Aspirin, Acetaminophen, and NSAIDs

Except for specific indications in rheumatologic diseases and for inhibition of platelet adhesion, aspirin is used less commonly as an analgesic in pediatrics because of its association with Reye’s syndrome. NSAIDs are most effective as pre-emptive agents, or for treatment of mild to moderate pain of somatic origin. Acetaminophen is used widely for minor pain and discomfort. Dosage guidelines for aspirin and other analgesics are summarized in Table 106-1. As excess dosing can cause hepatic failure in infants and children, maximal daily doses are also listed. Other NSAIDs, most commonly ibuprofen and naproxen, have gained wide acceptance in pediatric use, and are equally effective. For children who cannot tolerate oral dosing, ketorolac is available. NSAIDs provide a weak analgesia effect that tends to be more effective for pain that has an inflammatory component, as with postoperative pain. The concurrent use of NSAIDs can reduce opioid dosing needed for effective pain management. The use of more than one NSAID concurrently offers little therapeutic advantage and increases risks for side effects, such as gastrointestinal ulceration and bleeding. NSAIDs used in conjunction with corticosteroids augment risks of serious gastrointestinal complications.

Opioids

Opioids are the mainstay of treatment of severe pain, operative procedures, postoperative pain relief, and management of chronic painful medical conditions in neonates and children [Davies et al., 2008b; Berde and Sethna, 2002; Golianu et al., 2000; Stevens et al., 2000]. Opioids bind to brain and spinal cord opiate receptors to block neurotransmitter production. Mu-receptor agonists, such as morphine, codeine, fentanyl, hydrocodone, and hydromorphone, are effective drugs for all pain intensities and are first-line choices for pain management. Agonist–antagonist drugs are not recommended, as they have an analgesic ceiling, may precipitate withdrawal if given concomitantly with full agonists, and have limited routes of administration. Suggested guidelines for opiate-naive patients are shown in Table 106-1. As opiate agonists have no therapeutic ceiling, dosages should be adjusted to meet the patient’s individual needs. The overall safety and efficacy of opioids are well established. In children, as in adults, the risks of addiction after receiving opioids for pain are low; thus, clinicians should not hesitate to treat pain aggressively. However, increased risks for respiratory depression in infants less than 6 months of age require close cardiorespiratory and saturation monitoring in settings in which emergent airway management is feasible. Respiratory depression is seen more commonly when sedatives are used. Methadone, a long-acting opioid agonist, has been utilized in combination therapy for children with cancer pain, as it adds to the opioid analgesia and prevents or reverses a portion of the opioid tolerance [ Davies et al., 2008b; Gold et al., 2006]. Fentanyl is the drug most often used for short procedures because of its rapid onset (1–2 minutes), peak effect (3–5 minutes), and short duration of action (2–4 hours). Morphine has a somewhat slower onset (5 minutes), with a peak response at 10–30 minutes, and longer duration of action (3–8 hours).

Intravenous bolus or continuous infusions allow more rapid and reliable onset of analgesia that can be titrated easily and reversed quickly by narcotic antagonists. Patient-controlled analgesia can be used in children (generally over age 7 years), but younger children should be under parent or nurse control. Opioids also may be administered subcutaneously, orally, rectally, transdermally, or intranasally. For some procedures, oral transmucosal fentanyl citrate provides an alternative for conscious sedation, although emesis is a frequent side effect. Transdermal fentanyl patches with a drug-release rate of 12.5 μg/hour match the lower dose requirements for pediatric cancer pain control [Zernikow et al., 2009]. Rectal administration can be utilized when oral dosing is contraindicated; intramuscular injection should be used only as a last resort. Adverse effects of opioids include respiratory depression, sedation, vasodilatation, hypotension, bradycardia (fentanyl), muscle rigidity, urinary retention, ileus, seizures, and rigid chest (fentanyl given rapidly). Tolerance may develop more rapidly with continuous infusions and with use of synthetic opioids. Characteristic withdrawal symptoms, including irritability, hypertonicity, diaphoresis, fever, and emesis, can occur when opioids are discontinued abruptly after several days’ exposure.

