e2 Abstract Although decision making at the end of life most com monly rests with a child’s parents, there may be times when a parent requests therapies that are deemed inappropriate by the clinical t[.]
e2 Abstract: Although decision-making at the end of life most commonly rests with a child’s parents, there may be times when a parent requests therapies that are deemed inappropriate by the clinical team As truly futile treatment is difficult to define, deliberation over possibly inappropriate therapies should focus on intensive communication and negotiation, with hospital processes available for support and deliberation when this fails Rationing decisions should not be made for an individual patient when evaluating the appropriateness of a treatment at the end of life Rather, policies on rationing should be made at the institutional level The majority of deaths that occur in the pediatric intensive care unit so following a decision to withdraw or withhold lifesustaining treatments There is no legal or moral distinction between withdrawing and withholding treatment The doctrine of double effect supports the use of the titration of sedatives and analgesics to ensure comfort at the end of life Key words: Ethics, end-of-life decision-making, treatment, pediatric critical care, end-of-life care, double effect 19 Palliative Care in the Pediatric Intensive Care Unit ALISA VAN CLEAVE, EILEEN RHEE, AND WYNNE MORRISON Caring for children with life-limiting illnesses is an important role for the pediatric intensivist Over the past decades the overall mortality rate of pediatric intensive care units (PICUs) in US teaching hospitals has decreased by half, due in part to medical and technologic advancements.1 These advancements have increased the longevity of children with diagnoses that were previously uniformly fatal and have resulted in a growing number of children who live with chronic, lifelimiting conditions, many of whom are technology dependent Children with complex chronic illnesses represent an increasing proportion of hospitalized pediatric patients, many of whom require frequent care in the PICU.2 In this population of patients, the intensivist must evaluate the child’s illness trajectory, quality of life, symptom burden, and family preferences for care during each admission To adequately care for children with chronic life-limiting conditions, as well as those who are near the end of life, pediatric intensivists must have a high degree of competency in core palliative care skills, including communication, shared decision-making, appropriate limitation of interventions, and pain and symptom management This chapter explores the practice of palliative care in the PICU by intensivists, palliative care providers, and the interdisciplinary team Palliative Care Consults in the Pediatric Intensive Care Unit The compelling need for patient- and family-centered care (PFCC) and the broad range of pathophysiology that exists in ICUs demand a mixed model of integrative and consultative palliative care, 158 • • Pediatric intensivists must have a high level of competency in core palliative care skills, including communication, shared decision-making, appropriate limitation of interventions, pain and symptom management, and end-of-life care Mastery of communication skills is a vital part of critical care training When in doubt, talk less and listen more When considering the limitations of interventions, clinicians should elicit a family’s values, goals, and hopes for their child and develop recommendations for care aimed toward achieving those goals • • • • PEARLS Compassionate extubation is an important intensive care unit skill that requires meticulous planning and preparation for both family members and staff When the goals of care shift toward comfort, pain and symptom management must be prioritized, using both pharmacologic and nonpharmacologic interventions Indications for consultation by a specialty palliative care team include complex decision-making and communication support, advanced symptom management, need for enhanced family support, or transition to hospice which allows for a wider distribution of a limited subspecialty resource.3,4 Primary palliative care is an integrative model that focuses on maximizing and standardizing palliative care practices that clinicians routinely incorporate into the care of their patients.5,6 Secondary palliative care uses consultation of a palliative care team for complex, subspecialty-level problems Secondary palliative care helps ensure that there is adequate assessment and management of symptoms—as well as attention to emotional and psychological distress, practical and financial concerns, and spiritual and cultural needs—as part of comprehensive PFCC.3 Indications for specialty palliative care consultation include complex decision-making and communication support, symptom management, optimization of quality of life, hospice transition, and end-of-life care that extends beyond usual care practices Parents of critically ill children often face difficult, value-laden decisions amid bewildering amounts of information and an “irreducible amount of uncertainty.”7 Communication expertise around eliciting patient and family preferences and translating those preferences into decision-making are part of the core set of skills for intensivists Palliative care specialists can provide additional support and guidance