Palliative Care

C HAPTER

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PALLIATIVE CARE Kristen G. Schaefer, Janet L. Abrahm, and Joanne Wolfe

Palliative care is an approach to care for children and adults provided by an interdisciplinary team with a focus on individual patient and family goals, values, preferences, and relief of suffering in the face of serious illness. In a seminal 2003 report,1 and again in the recent “Dying in America: Improving quality and honoring individual preferences near the end of life”,2 the Institute of Medicine (IOM) defined the unique role of palliative care for children and adults with serious illness: “Palliative care seeks to prevent or relieve the physical and emotional distress produced by a life-threatening medical condition or its treatment, to help patients with such conditions and their families live as normally as possible, and to provide them with timely and accurate information and support in decision making.” The IOM report also outlined the need to improve patient access, education of health professionals, and research in palliative care (Table 92.1). In 2006, hospice and palliative medicine was accepted as a specialty by the American Board of Medical Specialties. In 2007, the Accreditation Council for Graduate Medical Education began accrediting fellowship programs, and the first qualifying examination in hospice and palliative medicine was given in 2008. Palliative care subspecialists provide clinical support in more complex situations and are also responsible for improving palliative care education, innovation and research, but all clinicians should integrate high-quality palliative care into their care of patients with serious illness.3 Palliative care practitioners have expertise in communication, in treatment of physical symptoms, and in relieving social, psychological, and spiritual and existential distress. Such care and assistance is not limited to people thought to be dying and should be available concurrently for patients receiving curative or life-prolonging treatments. Emerging evidence suggests that concurrent palliative care in serious illness can decrease costs and improve outcomes, and patient access to high-quality specialty-level palliative care is becoming the standard of care at most academic cancer centers2,4 However, overall rates of referral to palliative care remain low, particularly for patients with hematologic malignancies.5 Recently published oncology consensus clinical guidelines6 highlight the integral role of specialty-level palliative care throughout the cancer trajectory, and align with increasing public demand for better advance care planning and more timely referral to specialty-level palliative care and hospice.2 This chapter will review core elements of palliative care for children and then for adults in each section, with specific attention to growing awareness and understanding of palliative care needs in patients with hematologic malignancies.

• The heterogeneity of illnesses, many rare, requires the involvement of many disciplines and specialists. • Many children have genetic diseases, so there may be more than one affected child in a family. • The time course of some illnesses can be extremely variable. Pediatric palliative care may extend over years, even decades. • A broad developmental spectrum is represented, including changes in the individual child through time. • The underlying principles and ethics of palliative care are universal across the life span. However, as in all specialties, children bring with them unique issues and dilemmas. • A child or adolescent diagnosed with a life-threatening or lifelimiting illness throws an assumed sequence out of order. A time of role reversal is expected, when children will care for dying parents. When parents instead find themselves watching their child face death, a sense of tragic absurdity prevails. Not only is time shortened, but its order is shattered. A child or adolescent with a life-threatening illness represents a premature separation to the family. Even before the child has become a differentiated individual through a natural developmental sequence, that child is wrenched away. There is little preparation for separation by death when a psychological separation has not yet been effected. The adolescent who is beginning to negotiate an independent existence is often the hardest to face when that “moving forward” is irreversibly halted, or at least disrupted. A child has not even had the time to begin to form life goals. • The necessity for palliative care—the concept and the clinical approach—may emerge at different points in the illness trajectory, depending on the prognosis for the child, the decisions that must be made in choosing treatment options, and always, the management of pain and suffering in the provision of optimal quality of life. One of the foremost goals is to initiate palliative care for children earlier in the illness trajectory—in a proactive manner— so that effective care planning can be implemented. Care of the family, with a particular focus on the young siblings, is a priority.

COMMUNICATION

Pediatric palliative care is an emerging frontier in the comprehensive care of children.8 Specific issues related to palliative care for pediatric patients and their families include the following:9

Good communication can dispel fears of abandonment. Breaking bad news and discussing prognosis with patients with advanced disease are occasions when clinicians can demonstrate their commitment to an ongoing partnership with patients and families. Conversations must demonstrate respect for cultural differences and the conviction that psychosocial and spiritual growth can occur even at the end of life. If done well, the groundwork will be laid for further discussions of patient hopes and fears, goals, values, and spiritual concerns that form the basis of decisions about resuscitation and artificial life support. These conversations should be documented in the medical record, and recorded as a physician order when possible, for example, a signed MOLST (Medical Order for Life-Sustaining Treatment) or POLST (Physician Order for Life-Sustaining Treatment) form.2 Communication with a child…

• Smaller numbers of dying children than adults mean that there is less professional expertise and underrepresentation of children in palliative care protocols.