Procedural Sedation and Analgesia

Most minor procedures that are minimally invasive can be performed using a mild analgesic and sedation. Nonpharmacologic pain management strategies may also decrease the need for pharmacologic treatment. Among those demonstrated as effective are perioperative preparation, relaxation training, distraction, guided imagery, cognitive interventions, and hypnosis [Khan and Weisman, 2007]. More uncomfortable procedures require deeper sedation, more appropriately performed by anesthesiologists, intensivists, or emergency physicians trained in advanced life-support and experienced in deep sedation techniques and monitoring. The American Academy of Pediatrics has defined two levels of sedation less deep than general anesthesia [AAP, 2002]. Conscious sedation is a medically controlled state of depressed consciousness that:

Deep sedation is a medically controlled state of depressed consciousness or unconsciousness from which the patient is not easily aroused, loses protective airway reflexes, and cannot maintain an airway or respond purposefully to physical stimulation or verbal command. Guidelines for sedating children undergoing therapeutic or diagnostic procedures have been reviewed [Krauss and Green, 2000]. A recent review details procedural pain management in neonates [Lago et al., 2009].

Analgesia

Topical analgesia can reduce the discomfort of minor procedures or the local infiltration of anesthetic agents that should be used for lumbar punctures, difficult intravenous cannulation, and suturing. Useful topical compounds when skin is intact include TAC (tetracaine, epinephrine, and cocaine), LET (lidocaine, epinephrine, and tetracaine), and EMLA (eutectic mixture of topical anesthetics, prilocaine, and lidocaine). For very brief procedures, ethyl chloride or fluoromethane can be used to cool the skin and provide a limited analgesic effect. For procedures involving deeper structures or prolonged painful interventions, local anesthetics or peripheral nerve blocks are required. For procedures, fentanyl is preferable because of its faster onset, shorter duration, and lack of a histamine-inducing effect; transmucosal fentanyl (as lozenges or lollipops) offers procedural analgesia but often causes emesis. For longer-duration procedures, morphine or meperidine remains the preferable agent. Given either intramuscularly, intravenously, or enterally for painful procedures, ketamine induces profound analgesia, sedation, and immobilization while spontaneous respiratory activity, airway tone, and protective reflexes are maintained. It may be the drug of choice for emergency procedures when the patient has not fasted. Hallucinations, vivid dreams, and dysphoria are seen less commonly in younger children than in adults.

Sedation

Benzodiazepines are the most commonly used drugs for sedating children, particularly in pediatric intensive care. Midazolam is the preferred drug for procedural sedation. It has a rapid onset of action (1–5 minutes) and a short half-life (1–12 hours). Available for intravenous, intramuscular, intrathecal, or enteral routes, it provides potent sedation, some muscle relaxation, memory loss, and anxiolysis. Oral or intravenous routes are preferable for children; nasal administration causes an intense irritant response. As it has shorter duration than diazepam or lorazepam, midazolam is a more appropriate choice for brief procedural sedation. Oral diazepam avoids the pain of intravenous injection and the variable absorption of intramuscular doses. For painful procedures, benzodiazepine sedation should be accompanied by opioid analgesia, and the patient should be monitored for hypoxia or respiratory depression. Midazolam can be reversed by flumazenil. Barbiturates are regarded as the sedatives of choice for diagnostic imaging in children under 3 years of age, but provide no analgesia. Barbiturates lower the pain threshold, especially when pain is already present. Intravenous pentobarbital, rectal methohexital, and thiopental are the drugs most extensively used for procedural sedation. Chloral hydrate, an acceptably safe alternate sedative without analgesic efficacy, can be used for nonpainful procedures; the oral or rectal dose is 25–100 mg/kg (maximum dose, 2 g). Its use is largely restricted to sedation for electroencephalograms and diagnostic imaging in young children under 3 years of age. Its extremely long half-life in neonates mitigates against repeated administration [Mayer et al., 1991], and other concerns have been raised about potential carcinogenicity and genotoxicity in mice, even when it is given as a single low dose [Salmon et al., 1995]. Given at subanesthetic doses, intravenous ultra-short-acting agents (etomidate, methohexital, propofol, remifentanil, and thiopental) can be used for procedural pain management, but should be used only by certified practitioners experienced in dealing with the potential for over-sedation or rapid swings in consciousness.

