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Pediatric palliative care

Pediatric palliative care
Author:
Julie Hauer, MD
Section Editor:
David G Poplack, MD
Deputy Editor:
Laurie Wilkie, MD, MS
Literature review current through: Dec 2022. | This topic last updated: Jul 26, 2022.

INTRODUCTION — Pediatric palliative care is an interdisciplinary collaboration that seeks to improve the quality of life for children with life-threatening conditions, as well as their families. It focuses on preventing and relieving suffering, regardless of the stage of disease, and comprehensively addresses the physical, psychosocial, or spiritual needs of the child and family/caregivers.

Pediatric palliative care, including the approach to developing palliative care plans, establishing goals of care, symptom management, and end-of-life care in children, will be discussed in this topic. Other aspects of palliative care are discussed in separate topic reviews:

(See "Pain in children: Approach to pain assessment and overview of management principles".)

(See "Palliative care delivery in the home".)

(See "Ethical issues in palliative care".)

(See "Overview of spirituality in palliative care".)

(See "The initial interview in palliative care consultation".)

(See "Communication of prognosis in palliative care".)

(See "Overview of managing common non-pain symptoms in palliative care".)

(See "Palliative care: The last hours and days of life".)

DIFFERENCES BETWEEN PEDIATRIC AND ADULT PALLIATIVE CARE — Although the principles of palliative care are the same in children and adults, the implementation of care is substantially different because of the following:

Varied cognitive abilities and emotional maturity of the child or adolescent

Differences in emotional and psychological issues regarding seriously ill children

Necessity of dealing with the child, the parents/caregivers, and, in some families, siblings, grandparents, or other close family members

Authority for decision-making about care resides with the parents, not the child

Differences in the causes of life-threatening illness, with related differences in palliative care needs

Pediatric palliative care will be reviewed here. The many aspects of palliative care in adults are discussed separately. (See "Overview of comprehensive patient assessment in palliative care".)

PATIENT SELECTION — Palliative care is ideally integrated into the care of any child with a potentially life-threatening condition. Palliative care is appropriate for conditions in which any of the following apply [1,2]:

Progressive conditions for which curative treatment is possible but may fail – Examples include children with cancer, children awaiting solid organ transplantation, and children with certain types of complex cyanotic congenital heart disease (eg, hypoplastic left heart syndrome). (See "Hypoplastic left heart syndrome: Management and outcome", section on 'Choice of management'.)

Chronic progressive conditions for which long-term palliative care can help maintain quality of life – Examples include children with advanced cystic fibrosis, severe immunodeficiency, Duchenne muscular dystrophy, and other severe, progressive neuromuscular diseases. (See "Duchenne and Becker muscular dystrophy: Management and prognosis", section on 'Prognosis' and "Cystic fibrosis: Management of advanced lung disease", section on 'Advanced care directives'.)

Nonprogressive, irreversible conditions with extreme vulnerability to health complications – Examples include children with severe developmental disabilities, such as those with severe cerebral palsy, hypoxic brain injury, or brain malformations. (See "Cerebral palsy: Overview of management and prognosis", section on 'Prognosis'.)

Progressive conditions without a curative option – Examples include certain genetic conditions such as trisomy 13, trisomy 18, and type II osteogenesis imperfecta. It is important to note that as novel therapies emerge and change the disease trajectory, management may shift from a palliative focus to a more curative focus. Examples include nusinersen therapy for spinal muscular atrophy type I, gene therapy for certain metabolic and immunologic disorders (adrenal leukodystrophy, adenosine deaminase deficiency), or supportive therapies such as heart surgery for infants with trisomy 18. Nevertheless, palliative care teams remain a valuable resource in these settings as they can assist families with decision-making and provide ongoing support since some of these novel therapies are experimental and the outcomes may be variable. (See "Osteogenesis imperfecta: An overview" and "Approach to prenatal diagnosis of the lethal (life-limiting) skeletal dysplasias" and "Spinal muscular atrophy" and "X-linked adrenoleukodystrophy and adrenomyeloneuropathy" and "Adenosine deaminase deficiency: Treatment and prognosis".)

Pediatric palliative care is provided to children with a wide range of primary conditions, including cardiac [3,4], pulmonary [5], neurologic and neuromuscular [6], and severe genetic and metabolic conditions [7-9].

For families and providers caring for children with serious illness and making decisions about palliative care, web-based resources such as the Courageous Parents Network can provide support and guidance.

TIMING — Families of children with a diagnosis where a cure may not be possible are ideally introduced to palliative care shortly after the time of diagnosis [10-12]. Palliative care is introduced from the beginning of the treatment by acknowledging that it helps to ensure an ongoing focus on quality of life with relief of symptoms so that the child's enjoyment of life can continue [13-17]. Palliative care may be introduced at any of the following time points:

Prenatally, when a life-threatening fetal diagnosis is made

When a new life-threatening diagnosis is made in a child

When disease-modifying treatment for a chronic condition starts to have declining benefit

When disease burden starts to impact a child's quality of life

Children with chronic, potentially progressive conditions, such as cystic fibrosis or severe neurologic impairment, may benefit from the introduction of palliative care if symptom management is difficult or if there is significant suffering. This often becomes evident at a time of decline in health status, which results in uncertainty regarding outcome and impacts quality of life [18].

The introduction of palliative care does not preclude the use of potentially curative or disease-modifying treatments [11]. Nonetheless, common barriers to palliative care are overly optimistic estimates of prognosis and concerns by the medical team that families "are not ready" [19]. The available evidence suggests that integration of palliative care does not lessen parents' hope [15]. In addition, prognostic information that clinicians consider upsetting does not lessen a parent's desire for this information and is often considered important to decision-making [14].