When I first heard my diagnosis, one question kept going around and around in my head: “How long do I have, Doc?” — (12-year-old child)7

PEDIATRIC PALLIATIVE CARE: SPECIFIC ISSUES I just wish that I had armfuls of time.

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— (4-year-old child)7

Chapter 92  Palliative Care

TABLE 92.1

Summary of the Institute of Medicine Report: When Children Die: Improving Palliative and End-of-Life Care for Children and Their Families

Improve Organization and Delivery of Care Emphasis is placed on the development of care guidelines and protocols in all pediatric settings, the development of regional information programs and resources in rural areas, and policies and procedures for involving children in decision making Reform Financing of Palliative Services and Hospice Care Vast changes in public and private health coverage: add hospice, change eligibility rules, provide outlier payments, extend coverage for counseling family members and for bereavement follow-up Better Prepare Health Professionals Create educational experiences and curricula that will provide basic and advanced competence in palliative, end-of-life, and bereavement care Strengthen Research Base for Effective Care Emphases include appropriate quality-of-life measures, effective symptom management, impact of perinatal death on parents and siblings, impact of sudden death on family and professional caregivers, efficacy of bereavement interventions, models for provision of care, financing alternatives, effective strategies for educating professionals From Institute of Medicine: When children die: Improving palliative and end-of-life care for children and their families, Washington, DC, 2003, National Academy Press.

Children with serious hematologic disorders have usually lived with the illness over a prolonged period of months or years. Their knowledge, understanding, and awareness of their precarious life situation is often profound, at physical, cognitive, and emotional levels. The doctors think my bone marrow is fine for now—and for now is for now. — (8-year-old child)7 The protective stance of the past stated that disclosure to the child of his or her prognosis (and even, in some instances, the diagnosis) would cause increased anxiety and fear. Since the 1980s, however, a shift toward open communication has been evident.10 To shield the child from the truth may only heighten anxiety and cause the child to feel isolated, lonely, and unsure of whom to trust. In communication with the life-threatened child at any juncture in the illness, the precedent for a climate that enables such honest interchange is created from the time of diagnosis. The individual child’s competence and vulnerability serve as the context for decisions regarding disclosure at any point in the illness trajectory. Considerations about what or how much to tell include the child’s age, cognitive and emotional maturity, family structure and functioning, cultural background, and history of loss.7 These same factors apply at the end of life, with extreme sensitivity to how the parents have chosen to inform the child throughout the illness experience, how the child has understood and processed information up to this time, and what the child is now asking—implicitly and explicitly—about his or her situation. For adult patients with advanced cancer, having had a discussion about end-of-life wishes with one’s physician has been shown to correlate with less aggressive medical care near death, earlier hospice referrals, cost savings at the end of life, and improved bereavement adjustment for caregivers.2 Furthermore, having had conversations about end-of-life wishes is not associated with higher rates of major depressive disorder or more worry. Proactive, direct communication with patients and their families about approaching and managing the end of life can be a satisfying and rewarding part of patient care. However, more training in palliative care clinical and communication skills is needed during hematology/oncology fellowship and in continuing medical education settings to ensure competency in these areas.

TABLE 92.2

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Breaking Bad News

1. Make yourself, the patient, and the family comfortable 2. Find out what they know 3. Indicate that you are planning to tell them something that is unpleasant and may be disturbing 4. Find out whether they want to be told, or whether they want someone else to be told 5. Find out how much they want to know (i.e., the big picture versus all the details) 6. Tell them in words they can understand, allowing time for questions along the way 7. Respond to their feelings 8. Let them know that this is only the first of many discussions with you 9. Ask them to summarize what they heard you say; ask if they have further questions 10. Arrange your next meeting with them From Abrahm JL: Update in palliative medicine and end-of-life care, Annu Rev Med 54:53, 2003; and Back A, Arnold R, Tulsky J: Mastering communication with seriously ill patients: Balancing honesty with empathy and hope, New York, 2009, Cambridge University Press.

Breaking Bad News I just wanted to be told the truth and not too bluntly, but the truth nonetheless. So that I could make a decision myself, be able to accept it, decide what I was going to do about it. — Adult cancer patient seeking a second-opinion hematology-oncology consultation11 Table 92.2 contains an outline of the suggested steps to take when breaking bad news.12 For patients with advanced disease, the goal is to establish or strengthen trust, and reassure them that the physician is committed to caring for them even though their disease cannot be cured. To do this well, physicians need to believe that they have not failed the dying patient, even if medicine has.