Neuropathic Pain

A variety of neuromuscular and neurodegenerative diseases can cause both nociceptive pain (e.g., contractures or osteoarthritis) and neuropathic pain (e.g., nerve injury or entrapment) [Galloway and Yaster, 2000]. Neuropathic pain is seldom responsive to opioids, and often requires multiple modalities for effective pain relief. Infiltration or compression of nerves by tumors responds, to varying extent, to palliative radiation, steroids, surgical decompression, adjuvant drugs (tricyclics, antiepileptics), neurolytic nerve blocks, epidural or intrathecal blocks, and opioids (which are often ineffective) [Galloway and Yaster, 2000]. Phantom pain after amputation may respond to pre-emptive regional anesthesia, antiepileptics, tricyclic antidepressants, calcitonin, topical agents, or intrathecal medication. Nerve trauma may be treated with injected local anesthetics, steroids, neurolysis, antiepileptics, tricyclic antidepressants, or mexiletine. Neuropathies after chemotherapy can be treated with antiepileptics, tricyclic antidepressants, or mexiletine.

Tricyclic antidepressants (most commonly, amitriptyline, doxepin, or sertraline) are used as adjuvants to inhibit norepinephrine and serotonin reuptake, thereby increasing neurotransmitter tone at the cortical, brainstem, and spinal cord level. Pain relief is achieved more quickly than relief of depression. Given the quinidine-like effects of tricyclics on cardiac conduction, baseline and periodic electrocardiograms are recommended. Antiepileptic agents (carbamazepine and gabapentin) have been used, to a limited extent, in children to treat lancinating pain from peripheral nerve compression or injury and for peripheral neuropathies [Galloway and Yaster, 2000]. Mexiletine, an oral antiarrhythmic with potent analgesic effects, may be useful for some neuropathic pain resistant to tricyclics or antiepileptics. Since it can enhance the efficacy of other agents, it is usually used in combination with other drugs.

Pain in Children with Significant Neurological Impairment

Children with cerebral palsy or developmental delays experience pain, despite some reports of blunted pain responses or insensitivity to pain [Oberlander et al., 1999]. Patients with neurodevelopmental impairments have a variety of conditions that may cause pain: splinting and casting, dislocations and joint contractures, spasticity, pressure sores, feeding tubes, constipation, and diagnostic and therapeutic procedures. Pain from spasticity and muscle spasms can be treated with diazepam, dantrolene, oral baclofen, or botulinum toxin. Oral analgesia is least invasive and is therefore the preferred route; intramuscular injections may be more painful in children with decreased muscle mass. Subcutaneous and transdermal administration can be used for chronic administration.

Patients with neurologic impairment are more likely to have pain discounted or denied, and thus may have pain treated ineffectively. Providers believe that cognitively impaired children manifest more sensitivity to pain than children without impairment, and that children with mild cognitive impairment may over-react to pain [Breau et al., 2003]. In patients too impaired to communicate pain levels, behavioral responses should be regarded as surrogate measures of pain and the pain should be treated appropriately. To avoid over- or under-treatment when self-report is uncertain, it is important to choose a measure appropriate to the individual patient that can be measured over time. For children with significant neurologic impairments, the need for analgesia for acute procedural pain must be balanced by the recognition that systemic opioids may have undesirable side effects, such as respiratory depression or constipation.

