Adapted from Bicanovsky L. Comfort Care: Symptom Control in the Dying. In Walsh, D et al Palliative Medicine 1^st Edition, Saunders, An imprint of Elsevier Science, 2009.

• Profound progressive weakness

• Sleeping much of the time

• Little interest in food and drink

• Difficulty swallowing

• Disorientation to time, with increasingly short attention span

• Urinary incontinence or retention

• Oliguria or anuria

• Dropping blood pressure not related to hypovolemia, with rising, weak pulse

• Changes in respiratory rate and pattern, which may include a Cheyne-Stokes pattern characterized by oscillation of ventilation between apnea and tachypnea with a crescendo-decrescendo pattern in the depth of respirations

• Noisy breathing, airway secretions

• Mottling and cooling of skin

• Mental status changes, such as delirium, restlessness, agitation, and coma

Letting the family know when death is imminent

Parents and other family members are often fearful that if they step away from the bedside, they will not be present at the time of the child’s death. As such, it is often helpful to let family and loved ones know about the signs of impending death both to achieve a sense of preparedness about what to expect and to try to judge when presence at the bedside is helpful. To introduce this topic, simply ask whether it would be helpful to know more about what to expect as the child is dying. If a parent agrees to be informed, it is important to be clear and to provide short pieces of information, and then subsequently check if it is all right to continue.

It is also critical to warn parents that it may not be possible to know the exact moment of when the death will occur. Some families find comfort in knowing that children sometimes choose to die when their parent is not present as a last effort to protect the parent from further suffering, if such a choice is indeed possible.

Brain death

Typical signs of dying may not be present when a child is brain dead and is still receiving cardiorespiratory support. In children, brain death most commonly arises from traumatic brain injury due to child abuse, motor vehicle accidents, or asphyxia.⁶ Though there is general acceptance of the definition of brain death, it may still be difficult for parents and other loved ones to grasp this reality because the child may not appear dead. The clinical neurological examination remains the standard for the determination of brain death and has been adopted by most countries. The declaration of brain death requires not only a series of careful neurologic tests but also the establishment of the cause of coma, the ascertainment of irreversibility, the resolution of any misleading clinical neurologic signs, the interpretation of the findings on neuroimaging, and the performance of any confirmatory laboratory tests that are deemed necessary (Box 36-2).⁶ Despite medical consensus of the definition of brain death, not all religions officially accept this definition of death.⁷ Depending on the family’s beliefs and those of spiritual or religious advisers, families rarely disagree with this death pronouncement but conflict resolution may require ethics consultation and/or judicial involvement. Nonetheless, a thorough understanding of brain death can aid in conversations with families. Once cardiorespiratory support is discontinued, the typical signs of dying ensue.

BOX 36-2 Clinical Criteria for Brain Death in Adults and Children

Adapted from Wijdicks E. The Diagnosis of Brain Death. N Engl J Med 2001;344:1215–1221.

Coma

Absence of motor responses

Absence of pupillary responses to light and pupils at midposition with respect to dilatation (4-6 mm)

Absence of corneal reflexes

Absence of caloric responses

Absence of gag reflex

Absence of coughing in response to tracheal suctioning

Absence of sucking and rooting reflexes

Absence of respiratory drive at a PaCO₂ that is 60 mm Hg or 20 mm Hg above normal base-line values *

Interval between two evaluations, according to patient’s age

Term to 2 months old, 48 hours

>2 months to 1 year old, 24 hours

>1 year to <18 year old, 12 hours

>18 year old, interval optional

Confirmatory tests ^†

Term to 2 months old, 2 confirmatory tests

>2 month to 1 year old, 1 confirmatory test

>1 year to <18 year old, optional

>18 year old, optional

^* PaCO₂ denotes the partial pressure of arterial carbon dioxide

^† Confirmatory tests include cerebral angiography, electroencephalography, transcranial Doppler ultrasonography and cerebral scintigraphy

Key communication topics

Several communication topics are discussed in detail in Section 2, and will therefore not be covered in this chapter. Notably, however, anticipatory guidance can help prepare families for the child’s end-of-life course and ease the child’s and family’s distress. Proposed timing for key communication topics in children with advanced life-threatening illness is as follows:

• In advance of clear decline:

• Considerations about resuscitation status (Chapter 22)

• Discussions related to preferred location of care (Chapters 7 and 21)

• During clear decline:

• When death appears to be within days:

• Funeral and commemoration planning

• Following death:

• Bereavement guidance. (Chapter 5) Parents have reported finding face-to-face visits following the child’s death to be very helpful in their bereavement.^8,⁹

There are several key principles in managing the child’s final days. An analytical approach to symptom control continues, but usually relies on clinical findings rather than investigation. Drugs should be reviewed with regard to need and route of administration. Some patients manage to take oral drugs until near their death, but many require an alternative route. Finally, it is essential that the care team maintains effective communication and ensures that support is in place for the family. A daily visit for inpatients or a daily phone call at a planned time can be very reassuring for families. Experience suggests that clinician home visits from the hospital-based team are greatly appreciated throughout the entire palliative care course, and data suggest that this is especially valued at the end of a patient’s life.¹⁰

Importantly, even when the child may be comfortable and symptoms are well controlled, a caregiver’s mere presence during the final period can be very comforting to family members. Such a presence reinforces that the dying patient’s welfare remains important, and it provides support and guidance to the family at a time of extreme stress. It is critical to inform the family that although death may be imminent, the time frame may be hours to days. It is essential to ensure that someone will be available to pronounce the child’s death, especially when the child is not in the hospital.

