can continue to benefit from concurrent palliative care to maximize their quality of life.

Children who are dying often suffer with seven main symptoms that are managed most effectively within a holistic framework that includes but extends well beyond pharmacotherapy.¹⁶

NAUSEA AND VOMITING

Many children experience nausea and vomiting for a variety of reasons, including chemotherapy, other drugs, metabolic disturbances, central nervous system tumors, vestibular or middle ear pathology, gastrointestinal pathology, and anxiety or other conditioned responses. Treatment includes both pharmacologic and nonpharmacologic approaches. Medications commonly used include prochlorperazine, ondansetron, granisetron, scopolamine, dexamethasone, and benzodiazepines (use of metoclopramide has decreased since the black box warning about tardive dyskinesia in 2009 by the FDA). Unfortunately, scant data support the effectiveness of these drugs in the pediatric palliative care setting. Initial therapy should target the primary cause of the nausea or vomiting (e.g. dexamethasone for vomiting due to raised intracranial pressure from a brain tumor, or a benzodiazepine for nausea caused by anxiety). These medications can then be titrated to a maximal dose, and if that is ineffective, therapeutic control with another agent can be attempted. Often, symptom relief can be achieved only with multiple agents. Some of the most effective nonpharmacologic methods are providing small, frequent meals; giving medications after meals if possible; and eliminating smells and tastes that exacerbate the symptoms.¹⁷