Migraine and Headache

Symptomatic pain relief can be by oral, rectal, or intravenous routes, which have been reviewed elsewhere [Annequin et al., 2000]. NSAIDs or weak analgesics can be given in adequate doses for mild to moderate pain from tension headaches and many children with migraine attacks. Practitioners should be aware of the possible development of medication overuse headache when NSAIDS or opiates are used chronically. Practice parameters have been published for the evaluation and treatment of migraine headache in children and adolescents [Lewis et al., 2002, 2004]. With intractable migraine, dihydroergotamine and sumatriptan have been used in preference to opioids, due to the potential for abuse or dependence in patients with recurrent attacks. For migraine, the hallmark of effective control is treatment early in an attack. Intranasal administration of dihydroergotamine may be preferable due to the nausea and vomiting associated with some migraine headaches. Children with tension headaches improve with simple analgesia, and relief of any precipitating stressors. Once a chronic pattern is established, cognitive-behavioral techniques, such as biofeedback and relaxation therapy, may help alleviate headaches.

Identifying the Need

Whether providing primary care or subspecialty consultation, neurologists must learn when to address the palliative care needs of their patients and family members. For example, children with neuromuscular diseases, and their family members, have a diverse range of medical, psychological, and emotional needs that differ from those of dying adults. Providers should learn to recognize when the need for palliative care is becoming more salient with neurodegenerative diseases or progressive neuropathies, or as medical complications from a static encephalopathy impinge on quality of life (e.g., aspiration in patients with spastic quadriplegia). Medical information about expected outcome and services should be appropriate for the child’s developmental age, current cognitive capacity, and emotional stage, as well as sensitive to parents’ ability to accept their child’s clinical deterioration.

Given that there is often a significant degree of prognostic uncertainty, a series of questions can facilitate a shift in goals of care [Feudtner, 2007]. First, what has been the child’s health status and quality of life previously? Second, how likely is the child to survive the current life-threatening condition? Third, what degree of suffering is entailed in the recovery process if the child survives in the short term? Fourth, what is the anticipated quality of life in the future and how long is it expected to endure? Fifth, how likely are future crises or deteriorations? Answers to these questions can help inform the counseling process and facilitate later decisions about the appropriateness of further interventions.

Providers should allow time for the family to nurture their child, while recognizing the difficult emotional stresses that parents face seeing the acute change in their child’s condition or coming to grips with a certainty of neurologic compromise or death in the future. Providers should attempt to give parents and, when appropriate, children some sense of control when they often feel helpless and overwhelmed. Even when critically ill or dying, children benefit from being with and comforted by their parents. While to an extent remaining strangers at the bedside, physicians and nurses often become powerful and meaningful sources of support to parents [Meyer et al., 2002].

Transition in Goals of Care

Palliative care for children does not need to be restricted to those expected to die soon when therapeutic interventions fail or are withdrawn. Some neurologic conditions are associated with a predictably shortened life span (trisomy 13 and 18, neurodegenerative diseases, anencephaly or severe hydrocephalus, and many inborn errors of metabolism are examples), and the primary focus of care is limited to optimizing quality of life when a cure is not possible. Other diseases make prediction of life expectancy and quality of life more difficult (for example, traumatic or hypoxic brain injury, some central nervous system or spinal tumors, congenital myopathies and neuropathies, muscular dystrophy, or storage diseases). As it can be difficult to tell who will die early on in many central nervous system and neuromuscular disease processes, it seems appropriate to offer palliative care to children with progressive conditions at an early stage, transitioning from curative efforts to palliative care in response to their worsening condition. Accordingly, palliative care efforts can, and should, coincide with therapies aimed to cure the disease or limit its progression. With children old enough to express their preferences, it is important to address future choices for care before neurologic status deteriorates to the extent that they are no longer able to participate in health-care decisions. Parents must be given the information necessary to decision-making, but must also understand the limits of our ability to prognosticate treatment efficacy and outcome. Mention of palliative therapies, supportive care, or levels of non-intervention early in the course of a chronic or progressive life-threatening disease can soften the impact of non-intervention discussions later in the disease trajectory. An essential step is to clarify, and re-assess periodically, the goals of continuing, rather than limiting, on-going interventions as the chances for cure lessens (Figure 106-1).

