Neurologic Diseases

Published on 09/04/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

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39 Neurologic Diseases

It truly takes a village to meet the complex needs faced by children with neurologic conditions and their families. Front and center to this village are the parents and day-to-day care providers of such children, the ones who see the nuances of how a specific disease or condition manifests in an individual child. Medical teams bring expertise in the spectrum of problems seen in many children with similar conditions; parents often bring expertise in how these problems look in their child. Along with the medical needs of such children, families face myriad challenges that exist in the context of their hopes, fears, beliefs, values, the uncertainty of a their child’s condition, and the community where they live. Table 39-1 is an introduction to the expertise available from palliative care and hospice teams to assist with the needs of such children and their families.

TABLE 39-1 Team Member Expertise

These individual areas of expertise are critical to managing the multidimensional needs encountered. Though an individual of an interdisciplinary team will bring in expertise in one of these areas of need, each member will bring skills in assisting with all areas.
Social workers
Provide emotional support to family, psychosocial assessment and supportive counseling in collaboration with other providers for families adjusting to palliative care issues, link to psychosocial and mental health resources in the local community, anticipate legal and financial needs including guardianship, government programs that cover some medical expenses, and special needs trust, and direct to appropriate resources
Child life specialists
Assist siblings with the fears of having a sister or brother with complex healthcare needs, assist families with memory making and legacy of the child, provide bereavement support, provide education around appropriate grief responses of children
Chaplains
Support faith traditions and spiritual values that promote healing and hope, support families as they face loss and grief, identify religious and cultural factors that can shape how a family faces illness and suffering, provide a supportive presence for sick children and their families, provide support and advice in learning how to respond to the suffering witnessed by medical care teams
Nurses
Assist with bedside assessment of pain and other distressing symptoms, work with families and other care providers to determine what specific features in a nonverbal child with NI indicate specific distressing symptoms such as pain and dyspnea, translate this information into a care plan that is translatable to other care providers, listen in real time during times of emotional and spiritual distress for families
Physicians and advance practice nurses
Expertise in symptom management, serve as mediators between the medical teams and families, assist with advanced directives by working with medical teams and families, assist with translating goals of care to how medical interventions can or cannot meet those goals, review autopsy, tissue and organ donation, and Brain and Tissue Bank with families

Pediatric Palliative Care and Neurologic Conditions

Pediatric palliative care teams are commonly consulted to see children who have diseases and impairments of the nervous system. Of children enrolled in a pediatric palliative care project, 44% were categorized with a primary neurologic condition (24% of those were deemed progressive neurologic, 20% were CNS damage) and 15% frequently have associated neurologic impairment (10% with congenital anomalies and 5% with metabolic).1 Recent data for the Pediatric Advanced Care Team (PACT) at Children’s Hospital Boston and Dana Farber Cancer Institute categorized 37% neurologic and 10% genetic or metabolic.2 Unfortunately, little has been written about palliative care as it pertains to children with neurologic impairment (NI) and their families.

Families of such children face many challenges, including: lifelong conditions, living with hope and uncertainty, experiencing the sorrow of what is not possible along with the joy of small victories, difficulty in assessing and managing symptoms, and navigating through the care of many specialists. Palliative care teams are well suited to address the myriad psychosocial, spiritual, and physical needs experienced by these children and their families.

The personal challenges experienced by children and their families exist within the larger context of societal debates regarding medical decision making for such children. These discussions are guided and influenced by ethics, morality, religion, personal values, justice, resource usage, and quality of life. Case reports in the literature highlight the variability of this debate with examples of doing everything, possibly so as to not create the impression of discrimination based on disability3 and not offering treatment out of an assumption of poor quality of life.4 As society wrestles with these challenges, we must avoid bringing this debate to the bedside and instead be guided by legal and ethical knowledge while providing compassion and support.

Patient Population

There are a broad range of conditions with NI that benefit from pediatric palliative care.5 The conditions may be:

Conditions and suggested timing of palliative care consultation are summarized in Table 39-2; assistance with symptom management can occur at any point in time.

TABLE 39-2 Neurologic Conditions and Timing of Palliative Care Consults

Category Conditions Timing of consultation
Severe disability causing vulnerability to health complications and/or palliative after diagnosis

Intensive long-term treatment aimed at maintaining the child’s quality of life Curative treatment intended but may fail

Unique experiences and challenges

There are a number of unique themes experienced by children with NI and their families, including the lifelong nature of such conditions, limitations in acquiring independence, bereavement that includes loss of the intensive care provider role, lack of evidence for medical decision making, and challenges with symptom management. Support from interdisciplinary teams includes anticipating and exploring how each area affects an individual child and family. This section focuses on the themes that cross psychosocial and spiritual areas of need.

Loss is a recurrent theme, starting from the time of diagnosis. This theme is often repeated when what the child cannot do and the ongoing problems that cannot be fixed are reviewed at medical appointments. This journey often includes chronic sorrow, a phrase used to describe sorrow over time in response to ongoing loss.6 Examples may include loss of functional ability, loss of ability to meet nutritional and fluid needs through eating, and loss of health. Families are also simultaneously exploring meaning and hope in the context of their values, beliefs, relationships, and supportive networks. They may find joy in little victories, outcomes that were not expected, as they navigate hope, meaning, loss, and uncertainty.