Easing Child Distress

When a child is actively dying, and there is consensus that the primary focus of care should be on comfort, there is much that the interdisciplinary team can do to permit a more natural death experience. Continued medical interventions, including vital sign assessments, pulse oximetry, nonessential medications and blood work, may be disruptive and no longer beneficial to the child’s care. However, discussion of discontinuing these procedures is delicate and can be perceived as no longer caring for the child. This is especially true among families who are used to vigilantly monitoring their child. Parents should be made aware that discontinuing these procedures allows them time to be with the child without interference from intermittent procedures, wires and tape, and/or sounds from alarms. Gentle suggestions can be made that discontinuing monitors would enable the family to focus entirely on the child, rather than the surrounding medical equipment. As always, flexibility is essential. Some families may simply not want to forgo what they are used to and will often state that the vital sign assessment and/or the monitor will help them know when the child’s time has come.

Symptoms that need not be treated

Importantly, when a child is actively dying, comfort can be achieved without a focus on reversing the underlying causes of symptoms. For example, it may no longer be indicated to directly treat severe constipation if the child is not expected to live more than a few days. Rather, abdominal discomfort from constipation could be treated symptomatically with opioids, even though opioids may exacerbate constipation. Similarly, continued dressing changes for bedsores may be more painful and disruptive than opting for a more general approach of titrating symptom-relieving medications. Most importantly, when a symptom is not a priority for the child and the parent, this should guide treatment decisions. Again, these decisions should be individualized and discussed with parents so that they understand that it represents a change in care strategy rather than a decrease in care provided.

Drawing inward

The endpoint of the terminal phase is often marked by a turning inward, away from the external world, by the child. Cognitive and emotional horizons narrow, as 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. It is critical to explain this behavior as a normal and expectable precursor to death to the parents so that they do not interpret it as rejection.^11,¹²

Drowsiness and altered consciousness

Diminished wakefulness is also very common during the last days of life ¹³ and is often desirable for the child and family. However, it is also not uncommon for parents and loved ones to want to hold onto every wakeful moment possible. At times this desire can hinder titration of pain-relieving medications because of the worry of further limiting wakefulness. The team should continue efforts to try for a careful balance of comfort and an ability to interact, while at the same time encourage the family to continue their own interactions, such as gentle hugging or touching, talking or singing, with the hope that the child perceives such gestures. One family reread aloud the first book of the Harry Potter series to their dying child, hoping that the story would bring as much joy to him as it had previously.

Escalating pain, dyspnea, and agitation

Three highly common symptoms that may require intensive treatment efforts are escalating pain, dyspnea, and agitation during the final days of life. Full details for management of these distressing symptoms can be found in Chapters 28 31, and 32. One challenge to successful management is variability among providers in the approach to medication titration. Children’s Hospital Boston examined adequate symptom management in children with cancer at the end of life and found the following barriers to care:¹⁴

• Availability of nurses and physicians

• Identification of the team responsible for the patient

• Physician relying on the nurse at the bedside to recommend adequate pain medication to relieve symptoms of distress

• Delay in turn-around time from pharmacy in providing opioids to caretakers upon short notice

• Lack of knowledge and fear of opioid use in rapid escalation of doses

• Lack of understanding of the principle of double effect

• Fear on the part of the physicians and nurses of respiratory depression, addiction, hastening the death, and so-called euthanasia

• Variability in attitudes, practices, and experience of physicians and other members of the care team

In response to these findings, an interdisciplinary taskforce developed guidelines and a standardized order set to achieve greater consistency in medication titration and symptom management outcomes (Box 36-3). When a patient experiences a refractory symptom that cannot be captured through appropriate titration of symptom-relieving medications as described, then sedation to unconsciousness, or palliative sedation, may be indicated. Full details related to consideration, discussion and administration of palliative sedation can be found in Chapter 23.

BOX 36-3 Guidelines for Management of Escalating Pain, Dyspnea, or Agitation at the End of Life

These guidelines are intended for a patient at the end of life with a “Do Not Resuscitate” order in place, and with escalating pain, dyspnea, or agitation.

Palliative sedation may be considered, with experienced guidance.

With escalating pain, dyspnea, agitation:

Identify the doctor or nurse responsible for prescribing for patient.

Care nurse to remain at bedside, and prescriber to remain on unit until symptoms controlled.

Charge nurse or designee will be available to assist as needed until symptoms controlled.

Nurse will notify pharmacy of expected need for increased opioid use. A back-up continuous infusion supply will be maintained. Nurse will call for new infusion each time back-up solution is started.

No ceiling dose exists for symptom management at the end of life. The correct dose is the one that relieves the symptoms.

Loading Dose:

For patient already on opioids: administer loading dose of same opioid equal to 10% of total opioid dose from preceding 24 hours.

For patients not already receiving opioids: administer loading dose IV x1 as follows:

Morphine 0.1 mg/kg IV x 1 dose

Hydromorphone 0.015 mg/kg IV x 1 dose

Fentanyl 1 mcg/kg IV x 1 dose

Subsequent Dosing:

Doses may be given Q10 minutes prn end of life symptoms.