in particularly complex situations to help elucidate goals of care for patients and families.8 Communication The importance of communication in the intensive care setting has become abundantly clear over the past decades Healthcare providers communicate with families in many ways in the ICU, CHAPTER 19 Palliative Care in the Pediatric Intensive Care Unit but the hallmark of ICU communication is the family meeting In a typical family meeting, members of the medical team sit down with the patient’s family away from the bedside to formally discuss the patient’s care Commonly, these meetings are organized by the medical team to facilitate difficult conversations, including the delivery of bad news, discussions regarding goals of care, and end-of-life care preferences.9–12 Suboptimal Communication in the Intensive Care Unit Excellent bidirectional communication between providers and families is an essential part of providing comprehensive, familycentered care in the ICU.13–15 In fact, some families deem the quality of a physician’s communication skills more important than their clinical skills.16 Failure to provide adequate communication puts patients and families at risk for poor outcomes, including anxiety, depression, and posttraumatic stress disorder.17 Despite its importance, studies continue to reveal that communication between families and providers in the ICU is suboptimal.18–22 In a recent study,23 researchers interviewed parents of children who died in the PICU regarding the communication that occurred around their child’s end-of-life care More than 70% of parents gave constructive feedback to physicians regarding the way information was conveyed during their child’s terminal illness The most common issue raised in this study was physician availability and attentiveness to the families’ informational needs Other concerns included honesty, withholding of information, use of complex language, pacing of information delivery, providing false hope, body language, and affect during bad news delivery Families with Limited English Proficiency Families with limited English proficiency (LEP) are at even greater risk of receiving poor communication from healthcare providers despite using trained medical interpreters Patients with LEP are less satisfied with physician communication than English-speaking patients, including the degree to which physicians listen, answer questions, explain concepts, and provide support.24–28 A recent study compared the quality of communication during interpreted and noninterpreted PICU family meetings.29 Interpreted meetings had fewer elements of shared decision-making and a greater imbalance between physician and family speech In fact, LEP families spoke for less than minutes during meetings that lasted 43 minutes on average Though this finding may be cultural to some degree, it is difficult to argue that effective bidirectional communication can occur with so little family participation, especially when discussing such complex issues as the care of a critically ill child Family Meeting as an Intensive Care Unit “Procedure” Complex value-laden decisions, such as the decision to withhold or withdraw life-sustaining therapies, are typically made during ICU family meetings For that reason, conducting a family meeting with clear, compassionate bidirectional communication is a critical ICU “procedure” that must be effectively taught to all trainees.30 Importantly, because ICU family meetings are frequently organized when the medical team wants to discuss limiting or withdrawing lifesustaining measures, it is possible to foster a hidden agenda implying that, when family meetings are conducted well, families choose to limit or withdraw interventions In actuality, a successful family meeting is one in which the medical team elicits the patient’s and 159 family’s goals and values, and a care plan is crafted that achieves their goals and honors their values Communication Pearls Palliative care specialists receive extensive training on conducting difficult conversations; they can be consulted as a resource for both the medical team and family However, effective communication of difficult and complex information is a core competency for all intensivists, and these skills must be learned early in training and honed throughout one’s career A number of strategies exist to assist clinicians in conducting these conversations,31 one of which is the SPIKES protocol,32 summarized in Box 19.1 Phrases to Avoid Several antiquated phrases remain in the vernacular of healthcare providers that must be eliminated, such as “withdrawal of care,” “nothing more can be done,” and “there is no hope.” Care is never withdrawn from a patient in the ICU There is always more that can be done to help ensure comfort, provide support, maintain dignity, and create meaning Allowing families to maintain hope is important.33 Providers may worry that preserving hope and truth-telling are mutually exclusive However, research suggests the opposite: truthful disclosure of prognostic information, even in the setting of poor prognosis, is associated with increased parental hope.34 This may be because hope is not solely defined by a particular medical outcome Truthful prognostic information, when delivered compassionately, can allow parents to focus on achievable hopes, such as comfort, quality of life, and meaningful relationships Eliminating these phrases