Prognosis and Decision Making Surveys of bereaved parents indicate that physician communication about prognosis is not optimal. At the same time, emerging data suggest that bereaved parents consider high-quality communication as the most important value when reflecting on physician quality of care.2 Parents value clear information that is communicated sensitively and includes the child, when developmentally appropriate. When it comes to discussing prognosis, a majority of parents want as much information as possible.13 Furthermore, although many parents find prognostic information about their child upsetting, they still want prognosis to be discussed. The data suggest, however, that parents are overly optimistic about the child’s chances of cure in comparison to physicians, and this is especially true when the prognosis is uncertain. Being aware of these trends may help physicians to discuss prognosis with greater clarity. One side of my head says: “Think optimistic.” The other side says: “What if this treatment doesn’t work?” — (11-year-old-child)7 The child is often aware of the diminishing curative or life-prolonging options that he or she faces. It is at this time that the child may ask anxiously: “What if this medicine doesn’t work? What will you give me next?” The child experiences a profound sense of loss of control. It is at this time that families are confronted with a series of decisions regarding the nature and intensity of medical interventions they wish to pursue. This process can be excruciating: they do not want their child to suffer more, yet they often cannot tolerate the thought of

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missing an opportunity for a cure or prolonged time. The physician and team’s role shifts from leadership in recommending a curative treatment plan to the clarification of experimental and palliative options and consequences. In most instances, the parents make the decision; however, to varying degrees, when asked in a sensitive manner, children as young as 10 years of age are able and willing to talk about their experiences and end-of-life decisions.8 During the last decade there has been increased recognition of the child’s participation in making treatment decisions. Crucial to this process is an assessment of the child’s or adolescent’s ability to appreciate the nature and consequences of a specific medical decision. This becomes particularly complex when the wishes of the child differ from those of the parents. Because actual assessment tools are only in the early stages of development, professionals must rely exclusively on their clinical judgment to assess children’s understanding of the contingencies they are facing. This is often a juncture when input from members of the interdisciplinary team can be crucial: children often express their understanding, awareness, and thoughts about treatment options and living or dying to individuals other than their parents or primary physician. The following example illustrates the remarkable capacity of a young child to address the transition towards end of life. A 7-year-old girl told her parents that she was too tired to fight anymore, and that she wanted to give up. She added: “If I have to continue suffering, I would rather be in heaven.” These statements were major determinants in the parents’ choosing a palliative care plan without any further attempts at life-prolonging treatment. She went home on hospice care and died peacefully several weeks later.7 Adult patients and families also want to be prepared for the end of life, to be able to name someone to make decisions, know what to expect about their physical condition, have financial affairs in order, know that the physician is comfortable talking about death and dying, feel that the family is prepared for their death, have funeral arrangements in place, and have treatment preferences in writing.12 For this to happen, patients must know how long they are likely to have left to live. For adults with refractory hematologic malignancy, prognostic uncertainty about when to stop therapy in pursuit of cure and when end-of-life begins can be a barrier to palliative care referral.14 The median survival of older adults with leukemia is not that different from advanced pancreatic cancer or stage IV lung cancer … but we are stuck with this tail and so what do we do with that? We live on the tail, this 5% to 10% tail. — (Hematologic oncologist at an academic cancer center)15 Although most patients report wanting prognostic information from their oncologist, patients with advanced refractory disease may not ask about their prognosis, and oncologists often do not initiate the discussion. In one recent observational study of patients after their first oncologic visit for a hematologic malignancy,16 only half of patients were told unambiguous prognostic estimates for mortality or cure. Discussions of prognosis can be very painful for clinicians, who may experience feelings of guilt, failure, or sadness. Clinicians interested in improving their skills may refer to practical educational handbooks and articles that outline how to discuss these issues both with patients who want to know their prognosis and with those who do not or are ambivalent.17 Online resources for communication in serious illness training include the Center to Advance Palliative Care (capc.org) and VitalTalk (vitaltalk.org).

Impact on Hope The rigors of treatment regimens and the physical and emotional demands of the complex care required for patients with advanced disease tend to isolate patients and their families and focus all their

hopes on disease remission. They may have forgotten how to hope for anything else. Physicians and the teams they work with can help patients with advanced hematologic diseases develop new kinds of hope by encouraging them to reintegrate into activities that were meaningful before their disease began and they rearranged their lives around treatment schedules. Paradoxically, discussion about limited prognosis can lessen fears of abandonment and strengthen the trust patients have in the oncology team.12 As disease progresses, despite ongoing treatment, patients who have begun to reengage in non– treatment-related activities and who have developed a broader relationship with their physicians are more likely to understand that the physician is not abandoning them when he or she says that the goals of treatment should be comfort.

CAREGIVERS People who are not caregivers don’t understand the continuous burden of the role … the stress feels as if I’m constantly holding my breath. — (Family member about caring for a loved one with advanced disease)2 Families and other nonprofessional caregivers, who provide the vast majority of care for patients with advanced cancer, are stressed by the patient’s disability and degree of suffering, the lack of coordination of care, and underlying family, work, or financial pressures.18 As many as 32% of these caregivers either have a major psychiatric condition (panic disorder, major depression, posttraumatic stress disorder, or generalized anxiety disorder) or access mental health services after the patient’s diagnosis.2 Caregivers are likely to need the support of many members of the team therefore to continue in their difficult role.