Palliative care recognizes that there is always care to provide, such as attention to physical symptoms or emotional distress, regardless of the clinical situation [2,11,20]. In the setting of a poor prognosis, palliative care has a positive benefit for preparing families for later bereavement [17]. Parents and families who are poorly prepared in addressing quality of life tend to choose more intensive and invasive interventions that may prolong suffering near the end of life [13,21].

GENERAL APPROACH — Integration of palliative care for a child requires a customized care plan, developed based on goals that enhance the quality of life of the patient and their family. Goals and the care plan are made collaboratively with patients and their families.

Effective palliative care typically requires the following [22]:

Interdisciplinary team – One clinician rarely has the expertise or time to address all of the care needs of the child and family.

Communication and building relationships – Building a trusting relationship between the medical team and the child and family. Effective communication and availability are key elements in successful implementation of palliative care.

Developing care plans based on goals – Decision-making about ongoing medical treatment based upon the goals for the total care of the child and family, rather than treating a clinical problem in isolation. This can help to avoid the pitfall of performing an intervention out of a sense of needing to do something when parents and clinicians become distressed over the limits of medicine. The care plan needs to adapt to alterations in the needs of the patient and family as the disease progresses.

Symptom management – Managing symptoms (eg, pain, nausea, vomiting, and respiratory distress) due to the underlying disease and ongoing medical treatment. Management includes anticipating and preventing symptoms whenever possible.

End-of life care – Preparing patients and their families for end-of-life care, including bereavement support for families.

Interdisciplinary team — Palliative care is only possible with an interdisciplinary team as one discipline cannot address all of the needs of the child and family [2,23]. Disciplines required for the spectrum of palliative care needs of the child and family typically include a clinician, clinical nurse specialist or advanced practice nurse, social worker, child life specialist, and chaplain. Interdisciplinary teams can help ensure that the emotional, spiritual, physical, and practical needs of children and families are identified and met.

Physical – Clinicians, including physicians, clinical nurse specialists, or advance practice nurses, can bring expertise in symptom management. It is always important to remember that physical symptoms improve with attention to each of the other three domains. Palliative care clinicians also can serve as facilitators between other health care providers and families and can help coordinate medical care.

Psychosocial – Social workers provide psychosocial assessment and supportive counseling for the child and family and identify community services.

Emotional – Child life specialists provide skills in facilitating "communication" with children through activities that assist with emotional distress and can provide important understanding into the fears and wishes of the child.

Spiritual – Chaplains support faith traditions and spiritual values that can comfort families as they face uncertainty, changes in medical status, loss, or grief and promote hope and emotional healing.

Palliative care teams can also provide a supportive environment and assist members of the health care team in managing their own distress. Provider distress is understandable and common when caring for a child with serious illness.

Coordination of care — Optimal care is only possible when critical information is documented and communicated to all parties involved in the child's care. This coordination of care must take into consideration the following:

Locations of care – Locations of care include medical facilities (clinics, hospitals, and emergency services) and community sites in which the child lives (home, respite care, school, and sometimes community-based transportation).

Individuals involved in the child's care – This includes family, guardian or health care proxy (often a family member), home care nurses, providers of care in foster care or group homes, school nurses and teachers, respite care providers, bus drivers, health care teams (primary care providers, specialty clinicians, other members of these medical teams), and palliative care/hospice teams.

It is also beneficial to identify (with parental/caregiver agreement) what information should be shared with these individuals. This can lessen any sense of "Why are they doing this?" and help direct others involved in the child's care to focus on the primary goal of enhancing the quality of life for the child.

Documented information should include the following:

Goals of care and how these goals guide decisions

Health care and symptom management plans

Location of health care for acute illness

Resuscitation status

Care plans for a life-threatening event at home and school

Contact information for individuals with expertise and availability to assist at times of acute events (designated clinician or palliative care/hospice team)

Communication and building relationships — Communication is critical for effective medical care in all clinical settings [24,25]. It is based upon building a strong relationship between the care team and the child and family [26]. Patients with life-threatening illnesses and their families report great benefits of having a long-term relationship with their care team in order to promote open and effective communication [26]. They appreciate clinicians who take the time to get to know the patients as individuals and demonstrate a respect for the views of the child and family [26]. It is essential to reach out to both the parents and the child to gain their perspectives and explore their hopes, concerns, and goals of ongoing care.

Parents — The following open-ended questions are a good starting place to begin to understand the parents' perspectives:

Tell us about your child before they became ill.

What makes your child happy? Sad?

What is your understanding of your child's illness/condition?

What have the past few weeks or months been like? What are you anticipating?

What are your hopes? What are your worries?

In light of your understanding of your child's illness, what is most important to you and your family?

Parents have a broad range of beliefs of what they perceive they should do to be a good parent for their seriously ill child [27]. The process of adjusting to a change in prognosis requires transitioning from one set of goals (ie, curing the condition) to another (ie, reducing suffering and maintaining the child's quality of life) [28]. Hopeful patterns of thinking and the use of heuristics to simplify complex situations play particularly important roles in communication and parental decision-making [29-31].

Talking to children — When a child is facing a life-threatening condition, parents are the legal decision makers about treatment options, including the introduction of palliative or hospice care services. However, children should be involved in their treatment decisions at a developmentally appropriate level. Even young children can voice their preferences about treatment and care decisions [32,33]. Helping children understand what is happening to them physically, emotionally, and spiritually facilitates their comfort and reduces their anxiety. In particular, adolescents with terminal illnesses generally want to be involved in the medical decision-making process [34,35].

Children report that they are more comfortable with clinicians who take the time to communicate with them directly. The following are age-appropriate tips when talking with children [36].