Begin intermittent dosing, increasing every third dose, as indicated below:

If pain/dyspnea not relieved:

Doses given Q 10 min. prn

Example: Dose #1 is starting/loading dose 10 mg 1 x previous dose 10 mg Increase to 1.5 x previous dose 15 mg 1 x previous dose 15 mg Increase to 1.5 x previous dose 22.5 mg

If inadequate relief from first dose, give second dose.

If inadequate relief from second dose, notify prescriber of need for additional orders.

If good relief from either dose after 30 minutes, obtain order to begin and/or adjust continuous infusion.

If inadequate relief, continue the same sequence prn: 1x, 1.5x, 1x, 1.5x, 1x, 1.5x previous dose.

Pain Assessment may be by pain scale or be descriptive, such as crying, grimacing, moaning when moved.

Continuous Infusion Instructions:

Adjustments for Moderate Increase in Symptoms:

If at any time symptoms recur, resume intermittent dosing as recommended above, and then increase hourly continuous infusion dose accordingly.

For a patient who has been stabilized on Patient Controlled Analgesia and then experiences a mild to moderate increase in symptoms, increase continuous infusion and intermittent dose by 1.3-1.5 x current dose.

Always consider use of adjunctive therapy (see guidelines). If morphine equivalent > 5 mg/kg/hour, or hydromorphone > 0.8 mg/kg/hour, adjunctive therapy is strongly recommended.

Adjunctive Therapy:

For Anxiety/Agitation: (Recommended Starting Dose)

Intermittent Lorazepam: 0.05 mg/kg IV Q4h prn anxiety/agitation

Intermittent Haloperidol: 0.01-0.03 mg/kg/day, IV divided Q8h or Q12h prn hallucinations/agitation (adult dosing: 0.5-5 mg/dose)

For excess respiratory secretions:

Glycopyrrolate: 4-10 mcg/kg/dose IV every 3 hours prn secretions

Hyocyamine:

Children 2-12 years: 0.0625-0.125 mg PO/SL every 4 hours as needed; maximum daily dosage 0.75 mg

Children >12 years to Adults: 0.125-0.25 mg PO/SL every 4 hours as needed; maximum daily dosage 1.5 mg

Scopalomine:

Children >12 years and Adults: Apply 1 disc behind the ear every 3 days as needed

Noisy breathing

Breathing can become particularly noisy when death is imminent and is often described as the death rattle. This is more common in patients with primary lung disease or brain tumors.¹⁵ It is critically important to prepare family members for this possibility. Because this symptom is often present when the child is already unconscious, the child may not experience this as uncomfortable. However, transdermal scopolamine, l-hyoscyamine drops for smaller patients and glycopyrrolate can be helpful in drying secretions and diminishing this symptom.¹⁶^–¹⁸ Treatment of what the family perceives as suffering should be a priority, even if there are differences of opinion within the care team.

Terminal emergencies

Rare circumstances can result in the child experiencing sudden high distress and some are best treated using more invasive strategies. Patients should be assessed for their risk of experiencing these complications so that effective interventions can be readily available. Such emergencies include:

Delirium and/or Hallucinations

It is common for the child to become less coherent toward the end of life, however, overt hallucinations and agitation can be extremely distressing for both the child and family. Chapter 28 provides an extensive review of assessment and management of delirium. In truly end-of-life instances, the child may respond well to intermittent doses of haloperdol, which can be administered intravenously.

Ensuring comfort during special circumstances

There are times at the end of a child’s life when the process of either discontinuing certain medical interventions, such as fluids and nutrition, or beginning acts such as initiation of cardiopulmonary resuscitation, affect the experience of the child and family. When these special circumstances arise it is again helpful to prepare the family and child, if he or she is conscious.

When Fluids and Nutrition are Discontinued

Typically, as a child approaches end of life, there is little drive for drinking and eating, and older patients have indicated that this is not experienced as hunger or thirst.¹⁹ It is not uncommon, however, for children to be receiving medically administered fluid and/or nutrition near the end of life, and this may prolong dying without providing benefit in terms of comfort. Continued fluids and nutrition may even contribute to discomfort. For example, in a child dying from an advanced brain tumor, continued fluids can increase cerebral edema and headache. In a child with end-stage lung disease, continued fluid can increase secretions, and at times pulmonary edema, both of which can exacerbate dyspnea. In such circumstances, clinicians and parents may opt to discontinue medically administered fluid and nutrition. However, once discontinued and depending on how long a child is likely to survive, the child’s appearance may change over time. Anticipatory guidance is critical to helping the family through this end-of-life period. The following key considerations should be discussed:

Duration

Before embarking on this conversation, the clinician should assess how long the child is likely to survive without hydration and nutrition. Estimates should be given in terms of hours, days, or weeks, rather than an exact amount of time. The duration that a child may survive depends on multiple factors. One consideration is the condition of internal organs, especially the heart and kidneys. For example, if fluids and nutrition are discontinued in a child in a persistent vegetative state, but without other end-organ damage, the child may live for days to weeks. However, in a child with multisystem organ involvement, the duration is likely to be considerably shorter. One sign that can indicate that death is near is when the child becomes aneuric. It is uncommon for a child to live more than a few days once the kidneys have stopped functioning. Importantly, though a decision may have been made to discontinue supplemental fluid and nutrition if the child is receiving intravenous medications, especially as continuous infusions, these include some fluid and can greatly impact the duration of the child’s life. Estimates in these cases are much more difficult to make.