Loss of function often includes lifelong dependence on others for physical care and may include the same for decision making. Parents may have unspoken worries, such as what will happen if the child outlives his or her parents, and wonder how to factor this future worry into current decisions. As a child ages, questions of who will provide care, the location of this care, and who will make decisions for the child become relevant. Parents may worry about this child being a burden to another sibling while worrying about the quality of care at other facilities. For children living in facilities, such as medical foster homes, group homes, and long-term medical care facilities, another challenge is created when the legal decision maker is removed from the range of daily experiences, including suffering of the child. This can create conflicts when the child’s care provider, with a set of values, beliefs, and direct experience with the child, may judge a decision differently than the recognized decision maker. Other conflicting interests that often go unrecognized include financial interests of care facilities and personal interests of care providers, even when the best care for the child is the goal.

The care needs of children with NI often dominates a parents’ day, which may lead to limited emotional, spiritual, and physical reserves. This often leaves little energy to reflect and may result in decisions that are made during a time of crisis. The intensity of the care provider’s role can naturally result in this role becoming a prominent part of a parents’ identity. This may later impact bereavement when a parent experiences the loss of this role and loss of membership in parent support groups. In addition, being a long-term care provider can create a heightened sense of responsibility over outcome and loss of perspective over the intensive efforts being used to maintain the child’s level of health.

In the context of these challenges are the themes of hope and joy. It is not uncommon to identify a family saying, “I wouldn’t choose this, but I wouldn’t have it any other way” as they share the lessons learned through their child and the important role of this child in their family. Such families often experience simultaneous joy and sorrow that allows us to worry and celebrate with them as we join them and assist them on their journey.

Pediatric palliative care teams provide expertise through knowledge of themes that are universal to all children with life-threatening conditions, along with experience of themes that are unique to certain populations. This expertise allows teams to explore supportive approaches to healing and hope, meaning and values, relationships and connections, and grief and loss. Resiliency is an essential component of this process when a child and family live with a condition that is both lifelong, often experienced over years, and will result in an early death.

What to Expect: Life Expectancy Literature

Life expectancy data for children with NI, whether that data is related to the disability label, such as cerebral palsy, or related to a specific diagnosis that explains the disability, such as a genetic disorder, congenital syndrome, or metabolic disorder, provides a context but is of limited use for individual prognosis. Health problems that are life threatening can be divided into those related to the severity of the motor disability regardless of the cause, such as oral motor dysfunction and recurrent pulmonary aspiration, or related to the specific diagnosis that explains the disability, such as a genetic disorder with an associated cardiac defect or central apnea.

Information about life expectancy demonstrates a wide variation. The more severe the motor disability, such as the inability to lift the head up when prone, the more likely the child will not survive to adulthood.712 Survival for individuals with CP that includes severe impairment in cognitive function, motor ability, vision and hearing was 50% at 13 years and 25% at 30 years.9 It is beneficial to understand that CP is not a diagnosis but a developmental label indicating impairment of motor control as a result of non-progressive impairment of the central nervous system acquired at an early age. Information and experience from CP is relevant to any disease that results in severe motor impairment.

Even progressive diseases where decline occurs early in life, such as Tay-Sachs disease, often have a range of survival that may vary by several years. Prognostic information for specific diagnoses often lags the expanded survival from routine pulmonary management that assists with mobilization of secretions and treatment of acute respiratory illnesses. Worry over prognosis is further heightened when there is identification of neurologic impairment with no diagnosis. As palliative care clinicians we can assist through knowledge of the limitations of information that may be provided to families, use our knowledge and experience from all disorders with NI, and help anticipate and prepare for future events.

Framework for Approaching Prognosis, Uncertainty, and Decision Making

Literature and experience demonstrates a wide range in life expectancy, yet has provided limited information on how families approach this uncertainty. Studies13,14 of the experience of families with children dying from neurodegenerative conditions identify how they navigate uncharted territory and use strategies such as seeking and sharing information, focusing on the child, reframing the experience, and promoting the child’s health. Factors influencing parental decisions to limit or discontinue medical interventions include perception of their child’s suffering, likelihood of improvement, perception of their child’s will to survive, quality of life, previous experience with end-of-life decision making for others, and financial resources.15,16

Illness trajectory as a guide to decision making

Adult literature describes illness trajectories for cancer, organ failure, and frailty.17,18 Using the latter two trajectories and experience, Fig. 39-1 provides a hypothetical framework for reflecting on and anticipating the trajectory of a child with NI. The figure is intended to guide families through decision making by using a reflection on the past benefit of interventions to anticipating the probable and possible future benefit by hoping for the best, preparing for the worst.19

The hypothetical disease trajectory highlights that many health issues in children with NI progress gradually with initial benefit and return to health and functional baseline with treatment available. Over time, less return to baseline will occur from the interventions available, reflecting the inability to fix the problems that are secondary to the permanent NI. Predicting outcome at the beginning of the trajectory before any decline in health status is seen can result in pressure of past success20 when the outcome is better than predicted. Asking a parent of a child with NI to make a decision to limit interventions before the child has had any significant decline in health may feel as if the emphasis is on limiting interventions because of the disability. By identifying associated health problems, monitoring for changes in health status, and noting any decreased benefit from treatments, we can identify individuals with NI who are at risk for life-threatening events.