Children two to five years of age benefit from:

Simple, concrete information with adequate age-appropriate explanations

Comfort, reassurance, and the constant presence of caring family members

Involving parents in care and explanations

Answering questions calmly and with examples

Using play, puppets and dolls, expressive therapies, and storytelling for teaching and expression of emotion

Follow-up questions to "check in" about the child's understanding

Children six to nine years of age:

May become preoccupied with details and ask the same questions repeatedly

May feel responsible for the illness and need to be reassured that it is not their fault

Continue to find family to be very important and are reassured by the constant presence of caring family members

Still think concretely but benefit from more information about what is happening to them

Benefit from reading, playing, drawing, art, and music as appropriate modalities of intervention

Children 10 to 12 years of age:

Become very aware of feeling different around their peers at school

May exhibit stoic and brave responses in an attempt to protect their parents and caregivers

Benefit from a safe environment to explore fears, hopes, and expectations

May benefit from meeting with other children or caregivers away from their parents

May try to protect their parents and family

May benefit from reading, playing, drawing, art, and music interventions, along with peer-based support

Adolescents:

Display a wide range of responses to facing critical and life-threatening illness

Feel caught between finding independence and feeling the pull of dependence that can be brought on by illness

May have difficulty talking with non-ill peers because they feel "different"

Benefit from being matched with other adolescents also facing illness

Benefit from activity-based groups not focused on illness

Benefit from creative outlets, including art, dance, music, videography, blogging, and writing

Spirituality — Spirituality represents an individual's relationship to the meaning of life and his/her sense of peace, purpose, and connection to others [37]. For some families, spirituality may be found and expressed through an organized religion, but for others, spirituality is expressed outside of such structures. Interdisciplinary care that explores spiritual needs can provide an avenue for expression of the beliefs, values, hopes, and fears that ultimately guide decisions and impact grieving. Providing assistance to families regarding spirituality and discussion about death often includes the patient, parents, and the child's siblings. (See "Overview of spirituality in palliative care".)

Serious illness naturally raises questions for patients and parents of "why me?", with a desire to seek meaning at such times. Attending to the spiritual needs of the child and family can help the child manage these questions, prepare parents for such questions, and provide hope and comfort at the end of life [38-40]. Spiritual care may also assist with bereavement outcomes [41].

In children, components of spirituality include unconditional love, hope, safety, security, forgiveness, and legacy. The awareness of these needs and their meaning develop through childhood as follows [42]:

Infants and toddlers (0 to 3 years of age) – Needs include trust, a sense of hope and equanimity among others, and feelings of self-worth and love.

Preschool-age children (3 to 6 years of age) – In addition to the above, spirituality is imaginative and participation in ritual becomes important.

School-age children (7 to 12 years of age) – Concern over right and wrong and connecting ritual with personal identity are added components in this age group.

Adolescents (age 13 to 17 years of age) – Adolescents search for meaning, purpose, hope, the value of life, and an evolving relationship with a higher power.

Children also have a developmental understanding of death based upon age. Children understand death as a changed state as early as three years of age, universality by approximately five to six years of age, and personal mortality by eight to nine years of age [43]. In addition, the concept of death is often influenced by personal experiences and culture.

Hope — One barrier to initiating advanced care discussions is the fear of taking away hope [44]. Clinicians may also fear that they will create false hope if they support hopeful statements from a parent. However, some evidence suggests that parents who exhibit hopeful patterns of thinking and emotion may ultimately be more likely to institute limitations of care for their children compared with parents who are less hopeful [30]. Hope can be an effective adaptive approach to adversity for some and can be a means to convey commitment and love to one's child [45].

Establishing individualized goals of care — The medical team and family need to establish patient-centered goals of care with attention to relief of the child's and family's distress. The goals of palliative care do not necessarily exclude the goals of disease-directed therapy. For example, children with cancer and their parents often have simultaneous goals for palliative care to lessen suffering and for cancer-directed therapy to extend life [13].

The child's well-being should be the primary guide. The job of the parents and care providers is to watch, listen, and reflect honestly on what is in the best interest of the child. This process includes a reflection on the benefits and adverse effects of past interventions, and the expected outcome of current and future treatment. The following series of questions/issues are useful to consider and review with families while developing goals of care that can help instruct care plans.

Goals often identified by children and their families include comfort and quality of life. Are there other goals that are important to you (your child) and your family?

We know that children and their parents often have concerns that may change over time. These may include: Will I (my child) get better? Is there more that could be done? What are some of your worries?

Are you experiencing less benefit with less return to prior baseline over time from available chronic and acute treatments (eg, longer periods of illness or a shorter time between each episode)?

How much are you (is your child) able to enjoy the things that are important to you (your child), such as relationships and activities?

How many days each week are you (is your child) having difficult days?

If it would be helpful today, we can talk through some of the "what ifs" so as to address any worries you may have and to allow you to focus on loving your child. This may include discussing what to expect and developing a care plan for potential future needs, such as maintaining comfort.

We will carefully review whether interventions being considered will maintain or improve health, meet goals of care, or prolong a process of decline or suffering.

Medical decision-making

Initiating therapies of uncertain benefit — During the course of a life-threatening condition or when a life-threatening fetal diagnosis is made, any decision to implement therapies of uncertain benefit needs to be considered within the context of the goals of the child (or fetus) and family [11,12].

The following steps are useful in such circumstances:

Identify the likelihood of an intervention meeting the goals of care of the patient and family.

Know and discuss the evidence for the possible benefit and harm of the interventions available.

Define a time period in which the intervention would be expected to meet the identified goals.

