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[.]
Trang 1Abstract: 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 team As truly futile treatment is difficult to define,
delib-eration over possibly inappropriate therapies should focus on
in-tensive 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 do so following a decision to withdraw or withhold life-sustaining treatments There is no legal or moral distinction be-tween 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, pedi-atric critical care, end-of-life care, double effect
Trang 219
Palliative Care in the Pediatric
Intensive Care Unit
ALISA VAN CLEAVE, EILEEN RHEE, AND WYNNE MORRISON
• 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
achiev-ing 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 symp-tom management must be prioritized, using both pharmaco-logic and nonpharmacopharmaco-logic 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.
Caring for children with life-limiting illnesses is an important role for
the pediatric intensivist Over the past 2 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
ad-vancements.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,
life-limiting conditions, many of whom are technology dependent
Children with complex chronic illnesses represent an increasing
pro-portion of hospitalized pediatric patients, many of whom require
frequent care in the PICU.2 In this population of patients, the
inten-sivist must evaluate the child’s illness trajectory, quality of life,
symp-tom burden, and family preferences for care during each admission
To adequately care for children with chronic life-limiting
condi-tions, as well as those who are near the end of life, pediatric
intensiv-ists must have a high degree of competency in core palliative care
skills, including communication, shared decision-making,
appropri-ate 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
de-mand a mixed model of integrative and consultative palliative care,
which allows for a wider distribution of a limited subspecialty re-source.3 , 4 Primary palliative care is an integrative model that
fo-cuses on maximizing and standardizing palliative care practices that clinicians routinely incorporate into the care of their pa-tients.5 , 6 Secondary palliative care uses consultation of a palliative
care team for complex, subspecialty-level problems Secondary pal-liative 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 spiri-tual 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 “ir-reducible 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 addi-tional 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 2 decades Healthcare providers communicate with families in many ways in the ICU,
Trang 3CHAPTER 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
orga-nized 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,
family-centered 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
communi-cation puts patients and families at risk for poor outcomes,
in-cluding anxiety, depression, and posttraumatic stress disorder.17
Despite its importance, studies continue to reveal that
com-munication between families and providers in the ICU is
subopti-mal.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
con-cerns included honesty, withholding of information, use of
com-plex 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
pa-tients, including the degree to which physicians listen, answer
ques-tions, 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 4 minutes during meetings that lasted 43 minutes on
aver-age Though this finding may be cultural to some degree, it is
diffi-cult 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
Impor-tantly, because ICU family meetings are frequently organized when
the medical team wants to discuss limiting or withdrawing
life-sustaining measures, it is possible to foster a hidden agenda
imply-ing that, when family meetimply-ings 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
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 communi-cation of difficult and complex information is a core competency for all intensivists, and these skills must be learned early in train-ing 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 pa-rental 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 do 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
conversa-tion 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
emo-tions, families consistently report the importance of empathy from health-care providers The presence or absence of empathy can leave indelible marks on family members for years to come If unsure how to respond, lis-tening with empathic statements is always appropriate.
Summary: Provide a brief summary of the meeting, ensuring that you have
ad-dressed 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.
Trang 4Though 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
bear-ing witness to their sufferbear-ing will facilitate continued
collabora-tion 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
me-chanical support of the circulation, surgical interventions, renal
replacement therapy, intravenous (IV) medications, or medically
administered nutrition and hydration Forgoing such
interven-tions 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
other-wise do 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 do not
attempt resuscitation (DNAR) order Such orders historically
be-came necessary when medical care advanced to such a degree that
many “intensive care” interventions, such as mechanical
ventila-tion or cardiopulmonary resuscitaventila-tion (CPR), became the default
pathway to prolong life in most circumstances.36 , 37
The term DNAR is beginning to replace DNR (do not
resusci-tate), because DNAR does not presuppose that resuscitation
at-tempts 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
percep-tion of the success of CPR based somewhat on media depicpercep-tions.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
Clar-ity 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 do 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
cir-cumstances in which a clinician feels strongly that invasive
techno-logic 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 interven-tions that are already in place than it is to forgo pursuing new ones Therefore limitations of interventions may often begin as “nonesca-lation” 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 struc-tured to be compliant with Medicare guidelines.43 , 44 Importantly, hospices do not provide shift-based home nursing care for pa-tients 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 circum-stances, can provide continuous in-home care for up to 72 hours for patients who are actively dying Although hospital-based pal-liative 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
me-ticulous planning and symptom management Preparing the fam-ily 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 compas-sionate 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 ex-pected 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 help-ful 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 diffi-cult to assess distress and determine whether additional medica-tions for comfort are needed.49 , 50 It is possible to discontinue other interventions—such as vasoactive infusions, extracorporeal
Trang 5CHAPTER 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
symp-toms are part of routine ICU care (eTable 19.1; see also
Chapter 132) Medication choices may differ significantly
de-pending on how long a child is expected to live following removal
of ICU interventions, what sources of pain exist, or how
neuro-logically intact the patient is, although there is likely large
vari-ability between different centers.51eTable 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
depres-sion In patients without IV access, other routes of medication
administration (transdermal, sublingual, rectal, subcutaneous)
can be considered rather than increasing discomfort by
necessitat-ing 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
vomit-ing, 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
half-life Its NMDA effects can sometimes improve pain control in
pa-tients who have become tolerant to high doses of other opioids, and
it may also be effective in treating neuropathic pain Its long half-life can lead to drug accumulation, which may cause late-onset side ef-fects, 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 Simi-lar to methadone, it may have advantages for neuropathic pain Other important adjunctive pain control methods include re-gional 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 ad-juncts for pain relief due to malignant bone pain Nonpharma-cologic 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 consis-tent 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 noninva-sive positive pressure, can be considered if they enhance comfort, but there is no mandate to use them if they add to distress or pro-hibit 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 vomit-ing Ondansetron or metoclopramide are often effective Benzo-diazepines are also useful Phenothiazines, such as promethazine and prochlorperazine, are efficacious but can be very sedating They may also cause extrapyramidal side effects, which diphen-hydramine can help mitigate Olanzapine or haloperidol can be used when other agents are ineffective.59