Anticipating benefit also requires knowledge of how offered interventions will alter the clinical course. Some of the more common interventions for children with severe NI include anti-reflux surgery, tracheotomy, and spinal surgery. Most of the information in the literature is case reports or small series without rigorous evidence to indicate which patient is likely to experience benefit, morbidity greater than the intended benefit, or no alteration in the clinical course. In addition, these interventions are often approached as needed and required for the problems identified, rather than interventions to offer and consider. Fortunately, relevant information can be gained from the literature; a summary of information for these interventions is provided later in this chapter.

Support for the process of decision making is best done through a reflection of the child’s history in the context of emotional and spiritual needs that impact this process. Outlined are questions to consider and review with parents and care providers. It is helpful to acknowledge that some may be difficult to answer but help frame what parents are often already worrying about and considering. Such discussions should include opportunities to reflect on the successes and experiences that are enjoyed by the child. In addition, a comprehensive review of emotional coping, sources of stress, spiritual beliefs, and support networks, as examples, should be sought.

Starting the conversation:

Reflective questions with parents and care providers:

Here are some further questions for families and medical teams after goals have been identified and the child’s health trajectory has been reviewed:

Advance care planning: how to hope and prepare

Areas of need as a result of slow decline include planning for future problems, avoiding interventions of limited benefit, and assistance for long-term caregivers.21 A goal is to facilitate planning by giving physicians permission to discuss what-if scenarios while giving parents permission to maintain hope. Palliative care can assist with this process by exploring psychosocial, spiritual, and physical needs that impact on decisions, such as the worry of giving up, a sense of needing to do something, and the fear that treating physical suffering will result in an early death. Given the inherent challenge of determining when goals shift from preserving health status to preserving comfort, medical, and palliative care are ideally integrated together for children with NI.22,23 Through this process of exploration with families and by integrating symptom-directed treatment into medical care plans, we can minimize the impression of choosing treatment that preserves life vs. comfort care that means giving up.

As palliative care teams attend to spiritual and emotional needs of patients and families, this ideally creates a healthy space for this reflective and anticipatory work. As parents encounter decisions for interventions in children with NI, it is helpful to acknowledge the range of decisions made by families. This naturally creates distress for both parents and medical teams; why is this decision, whether to do or not to do, acceptable when other families would choose differently? Palliative care teams can assist through identification of the details relevant to each family and communication of information to medical teams. Suggestions for follow-up include:

Resuscitation is another important part of advance care planning. Discussing resuscitation requires an honest assessment of the likelihood of benefit and possible burden. It is important that we assume responsibility for this assessment and provide directive guidance so as to not imply parental responsibility for such decisions. Many hospital-based resuscitation order forms now include advance care planning for other medical interventions. This can serve as a prompt to review interventions beyond intubation and cardiac resuscitation. Ideally, these discussions take place grounded by extensive knowledge of the child and family that then allows a comprehensive integration of spiritual, psychosocial, and medical details into care plans.

Several details about resuscitation are important for children with NI:

This is also a time to review that a decline in health is not a result of the care provided at home or a result of decisions made. Rather, it reflects the health problems that cannot be cured or fixed, while providing reassurance that interventions will be used for as long as the parents identify them as meeting goals. Several articles provide further beneficial communication strategies.24,25

Leo was admitted to the hospital with another respiratory exacerbation. Soon after admission, he was intubated and identified to have respiratory syncytial virus (RSV). A palliative care consultation was obtained 1 week later. Information identified included an overall decline in health, along with diminishing benefit from chronic treatments and use of antibiotics and steroids during acute exacerbations. Prior evaluation included sleep study negative for obstructive apnea but persistent oxygen desaturation to the mid 80s, a normal upper gastrostomy series (UGI), normal inflammatory markers, and decreased lung volumes noted on chest x-ray. A team meeting was arranged to include Leo’s primary physician, pulmonologist, the intensive care physician, as well as the chaplain, social worker, child life specialist, and physician of the pediatric palliative care team. Leo’s primary physician wants to give hope to the family by identifying the problem as RSV. Questions identified to facilitate discussion include: Is the prolonged course of ventilation more a result of RSV or chronic changes to the lungs? Would a tracheotomy help? What timeline would be expected to allow recovery to extubation, if this is possible? How much enjoyment or suffering is Leo experiencing between illnesses? Discussion identified that a child of this age would not be expected to require mechanical ventilation from RSV. The RSV was a trigger but the decline to respiratory failure indicates significant lung damage that is likely not reversible. A tracheotomy would not help but would be used if chronic ventilation was used. A timeline of 1 to 11/2 weeks was identified as sufficient for recovery.

During this meeting, family and care providers identified goals of comfort, quality of life, and the opportunity to remain home where he is most comfortable. Family and home care nurses continue to hope for recovery and as much time as possible with Leo. They do not anticipate using surgical interventions but need time to consider the options available. A follow-up team meeting is arranged for 1 week later, recognizing that the decisions will depend on whether recovery to extubation occurs.

Documenting, Communicating, and Coordinating Plans of Care

Great care is possible only when information is identified, documented, and communicated to those involved in the child’s care. This can ensure that previously defined care plans are carried out, which protects the child from interventions that are anticipated to cause potential harm without long-term benefit. It can be beneficial to others to summarize why certain interventions have been limited. This can lessen a sense of giving up and help direct those involved with the child to what we can do; that there is always care to provide.