Discuss a backup plan in case the hoped-for benefit does not occur or increased suffering occurs. This may include stopping the treatment or shifting the overall focus from curative treatment to comfort measures.

To avoid misunderstanding during these discussions, it should be made clear to the family that the care team is not trying to limit treatments that may have reasonable benefit. The goal is to protect the child from interventions with little or no benefit that may harm or prolong suffering and to provide care that maximizes comfort throughout the child's life.

Resuscitation and life-sustaining measures — In a child with life-threatening illness, invasive and life-sustaining interventions, such as resuscitative measures (eg, intubation), are available even at the end of life. Prior to a life-threatening event, it is important to consider whether such interventions should be performed. Early discussion allows children and parents to make decisions that fit with their values and goals of care [10,12]. This assessment should consider whether any of the proposed interventions (eg, intubation, chest compressions, defibrillation) will result in a desired outcome.

Discussing resuscitative and life-sustaining efforts requires an honest assessment of the likelihood of benefit and possible harm and is based upon the steps discussed above. It is important that the care team assume responsibility for this process so as to not imply undue parental responsibility for any decision regarding life-sustaining measures. This is ideally a process of shared decision-making. As an example, avoid asking "Do you want your child to be intubated?". This focused question implies offering something that would benefit the child and shifts the decision-making burden to the parents. It is also important to reassure patients and families that a decision to forgo life-sustaining measures will not alter ongoing care of a patient.

This is also a time to remind families that when there is a decline in health, it is not a result of any decisions made to limit interventions or of the care provided at home but rather a result of the health problems that cannot be "cured" or "fixed." The family should be reassured that interventions will continue as long as they maintain sufficient benefit to quality of life.

Perinatal palliative care — Assisting families facing a life-threatening fetal diagnosis involves unique challenges yet utilizes the principles outlined above. Families benefit from the opportunity to prepare and plan prior to delivery. This planning involves supportive care for the mother during labor and delivery, involvement of the father or other coparent when appropriate, and care plans that account for variable outcomes of the baby. A birth plan can be developed that indicates the care plan for the baby following delivery. Communication is an essential part of planning to ensure that interventions that are consistent with the parents' goals for their baby are implemented. This may involve a decision not to resuscitate (including intubation and insertion of intravascular access) and to focus on the infant's comfort while parents maximize their time together as a family. When appropriate, parents can have supportive plans in place if the infant survives, such as identifying home-based palliative and hospice care. There are a number of excellent resources to provide guidance [46,47].

Health care proxy and guardianship — Appointing a health care proxy allows a patient over 18 years of age to designate someone to make medical decisions if the patient is unable to make or communicate those decisions. A social worker or other trusted team member involved with the patient can assist with the process of identifying someone the patient trusts and filling out the necessary documentation. This process also provides an opportunity to discuss goals of care, revisit key medical decisions, and assure a clear understanding of the patient's preferences.

For those unable to participate in decision-making (incapacitated), guardianship involves obtaining the legal authority to make decisions for another person. Parents must seek guardianship if their child will remain incapacitated once 18 years of age.

SYMPTOM MANAGEMENT — Symptoms commonly identified in children with life-threatening illness include [48-52]:

Pain

Dyspnea

Sleep disturbance

Nausea and vomiting

Anorexia and weight loss

Fatigue

Depression and anxiety

Delirium and agitation

Anemia and bleeding

Seizures

These are often distressful for both the child and family. As a result, management includes preventive measures and treatment directed towards reducing or eliminating symptoms (table 1).

Nonpharmacologic interventions — Nonpharmacologic interventions are an essential part of symptom-management strategies. These include:

Physical measures, such as cuddling, massage, heat, cold, and physical and occupational therapy

Cognitive behavioral measures, such as guided imagery, meditation, hypnosis, distraction, storytelling, music, and art therapy

Increasingly, complementary and alternative interventions (CAM) are being used to treat physical and psychological symptoms in pediatric oncology patients [53-56]. Several of these interventions, including hypnosis and mind-body therapies, are efficacious interventions for pain and anxiety in children [56,57]. Hypnosis has been effective in lessening procedural distress, reducing procedural time, and reducing procedural pain and anxiety for children [55,58]. Acupuncture has also been reported to reduce pain and anxiety in children [55]. (See "Complementary and integrative health in pediatrics", section on 'Complementary therapies'.)

Pain — Pain is a common symptom that often causes distress for both the child and family. Effective pain management includes identifying pain and assessing its severity, preventive measures, and treatment to either eliminate or reduce pain, which is discussed in detail separately. (See "Pain in children: Approach to pain assessment and overview of management principles" and "Cancer pain management with opioids: Optimizing analgesia".)

Dyspnea — Dyspnea is the experience of feeling short of breath, which may be due to difficult or painful breathing. It is a common symptom found in numerous medical disorders. Objective measures (respiratory rate, oxygen saturation) and clinical observations do not necessarily correlate with the patient's perception of breathlessness. A child may be able to report the degree of distress associated with dyspnea using a scale similar to the visual analog scale used for pain. Although instruments are available for assessing dyspnea, including the Respiratory Distress Observational Scale, which is used in adults unable to report about symptoms, they have not been validated in children [59,60]. (See "Assessment and management of dyspnea in palliative care".)

Treating dyspnea is typically focused on identifying and treating the underlying cause. Causes of dyspnea include:

Pulmonary disease (eg, progressive neuromuscular disorders and primary interstitial disease)

Anemia

Airway obstruction (eg, from tumor)

Heart failure from cardiac disease

Opioids are the primary therapy used to alleviate dyspnea that persists despite optimal medical treatment of identified causes. Other useful interventions include a trial of oxygen, cool air from a fan or open window directed at the face, repositioning, hypnosis or other relaxation techniques, and lorazepam for associated anxiety.