will help ensure that families not feel abandoned by the medical team • BOX 19.1 SPIKES: A Protocol for Delivering Bad News Setup: Find a private space with adequate seating Never conduct a conversation of this nature without sitting down Patient perspective: Begin by allowing the patient or family to share their understanding, concerns, and goals for the meeting Always listen before you talk Invitation: Obtain the family’s permission to give them the information you want to share This can also be an opportunity for a “warning shot,” or a phrase that prepares the family for difficult news As an example, one could say, “Unfortunately, I have some difficult information to share with you Is it all right if I talk about that during this meeting?” Knowledge: Deliver the information clearly and compassionately Go slowly, and allow for silence Families may only hear the first piece of bad news delivered before their emotional response prevents further comprehension Resist the temptation to continue delivering information if the family is having an emotional response Instead, acknowledge, validate, and explore their emotions Emotions: Though some providers may feel uncomfortable addressing emotions, families consistently report the importance of empathy from healthcare providers The presence or absence of empathy can leave indelible marks on family members for years to come If unsure how to respond, listening with empathic statements is always appropriate Summary: Provide a brief summary of the meeting, ensuring that you have addressed the goals and concerns laid out by the family at the start of the meeting State the next steps, and plan for future conversations Modified from Baile WF, Buckman R, Lenzi R, et al SPIKES—A six-step protocol for delivering bad news Oncologist 2000;5(4):302–311 160 S E C T I O N I I I Pediatric Critical Care: Psychosocial and Societal Though protocols and guidelines can be helpful, all providers will encounter situations in which it is difficult to know what to say or how to proceed When this occurs, we are reminded by Elaine Meyer of the importance of “being present, not perfect.”35 Connecting with patients and families on a human level by bearing witness to their suffering will facilitate continued collaboration amid even the most difficult circumstances Limitation of Interventions One of the most difficult options for a family and the medical team to consider when a child may be dying is whether advanced technologic supports offer any benefit Such supports can include invasive or noninvasive mechanical ventilation, medical or mechanical support of the circulation, surgical interventions, renal replacement therapy, intravenous (IV) medications, or medically administered nutrition and hydration Forgoing such interventions requires that the family and medical team agree that such therapies offer little chance of benefit, that the pain or suffering they cause is not worth the hoped-for benefit, that the therapies no longer provide a reasonable quality of life, or that they otherwise not help to achieve important goals for the child and family Do Not Attempt Resuscitation Orders An important tool when determining desired goals is the not attempt resuscitation (DNAR) order Such orders historically became necessary when medical care advanced to such a degree that many “intensive care” interventions, such as mechanical ventilation or cardiopulmonary resuscitation (CPR), became the default pathway to prolong life in most circumstances.36,37 The term DNAR is beginning to replace DNR (do not resuscitate), because DNAR does not presuppose that resuscitation attempts will be successful Some centers have shifted terminology for DNAR orders even further by calling them allow natural death (AND) orders.38,39 The general public may have an inflated perception of the success of CPR based somewhat on media depictions.40 A physician’s willingness to share one’s medical opinion regarding the likelihood of success of CPR, especially if that likelihood is low, may be helpful in a family’s decision-making Using phrases that focus on what will be provided (e.g., comfort) rather than on what will be withheld (e.g., resuscitation) may help families understand the reasoning behind such choices How choices are presented, or “framed,” may affect patient and family decisions.41–43 In discussions with the patient and family, it is imperative that the clinician elicit the family’s overall goals rather than presenting a list of all possible interventions and asking for a yes/no answer to each Once the family members have articulated their goals (e.g., “going home,” “avoiding painful procedures,” or “waiting to see if our child can get back to baseline”), then the clinician can determine what interventions might help achieve those goals DNAR orders are not “all or none,” and a range of interventions could make sense depending on the clinical circumstances Clarity in the orders is important, however, especially if multiple transitions in care providers may occur During these discussions, it is important to avoid phrases such as “Do you want us to everything for your child?” Such phrases are nonspecific and imply that “doing everything” is the right course of action,44 because the converse is to “do nothing.” In circumstances in which a clinician feels strongly that invasive technologic support will not lead to long-term benefit, it is