Fig. 106-1 Model for concurrent components of palliative care.

(Modified from Michelson KN, Steinhorn DM: Pediatric end-of-life issues and palliative care, Clin Pediatr Emerg Med 8[3]:212, 2007; Feudtner C: Collaborative communication in pediatric palliative care: a foundation for problem-solving and decision-making, Pediatr Clin N Am 54:583, 2007.)

Providers should facilitate the transition from curative/life-extending care to supportive efforts intended to improve quality of life. They may need to initiate discussions about changing treatment goals when parents have difficulty admitting that end-of-life care is needed [Callahan, 2000], particularly when parents have adapted to the demands of caring for children with life-threatening chronic conditions and to the diminished quality of life associated with many of these conditions (e.g., spinal muscular atrophy, unresponsive central nervous system tumors, or states of minimal consciousness). Acute brain injury (such as perinatal asphyxia, and other sudden catastrophic deteriorations in neurologic status) can make the transition to palliative care difficult, as physicians often cannot offer clear prognostic certainty on survival or later neurodevelopmental functioning. With emergent conditions, there is often little time for difficult decisions or coping with complex emotions, and the transition from curative interventions to palliative care can seem abrupt [Frager, 1996; Sahler et al., 2000].

Levels of Care

The shift in treatment goals, however, does not imply that on-going medical interventions aimed at treating or curing the child’s disease (such as blood product administration, oxygen, or antibiotics) must be abandoned, as they typically are in adult hospice care. Some potentially beneficial treatments entail significant burdens, and it becomes important to clarify specific measures that will continue to be utilized, as well as what level of non-intervention appears most appropriate. An order proscribing all or selected resuscitative measures in the event of a cardiac or respiratory arrest (a “Do not resuscitate” [DNR] or “Do not attempt resuscitation” [DNAR] order) is often the first step. This order represents a selective non-intervention order in the event of a respiratory or cardiac arrest, not a half-hearted resuscitation (“slow code”) of a child deemed likely to die. When providers and parents perceive that further medical therapy offers no benefit, that on-going interventions are inappropriate, or that the present quality of life is untenable, more extensive discussions about withholding or withdrawing specific life-sustaining therapies may be indicated [Wall and Partridge, 1997]. Withholding of life-sustaining measures involves an agreement not to institute specific medical measures that would otherwise be indicated medically. This level of non-intervention is reserved more often for children with a grim prognosis or whose quality of life would not be improved by more aggressive interventions. Such a level of non-intervention might be deemed appropriate for infants with trisomy 13 or 18, cerebral or spinal tumors, severe central nervous system malformations, or severe hydrocephalus. Interventions deemed futile are not required and should be discontinued [Paris et al., 1999] – for example, in patients with brain death, anencephaly, lethal neuropathies or myopathies, or untreatable inborn errors of metabolism. Withdrawal of life-sustaining measures involves the discontinuation of medical interventions currently in use but deemed either futile or no longer appropriate to the child’s best interests, accepting the possibility that death may be hastened. While ethically equivalent, withholding and withdrawing often feel different at the bedside and can raise emotional responses for parents or providers. Whenever support is withheld or withdrawn, it is appropriate for the primary physician to discuss DNAR status with parents and the health-care team before writing the order.

Communication

Patterns of communication sensitive to the child’s developmental age and neurological status and to the parents’ emotional state are critical to good palliative care for children. The process by which collaborative communication in pediatric palliative care can set the foundation for problem-solving and decision-making has been reviewed in detail elsewhere [Feudtner, 2007]. The degree to which the child can participate in decision-making increases as children develop increasing cognitive capacity (over the age of 7). The use of pictures, stories, toys, music, or other family rituals can help younger children communicate their preferences. On the other hand, infections of the nervous system, hypoxia-ischemia, neurodegenerative diseases, or traumatic brain injury can diminish older children’s decision-making capacity. Whenever the child has the cognitive capacity to participate, it is important for providers to allow the child the time to express hopes, dreams, and fears [Back et al., 2003].