We typically use low-dose opioid therapy in children with refractory dyspnea. A suggested starting dose for morphine sulfate in a child who has not previously received opioids is 25 to 30 percent of the dose used for pain (ie, 0.1 mg/kg per dose administered orally every three to four hours as needed; maximum initial dose 5 mg). If the child is already receiving an opioid agent, the dose is increased by 30 percent.

Studies involving adult patients with cancer, chronic lung disease, and cardiac disease have demonstrated improvements in refractory dyspnea with morphine sulfate, without development of respiratory depression [61-64]. Although similar data are lacking in children, it is reasonable to expect similar benefits.

Sleep disturbance — Sleep disturbances in children who are seriously ill include difficulties in falling asleep, trouble staying asleep, early-morning awakening, complaints of nonrestorative sleep, and periods of too much sleep. (See "Insomnia in palliative care" and "Sleep disorders during and after cancer in children".)

Sleep may improve with management of contributing problems (eg, pain, depression, medications). Treatments include pharmacologic and nonpharmacologic modalities.

Psychological and behavioral interventions include stimulus control, sleep restriction, sleep education, relaxation training, and combinations of these therapies [65].

Pharmacologic treatments include melatonin [66], antidepressants (eg, tricyclic antidepressants and trazodone) [67-69], clonidine [70], and antipsychotics [65,71]. Benzodiazepines tend to be overused, leading to dependency and tolerance, and should only be used in a time-limited manner [72].

Nausea and vomiting — Nausea and vomiting are common symptoms in patients with chronic diseases that may be due to the underlying disorder or its treatment. The underlying pathophysiology is a complex process that involves abdominal vagal afferent input, vestibular system, and two areas of the brainstem: the area postrema (referred to as the chemoreceptor trigger zone) and the nucleus tractus solitarius (referred to as the vomiting center) (figure 1). Neurotransmitters of primary importance within this process include histamine, dopamine, 5-hydroxytryptamine (serotonin), and substance P (neurokinin-1 receptor). (See "Assessment and management of nausea and vomiting in palliative care".)

Symptom management is based upon determining and eliminating the underlying cause. Medications can cause nausea, and it may be necessary to discontinue or replace them. As an example, opioids are a common source of nausea and vomiting, as they stimulate the area postrema mediated through dopamine 2 receptors and the vestibular system, and also cause constipation. Treatment includes rotation of different opioids, prevention of constipation, and the use of dopamine receptor antagonists.

In addition, therapy for nausea and vomiting includes management of electrolyte abnormalities, managing mucositis, treating constipation, and the use of antiemetic therapy, if needed. In children, several classes of antiemetic drugs are available that generally antagonize the neurotransmitter receptors involved in the physiology of nausea and vomiting (table 1). (See "Characteristics of antiemetic drugs".)

Anorexia and cachexia — Anorexia and cachexia are commonly seen with progression of a life-threatening disease. Contributing factors include [73,74]:

Pain

Medications that impair taste or decrease hunger

Depression

Gastrointestinal problems, such as nausea, constipation, gastritis, mucositis, oral candidiasis, and dysphagia

Treatment should be directed at identifiable causes. Disease treatment can improve these symptoms. Pharmacologic interventions that can increase appetite include megestrol acetate, cannabinoids, and corticosteroids. Their benefit may be limited and may not result in an increase in body mass even when an increase in appetite is seen [75,76]. (See "Assessment and management of anorexia and cachexia in palliative care".)

In oncology patients, poor nutritional intake and increased metabolic demands related to cancer appear to be the primary underlying causes. The clinical features, pathogenesis, and treatment of cachexia due to cancer are discussed in detail separately. (See "Pathogenesis, clinical features, and assessment of cancer cachexia" and "Management of cancer anorexia/cachexia".)

Hydration and nutrition — Some children may develop swallowing impairment as a manifestation of disease progression (eg, due to brainstem involvement of a central nervous system tumor, progressive neurologic disease, or other progressive disease). In such circumstances, enteral or parenteral nutrition and hydration may be considered, and it is imperative to discuss and balance the benefits of the intervention with the potential complications as the child's disease progresses [77]. The discussion should include the following issues:

Identify interventions that may improve anorexia and cachexia (eg, treatment for constipation and nausea), but balance the information with acknowledgment that these interventions may not be successful in improving the child's appetite or weight as a result of disease progression.

Inform the family/caregivers that providing enteral and parenteral nutrition may not reverse the cachexia seen in some patients with chronic life-threatening disease (eg, cancer) [78-80].

Review the potential adverse effects of enteral nutrition, including nausea, vomiting, aspiration, oral secretions, and edema as the body becomes unable to process medically provided nutrition and hydration.

Inform the family/caregivers that children can live comfortably for long periods of time with minimal fluids and nutrition.

Review the child's goals of care and discuss strategies that are consistent with the goals. For some families, placement and use of a gastrostomy tube may not fit with goals of care. Other families may choose to use a feeding tube for nutrition but to give tastes for pleasure by mouth, especially when eating provides significant pleasure to the patient.

Discussing nutrition and hydration can be difficult given the deep instinct of parents to provide nutrition to their children and its association with comfort. Understandably, this makes it difficult for families to consider that artificial nutrition may not provide benefit or may actually cause harm. (See "Overview of managing common non-pain symptoms in palliative care", section on 'Limited role for artificial nutrition and hydration' and "Stopping nutrition and hydration at the end of life".)

Fatigue — Fatigue is identified by parents as the most common symptom in the last month of life and the source of greatest distress for children with cancer [48,50]. This symptom is also the least likely to be addressed [48].