RELIEF OF SUFFERING Suffering includes physical, psychological, social, and spiritual or existential dimensions.

Symptom Management in Children Therapist: If you could choose one word to describe the time since your diagnosis, what would it be? — Child: PAIN.7 Maintaining patient comfort is a critical issue throughout treatment, as well as during the end stages of life. Although effective pain control is a hallmark of palliative care, pain is only one of many distressing symptoms. The spectrum of physical symptoms includes (although it is not limited to) dyspnea, fatigue, seizures, loss of appetite, nausea and vomiting, constipation, and diarrhea. Early studies among bereaved parents or children who died of cancer indicate that optimal symptom management is still far from being achieved, even in major pediatric teaching centers,19 and in one study more than 10% of parents had considered hastening their child’s death; this was more likely if the child was in pain.20 A recent study among children with advanced cancer confirmed high suffering from physical and psychosocial symptoms according to the children themselves.21 Relief of a child’s end-of-life distress may have long-lasting implications for bereaved parents, who are negatively affected by the child’s experience of pain years beyond the death. Psychologic symptoms such as depression and anxiety are also prevalent in children at the end of life.19 Children also experience existential concerns. Creating opportunity for communication around these sources of distress involves using creative strategies that incorporate the developmental stage of the child. Strategies may involve verbal communication using open-ended questions such as “What are you hoping for?” and “What are you worried about?” However, many children communicate best through nonverbal means such as artwork and music. Children may be more willing to

Chapter 92  Palliative Care

“talk things over” with puppets or stuffed animals rather than real people. Importantly, euphemistic expressions about death can be confusing or even frightening (for instance, equating death with sleep may result in the child’s being afraid of going to bed) and should be avoided. Needless to say, parents of children with hematologic malignancies also experience distressing symptoms such as anxiety, depression, and spiritual and psychosocial concerns. Recognition of these by the pediatric clinician may serve families well while the child is alive and during bereavement.

Symptom Management in Adults Patients with hematologic malignancies experience a high physical and psychologic symptom burden, similar to patients with metastatic solid tumors, especially during periods of treatment, when hospitalized, or with advanced disease.22 Pain and antiemetic therapy for adults is reviewed elsewhere in this volume (see Chapter 91). Anxiety, depression, delirium, and control of symptoms occurring in the last days of life are reviewed subsequently. Among social sources of distress are financial concerns and, with increasing debility, loss of independence and sense of contribution and efficacy. Worries about burdening the family or that the family will fail them when they really need them may lead patients to request physician-assisted suicide. Social workers are the key team members who can help alleviate or at least ameliorate these sources of distress, and can help the caregivers cope. Physicians should also explore religious and spiritual concerns, and understand what rituals will be important at the end of life. Spiritual and existential distress occur when individuals are unable to find sources of meaning, hope, love, peace, comfort, strength, and connection in life, or when there is dissonance between their beliefs and what is happening to them. Patients who use “positive” religious coping (e.g., prayer, feeling a sense of connectedness to a religious community, having a positive relationship with God) have been found to have better mental health status, growth in the spiritual dimension with stress, and a better overall quality of life. Patients who use “negative” religious coping (e.g., ascribe their illness to a punishing God or one who has abandoned them) have a poorer quality of life.23 Clinicians should therefore include either a formal or informal spiritual assessment for all patients diagnosed with serious illness.

PSYCHOLOGIC CONCERNS Clinicians must assess and attend to patients’ and families’ psychosocial distress, including developmental issues, meaning and impact of illness, coping style, impact on sense of self, relationships, stressors, spiritual resources, economic circumstances, and physician–patient relationship; they must be able to distinguish normal human reactions of grief, sadness, despair, fear, anxiety, loss, and loneliness in patients facing the end of their lives, from clinical anxiety and depression.24 Up to 50% (or more) of patients with advanced cancer meet criteria for a psychiatric disorder when the diagnosis of adjustment disorder is included.25

Anxiety and Depression It is noteworthy that depression and anxiety are often not recognized as symptoms in children, and in many instances are inadequately addressed. Significant anxiety is found in approximately 25% of adult patients with cancer,25 and anxiety symptoms can interfere with their ability to receive care. Patients with panic disorders, agitated depression, phobias, obsessive-compulsive disorder, delirium, posttraumatic stress disorder, or adjustment disorders can all present with anxiety. Anxiety in dying patients may arise from worries about the future (uncontrolled symptoms, family concerns, or concerns about death),