Change in Appearance

With discontinuing fluids and nutrition, the child will appear increasingly dehydrated and this can be difficult to bear. In infants, the fontanel will become sunken. Eye sockets will become sunken in all children. Depending on how malnourished the child was to start, the abdomen may become concave. Mucous membranes become increasingly dry and secretions very thick. The skin will become looser. Though changes are likely, this does not mean the child is feeling uncomfortable. Nonetheless, the physical appearance of the child may invoke images of malnourished children, and although parents may understand that the child is not suffering, emotionally the changes may be unacceptable to them. As such, it is important to offer parents the opportunity to express their worries, and it may be even helpful to reinstitute some hydration at times.

What Can and Should Be Done

As noted, most children do not express discomfort when not receiving medically administered fluid and nutrition, especially as consciousness lessens. Nonetheless, simple measures can be helpful to both the child and family. Sips of desired liquid and small bites of any food requested can provide a great deal of comfort. Administration of artificial tears can prevent painful cornea abrasions as the eyes become dryer. Gentle moistening of the mouth with water-soaked toothettes or ice chips can also be extremely comforting, as is moistening of the lips with a damp cloth or petroleum hydrolatum. Gentle repositioning to prevent bedsores is also helpful. Finally, as described in Chapter 31, opioids should be titrated appropriately in response to any expression of distress.

When the Ventilator Is Discontinued

One of the most common ways a child typically dies in the intensive care unit (ICU) is through the discontinuation of ventilator support.²⁰^–²² Though difficult to know with certainty, this decision is typically made when there is consensus that the underlying cause of ventilator dependence is irreversible, and continued ventilatory support will not result in a meaningful quality of life. The following considerations are important when the patient, when involved, the family, and the healthcare team reach consensus that ventilator support will be discontinued.

The counseling of families ²³ is a critical aspect of care for the patient who is to be removed from a ventilator. Before withdrawal, the following issues should be discussed:

• Potential outcome of ventilator withdrawal: Clinical experience suggests that even when there is clear consensus that the child will not survive for long following discontinuation of ventilator support, at times a child will unexpectedly breathe on his or her own. This is especially possible in the newborn who was initially intubated for apnea. Thus preparing the family for the possibility that the child may survive is essential. In most situations, however, when all other life-sustaining treatments have been stopped, including artificial hydration and nutrition, outcomes include rapid death within minutes, that is when patients are on maximal respiratory support, or death in hours to days. More specific prognostication is often not possible.

• Assurances about comfort: It is important to prepare the family for the possibility that breathlessness may occur, but that it can be managed. Confirm that medication will be available to manage any discomfort and that the patient will likely need to be kept asleep to control symptoms. It is helpful to further explain that involuntary moving or gasping does not reflect suffering if the patient is properly sedated or in a coma. Explain that the family can be at the bedside throughout withdrawal of the ventilator.

• Reinforce the decision: Even though a family is able to make a definite decision for ventilator withdrawal, such a decision is always emotionally difficult. It is helpful to provide reassurance they have made the best decision possible for their child.

Options for Ventilator Withdrawal

Two methods have been described for ventilator withdrawal:²⁴ immediate extubation and terminal weaning. The clinician’s and patient’s comfort, and the family’s perceptions, should influence the choice. In immediate extubation, the endotracheal tube is removed after appropriate suctioning. Humidified air or oxygen is given to prevent the airway from drying. This is the preferred approach to relieve discomfort if the patient is conscious, the volume of secretions is low, and the airway is unlikely to be compromised after extubation. A patient likely to experience significant hemoptysis, for example, may benefit from a terminal wean with the gradual reduction in oxygen and/or ventilator rate at a pace not faster than pharmacologic sedation is administered to treat objective signs of distress from the effects of hypoventilation and hypoxia. In terminal weaning, the ventilator rate, positive end-expiratory pressure (PEEP), and oxygen levels are decreased while the endotracheal tube is left in place. Terminal weaning may be carried out over a period of as little as 30 minutes, or it can be longer. If the patient survives and it is decided to leave the endotracheal tube in place, a Briggs T-piece can be placed.

The following are suggested steps to ensure smooth withdrawal of ventilator support:

• Encourage family members to make arrangements for clergy, special music or rituals that may be important to them.

• Document clinical findings, discussion with family and plan of care.

• Turn off all monitors and alarms, when agreed upon by family.

• Remove any unnecessary medical paraphernalia, such as the nasogastric tube.

• Maintain intravenous access for administration of palliative medications.

• Suction patient and set the oxygen to 0.21; observe for signs of respiratory distress.

• Adjust medications as needed to relieve distress before proceeding further.

Neuromuscular Blockade (NMBA)

Neuromuscular blocking drugs have no sedative or analgesic properties and may mask symptoms of suffering at the end of life. As a general rule, therefore, pharmacologic paralysis should be avoided at the end of life. In most cases, the effect of these agents can be reversed or allowed to wear off within a short period of time, allowing for the withdrawal of mechanical ventilation in the absence of the confounding effects of paralysis. Patients who have been receiving NMBAs chronically for management of their ventilatory failure occasionally can present a more difficult ethical dilemma. In some situations, restoration of neuromuscular function may not be possible for several days or even weeks. When faced with this problem, the clinician must choose between withdrawing the ventilator while the patient is paralyzed versus continuation of life support well beyond the point at which the patient and family have determined that the burdens of such treatments outweigh the probable benefits. In this circumstance, it may be preferable to proceed with withdrawal of life support despite the continued presence of neuromuscular blockade.