Factors that contribute to fatigue include anemia, infection, pain, impaired nutrition, deconditioning, sleep disturbance, depression, anxiety, and medications [81]. In children with advanced disease, opioid analgesics may contribute to diminished levels of energy and decreased ability to concentrate [82]. Assessment instruments are available to assess fatigue in children [83,84]. (See "Overview of fatigue, weakness, and asthenia in palliative care".)

Treatment is generally focused upon the underlying cause of fatigue (eg, anemia). A multimodal approach to treatment may be most effective. Interventions include:

Treatment of depression, anxiety, and/or sleep disturbance, if present.

Exercise programs within the child's tolerance level.

Rest and frequent naps [85].

Modifying activities (including playing/socializing).

Use of psychostimulants, such as methylphenidate, can be used to increase wakefulness [86]. Given the short duration of action, the timing of doses can be controlled to coincide with important events during the day, such as visits with family and friends.

Depression and anxiety — Children with a life-threatening illness commonly experience symptoms of depression and anxiety [87,88]. Such symptoms can be difficult for children to separate out from physical symptoms [89]. They are often interrelated symptoms, and addressing each of them is usually important to the relief of the others [90]. These symptoms vary in severity from "feeling sad and anxious," a normal adaptive response, to those that meet diagnostic criteria for a psychiatric disorder (eg, major depressive disorder), which causes significant distress or interferes with day-to-day function [48,50,87,88,91-98]. (See "Pediatric unipolar depression: Epidemiology, clinical features, assessment, and diagnosis".)

Both psychotherapeutic and psychopharmacologic treatments are used in treating depression and anxiety in children with life-threatening illnesses. Management of depression in these children is based upon the same principles of treatment for depression in adult patients receiving palliative care or those with cancer, or physically healthy adolescents. (See "Overview of anxiety in palliative care" and "Assessment and management of depression in palliative care" and "Management of psychiatric disorders in patients with cancer".)

These include:

Nonpharmacologic therapy – Child life specialists, child psychologists, or other trained experts are essential members of the team to assist children with expressing emotional symptoms of distress through age-appropriate activities. In particular, guided imagery and hypnosis are effective tools in children with depression and anxiety [99].

Pharmacologic therapy – Psychopharmacologic treatment can be considered for children with symptoms of depression and anxiety that persist despite nonpharmacologic interventions or in those with more severe symptoms that meet diagnostic criteria for a psychiatric disorder.

Agents commonly used for treating depression in the palliative care setting include selective serotonin reuptake inhibitors (SSRIs) and psychostimulants (eg, methylphenidate) [100-102]. (See "Assessment and management of depression in palliative care", section on 'Pharmacotherapy'.)

Benzodiazepines are commonly used for some short-term management of anxiety symptoms. SSRIs are often used to manage chronic anxiety or to treat mixed symptoms of anxiety and depression. (See "Overview of anxiety in palliative care", section on 'Pharmacotherapy'.)

Delirium and agitation — Delirium and agitation are challenging problems for many seriously ill patients.

Delirium – Delirium is a disturbance of consciousness and altered cognition that develops acutely (usually hours to days) and tends to fluctuate during the course of the day. It is most commonly seen in patients with complex underlying medical conditions. (See "Diagnosis of delirium and confusional states".)

Causes include the following:

Medications (opioids, anticholinergics)

Metabolic disturbances (infection; dehydration; and renal, liver, and electrolyte abnormalities)

Brain metastases

Sources of discomfort (pain, dyspnea, muscle spasms, position, constipation)

Psychosocial contributors (emotional and spiritual distress, vision or hearing impairment)

Agitation – Agitation is an unpleasant state of arousal. It may present as loud speech, crying, increased motor activity, increased autonomic arousal (eg, diaphoresis, increased heart rate), inability to relax or concentrate, or disturbed sleep-rest pattern. Although symptoms of agitation can overlap with anxiety, they generally tend to have more motor than psychological manifestations.

Management of both delirium and agitation involves treating the underlying cause when possible. Medications that can help manage the symptoms of delirium and agitation include benzodiazepines and antipsychotic medications. It is also important to rule out conditions that give the appearance of agitation, such as akathisia (an unpleasant state of motor restlessness), from antidopaminergic medications, myoclonus, withdrawal from opioids, and paradoxical reactions from medications. (See "Delirium and acute confusional states: Prevention, treatment, and prognosis" and "Overview of managing common non-pain symptoms in palliative care", section on 'Delirium'.)

Anemia and bleeding — Children with anemia may present with fatigue, dyspnea, or significant dizziness. In these patients, a red blood cell transfusion may improve their quality of life [103]. As time progresses, the symptomatic benefit from a transfusion may decrease as the disease progresses, offsetting the benefit compared with the risk of transfusion-related complications.

An increased risk of bleeding can occur in children with cancer due to a decrease in either platelet count or function, typically caused by an adverse effect of therapy or underlying disease. In these patients, mucosal bleeding can sometimes be controlled with aminocaproic acid given orally or intravenously to inhibit fibrinolysis [104]. Topical options include fibrin sealants [104]. The tannins present in black teas can also help to stop bleeding. At home, patients can press a wet tea bag onto bleeding gums. More significant bleeding may require platelet transfusions.

Seizures — Seizures do not cause significant suffering for the patient beyond damage caused by the unconscious movement, but they are highly distressing for families. Seizures may occur near the end of life as a result of central nervous system disease, intracranial hemorrhage, electrolyte abnormalities, fever, or hypoxia. Management options include the transmucosal administration of antiepileptic therapy. (See "Seizures and epilepsy in children: Refractory seizures", section on 'Home rescue therapy (transmucosal antiseizure medications)'.)