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isolation from loved ones, sepsis, hypoxia, metabolic abnormalities, withdrawal from alcohol, opioids or benzodiazepines, drug reactions (e.g., akathisia from metoclopramide, phenothiazines, and butyrophenones; paradoxical agitation from benzodiazepines and olanza­ pine), and uncontrolled pain. Nonpharmacologic treatments, such as relaxation training, hypnosis, supportive psychotherapy, and counseling, are very effective. Pharmacologic treatments usually include benzodiazepines (e.g., the short-acting lorazepam, starting dose 0.5–2 mg every 8 hours as needed; or long-acting clonazepam, starting dose 0.25–0.5 mg orally (PO) two-times daily), selective serotonin reuptake inhibitors (SSRIs; see later), and, when there is evidence of delirium, neuroleptics (see later). It is estimated that 5–26% of patients with advanced cancer meet criteria for a major depressive disorder, and patients with high symptom burden such as patients facing bone marrow transplantation are at higher risk.25 It can be difficult to discern which patients with advanced disease are depressed or grieving. The usual somatic signs of depression or grief (e.g., anorexia, sleep disturbances, fatigue, or weight loss) are common in this population. Depressed patients, however, will be anhedonic and feel worthless, guilty, hopeless, or helpless. Grieving patients, in contrast, are very sad, but they are able to find happiness in some circumstances and can plan for the future.25 Pain, a past or family history of substance abuse, depression, or bipolar illness are major risk factors for depression. Terminally ill patients responding “Yes” to the screening question “Are you depressed?” are very likely to be confirmed as depressed in a more comprehensive evaluation. Useful follow-up questions include “How do you see your future?” “What do you imagine is ahead for yourself with this illness?” “What aspects of your life do you feel most proud of? Most troubled by?” As part of the treatment of depression, pain must be brought under control. Counseling can explore patient fears, provide emotional support, and help patients review their lives and find the meaning and areas of accomplishment in them. A variety of models of therapy are used, and none has been shown to be superior over the others. The psychostimulants dextroamphetamine and methylphenidate (2.5–5 mg, 8 AM and noon; maximum dose 60 mg daily) often act within a few days. The SSRIs are the first choice when immediate onset is not needed because they usually take several weeks to show effect. Useful agents include citalopram (Celexa) and paroxetine (Paxil; 10 mg PO daily initially; maximum 40 mg PO daily); escitalopram (Lexapro; 10 mg PO daily initially; maximum 20 mg PO daily); sertraline (Zoloft; 50 mg PO daily initially, maximum 200 mg PO daily); fluoxetine (5–10 mg PO daily initially; maximum 60 mg PO daily); and the serotonin–norepinephrine reuptake inhibitor venlafaxine (Effexor; 37.5 mg PO twice daily initially; maximum 225 mg PO daily). Venlafaxine inhibits norepinephrine, serotonin, and dopamine reuptake. Major side effects of the SSRIs include hyponatremia, sexual dysfunction or loss of libido, and gastrointestinal complaints (e.g., nausea, diarrhea, and foul-smelling flatus). Modafinil may also be an effective adjuvant agent to reduce SSRI-related sedation. The exact mechanism of action of mirtazapine (Remeron; 15 mg PO at bedtime initially; maximum 45 mg PO at bedtime) is unknown. If the patient is expected to live longer than weeks to a few months, a stimulant and an SSRI should be started simultaneously, and the stimulant can be titrated off several weeks later. Tricyclic antidepressants are less useful in these patients because of their sideeffect profile. If the patient does not respond to first-line agents, a psychiatrist should be consulted. Referral to a psychiatrist is also necessary when the physician is unsure of the diagnosis; the patient is psychotic, confused, or delirious; the patient previously had a major psychiatric disorder; the patient is suicidal or requesting assisted suicide; or there are dysfunctional family dynamics.

Delirium Delirium occurs in up to 80% of patients dying from advanced cancer and can cause distress and anxiety in caregivers.12 Delirious

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patients can be agitated, hypoactive, or vacillate between the two. Symptoms of delirium include insomnia and daytime somnolence, nightmares, restlessness or agitation, irritability, distractibility, hypersensitivity to light and sound, anxiety, difficulty in concentrating or marshaling thoughts, fleeting illusions, hallucinations and delusions, emotional lability, attention deficits, and memory disturbances. Validated delirium screening and severity tools are available, but a comprehensive psychiatric evaluation is recommended to exclude other disorders, such as anxiety, minor depression, anger, dementia, or psychosis. The cause of delirium is often never determined and is frequently multifactorial. Medications, especially opioids, nonsteroidal antiinflammatory drugs, and high-dose corticosteroids, commonly contribute. Opioid-induced central nervous system toxicities are more common in patients with renal dysfunction, on high doses of opioids for long periods of time, with impaired cognition before starting the opioids, with dehydration, or taking other psychoactive drugs. Other causes include metabolic abnormalities (hypercalcemia, hyperglycemia, or uremia), malnutrition, hypoxia, fever, infection, uncontrolled pain, hepatic failure, primary brain tumor, and brain metastases. Treatment for delirium should begin while the underlying cause(s) are being treated. In addition to the medications listed in Table 92.3, it is helpful to make the patient’s surroundings as familiar as possible, restore aids to hearing and sight if they are needed, reorient the patient frequently, and have family members, friends, or well-known caregivers present.