Information to consider documenting includes: goals of care and how these goals guide decisions, heathcare and symptom management plans that meet these goals, naming the legal decision maker, the location for acute illness, resuscitation status, care plans for home and school in the event of a life-threatening event, and contact information for individuals with expertise and availability to assist at times of acute events. Those who should receive this information include the family, healthcare proxy or legal decision maker, home care nurses, providers of care in foster care or group homes, school nurses and teachers, respite care providers, bus drivers, healthcare team members, and palliative care and/or hospice teams.

Symptom Management

Children with NI experience pain more frequently than the general pediatric population.2629 Caregivers of children with severe cognitive impairment reported 44% experiencing pain each week over a 4-week interval. Pain frequency was higher in the most impaired group of children.27 In a study of nonverbal cognitively impaired children, caregivers reported that 62% experienced five or more separate days of pain and 24% experienced pain almost daily.29 In addition, children with severe-to-profound cognitive impairment were found to have elevated pain scores at baseline on two pain assessment scales.30

Other distressing symptoms commonly encountered in children with NI include: 3133

In addition, depression and anxiety may be experienced at an increased rate by children with a muscular dystrophy (MD) such as Duchenne MD.34 Unfortunately, few studies have explored symptom management in children with NI both during life and at the end of life.

General approach

This section will outline management of distressing symptoms for children with NI (Fig. 39-2). This allows the healthcare provider to consider interventions that may benefit several problems. This can be helpful because it is not always possible to determine which symptoms or problems are the primary source of distress and which ones are the secondary manifestations in these children. Is the nonverbal neurologically impaired child irritable and in distress because of spasticity or is the spasticity secondary to underlying pain? Are the signs and symptoms of dysautonomia caused by pain, mimic the appearance of pain, or do the associated problems of dysautonomia contribute to pain? Given these challenges, it is helpful to focus on all potential sources of distressing symptoms, including neuropathy. The focus of this section will be on nonverbal children with NI given the inherent challenge with this group.

image

Fig. 39-2 Multidimensional symptom assessment and management.

(These symptoms are sources of agitation, distress, and irritability in neurologically impaired children.)

There is regional and international variation in medication selection for the variety of problems and distressing symptoms encountered. Medications provided in the tables indicate mechanisms of action so that medications with similar properties available in different countries can be identified.

Assessment

The options for assessing presence and severity of pain include self-report, observational assessment of behaviors in nonverbal children, and assessment of physiological markers. Knowledge of the child’s cognitive level allows selection of a validated pain rating tool appropriate for the level of intellectual function such as The Poker Chip tool, the Oucher,35 and the Wong-Baker FACES pain rating scale36 for children at a cognitive level of 5 to 6 years.

Observational tools are available for individuals unable to report pain. Specific distress behaviors have been associated with pain and are very helpful in quantifying and monitoring pain in children unable to provide self-report. The reliability and validity of behavioral observations are highest when the pain is acute in nature, such as that associated with medical procedures. Behavioral measurement must be assessed in the context of sources of distress because it may be difficult to distinguish between pain behaviors and those resulting from other types of distress, such as hunger. Observational tools rely on assessing the following items:

Studies of physiologic measures including vital signs, diaphoresis, and hormone levels such as cortisol have predominantly focused on the pain associated with invasive and surgical procedures. None has proved to be reliable, sensitive, and specific for chronic pain. In addition, such measures may be altered by autonomic dysfunction in children with NI. Physiologic measures may have a role in monitoring pain when used to supplement more reliable information.

Assessment Tools

Observational pain assessment tools assist with identifying the presence of pain and monitoring improvement in pain when an intervention is introduced. (Box 39-1).3749 In a comparison of the revised-Face, Legs, Activity, Cry, Consolability (r-FLACC) tool, the Non-Communicating Children’s Pain Checklist-Postoperative Version (NCCPC-PV), and the Nursing Assessment of Pain Intensity (NAPI), the r-FLACC and NAPI were identified as having a higher overall clinical utility based on complexity, compatibility, and relative advantage.37 The r-FLACC was the tool most preferred by clinicians in terms of pragmatic qualities.

Evaluation

This section discusses the sources of pain in neurologically impaired children.

Neuropathic: Peripheral Neuropathy and Central Pain

Neuropathic pain conditions are those associated with injury, dysfunction, or altered excitability of portions of the peripheral, central, or autonomic nervous system. It can be caused by compression, transection, infiltration, ischemia, or metabolic injury. Common features of neuropathic pain conditions include: descriptors such as burning, shooting, electric, or tingling; motor findings of spasms, dystonia, and tremor; and autonomic disturbances of erythema, mottling, and increased sweating.50

There are many reasons to consider neuropathic pain in children with NI. Experience shows that a nocioceptive pain source may not be identified or pain may continue despite treatment of an identified source. “Screaming of unknown origin” was used to describe children with neurologic disorders, severe developmental delay, neurodegeneration, or severe motor impairments with persistent agitation, distress, or screaming, acknowledging that evaluation often does not identify a specific nociceptive cause.52 Pain in children with NI is typically thought to be nociceptive in origin; however, after repeated injury or surgery, neuropathic pain may also occur.53 In one case series, 6 children with cerebral palsy developed neuropathic pain following multilevel orthopedic surgery.54 Onset of symptoms ranged from 5 to 9 days. Interventions included gabapentin in 4 children, amitriptyline in 2, and transcutaneous electrical nerve stimulation for 5, with improvement in symptoms over variable periods of time. Although not reported, experience in nonverbal children with NI identifies development of crying spells increasing in intensity weeks to months following major surgery, such as for neuromuscular scoliosis. Medications used for neuropathic pain include opioids, tricyclic antidepressants such as nortriptyline and amitriptyline, and anticonvulsants such as gabapentin, pregabalin, carbamazepine, phenytoin, valproic acid, and lamotrigine.50