Discussions about treatment options should review the child’s current status, estimate the presumed disease trajectory, characterize the quality of life that each treatment choice would entail, and, finally, assist the child and parents in selecting which measures should and should not be done. Given the frequency of prognostic uncertainty in many pediatric conditions, it may be helpful to ask a series of questions to provide the best information on which to base decisions [Feudtner, 2007] (Figure 106-2). As the disease progresses, it may be necessary to reframe the situation by shifting from a goal of curing the disease to a goal of caring for the patient. The primary focus is to determine what is important to the child and the family: longer life, pain-free, time with family members, specific desires, environment and setting, time to say goodbye. The next step is to devise a collaborative care plan that respects the patient’s emotional and spiritual needs, incorporates family values, and supports family involvement in health-care decisions. When time allows, such conversations are best held by continuity providers over a period of time, giving cognitively capable patients or parents time for questions, second-opinion consultation, talks among family members, reflection, deliberation, and informed decision-making. Repeated discussions empower informed decision-making, and help minimize the guilt some parents feel about their decisions after their child dies.

Fig. 106-2 Questions to address prognostic uncertainty in palliative care decisions for children.

(From Feudtner C: Collaborative communication in pediatric palliative care: a foundation for problem-solving and decision-making, Pediatr Clin N Am 54:583, 2007.)

A structured approach can help physicians deliver difficult news and negotiate goals of care (Box 106-5). Critical aspects of the communication process include simple, direct, clear, and honest explanations that relate the expected outcomes of each treatment option without prejudicing or constraining the choices. It is also important that the information neither overwhelms the parents nor leaves them in despair. Providers should avoid promising outcomes that they do not believe are reasonably feasible. Neurologists play a key role in communicating the expected neurologic outcome after a life-threatening illness; neurology consultation can help parents understand expected functional status when there is little chance for an acceptable outcome. To minimize the sense of giving up or abandoning their child, it is critical that parents understand that the goal of care will be to do everything that should be done, rather than everything that can be done.

Box 106-5 Protocol for Palliative Care Communications

SPIKES: A Protocol for Delivering Bad News and Negotiating Goals of Care

Setting: create environment conducive to communication

Perception: determine what the family knows

Invitation: determine how the family would like information delivered

Knowledge: provide information

Emotions: empathetically support patient

Summarize: set realistic goals, make a treatment plan, and follow through

Repeat: review and revise frequently

Conflicting perspectives occasionally complicate end-of-life discussions. Attempts to understand the perspectives of all of the parties in the decision-making process better may result in a more collaborative process [Feudtner, 2007]. This may require providers to explore their own attitudes, as well as clarifying those of the patient and family. The use of open, direct, and supportive language is critical to consensus-building and resolution of potential conflict [Feudtner, 2007].

Health Care Decision-Making

As children usually have no previously documented preferences for health-care decisions, parents and physicians must assess when the burdens of care are no longer in the best interests of the child. The first issue is to identify and involve those who will be making health-care decisions. To the level of their developmental capacity, older children themselves have the right to determine what quality of life means to them, with support from their parents and physicians. Younger children necessarily depend on their parents as the decision-makers.

Parents (or the competent adolescent) determine the quantity of information necessary for them to make an informed decision. If decisions seem not to reflect a full understanding of the disease, the goals of care, or expected outcomes, further discussions facilitate review of the information and reflection on treatment options. Decisions that do not concur with the provider’s choices should be respected when taken in the child’s best interests. Advanced directives regarding care preferences may have no legal relevance in pediatric patients [Pierucci et al., 2001], although the process may afford older children a more participatory role in making end-of-life decisions [Zinner, 2009]. In general, parents are assumed to be the primary proxy decision-makers for young children; however, their discretion is not limitless. When consensus is difficult to reach, ethics committee consultation can help clarify impediments to decision-making. In rare situations in which parents’ judgment may be questionable or the child’s best interests remain uncertain, providers may need to advocate for the child or even refer to child welfare services. A process of guided autonomy can support the child’s and the parents’ involvement in treatment decisions while preserving some autonomy and minimizing the undue emotional strain of making decisions to limit care. Once the goals are clarified and decisions have been made, the care plan should be disseminated to the entire health-care team, and any specific limitations to medical interventions clearly documented in the patient’s chart. It is critical that decisions to limit the invasiveness of care not lead to a “do nothing” environment [Feudtner, 2007].