MANAGEMENT CONCERNS DURING THE LAST DAYS OF LIFE Evidence suggests that children with advanced cancer who receive concurrent home-based palliative care have improved quality of life at the end of life and are more likely to die at home.26 For some

TABLE 92.3

families, there is the possibility of planning ahead and choosing a setting for their child’s death—home, hospice, or hospital. The child may express a preference about where he or she feels safe or prefers to be. Clear information about how the child is likely to die and professional support to validate the family’s choice are crucial. Even more important is the explicitly stated “permission” from all members of the professional team that the family may change their choice freely at any time—that all options remain open and that no decision is irrevocable. In the past, siblings were rarely included in these discussions and were often inadequately prepared for the eventuality of a child dying at home. It is only recently that their voices are beginning to be heard.

The Dying Child Therapist: Are you in any pain? Does anything hurt? Child: My heart. Therapist: Your heart? Child: My heart is broken. I miss everybody.7 The distillation of anticipatory grief to its essence marks the imminence of death. At times imperceptibly, at other times dramatically, the child who has been living with the illness is transformed into a dying child. The end point of the terminal phase is often marked by a turning inward on the part of the child, a pulling back from the external world. Cognitive and emotional horizons narrow, because all energy is needed simply for physical survival. A generalized irritability is not uncommon. The child may talk very little and may even retreat from physical contact. Although such withdrawal is not universal, a certain degree of quietness is almost always evident. The child is pulling into himself or herself, not away from others. This behavior is a normal and expectable precursor to death—a form of preparation for the ultimate separation that lies ahead.7

Treatment of Delirium (Adult Patient)

Drug

Dose

Typical and Atypical Antipsychotics 1–4 mg PO, or 0.5–2 mg SQ, IV Haloperidol qhs or bid to tid

Comment Do not exceed 20 mg in 24 hours Can add the same dose q4h prn Maintain the patient on the effective dose (divided into a bid dose) for 3–4 days, then taper over 1 week, as tolerated Oral dose is 60–70% as potent as parenteral dose

Quetiapine

25–200 mg PO qhs

Particularly useful in elderly patients with evening delirium Start 25 mg hs for 3–4 days

Olanzapine

2.5–5 mg PO/SL

Start 2.5–5 mg PO/SL qhs to bid (2.5 mg for elderly patients); can use q 4–6 hours prn agitation Maintain the patient on the effective dose (divided into a bid dose), then taper over 2 weeks, as tolerated; also antiemetic

Aripiprazole

5 mg PO qd

Do not exceed 30 mg Does not prolong and may shorten QTc

Chlorpromazine Benzodiazepinesa Lorazepam

12.5–1000 mg PO/IV/PR

Sedating; may cause significant hypotension

0.5–1 mg q 1–2 hours

Add to antipsychotic for patients with an agitated delirium Tablets can be used PR for terminal delirium

Diazepam

5–10 pm PO bid

Useful PR for patients unable to take oral medication

Clonazepam

0.5–5 mg PO/SL bid to tid

Tablets have been used PR for terminal delirium; do not exceed 20 mg/24 hours

Midazolam

30–100 mg IV/SQ over 24 hours

IV drip or subcutaneous infusion for terminal delirium

a

Caution: Any benzodiazepine may exacerbate delirium, especially in older adults (>70 years of age). q, Every; hs, at bedtime; d, day; bid, twice a day; tid, three times a day; prn, as needed; IM, intramuscularly; IV, intravenously; PO, orally; SL, sublingual; PR, rectally; SL, sublingually; SQ, subcutaneously. Modified from Abrahm JL: A physician’s guide to pain and symptom management in cancer patients, ed 3, Baltimore, 2014, Johns Hopkins University Press; and Miovic M, Block S: Psychiatric disorders in advanced cancer. Cancer 110:1665, 2007.