Symptom Control

The most common symptoms related to ventilator withdrawal are breathlessness and anxiety. Opioids and benzodiazepines are the primary medications used to provide comfort, typically requiring doses that cause sedation to achieve good symptom control. The dose needed to control symptoms will depend on the neurological status of the patient and the amount of similar medication used up to the time of extubation. In unconscious patients, it should not be assumed that they will not experience distress, and symptom-relieving medications should be administered in advance of planned extubation. Patients who are awake at the time extubation or in whom significant amounts of opioids and benzodiazepines have been used previously will require greater dosages or a change to a barbiturate to achieve symptom control.²⁵ Medication titration should proceed according to guidelines discussed in Chapter 23.

Clinical Vignette

Jamal was a 3-year-old boy with a history of hypoxic ischemic encephalopathy who was admitted to the intensive care unit with aspiration pneumonia requiring intubation. This was the fourth intensive care unit admission in 6 months. After 3 weeks of care in the ICU, and several unsuccessful attempts at extubation, the family was approached about tracheostomy placement and chronic ventilation. After extensive consultation with the pediatric palliative care team, his parents came to the decision that Jamal had been through enough, that despite the doctors’ best efforts his condition continued to deteriorate. They expressed the desire that he be allowed to die. They also expressed the hope that he die at home, despite the fact that they lived 3 hours away in a rural area.

Extensive discussions were held with the ICU staff about the following concerns:

• How could the ICU team be sure that he would be comfortable if extubation were to take place at the home?

• What if the family changed their minds and asked for him to be reintubated?

• Is it fair to use hospital resources, such as the transport team, to transfer the child home if these same resources might be needed to save a child’s life?

Many interdisciplinary conversations were held to ease the worries of the ICU team. Arrangements for support at home were made in advance of the transport. Ultimately, Jamal was taken home by the transport team and the palliative care physician. He was stable during the journey. His extended family was at his home when he arrived. They said their farewells, and Jamal received the sacrament of the sick from his local priest. Also present were his pediatrician and two nurses from a local hospice. Jamal received 2 mg morphine and 1 mg midazolam, and was then removed from the ventilator. He was given four morphine rescue doses and two midazolam rescue doses through his pump for respiratory rate of greater than 50 breaths per minutes and increased work of breathing as assessed by clinicians or his parents. He died peacefully in his mother’s arms 75 minutes after extubation. The family was extremely grateful that he was home to die.

Easing Family Distress

During a resuscitative effort

Approximately 75% of children who experience pulseless cardiac arrest in the hospital, and 90% of those who experience an out-of-hospital arrest will die.^26,²⁷ Most hospitals are instituting efforts to pre-empt unexpected cardiorespiratory arrest by instituting protocols to promote early transfer to the ICU.²⁸ Despite these efforts, codes continue to occur and efforts should be made to attend to the family’s distress. Such efforts include identifying a point person for the family, who can serve as a constant presence during the resuscitative effort, provide regular updates, answer questions and make every effort to meet the family’s needs. Families are now regularly invited to be present during resuscitative efforts. The data suggest that when they are present, there is less anxiety, litigation, and second-guessing regarding the efforts and competence of the staff providing that care.²⁹

The lingering child

Clinical experience suggests that it is not uncommon for families to express distress regarding the length of time it is taking for the child to die when active dying lasts for hours or days. It is impossible to imagine the enormity of the distress associated with this period of time, the ambivalence of feelings related to wanting to hold on to every moment versus not being able to bear a moment longer at the child’s bedside. Anticipatory guidance aimed at preparing families for this period can be helpful. Further, suggesting ways to be with the child can also ease family distress. Communication strategies include:

• Touch, such as lying beside the child, gentle massage, and hugging,

• Sound, family’s spoken words, including reading favorite books, reflecting on memories, music, and prayer, much of which depends on family background and communication style,

• Sight, such as looking at photos and other memorabilia, and adjusting the lighting to family preference,

• Smell, depending on family culture incense and other familiar smells may be calming,

• Doing, continuing with caring through application of damp cloths to forehead, moistening lips, or gentle suction when helpful is also part of the process.

Management of visitors is a key role of the healthcare team. For some families, being surrounded by extended family and friends is critical, for others it is unbearable. Inquiring about what is most comforting is essential.

Throughout this period, attention to the child’s comfort remains the top priority and if suffering is perceived by loved ones and clinicians, medications should be further titrated.

Hastening death

Rarely, a parent will overtly request that the child’s death be intentionally hastened,³⁰ and this is most commonly associated with parental fears about child suffering. Euthanasia is legal only in the Netherlands^22,^31,³² and is for children either younger than 1 year old or older than 12 and who are considered able to provide assent. Euthanasia for minors is also under consideration in Belgium.³³ There are no legal hastening options for minors in North America. Yet, if the parent requests hastening, it is important to respond with more than just a statement that hastening is not legally permissible. Just as other requests should be explored to identify underlying meaning and fears, so should this type of request. Explanation of legal and effective alternatives, such as further titration of symptom-relieving medications and palliative sedation, is helpful.