acceptable to recommend against CPR or intubation as a way to protect the child from interventions that will not help.45 Making a recommendation is an important part of shared decision-making; recommendations should be based on the goals and values articulated by the patient and family.46 Although the ethical justification for withholding a therapy is exactly the same as for withdrawing a therapy, it may be psychologically more difficult for some families to stop interventions that are already in place than it is to forgo pursuing new ones Therefore limitations of interventions may often begin as “nonescalation” plans, with consideration of withdrawal of an intervention when the clinical trajectory becomes clear Hospice Support in the Home During discussions around goals of care, some families may share that allowing their child to die at home would be a great source of comfort or meaning In certain clinical scenarios, this option may be feasible and appropriate to offer Hospice agencies are invaluable partners specifically skilled to coordinate and facilitate such a care plan Hospices provide comfort-oriented medical care and psychosocial support to patients with life-limiting illnesses and their families US hospices are independent agencies structured to be compliant with Medicare guidelines.43,44 Importantly, hospices not provide shift-based home nursing care for patients Hospice nurses visit patients on a regular basis (from twice weekly to monthly, depending on needs) to help caregivers assess symptoms and manage changes in clinical status They are also on call 24 hours a day for support by phone and, in special circumstances, can provide continuous in-home care for up to 72 hours for patients who are actively dying Although hospital-based palliative care teams and community-based hospices are distinct, pediatric palliative care teams work closely with hospices to help ensure a seamless transition from hospital to home.45,47 Compassionate Extubation Discontinuing mechanical ventilation (now called compassionate extubation) is an important skill for all intensivists It requires meticulous planning and symptom management Preparing the family is an important first step This includes determining who should be present, asking whether the family member to be present would like to hold the child during extubation, and distinguishing between expected signs that are part of the dying process (e.g., color change, noisy breathing) versus signs that would be treated with additional medication Providers should avoid overly precise predictions of how quickly a patient will die following compassionate extubation, as some patients may breathe longer on their own than anticipated Providing a range of time, such as “minutes to hours” or “days to weeks,” gives families a general idea of expected time course Preparing medications ahead of time to treat anticipated symptoms is important; having a titration plan in place may help staff assess and respond to distress.48 It is sometimes helpful to decrease ventilatory support shortly before extubation to assess whether the patient develops dyspnea If the patient appears uncomfortable on lower ventilatory settings, additional doses of medication can be given before extubation Although medication for dyspnea and agitation is essential, neuromuscular blockade should not be administered if a ventilator is being withdrawn Neuromuscular blockade may hasten death; it also makes it difficult to assess distress and determine whether additional medications for comfort are needed.49,50 It is possible to discontinue other interventions—such as vasoactive infusions, extracorporeal CHAPTER 19 Palliative Care in the Pediatric Intensive Care Unit circulatory support, or supplementary oxygen—while awaiting resolution of neuromuscular blockade before extubation Pain and Symptom Management When the goals of care shift to comfort, it is important to pay close attention to medication management and symptom control Common symptoms at the end of life include pain, dyspnea, anxiety, and agitation Many medications that treat these symptoms are part of routine ICU care (eTable 19.1; see also Chapter 132) Medication choices may differ significantly depending on how long a child is expected to live following removal of ICU interventions, what sources of pain exist, or how neurologically intact the patient is, although there is likely large variability between different centers.51 eTable 19.1 includes typical starting doses, but doses will need to be at a significantly higher level if a patient has developed tolerance Medications should be titrated to effect, with the maximum dose dictated only by side effects For opioids and benzodiazepines, bolus doses and infusion rates may be repeatedly increased by 20% to 50% until symptoms are controlled, which typically occurs before respiratory depression In patients without IV access, other routes of medication administration (transdermal, sublingual, rectal, subcutaneous) can be considered rather than increasing discomfort by necessitating needle sticks and procedures to maintain venous access Medication Management Opioids Several commonly used opioids are listed in eTable 19.1 Opioids treat both pain and dyspnea and may also have sedating effects They work via central nervous system µ-receptors Potential side effects include constipation, nausea, pruritus, urinary retention, and respiratory