Chapter 92  Palliative Care

TABLE 92.4

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Treatment of Common Problems in the Final Days (Adult Patient)

Problem

Agent(s)

Routes, Doses

Baseline pain

Concentrated oxycodone or morphine solution Morphine or hydromorphone tablets Fentanyla Methadone liquid Acetaminophen, naproxen Dexamethasone (requires compounding)

PO/SL q4h around the clock; individualized PR q4h; individualized Transdermal; individualized PO; individualized PR tid to qid PR daily to bid

Breakthrough pain

Concentrated oxycodone or morphine solution Fentanyla

PO or per gastric tube q4h around the clock; individualized Transmucosal (buccal, sublingual); individualized

“Death rattle”

Scopolamine Hyoscyamine Glycopyrrolate Atropine

Transderm Scop patch 1–3q3 days; gel 0.125–0.25 SL tid to qid 0.2–0.4 mg IV tid to qid or 1–2 mg po bid to tid 0.4 mg SL q4–6h

Dyspnea (anxiety)

Lorazepam Chlorpromazine

1 mg PO, SL, q2–4h 25 mg PO, PR q4–12h; or 12.5 mg IV q4–8h

Dyspnea (other)

Morphine/oxycodone Morphine

5–10 mg SL oral concentrate q2h 2–4 mg IV q1h

Nausea

Combinations of lorazepam, metoclopramide, dexamethasone, and/or haloperidol

PR q6h; compounded suppositories with desired agents (depending on presumed cause of nausea)

Anxiety

Lorazepam Diazepam suppository

1 mg PO, SL, q2–4h 5–10 mg PR daily

All liquid PO medications can be given per gastric tube. a Fentanyl only for opioid tolerant patients. h, Hour; IV, intravenously; PO, orally; PR, rectally; q, every; qid, four times a day; SL, sublingually; tid, three times a day. Modified from Abrahm JL: A physician’s guide to pain and symptom management in cancer patients, ed 3, Baltimore, 2014, Johns Hopkins University Press.

Adults Common physical symptoms that occur in the last week to days before an adult’s death from cancer include pain (70%), noisy or moist breathing (60%), urinary incontinence or retention, dyspnea, and nausea and vomiting.12 Patients may also experience fatigue. Hunger and thirst are unusual. Treatments for problems at the end of life are reviewed in Table 92.4. Patient and family wishes and options about the setting for end-of-life care should be explored. Some evidence suggests that patients with cancer who die at home have better quality of life, and their caregivers have better bereavement outcomes than cancer patients who die in the hospital. However, given the potentially high symptom burden in the final moments of life, such as bleeding or dyspnea, more research is needed to understand barriers to hospice care and patient and family outcomes in hematologic malignancies.27

HOSPICE PROGRAMS In the four weeks prior to his death, my father lived under the care of five different institutions in two states. Only the last place, the hospice, appeared willing or able to provide care and comfort to a man who was obviously at the end of his life.2 — (Bereaved family member) In the United States, most hospice care takes place in the home, although patients can be admitted to nursing homes for brief periods (usually 5 days) to provide a respite for the family caregivers, or to the hospital (usually for up to 14 days) if symptoms cannot be controlled at home. Early referral to hospice programs improves outcomes, and in many cases hospice care is the only effective way to support these patients and families at home at the end of life. The Medicare Hospice Benefit does not require a do not resuscitate status, but it does require that the attending physician and the hospice medical director certify that the patient has a prognosis of 6 months or less to live if the disease follows its usual course. Medicare

reimburses hospice programs about $186 per day per patient (as of fiscal year 2013) to provide the routine care described in Table 92.5. Therefore, the cost of transfusions typically required for many patients with hematologic malignancies, even at the end of life, may make it difficult for hospice programs to enroll patients insured by Medicare alone. Other insurance programs may allow their patients to receive transfusions and hospice care. Notably, many children are not referred to hospice because their illness experience is inconsistent with hospice specifications—prognosis is uncertain; there is a blending of goals, which can result in more costly health care; and providers lack pediatric expertise. Importantly, the Patient Protection and Affordable Care Act now requires state Medicaid programs to allow children with a life-limiting illness to receive both hospice care and curative treatments concurrently; the full effect of this change remains to be seen.

BEREAVEMENT Bereavement follow-up by the professional team is an intrinsic component of comprehensive pediatric palliative care. Bereaved families often express the sentiment of a double loss: loss of their child and loss of their oncology team whom they have known and trusted, often over months and years. Parental grief has been recognized as more intense and longer lasting than other types of grief. Contact from a team member after the child’s death can assuage the family’s sense of abandonment and the palliative care team can serve a crucial preventive role by identifying families at particular risk for prolonged grief disorders and identifying resources for them. Each bereaved person’s loss is unique, but many people manifest similar symptoms of grief, some of which become less persistent as they rebuild their lives. Recurrent intense symptoms typically occur at the anniversary of the death of the patient but can occur at unpredictable times, induced by reminders of the deceased. Survivors appreciate calls or letters from the patient’s physician and nurses. For patients enrolled in hospice programs, a formal bereavement program is offered for the family throughout the first year after the patient’s