Persistent Vegetative State

A small proportion of children who survive critical illnesses continue for varying periods of time in a persistent vegetative or a minimally conscious state. While the exact incidence is unknown, the estimated 6000 infants and children that live in this state present a special challenge to clinicians and ethicists alike [Ashwal et al., 1992]. Although there is no consensus as to whether children in a persistent vegetative state experience pain and suffering, it seems prudent to treat signs or symptoms in children in a persistent vegetative state to minimize pain and suffering. Decisions to withdraw or withhold life-sustaining measures should be made in collaboration with parents only when neurodiagnostic testing and a sufficient period of observation suggest there is no chance for a meaningful recovery. Decisions to forego supportive care, especially those involving the withholding of fluids or hydration, remain controversial, even when deemed appropriate responses to the child’s medical situation. Such decisions present complex emotional and ethical challenges to clinicians and family members [Carter and Leuthner, 2003].

Environment for Death and Dying

Some parents may prefer continued hospital care where skilled nursing and medical care offsets the limitations to privacy and the inhospitable ambience of most inpatient settings. A private space for families to gather before and after the child’s death is recommended (e.g., a screen for privacy or, better, a separate comfort care room) and should not impede nursing care or prompt medical attention to pain and symptoms. Other inpatient options include transfer to a lower level of care or to a hospice, although some parents may feel abandoned by changes in care providers late in their child’s disease. Death at home is preferred by a significant proportion of children and parents [Pierucci et al., 2001]. An increasing proportion (approximately 25 percent) of deaths from neuromuscular disorders occur in the home setting, second only to deaths from malignancy [Feudtner et al., 2007]. Parents who opt for home care must be supported adequately in managing terminal care for the child outside the hospital. Plans for a death at home require a continuity provider skilled in managing pain and suffering, and able to provide 24-hour coverage from discharge through the child’s death and the parents’ bereavement.

Support During Dying

When death is near, providers can prepare parents for the child’s imminent death by envisioning expected changes in the child’s appearance, advising them as to likely timing, and assuring them that pain or suffering will continue to be treated aggressively. Parents usually choose to hold their dying child; this should be facilitated and parents’ well-being ensured, as best as possible. Mention can be made of discussions that will be required after the child dies, such as autopsy or organ donation. Some families perceive organ donation as a positive aspect within the tragedy of their child’s death (e.g., after traumatic brain death or sudden infant death syndrome). In other cases, such as anencephalic infants, organ donation continues to pose difficult medicolegal issues for clinicians. Overall support to a family while a child dies involves a balance between respect for privacy and close, but non-invasive, attention to the child’s and the family members’ needs. Neurologists caring for terminal patients can turn to primary care pediatricians, social workers, clergy, psychologists or psychiatrists, and other sources of support as parents’ adjustment dictates.

Assessment and Treatment of Symptoms

A primary focus of palliative care is the relief of distressing symptoms that detract from the child’s enjoyment of life. Providers should discuss symptom management in advance to reassure patients and parents and to educate them as to what may occur as the disease progresses. Providers should re-evaluate the efficacy of pain and symptom management as the disease progresses. Some neurologists will feel comfortable managing pain and other end-of-life symptoms, and will follow the child throughout the course of the disease; some consultant neurologists may prefer to consult the primary care pediatrician or pediatric palliative care specialists with expertise in symptom management.