Visceral Hyperalgesia

The gastrointestinal tract is commonly identified by parents as a source of pain in neurologically impaired children. It has been identified as the most frequent source of all episodes of pain in children with severe cognitive impairment.27,55 Pain of unknown cause was the most intense, followed by pain attributed to the bowels, gastrointestinal tract, and digestive pain. In children with NI, significantly higher rates of pain were reported in those with a gastrostomy tube and those taking medications for feeding, gastroesophageal reflux, or gastrointestinal motility.28 This association between pain and gastrointestinal symptoms led to the hypothesis that some children with NI experience visceral hyperalgesia that benefits from gabapentin.56 This potential source of pain is reviewed in more detail in the section on retching and vomiting.

Management

This section outlines management strategies for pain and other common symptoms encountered in neurologically impaired children. Some symptoms are a result of the underlying impairment of the nervous system, may indicate a medical problem to treat, often benefit from interventions that target symptoms, and may be exacerbated by pain. Finally, symptoms such as vomiting and feeding intolerance that persist despite comprehensive evaluation, management of contributing problems, and use of symptom management strategies, may be informing us of an irreversible decline in the child’s overall neurological function and health status.

General Approach to Management of Distressing Symptoms in Nonverbal Children with NI

Good pain management starts with an assessment for treatable sources of pain, such as urinary tract infection, gastroesophageal reflux, and pancreatitis. For chronic sources of nociceptive pain, such as hip subluxation, pain management is guided by the principles of the World Health Organization (WHO) analgesic ladder.52,5961

In addition to assessment for nociceptive sources of pain, neuropathy should be considered for the reasons previously discussed. For persistent distress that suggests pain, nonverbal children with NI often benefit from a medication trial that targets pain syndromes such as neuropathic pain, visceral hyperalgesia, or central pain. Medications best studied for these sources of nerve pain include gabapentin and tricyclic antidepressants (TCAs) (Table 39-3). There are reasons to start with gabapentin, including a good safety profile and no interactions with other drugs. Gabapentin has been found to be safe in children at doses up to 78 mg/kg/day.62 Pharmacokinetics indicate that children under 5 years of age require the highest doses.63 Gabapentin is readily available in an oral solution. Pregabalin is an alternative option known to have higher oral bioavailability compared to gabapentin (90% vs. 33% to 66 %) with a linear increase in plasma concentration with increasing doses compared with gabapentin’s decreasing bioavailability at higher doses. At this time there is no data to indicate that this results in a greater clinical benefit for children.

TABLE 39-3 Medications for Neuropathy and Central Pain

Gabapentin

Nortriptyline

Adapted from Hauer JM. Respiratory symptom management in a child with severe neurologic impairment. J Palliat Med. 2007; 10 (5): 1201-1207.

Spasticity

Spasticity is an involuntary, velocity-dependent, increase to muscle tone that results in resistance to movement. Spinal and cortical injury can alter inhibitory and excitatory messages to the motor neuron, resulting in spasticity. Spasticity may be mistaken for seizure activity but is typically not as rhythmic or symmetrical. Factors that can exacerbate spasticity include acute illness, discomfort such as from constipation, and pain. For this reason, neuropathy may be a poorly recognized contributing factor to spasticity in children with NI.

Treatment is intended to reduce the excessive muscle tone, with the goal of improving a patient’s functional capacity and comfort. Various therapeutic interventions are available, including physical therapy, medications, and surgery. There are few randomized controlled trials of oral drugs, especially in children. There is weak evidence demonstrating the efficacy of medications such as benzodiazepines, baclofen, dantrolene, and α2-adrenergic agonists of clonidine and tizanidine.64,65 There have been only two trials comparing drugs, tizanidine vs. diazepam and tizanidine vs. baclofen, with no significant differences noted between medications in either trial.66,67 Adverse drug reactions are common, and highest with dantrolene (64% to 91%).64 Side effects commonly seen with all medications include sedation, drowsiness, and muscle weakness. Side effects tend to be dose related and disappear when doses are reduced. In patients with multiple sclerosis, evidence identifies benefits of treating spasticity with gabapentin without experiencing sedation.68 This may indicate benefit to children with demyelinating or other neurodegenerative conditions but warrants further study.

Typically, children with NI referred to palliative care are commonly on one of these medications or have an intrathecal baclofen pump. The role of palliative care clinicians can be to search for factors that can exacerbate spasticity, such as pain, and offer therapeutic symptom management trials, rather than increasing or adding other interventions for spasticity.

Autonomic Dysfunction

Autonomic dysfunction, also called dysautonomia, is common in children with NI. Other terms used include autonomic storm, sympathetic storm, paroxysmal sympathetic hyperactivity, and paroxysmal autonomic instability with dystonia. In children with NI, dysautonomia typically reflects hypothalamic impairment. Autonomic dysreflexia is used most commonly to describe autonomic dysfunction seen in patients with spinal cord injury above the splanchnic sympathetic outflow, which is at or above T6, though T6-T10 may be susceptible.