END-OF-LIFE CARE — At some point, a cure becomes no longer realistic or disease-modifying therapy is no longer beneficial in some children with life-threatening illness and the goals of treatment shift. Care at the end of life is focused on comfort for the dying child and minimizing suffering (eg, distress and anxiety) for both the patient and family. It requires understanding and anticipating the needs of the patient and family and managing the social, legal, economic, and institutional circumstances that surround the child's death. Although preparing families for a child's death can be difficult, it is tremendously beneficial for families. A number of factors contribute to the quality of end-of-life care provided to children in the inpatient setting (table 2) [105].

Planning for the death of a terminally ill child requires an honest discussion of plans that will meet goals of care, identify current and future care needs, and determine what resources in the community are available to meet these needs [12].

Preparation and support — Guidance in the preparation and support of a child and family as the child's death approaches is aided by the following steps:

Parents and caregivers can be encouraged to maintain their daily and familiar routines to help families cope by keeping control in some aspects of their lives.

Family distress can be lessened by reviewing anticipated changes in appearance that occur as death approaches [16]. Physical changes include weakness and fatigue, decreasing appetite and intake, skin changes (eg, cooling and mottling), sedation or confusion, changes in the pattern of breathing, increase in noise with breathing from respiratory secretions, and loss of ability to close the eyes.

Families benefit by replacing their usual care routines and interventions for their child with care directed to the child's comfort. This can include mouth care with moistened sponges, personal closeness and contact, selecting favorite music, and reading stories.

The clinician can proactively prescribe and ensure availability of medications that manage anticipated end-of-life symptoms (table 1) [106]. (See 'Management of end-of-life symptoms' below.)

Management of end-of-life symptoms — The care of a terminally ill child should ensure that the child is without pain or distress. Symptoms that may require management at the end of life include pain, respiratory distress, and agitation. Management of each of these symptoms is addressed in previous sections (see 'Symptom management' above), summarized briefly here (table 1), and discussed in greater detail separately. (See "Palliative care: The last hours and days of life", section on 'Management of specific symptoms'.)

Pain and dyspnea – Opioids are the mainstay of management of pain and dyspnea at end of life. For patients with significant opioid tolerance or neuropathic pain that is poorly responsive to opioids, ketamine may be of benefit as a co-analgesic [107-109]. (See "Pain in children: Approach to pain assessment and overview of management principles", section on 'Opioids' and 'Dyspnea' above.)

Agitation and anxiety – Treatment generally consists of benzodiazepines (eg, lorazepam and/or or continuous midazolam) or an antipsychotic drug (eg, haloperidol). (See 'Depression and anxiety' above and 'Delirium and agitation' above.)

Seizures – Seizures are treated with midazolam, lorazepam, or diazepam. (See 'Seizures' above.)

Respiratory secretions – Respiratory secretions that result in noisy breathing can be treated with an anticholinergic agent (scopolamine, hyoscyamine, or glycopyrrolate). (See "Palliative care: The last hours and days of life", section on 'Airway secretions'.)

The role of artificial nutrition and hydration – Like other medical interventions, the decision to provide artificial nutrition and hydration should be evaluated by weighing its benefits and potential complications in light of the clinical circumstances and goals of care with the parents/caregivers and other family members [110,111]. It is important that the family be ready to discuss whether it is better to initiate or forgo artificial nutrition and hydration for the child based on their understanding of the child's quality of life and the effects of administering or withholding nutrition and hydration [112]. If the decision is made to withhold it, support from the care team for the family is imperative as there are often feelings of guilt. The pros and cons of administering artificial nutrition and hydration in terminally ill patients are discussed separately. (See "Overview of managing common non-pain symptoms in palliative care", section on 'Limited role for artificial nutrition and hydration'.)

Palliative sedation – Although rarely needed, palliative sedation is used as a last resort when all other methods of controlling pain, dyspnea, agitation, and myoclonus have failed [113-115]. It provides enough sedation so that a child with a terminal illness becomes unconscious, with the goal to relieve their suffering [113,114]. Benzodiazepines or barbiturates are the agents most commonly used as palliative sedatives [116]. Further discussion of palliative sedation is covered separately. (See "Palliative sedation".)

Location of death — Planning for the death of a terminally ill child includes deciding upon the preferred location of death [12]. Locations include the home, hospice facilities, and inpatient hospital units. Parents/caregivers who are able to plan for the location of death generally feel more prepared for the child's death and are less likely to have doubts or regrets later on about the setting of their child's death [117].

Planning begins with the palliative team explaining treatment options. An important part of the planning process is involvement of the child when appropriate. When the preferred site is at home, discharge planners and social workers help arrange for a hospice service or for other resources within the community.

One study of 140 parents who lost a child to cancer reported that planning for the location of death resulted in more home deaths compared with no planning (72 versus 8 percent) [117]. For children who died in the hospital, planning resulted in more deaths in the inpatient units rather than the intensive care unit and fewer children being intubated.

Hospice services — Increasingly, there are hospice services that fulfill the needs of terminally ill children.

Benefits of hospice – Hospice teams are ideally suited to provide comprehensive services to the dying child, siblings, parents/caregivers, and extended family [118,119]. The hospice team is interdisciplinary and includes clinicians, nurses, social workers, chaplains, bereavement staff, child life specialists, and mental health and complementary medicine therapists. In a retrospective survey, parents of children who received end-of-life home hospice services as part of their oncology care more often reported their child had fun (70 versus 45 percent), experienced events that added meaning to life (89 versus 63 percent), and died at home (93 versus 20 percent) compared with parents of children who died without home hospice involvement [119].