Pain should be treated aggressively with analgesics. When there is severe or unremitting pain at the end of life, terminal sedation with opioids is used to alleviate suffering, recognizing that the opioids may suppress respiratory drive in debilitated patients and may thereby hasten death. The risk of death is justified because there is no alternative that alleviates suffering while lessening the risk of death. Fears of addiction to opioids, the mainstay of pain management in end-of-life care, on the part of providers or family members are unwarranted. Paralytics are not appropriate when life support is being withdrawn [Pierucci et al., 2001]. Anxiolytics may help terminal agitation and perhaps air hunger experienced in the terminal phases. Many neurologists will feel comfortable treating seizures, insomnia, and depression in dying patients, but may prefer to have primary care pediatricians or palliative care specialists manage other frequently encountered end-of-life symptoms (dyspnea, anxiety, fatigue, fever, bleeding, nausea, skin breakdown, anorexia, dehydration, and constipation). Despite shared concerns for patient well-being, treatment of other symptoms experienced by dying patients has been inconsistent [Pierucci et al., 2001; Wolfe et al., 2000; Partridge and Wall, 1997]. Attempts should be made to organize a care plan allowing a death without suffering (either at home or in hospital) for any child in need, regardless of socioeconomic circumstances.

Developmental, Emotional and Spiritual Concerns

Care for a dying child requires candor, openness, and an emotional availability to the child’s experience of illness and imminent death. Children need to come to understand death and dying in terms of their developmental age. Research indicates that they come to understand it as a change of being by age 3, and as a universal fact of living by age 5–6, and have gained a concept of their personal mortality by age 8–9 [Himelstein et al., 2004]. Spirituality is one way that patients and families cope with death and dying, whether or not they adhere to any specific religion. Approaches that respect the children’s spiritual nature at varying developmental stages will allow providers to explore issues of love, hope, security, loneliness, and concepts of the legacy that the child will leave to surviving parents, siblings, friends, and schoolmates. Family routines and rituals add familiar structure and a sense of normality as the medical condition worsens, and should be encouraged whenever possible.

Bereavement

Children are aware of dying and disease; they understand much more about death and dying than adults often realize. Parents who sense that the child is aware of imminent death more often regret not having talked with the child than do parents who do not sense this awareness in the child, and typically parents who talk with their child about death do not regret doing so [Kreicbergs et al., 2004]. Children grieve as much as adults, although in different ways. Dying children may grieve about their impending death, the loss of functional status, or the inability to participate in current or future events. They may fear how their family members will cope after their death. Dying children and their siblings do not need to be protected from death and dying as much as they need help making it understandable. When heritable neuromuscular disorders pose a future risk to affected siblings, particular care is necessary to deal with parents’ sense of guilt, parents’ and siblings’ grief, concerns about subsequent pregnancies, or family fears of illness in the future.

In general, the grieving process should not be rushed. Providers should allow family members the opportunity to spend time grieving with their deceased child alone. In the immediate moment, there is a time to talk and a time to be silent; it may be helpful to celebrate their child’s life, recap the family’s experience, value their loss and their emotional responses, and help them contact family members and friends. Parents should be offered religious observances, social services support, family support groups, or other supportive measures to help them cope. Excluding children may foster a loss of trust or a sense of resentment. Funerals can be ways in which children can participate in remembering and valuing the dead child. In some cases, psychological counseling may be offered as a way to help parents and siblings work through the emotional trauma.

Follow-Up Conference

Many physicians arrange a post-death conference to see how the parents are coping and help them resume more normal functioning in the aftermath of the child’s death. A meeting can review autopsy results and clarify aspects of the diagnosis, etiology, and end-of-life events; it can provide a measure of solace by showing that providers remember the child and continue to care about the family. It is often an opportunity to put to rest parents’ questions about what “might have been,” to minimize tendencies to blame themselves or their child’s providers, to detail the implications of heritable neurologic disorders for siblings or subsequent pregnancies, and to offer referrals to counseling or support groups. It can also indicate ways in which providers might improve end-of-life care for future patients.