Clinical Vignette

Eddie was 6-year-old boy with advanced metastatic alveolar rhabdomyosarcoma. The primary lesion involved his face and neck, and he also had pulmonary and bone marrow metastases. His parents wanted to be prepared for what to expect for his end-of-life course and the palliative care team offered the following information. One significant concern was that Eddie’s tumor would progress locally faster than in his lungs and bone marrow and, as a result, he was at high risk for upper airway obstruction and suffocation at the end of life. The family was also told about progressive dyspnea and fatigue, should other sites of disease progress faster. Upon hearing these possibilities his father, Charlie, asked whether Eddie’s life could be intentionally ended when he reached a stage when his life was no longer worth living. Rather than focusing on the illegal nature of such an action, Charlie was asked more about his worries and he described immense concerns about Charlie’s experience of suffering. In response, the physician gently informed him that we may not be able to tell when life was not worth living from Eddie’s perspective, but that every effort would be made to try to ensure his comfort. Eddie’s father was told about strategies for intensive symptom management and palliative sedation, if necessary.

Eddie did not suffer from dyspnea or upper airway obstruction. He simply became less and less responsive, and required minimal pain-relieving medications. He died peacefully surrounded by family, friends, and clinical caregivers. Several weeks following his death, Charlie reflected that had Eddie’s life been intentionally ended, it would have been treating his own suffering, and not Eddie’s.

Participating in prayer with families

Clinicians are, at times, asked to pray for a child or to lead a family in prayer. This may be an indication of unmet spiritual needs and further exploration of whether it would be helpful to involve the chaplain, if he or she is not already involved, may be warranted. Regardless, the clinician may feel conflicted about whether to participate in or lead a prayer on behalf of the patient because of considerations of professional-personal boundaries and his or her own religious beliefs or spirituality. Consideration of the following options, which attempt to respect the integrity of the clinician’s beliefs and to be supportive of the family’s needs, may be helpful.^34,³⁵

1. Praying for the patient: It may be suitable for clinicians to pray if they feel comfortable doing so and such prayer is consistent with their own beliefs.

2. Sit with patient and family while the family prays: A clinician who is uncomfortable praying with the family may choose instead to sit quietly in supportive company while they pray. In this way, clinicians lend support to the patient and family without explicitly endorsing a particular belief system.

3. Respectfully decline: Clinicians may also respectfully de-cline to pray with the family. To avoid the patient feeling rejected, the clinician may want to say, “Because of my own values, I am unable to participate; however, please know that you will be in my thoughts.”

Memory making

There are numerous activities that families can take part in during the final periods of the child’s life that contribute to the legacy and family memories. The following activities are commonly gently offered to families:

• Prints of the hands and feet, or making molds

• Obtaining a lock of hair

• Making of a memory box or book

• Photographing and videotaping

• Journaling

It is important to offer a variety of possibilities, because activities will appeal to different family members. At the same time, respecting the family’s decision to decline is also critically important.

At the Time of Death

There is no true standard for declaring that a patient has died. When a clinician enters the room and is not known to the family, an introduction and explanation of his or her purpose is warranted. Empathetic statements, such as “I am sorry for your loss,” can ease the distress, as can telling the family that they are welcome to stay while their loved one is examined.

The pronouncement ³⁶

Consider the following steps to pronounce the death:

1. Identify the patient by the hospital ID tag.

2. Note the general appearance of the body; ensure that the patient is not overtly hypothermic at the time of this assessment.

3. Ascertain that the patient does not rouse to verbal or tactile stimuli. Avoid overtly painful stimuli such as deep sternal pressure.

4. Listen for the absence of heart sounds; feel for the absence of carotid pulse.

5. Look and listen for the absence of spontaneous respirations.

There is no standard amount of time for steps 4 and 5; at Children’s Hospital Boston it is recommended to observe for 60 seconds.

6. Record the position of the pupils and the absence of pupillary light reflex.

7. Record the time at which assessment is completed.

8. Once completed, it is considerate to again make an empathetic statement acknowledging the family’s loss.

Documentation in the medical record

A simple statement in the medical record is all that is required, “Called to pronounce (name); chart the findings of physical examination and note the date and time of death.” The following additional items should also be noted:

• If attending physician and family, if not present, were notified

• If the organ bank was notified, which is required in the United States

• If the coroner or medical examiner was notified, which is required in some states for a child death regardless of cause

Death certificate completion

There is tremendous variability in diagnoses used for completion of the death certificate; however, experience suggests that what is written can hold great importance for family members. Guidelines are readily available. In the United States, the National Association of Medical Examiners provides the following guidelines:³⁷

1. Make every effort to report an etiologically specific underlying cause of death.

2. Try to use a Sequential Part I Format, if possible.

3. Err on the side of reporting too much rather than too little.

4. Do not report mechanistic terminal events such as:

• Cardiac arrest

• Asystole

• Cardiopulmonary arrest

• Respiratory arrest

• Electromechanical dissociation

• Ventricular fibrillation

5. Do not report symptoms or signs.

6. Do not report a condition if its existence in the patient is obvious based on another reported condition.

7. Do not oversimplify.

8. Do not use abbreviations.

After Death Care

The most important message to relay to parents is that nothing needs to be done in a hurry when their child dies. This is very much a private time for family to say their individual goodbyes. Saying goodbyes and performing rituals are important because they enable parents, siblings, and other family members to express their love, sorrow, relief, regrets, and share precious memories. Many will be guided by religious practices, which can vary considerably (Table 36-2). Washing the child for the last time, dressing the child in special clothes, taking photos, playing favorite music, praying together, touching and cuddling the child, talking to the child, taking foot and handprints, cutting a lock of hair and writing a message or poem for the child are all examples of rituals that families have found helpful and necessary.