1494 TABLE 92.5

Part VIII  Comprehensive Care of Patients with Hematologic Malignancies

Specialty Level Palliative Care Versus Hospice Care

Palliative Care

Hospice Care

Interdisciplinary Model of Care • Clinical specialty, offers expert: • Symptom management and communication • Psychosocial and spiritual care • Inpatient, outpatient and home care consultations to the primary team • Coordination of care among treating teams Eligibility • Any patient with serious or life-threatening illness • Any stage the illness • Concurrent with curative or disease-directed therapies Interdisciplinary Consult Team Palliative care physicians, advance practice nurses, physician assistants, nurses, social workers, chaplains, and bereavement counselors and others

Interdisciplinary Program of Care • Medicare hospice benefit, delivers: • Symptom management and communication • Psychosocial and spiritual care • Home, inpatient, or respite care in a nursing home under the direction of the patient’s physician • Continuity with referring care team Eligibility • Estimated 6 months or less prognosis • Eligible for Medicare or secondary insurance • Focus is quality not life prolongation Interdisciplinary Care Team Hospice medical director (physician), advance practice nurses, physician assistants, nurses, social workers, home health aides, chaplains, volunteers, administrative personnel, medical consultants, occupational therapists, physical therapists, speech therapists, and bereavement counselors.

death. After the formal program ends, the bereaved are welcome to continue to participate in any bereavement activities that have been meaningful to them. At the time of death, survivors may seem numb, confused, or dazed and experience disbelief. By the second month after the death, yearning has replaced disbelief. During the next months, disbelief, depressed mood, and yearning decline gradually, and by 6 months after the death, most people will have accepted the reality of the death and are beginning to think about reengaging in relationships and work, discovering new meaning and purpose. Siblings are especially vulnerable in the year following a child’s death.28 By a year or two, most survivors have accommodated to their loss. They become aware of the changes that must be made if they are to resume old relationships and responsibilities, or to establish new ones and risk recurrent loss. About 10–20% of survivors, however, suffer either from depression and/or from a symptom complex previously called complicated grief, now identified as prolonged grief disorder.12 Patients with depression manifest symptoms of sadness, anhedonia, and psychomotor retardation, but they are not yearning for the deceased or unable to accept the death. Depressed survivors benefit from counseling and consideration of pharmacologic treatment. Patients with prolonged grief disorder, in contrast, have grief symptoms that last beyond 6 months and cause functional impairments. Such patients are at increased risk for medical and psychiatric illness and should be referred for psychiatric or spiritual counseling. Persons at higher risk for this disorder include those with a history of attachment disorders (childhood abuse, childhood separation anxiety), aversion to lifestyle changes, being unprepared for the death and unsupported after it, and a particularly interdependent relationship with the deceased.

SELF-CARE FOR CLINICIANS Even while providing steady care for the patient and family, professional caregivers are often experiencing their own distress in a sort of parallel process. The professional often feels anguish and helplessness in witnessing a child endure pain and suffering—physical or psychic. He or she often identifies with the parents of the child. This reaction intensifies when the caregiver is also a parent, especially if his or her healthy child is the same age as the patient. For the caregiver who does not yet have children, the specter of a fatally ill child may loom threateningly. In surveys, medical and nursing staff often cite the personal pain of losing a child as the most difficult experience in their work with dying children. Special attention should be paid to the

grief experienced by trainees with little previous experience with death and dying. Interns are in special need of emotional support following a patient’s death. Reviewing each death on the next morning’s rounds provides the needed debriefing and shows respect for the patient who has died. When possible and it feels appropriate, clinicians can write a card or attend the funeral or memorial service, which may facilitate closure. For all these reasons, the professionals who engage in this extraordinarily rich and demanding work articulate significant needs for support themselves. Otherwise, the toll of cumulative unresolved grief exacts a heavy toll in their personal and professional lives. A cohesive team and/or the opportunity for individual and group consultation are crucial for those who are intimately engaged in repeated cycles of attachment.

CONCLUSION AND FUTURE DIRECTIONS Patients with hematologic malignancies and their families face unique challenges compared with patients with solid tumors. These challenges include: the need to undergo high-risk treatments with significant symptom burden and prolonged hospitalization to achieve cure; significant prognostic uncertainty; difficult decisions about the benefits and burdens of life-sustaining transfusions of blood products when the prognosis of the cancer is weeks to months; increased likelihood of receiving more aggressive care in the last month of life; and more deaths in the hospital.5,22,27 There is growing evidence that integration of palliative care into the care of patients with hematologic malignancies is achievable and can improve patient and family outcomes.29 Appropriate triggers for members of the hematologic oncology team to request a palliative care consultation could include patients with high risk of refractory symptoms (e.g., severe graft-versus-host disease) or high mortality risk (e.g., relapse after bone marrow transplant, or hospitalized patients with end-stage disease). Research is needed to characterize and alleviate the symptom burden of patients with hematologic malignancy at all points in their illness trajectories,30 and measure the effectiveness of specialty-level palliative care interventions to relieve patients’ and families’ suffering.14

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