depression.52,53 Side effects should be anticipated and prevented if possible (e.g., with a bowel regimen) Intractable side effects can sometimes be managed by rotating to another agent in the class.54 Some side effects, such as nausea and vomiting, may resolve over time Distinguishing features of specific opioids are important to mention Codeine should be avoided because approximately 10% of the general population lacks the hepatic enzyme necessary to convert it to morphine, and up to 35% of children demonstrate inadequate conversion to morphine.55 Meperidine should also be avoided because its metabolite, normeperidine, can accumulate and cause seizures Fentanyl is commonly used in ICUs because of its rapid onset and titratability, but it can be problematic at the end of life if used for longer than brief periods because tolerance can develop rapidly However, the transdermal (patch) form is often useful for patients who are unable to tolerate enteral medications and no longer have IV access Morphine leads to histamine release, which can cause pruritus and hypotension, which may improve with rotation to hydromorphone At very high doses, morphine has neuroexcitatory effects that cause hyperalgesia, delirium, and myoclonus Morphine should also be avoided in renal failure, as accumulation of its metabolites causes myoclonus Methadone Methadone differs from other opioids and therefore bears special mention It is a µ-receptor agonist, as well as an N-methyl-d-aspartate (NMDA) receptor antagonist It has a long and highly variable halflife Its NMDA effects can sometimes improve pain control in patients who have become tolerant to high doses of other opioids, and 161 it may also be effective in treating neuropathic pain Its long half-life can lead to drug accumulation, which may cause late-onset side effects, such as obtundation Careful adjustment of dosing schedules is required Methadone has many drug–drug interactions that require careful review It can also prolong the QT interval; thus it is prudent to screen patients with an electrocardiogram before its initiation Other Pharmacologic Agents Acetaminophen or nonsteroidal antiinflammatory drugs (e.g., ibuprofen, naproxen, ketorolac) may be useful adjuncts to the medications shown in eTable 19.1 However, doses of these medications cannot be escalated because of the risk of toxicity Combination agents, such as acetaminophen with oxycodone, should be avoided because acetaminophen limits the ability to escalate the opioid component Ketamine is a dissociative anesthetic that also has NMDA effects It offers excellent pain control and may have opioid-sparing benefits However, it can cause disturbing hallucinations and delirium Similar to methadone, it may have advantages for neuropathic pain Other important adjunctive pain control methods include regional anesthesia (nerve blocks, epidural, or spinal anesthesia), lidocaine patches, and occasional treatment with steroids Symptom Management Pain A multitude of agents are available for the treatment of pain, many of which are discussed in other chapters (see Chapter 132) Neuropathic pain may be treated by methadone, gabapentin, or amitriptyline Steroids and IV bisphosphonates are useful adjuncts for pain relief due to malignant bone pain Nonpharmacologic adjuncts to pain control may also be useful Research suggests that integrative therapies, such as art and music therapy, can be effective adjuncts for pain treatment in children with cancer.55 Dyspnea Opioids are the mainstay of treatment for dyspnea Nebulized opioids are sometimes used, although they have not shown consistent benefit in controlled trials.47,57 In addition to opioids, dyspnea may be improved with a fan blowing in the patient’s face Other respiratory support, such as supplementary oxygen and noninvasive positive pressure, can be considered if they enhance comfort, but there is no mandate to use them if they add to distress or prohibit patient disposition to another location (e.g., home or outside of the ICU) that would be preferable to the family Agitation and Anxiety Benzodiazepines are often useful to treat agitation or anxiety at the end of life and may also help decrease opioid requirement.58 Low doses may be sufficient, but some patients may require escalation to sedating doses A calm, quiet environment can be helpful but so can distracting or enjoyable activities Nausea and Vomiting Several agents are available for the treatment of nausea and vomiting Ondansetron or metoclopramide are often effective Benzodiazepines are also useful Phenothiazines, such as promethazine and prochlorperazine, are efficacious but can be very sedating They may also cause extrapyramidal side effects, which diphenhydramine can help mitigate Olanzapine or haloperidol can be used when other agents are ineffective.59 ... you want us to everything for your child?” Such phrases are nonspecific and imply that “doing everything” is the right course of action,44 because the converse is to “do nothing.” In circumstances... shared decision-making, appropriate limitation of interventions, and pain and symptom management This chapter explores the practice of palliative care in the PICU by intensivists, palliative care... information was conveyed during their child’s terminal illness The most common issue raised in this study was physician availability and attentiveness to the families’ informational needs Other