TABLE 36-2 Examples of Religion and Care at the Time of Death

Clinical Vignette

Laila was a 2-month-old girl with junctional epidermolysis bullosa, an often fatal disorder involving fragility of the skin and mucous membranes. Laila was being cared for in the neonatal intensive care unit, and with time, the primary hopes of her parents became focused on ensuring her comfort. Laila’s parents were newly arrived immigrants from China and they were living with paternal grandparents, also first generation immigrants. All family members were often present at the bedside. Laila’s mother, Hua, sought support from the pediatric palliative care team social worker to help reconcile two concerns. The first was that related to the families’ cultural background, photographs were not permitted. Hua felt caught between respecting tradition and her elders and wanting to have an image of her daughter. Through strategizing with the care team, she decided to seek permission from the family to obtain a passport for Laila. The family agreed, hoping that should Laila’s condition improve she could be brought to China for herbal remedies. The social worker arranged for representatives from the passport agency to come to the hospital to complete the application, which included taking a photograph. The passport was expedited and arrived just prior to Laila’s death.

Hua also said her in-laws had told her that she would need to leave Laila at the hospital following her death and they would not permit burial. They believed that if Laila were to be buried, her spirit would interfere with Hua being able to attach to future children. Despite this advice, Hua was desperate to know the place where Laila would be buried. Arrangements were made to bury her in the hospital’s burial grounds, where Hua could visit without the knowledge of her family.

When the family is ready, they need to phone the funeral home. If preparation for death has been encouraged, then most families will have already chosen the funeral director they wish to use. Parents need to inform the funeral director of the time they wish for the child’s body to be collected. It is very important that parents remain in control of the timing and they are not hurried. Funeral directors are on call 24 hours a day and parents can phone at any time. There may be an extra cost if the child needs to be taken after normal working hours.

If the family plans to be with the child’s body for a prolonged duration, then it may be helpful to gently prepare them for how the body will change over time.

Livor mortis

One of the early changes that can be observed is livor mortis, also referred to as lividity, postmortem hypostasis, vibices, and suggilations.³⁸ This is a physical process. While the individual is alive, the heart circulates the blood. When death occurs, circulation stops and the blood begins to settle, by gravity, to the lowest portions of the body. This results in a discoloration of those lower, dependent parts of the body. Although beginning immediately, the first signs of livor mortis are typically seen about 1 hour following death, with full development being observed 2 to 4 hours following death.³⁸

Rigor mortis

Rigor mortis is a chemical change resulting in a stiffening of the body’s muscles following death, resulting from changes in the myofribrils of the muscle tissues. Immediately following death, the body becomes limp and is easily flexed.³⁸ As ATP is converted to ADP and lactic acid is produced lowering the cellular pH, locking chemical bridges are formed between actin and myosin resulting in formation of rigor. Typically, the onset of rigor is first observed 2 to 6 hours following death and develops over the first 12 hours. The onset begins with the muscles of the face and then spreads to all of the muscles during the next 4 to 6 hours. Rigor typically lasts from 24 to 84 hours, after which the muscles begin once again to relax.

Death in the hospital

Once the family has left the hospital, nurses typically prepare to do postmortem care by two staff members who can support one another. Preparation of the body will vary depending on hospital policy, religious and cultural preference, and whether or not the body will be examined by the medical examiner and/or an autopsy will be performed (see Table 36-2). When the body is formally examined, all indwelling lines and tubes must remain intact. Appropriate pads should be placed over puncture or wound sites and under the perineum to contain body fluids if necessary. The body should be dressed in a hospital gown or clothing designated by family.

Non-Medical Examiner Cases

If permission for autopsy is anticipated, all indwelling lines and tubes must remain intact, depending upon type of autopsy. This enables the pathologist to perform a more accurate examination. Parents who object to this practice may communicate their wishes for modifying the autopsy consent form. After consulting with the family as to specific religious or cultural practices:

• Consider warming lights to assist in maintaining some of the body’s warmth.

• Bathe the body and attempt to restore as normal an appearance as possible. Some parents may wish to participate in bathing and dressing their child. This should be offered.

• Families may request special handling or positioning.

• Contact the appropriate chaplain for assistance, if indicated.

In most hospitals, the body is transported to the morgue in anticipation of being picked up by the funeral home. Body bags and special stretchers are used so that others will not recognize that a body is being transported. At times families object to the child’s body being transported to the morgue and every effort should be made to accommodate such requests. In addition, certain religious observances require different handling of the body (see Table 36-2).

Debriefing staff

No matter how common or rare it is for staff to care for a child through death, it is always helpful to create a safe and nonjudgmental forum for staff to reflect on the experience. As a example, in one setting staff bereavement rounds take place biweekly and each child who died during the period is discussed. The goals of such discussions are fourfold:

1. To reflect on the care delivered to the child and family regarding what went well and what could be improved upon,

2. To report on the bereavement experience of the family to ensure that their support needs are being met,

3. To celebrate the life of the child,

4. To support one another.

Because of role modeling by senior staff, these rounds are generally well attended.