The autonomic nervous system regulates body temperature, respiratory rate, heart rate, blood pressure, intestinal motility, urination, salivation, perspiration, and pupillary size. Symptoms that are more commonly seen in children with NI and associated dysautonomia include: tachycardia or bradycardia; hypertension; hyperthermia, or hypothermia; skin changes including pallor, flushing and redness of the face, and/or body; vomiting, retching, bowel dysmotility, and constipation; urinary retention; abnormal sweating; increased salivation; posturing; and agitation.

As with other symptoms, evaluation should include a search for factors that may exacerbate the features of dysautonomia, including a review of potential sources of pain. Treatment for dysautonomia in neurologically impaired children has been poorly studied. Treatment options include benzodiazepines, bromocriptine, clonidine, oral and intrathecal baclofen, beta agonists such as metoprolol, and morphine sulfate. Literature is limited to case reports, predominantly in patients with traumatic brain injury. In a review of case reports of hypothalamic dysfunction, cyproheptadine was identified to benefit 4 individuals, including minimizing or eliminating symptoms of temperature instability, diaphoresis, vomiting, and abdominal pain.69 Other interventions used in patients in this review without benefit included antiepileptics, haloperidol, diazepam, and clonidine. A recent case series of 6 patients following traumatic brain injury identified improvement with gabapentin when symptoms of agitation, dysautonomia, and spasticity persisted despite treatment with bromocriptine, clonidine, ITB pump, metoprolol, morphine, and benzodiazepine.70 Finally, 13 out of 15 individuals with familial dysautonomia experienced a decrease in symptoms of nausea, retching, tachycardia, and flushing on pregabalin.71 Most patients were already using clonidine and a benzodiazepine. In children with NI, a therapeutic trial for pain should be considered because it is difficult to determine when features of dysautonomia are indicating underlying pain. In addition to scheduled medications, children with intermittent autonomic storms, often manifested by an acute onset of facial flushing, sweating, tachycardia, retching, agitation, and stiffening, may benefit from use of clonidine, diazepam, and morphine sulfate as needed during these episodes.

Medication Toxicities

Although uncommon, the features of serotonin syndrome and neuroleptic malignant syndrome include autonomic dysfunction and can be a result of medications used in this population. Awareness and monitoring for changes from a child’s baseline can alert us to these possible drug effects. Features of serotonin syndrome include tachycardia, hypertension, hyperthermia, diaphoresis, mydriasis, increased bowel sounds, hyperreflexia, clonus, agitation, and rigidity.72 Drugs commonly implicated include selective serotonin reuptake inhibitors (SSRIs). Other drugs reported, often when used in combination, include: trazadone, fentanyl, tramadol, risperidone, ondansetron, metoclopramide, and valproate. Management includes removal of causative medications, use of cyproheptadine as a 5HT2 antagonist, and supportive care for other associated problems. Features of neuroleptic malignant syndrome are similar and include muscle rigidity, autonomic dysfunction, and altered mental status, most commonly caused by dopamine antagonists such as haloperidol. It has also been associated with tricyclic antidepressants and some anticonvulsants.73

Abnormal Movements: Dystonia and Posturing

Dystonia is a movement disorder that causes the muscles to contract and spasm involuntarily resulting in twisting, repetitive movements and abnormal postures. Dystonia may be a primary disorder of movement, it may be secondary to diseases and insults that injure the central nervous system, or it may be a result of medications such as neuroleptics. Interventions include physical therapy, medications, and botulinum toxin for focal muscle groups. There have been few studies evaluating the effectiveness of medications for secondary dystonia. Those considered include benzodiazepines, baclofen, dopaminergic agents such as bromocriptine and levodopa (Sinemet), and anticholinergics such as trihexyphenidyl (Artane). Palliative care physicians can assist by assessing for distressing symptoms such as pain that can worsen the movement disorder.

Posturing is a term used to describe both findings of abnormal position, such as arching of the back, and clinical exam findings, such as decorticate, decerebrate, and opisthotonos. Lack of nomenclature, assessment tools, and the variability of neurologic impairment results in a variation of assessment, should the movement be labeled dystonia, posturing, or autonomic storm, and approaches to management, as evident by the large number of medications for consideration. Again, palliative care teams can assist by bringing a comprehensive approach to assessment including a thorough consideration of neuropathy as a source of symptoms.

In addition, clinical findings of posturing can be informative as pre-terminal signs of progressive injury to the central nervous system in children with neurodegenerative conditions, indicating loss of inhibitory control. These findings can include: decorticate posturing as indicated by rigid extension of the arms and legs, downward pointing of the toes, and backward arching of the head; decerebrate posturing with rigidity, flexion of the arms, clenched fists, and extended legs; and opisthotonos with rigidity and severe arching of the back, with the head thrown backward. These can also be intermittent findings in children with severe injury of the CNS.