Barriers – Despite the recognition that pediatric hospice services are effective in providing palliative care in the home of a dying child, there remain barriers in the provision of hospice services for terminally ill children [118]. These include:

Limited hospice services – Many fewer children than adults die, which creates a challenge of maintaining trained staff that are able to provide palliative care to children.

Cost and reimbursement of hospice care – Hospice reimbursement is often based on a per-visit mode (per diem) at a rate often ranging from $125 to $190 per day. This rate is intended to cover all of the personnel, medications, equipment, and supplies that the child needs. In the United States, there may be requirements for insurance coverage, such as a life expectancy of no more than six months and an absence of life-sustaining treatments (eg, chemotherapy).

Unfamiliarity or discomfort with hospice care – Many clinicians caring for children are unfamiliar with palliative pediatric care and hospice services. As a result, referrals to hospice services are limited.

Funding – In the United States, the Patient Protection and Affordable Care Act (PPACA) allows terminally ill children who are enrolled in a Medicaid or state Children's Health Insurance Plan (CHIP) hospice benefit to concurrently receive curative care related to their terminal health condition [120].

When death occurs — In the United States, local regulations generally require that a clinician pronounce death and complete a death certificate. If hospice is involved, the family should be directed to call the hospice and a nurse will generally come to the home and pronounce the patient.

For patients who die in the hospital, the covering clinician is typically summoned to pronounce the patient. The clinician should inform the family that the child has died in a clear and compassionate manner. The clinician should also inform the family of the option to pursue autopsy and organ or tissue donation. Family communication regarding organ donation is discussed separately. (See "Assessment of the pediatric patient for potential organ donation", section on 'Family communication'.)

In many areas, local regulations require that pediatric deaths be reported to the local medical examiner (coroner), who may choose to investigate the death and may perform an autopsy [121]. This typically happens when the circumstances surrounding the death are uncertain. In cases where the child's death was expected, examination by the coroner is usually not necessary; however, the decision is at the discretion of the coroner. If the coroner declines the case, the family can be offered the option of pursuing a medical autopsy. Autopsy may provide important information both for the care team and family. In particular, the review of autopsy results with a trusted provider may allow lingering questions or concerns that are causing parental distress to be answered [122].

In other countries, different procedures regarding pronouncement of death and notifying local authorities are used.

Bereavement — Bereavement after the death of a child for parents, siblings, and other members of the family can be profoundly distressing, complicated, and prolonged [123-127].

Parents – The loss of a child distinct from other forms of bereavement for many reasons, including [123,125,128]:

The death of a child is against the natural order of events

Parents need and believe in their ability to protect their child

Witnessing the suffering of one's child is devastating

Parents may experience a catastrophic loss of control and potentially loss of their parental identity

Siblings – Bereaved siblings may experience social isolation and withdrawal [129,130]. Involving siblings throughout the illness trajectory, allowing them to have safe outlets for their feelings, and creating opportunities for their needs to be identified and met have all proven to be useful interventions [129].

Bereavement follow-up is an intrinsic component of comprehensive palliative care. Resources to help grieving families include [25,131]:

Continued contact with the care team [12]. Families may continue to have medical questions or misconceptions following the death, which a trusted team member can address. Often, the family has established a significant bond with the team, and continued support avoids a sense of abandonment on the part of the family. In particular, care teams should reach out to families at anticipated difficult times, such as the first holiday season, first anniversary of the child's birthday, and first anniversary of the death.

Support groups are often helpful to bereaved families. Parents who have access to psychosocial support prior to the child's death are better able to work through their grief [132].

Educational materials about the process of grief and mourning. There are resources that are written both for adults and children.

Grief counseling with therapists and/or clergy.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Palliative care".)

SUMMARY AND RECOMMENDATIONS

What is palliative care? – Pediatric palliative care utilizes an interdisciplinary team to focus on preventing and relieving suffering in children with life-threatening illnesses, regardless of the stage of disease. It comprehensively addresses the physical, psychosocial, and spiritual needs of the child and family. (See 'General approach' above and 'Interdisciplinary team' above.)

Patient selection – Conditions in which palliative care is beneficial include those in which curative treatment is possible but may fail (eg, cancer), progressive conditions without curative treatment (eg, trisomy 13), chronic conditions with intensive medical long-term therapy (eg, advanced cystic fibrosis), and severe nonprogressive conditions with health complications (eg, severe cerebral palsy). Palliative care is optimally introduced shortly after a life-threatening diagnosis is made or, in children with chronic diseases, when there is a decline in health status from baseline. (See 'Patient selection' above and 'Timing' above.)

Key aspects of palliative care – Effective palliative care includes the following:

Strong relationship between the medical team and the child and family, resulting in successful, open, and effective communication. (See 'Communication and building relationships' above.)

Establishing goals of care that are focused on quality of life and preventing and relieving suffering. (See 'Establishing individualized goals of care' above.)

Medical decision-making that is consistent with the established goals of care, particularly regarding decisions to initiate therapies of uncertain benefit and to provide resuscitation and life-sustaining measures. (See 'Medical decision-making' above.)

Managing symptoms such as pain, dyspnea, sleep disturbance, nausea and vomiting, anorexia and weight loss, fatigue, depression and anxiety, delirium and agitation, anemia and bleeding, and seizures. (See 'Symptom management' above.)

Preparation and support of the child and family for end-of-life care including management of end-of-life symptoms, selection of preferred location of death, and discussion of the options of autopsy and organ donation. (See 'End-of-life care' above.)

Bereavement support for the family following the death of the child. (See 'Bereavement' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Barbara L Jones, PhD, and Joanne Wolfe, MD, who contributed to an earlier version of this topic review.

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