36 Easing Distress When Death is Near

What is a good death? When asked, many adults facing the end of life hope to control pain and other distressing symptoms, have a sense of preparation for death, and achieve a sense of completion; however, other factors important to quality at the end of life differ by the individual.¹ For some, a child’s death can never be good. Nevertheless, as palliative care clinicians, we can aspire to enable a better death experience for the child and family when we are faced with the inevitability of a child dying. This chapter will review strategies aimed at trying to achieve this outcome.

Importantly, when a child is dying, all care goals may not be uniformly focused on easing suffering. Recent studies affirm the common clinical experience that even when a child’s illness is said to be incurable, parents will hope to extend life concurrent with ensuring comfort.^2,³ Family values will differ: While some aspire for a peaceful end of life experience for their child, others value another approach, as stated by one father:

“The battle with the dragon, or the intent and the need, or the struggle to come to grips with him, or just coexist with him even, threatened to consume our lives. In the sense that the battle against the illness and all the circumstances—logistical and practical, and medical, and financial, and interpersonal…that became all consuming, just like the dragon’s fiery breath…just like his fiery breath is understood to be all consuming, literally obliterating either an individual, or a number of individuals, or a whole village.”⁴

In other words, it is a fight to the bitter end. As clinicians, however, it is critical to remember that whatever the primary goal of care and setting, care should never be at the exclusion of ensuring comfort.

Of note, studies that assess quality of life at the very end of life in children are limited. Thus clinical experience based upon consensus and the existing literature guide the content of this chapter.

Interdisciplinary Caring

During this most intimate of clinician-family experiences, the care of a dying child, exquisite collaboration among all involved is required to meet the needs of the child and family. Table 36-1 highlights key roles and activities of the interdisciplinary team. No matter where the child is being cared for, short huddles or meetings among team members may be helpful to maintain open lines of communication.

TABLE 36-1 Interdisciplinary Roles in the Care of the Imminently Dying Child

Clinician	Discipline specific roles	Interdisciplinary roles
Primary physician/ Nurse practitioner	• Leads discussions related to establishing and re-evaluating goals of care and care plans • Therapeutic recommendations to relieve distress • Request for organ donation and autopsy	• Presence • Anticipatory guidance • Assessment and relief of child and family distress • Respond to questions and/or concerns • Perform little acts of kindness
Nurse	• Administration of distress relieving interventions • Ensure the setting is equipped to meet child’s and family’s needs with regard to staff, medications and equipment • Gatekeeper when requested by family • Communicator with larger team regarding bedside experiences of child and family
Psychosocial clinician	• Provide individual or family psychotherapy as a continuation of an ongoing process, or as needed on a crisis basis • Facilitate legacy-building opportunities • Assist with advanced care planning regarding after-death steps such as wake and/or funeral arrangements • Consider larger family and community needs such as siblings and school
Child life specialist	• Play activities to allow legacy-building opportunities • Play activities for child if able, siblings and other children to promote expression of feelings regarding child’s death • Play activities for siblings and other children to create opportunities to escape from the intensity of being with a dying child
Chaplain	• Assess and respond to spiritual needs of child and family • Ensure that clinicians are familiar with and respectful of family rituals related to death and dying • Meet with community spiritual leader as indicated
Pharmacist	• Advise about distress-relieving medications • Ensure adequate supply of distress-relieving medications

Recognizing When Death Is Imminent

Dying is a dynamic process. Though the process is influenced by many factors, it is remarkable how similar it can be despite very different underlying illnesses. Clinical experience suggests that when approaching death, a child typically experiences the following constellations of findings. The child is often bedridden, semiconscious, with little or no oral intake, and changes in pulse, respiration and peripheral circulation may also be apparent (Box 36-1).⁵

BOX 36-1 Signs of Impending Death

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.