Sleep Disturbance

Treating sources of distress will often improve sleep in children with NI. Pharmacologic treatments to consider include melatonin,79,80 antidepressants such as tricyclic antidepressants and trazodone,8183 clonidine,84 and antipsychotics, especially for patients with delirium.85 When using melatonin for children with NI, some advocate that as the severity of disability increases, higher doses, occasionally even up to 15 mg, may be beneficial.80 Benzodiazepines tend to be overused, leading to dependency and tolerance, and should only be used in a time-limited manner and discontinued or weaned following short-term use.86 Nonpharmacologic interventions include a consistent bedtime and routine, quiet, a dark environment, and stimulus control.85 When sleep problems are linked to symptoms such as pain or dyspnea, or correlated with an underlying problem such as obstructive apnea, treatment of the underlying condition may improve sleep.

Respiratory Health in Children with NI

Children with severe neurologic impairment have a high incidence of respiratory problems.88 Recurrent respiratory illness leading to respiratory failure is the most common cause of mortality in children with severe cerebral palsy.10,11 Aspiration is a frequent factor, identified in 31% to 68% of children with cerebral palsy.8991 Aspiration pneumonia in neurologically impaired children is best understood as an acute exacerbation resulting from chronic contributing factors. Those factors include:

In children with severe NI and recurrent respiratory illnesses, it is misleading to parents to label respiratory exacerbations as pneumonia or aspiration pneumonia because this can give the impression of a reversible problem. Instead, it can be helpful to acknowledge that the goal of chronic and acute treatment is to minimize the effect of these factors and maximize recovery and maintenance of health while we prepare for diminishing benefit.

Other factors that are considered to contribute to a decline in respiratory function include gastroesophageal reflux disease (GERD) and neuromuscular (NM) scoliosis. Evidence for the benefit of interventions is unfortunately limited, but includes:

Although the improvement in life expectancy for the most medically fragile children with cerebral palsy is believed to be a result of gastrostomy feeding tubes,92 there are no studies to guide which children benefit.93 Gastrostomy feeding tubes have not been demonstrated to prevent aspiration pneumonia in adults with advanced dementia.94
Anti-reflux surgery does not routinely alter respiratory symptoms or the frequency of pneumonia, though it is often offered for this purpose.9599 As outlined previously, other factors not altered by anti-reflux surgery contribute to respiratory problems. In addition, it can result in or worsen pre-existing retching, a distressing symptom seen in children with NI.100
A review of the indications for tracheotomy identified a mortality of 27% in neurologically impaired patients compared with an 11% or less mortality for conditions of airway obstruction. The review found mortality from upper airway obstruction to be 11%, vocal fold paralysis 8%, and no deaths from craniofacial abnormality.101 This likely reflects greater benefit for airway obstruction vs. less benefit for children with NI and respiratory compromise from multiple factors, an observation further supported by a recent retrospective review of outcomes following tracheotomy.102
The outcome following spinal surgery for neuromuscular scoliosis in 288 children with cerebral palsy was reviewed.105 The average age at time of surgery was 13 years 11 months, with a standard deviation of 3 years 4 months. Mortality included three perioperative deaths, a mean survival of 4.3 years for the 33 other deaths, and a mean estimated survival of 11.2 years for all patients. Missing from this analysis was a stratification of patients based on pulmonary status before surgery and a review of anticipated survival without surgery so as to account for differences in children with multiple factors impacting respiratory health vs. children with restrictive lung disease with no other risk.

Dyspnea

Dyspnea is the experience of shortness of breath, difficulty breathing, or uncomfortable breathing. It is a common symptom of numerous medical disorders. Included are diseases of the lung parenchyma and obstruction of the airway, which can occur from repeated micro-aspiration with resulting infection, inflammation, bronchiectasis, and lung scarring over time.

Measures of respiratory rate, oxygen saturation, blood gas levels, and family perception do not necessarily correlate with the patient’s perception of breathlessness. Instruments such as the Respiratory Distress Observational Scale (RDOS) are being evaluated for use in adults unable to report about dyspnea.106 When assisting parents in assessing for dyspnea in a nonverbal child, strategies are similar to those used in pain assessment of such children. Assessment can include asking a parent if he or she has observed the child to be in distress or appear anxious during a respiratory exacerbation. Behaviors indicating distress may include facial expression, appearing restlessness, or becoming withdrawn. It can also be helpful to instruct parents and care providers to breathe along with the child for 1 minute as an indirect indicator of the child’s effort of breathing.

Treating dyspnea is typically focused on identifying and aggressively treating the underlying cause. Evidence is established in adults for using interventions to alleviate dyspnea that persists despite maximum medical management of identified causes. These interventions include an oxygen trial, cool air from a fan or open window, repositioning, lorezepam for associated anxiety, and morphine sulfate. A recent review from the American College of Physicians identified strong evidence for treating adults with dyspnea from chronic lung disease with short-term opioids.107,108 Evidence includes demonstration of significant improvement in refractory dyspnea in participants completing a randomized, double blind, placebo-controlled crossover study with no significant episodes of sedation.109 Several studies have demonstrated the safety of using morphine sulfate for management of dyspnea that occurs despite maximum treatment of the underlying cause without development of respiratory depression.110112 A suggested starting dose for an opioid naive patient is 25% to 30% of the dose used for pain with a maximum starting dose of 5 mg orally. If the patient is already on an opioid, increase the dose by 30%.