Staff commemoration

With the death of a child, whether that child has been cared for by staff for a short or long time, there are various ways in which to commemorate the child. At the same time, these actions often provide opportunities for self-care. In a study of more than 200 pediatric critical care specialists, 79% contacted families following the death at least sometimes, 72% attended funerals, and only 2.5% thought that it was inappropriate for clinicians to attend funerals. A total of 76% agreed that follow-up contact helps the family, whereas 47% agreed that follow-up contact helps the physicians.³⁹ For a more detailed description of staff commemorative activities see Chapter 18.

The condolence letter or card is one of the simplest gestures that can be made by a clinician and is typically highly valued by family members. Personal experience suggests that these notes are kept indefinitely and become a part of the child’s legacy. A good condolence letter has two goals: to offer tribute to the deceased and to be a source of comfort to the survivors. One of the greatest hurdles to writing these letters is simply finding the right words. Bedell and colleagues offer some concrete suggestions:⁴⁰ One can begin the letter with a direct expression of sorrow about the death, such as “I am writing to send you my condolences on the death of your son.” Typical themes to include in the letter are:

• A personal memory of the patient and family,

• Specific references to qualities of the child, such as courage, humor, and kindness, can bring life to the letter,

• An acknowledgment of all the family did for the child,

• A statement that it was a privilege to have participated in the child’s care,

• Let the family know that it is fine to contact caregivers with questions or concerns,

• Conclude with a statement that the child will be remembered, which can also be very comforting.

Clinician acts of kindness and commemoration following a child’s death should not be taken lightly. In a qualitative study ⁴¹ of bereaved parents whose child was in the care of the ICU three critical themes were identified:⁴¹

1. Parents place great importance on the hospital’s memorial service, and on staff presence at such a service.

2. Parents often find it difficult to return to the hospital after the child’s death, but attending the memorial service is common and helps with their bereavement.

3. Parents greatly appreciate receiving cards and clinician efforts to telephone, visit and attend the funeral and these little acts of kindness are recalled months, even years, later. Conversely, parents express disappointment when clinicians did not engage in these activities.

It is increasingly common for palliative care programs to offer longer-term bereavement support to families and these efforts are described in detail in Chapter 5.

Summary

Caring for a dying child is a truly intimate experience and a privilege that brings with it great sadness, but also enormous gifts, especially when clinicians are prepared to participate in this process and are given the opportunity to provide unhurried, compassionate care. The following case exemplifies such an experience.

Clinical Vignette

Mary was a 16-year-old girl with an advanced brain tumor and spinal metastases. Together with her family, her goals were to continue to strive for cancer control, but at the same time enjoy as much time at home in her community, which was several hours away from the hospital. She was passionate about attending school, being with family and friends, and painting (Fig. 36-1). Comprehensive support was put into place at home and Mary’s many symptoms were managed proactively. Her medication regimen was extensive including the following:

Fig. 36-1 Painting by Mary in the last year of her life.

Oral Medication	Indication
Dexamethasone 2 mg qid	Cerebral edema
Omeprazole 20 mg bid	Gastric prophylaxis
Bisacodyl 2 tabs bid	Constipation
Ondansetron 8 mg tid	Nausea
Metoclopramide 10 mg tid	Nausea
Methadone 15 mg tid	Pain
Methylphenidate 15 mg qam, 15 mg qnoon	Daytime drowiness
Celecoxib 100 mg bid	Antiangiogenic agent
Nortryptiline 20 mg qhs	Neuropathic pain
Gabapentin 300 mg tid	Neuropathic pain
Baclofen 15 mg tid	Muscle spasm
Sertraline 100 mg qhs	Depression, anxiety
Trazadone 25 mg qhs	Depression, insomnia

Unfortunately, 1 month before death, Mary was admitted to the hospital in pain crisis and it took 3 days before her distress could be eased. The team was concerned that the only way to achieve comfort would be through palliative sedation, and Mary’s family was prepared for this possibility. But in true Mary fashion, despite substantial escalation of symptom-relieving medications, she woke up and was comfortable. The next phase of conversation related to whether it would be possible to transition Mary back home. Unfortunately, her home hospice team believed that they could not manage her complex regimen. Instead, she resided in the hospital’s homelike suite, “The Comfort Care Room,” for the final weeks of life. Fortunately, her community came to her. Mary continued to be involved in decision making. One such decision was related to her continuing to take food by mouth. Despite many hours of being asleep a day, Mary would regularly wake up in the morning and asked to be wheeled down to the cafeteria for a breakfast of bacon, eggs, and potatoes. However, her ability to swallow effectively was diminishing and she was at high risk for aspiration. Nonetheless, she chose to continue her passion of eating breakfast. Comfort was achieved through a modified medication regimen.

Intravenous Medication	Indication
Dexamethasone 4 mg qid	Cerebral edema
Ondansetron 8 mg tid	Nausea
Methadone 10 mg qid	Pain
Fentanyl 225 mcg/h PCA with 180 mcg bolus q6min	Pain
Ativan 1.75 mg IV q4h, 1 mg IV q1h prn	Anxiety
Haldol 1 mg IV q6h	Anxiety, agitation, hallucinations
Scopolamine patch	Excess secretions