As a decline in respiratory status occurs despite management of these contributing factors, respiratory distress during exacerbations is likely an under-recognized symptom in children with NI. Using evidence for managing dyspnea in adults, children deserve symptom management of respiratory distress incorporated into medical care plans (Table 39-4). 113 For example, the table identifies antibiotics that provide coverage of anaerobic bacteria along with other oral bacteria when treating children who chronically aspirate oral secretions.114118 Morphine sulfate is included in Table 39-4 to remind us to ask if there is distress during respiratory exacerbations and to include that information in care plans when distress is identified. This allows integration of symptom management into acute medical care plans in advance of seeing limited benefit from chronic and acute medical interventions. Overall, this will assist with future decisions by assuring that comfort is part of the care plan and avoid decisions to use interventions offered being made solely out of fear of suffering.

TABLE 39-4 Respiratory Home Management: Medical and Symptom Treatment Strategies

Chronic interventions
Suctioning As needed for comfort
Oxygen Assessed by appearance of patient or by oximeter
Albuterol nebulizer Every 3-4 hrs for coughing, wheezing, congestion
Ipratropium (Atrovent) nebulizer Every 3-4 hrs for coughing, wheezing, congestion
Saline or Mucomyst nebulizer As needed for thick secretions
Chest physiotherapy or vest 2 times/day, increase to 4 times/day with increased symptoms*
Nebulized budesonide (Pulmicort) 2 times/day, increase to 4 times/day with increased symptoms*
Salmeterol (Serevent) Family history of allergies or benefit from daily albuterol
Acute interventions
For respiratory exacerbations from chronic aspiration
Clindamycin, Augmentin or Levofloxacin/Moxifloxacin 10-14 days
Systemic steroids (prednisone) 5 days
Additional interventions
For symptom management and end-of-life care
Fan on face Relieves sensation of breathlessness
Morphine sulfate

Glycopyrrolate (Robinul), scopalamine, hyoscyamine

* Symptoms include increased coughing, secretions, congestion, respiratory rate and breathing effort.

Use when respiratory symptoms persist or worsen despite an increase in chronic interventions.

Include with third or fourth exacerbation, sooner if symptoms return within two months of antibiotic course.

§ Features suggesting respiratory distress include facial expression such as grimacing, appearing restlessness, having an anxious look, stiffening, tears, or becoming withdrawn.

Adapted from Hauer JM. Respiratory symptom management in a child with severe neurologic impairment. J Palliat Med 2007; 10(5):1201-1207. Table 39-1. Reprinted with permission from Journal of Palliative Medicine.

Leo was extubated 1 week later, before the next team meeting. The family had discussed what they would want for Leo when the next respiratory illness occurred. They believed they understood what medical interventions in the hospital could and could not do for Leo. A home care plan was outlined and hospice introduced to help assist with the family’s goals of maintaining Leo’s comfort and care at home. The home care plan included medical interventions to initiate during acute respiratory exacerbations, including several rotating antibiotics and a short course of prednisone. The goal was to use such acute interventions as long as they met the goal of improving Leo’s respiratory health status during times of decline without resulting in complications. There was understanding that the benefit of these interventions would lessen over time as they do not eliminate the factors contributing to respiratory decline. The care plan also incorporated symptom management with morphine sulfate. A detailed review identified specific symptoms that indicate when Leo is in distress.

During the next respiratory exacerbation, morphine sulfate was used along with the home treatment plan of an antibiotic and prednisone. Leo’s care providers were pleased with how much more comfortable Leo appeared during this illness. They were relieved that they did not need to choose between treatment for medical benefit and treatment for comfort. They are more certain with their goals for Leo and feel prepared for the time when the home treatment plan may no longer benefit him, but are certain they can maintain his comfort throughout life with guidance in dose adjustment as needed.

For such reasons, we likely underuse morphine sulfate when managing respiratory distress, instead reserving its use for comfort at the end of life. Integrating symptom management earlier can minimize interventions being pursued out of fear of the patient suffering without the intervention. There is a need to study the integration of morphine sulfate into the care plans of neurologically impaired children with recurrent respiratory exacerbations. Waiting until the goal is comfort care rather than treatment focused delays initiation of comfort strategies.

Secretions

Secretions occur for various reasons in children with NI. It is helpful to consider sialorrhea separately from thicker respiratory secretions. Sialorrhea involves thin, watery secretions that can pool out of the mouth as a result of diminished oral sensation of saliva. There is ample evidence of the benefit of medications such as glycopyrrolate and botox or surgical ligation of the salivary ducts in diminishing sialorrhea. In contrast, an increase in respiratory secretions in such children is likely due to multiple factors, including an increase in pulmonary secretions as a result of inflammation from aspiration of oral secretions and mucous that is difficult to mobilize. Management is directed at minimizing the development of such secretions through use of inhaled steroids and assistance with clearance through bronchial drainage techniques and devices such as the pulmonary vest and cough assist. Medications are available to decrease secretions but should be used with caution in this population because they can result in thicker secretions that are more difficult to mobilize. They may have a more beneficial role when comfort is the primary goal of care. Medications with anticholinergic properties that have been used for management of secretions at the end-of-life include scopolamine, hyoscyamine, and atropine, tertiary amines that may cause sedation and delirium as they cross the blood brain barrier. Glycopyrrolate is another option, which may be less sedating. No evidence exists indicating that one is more beneficial than another. Decreasing total fluid intake by 25% or more is another simple intervention to consider for management of excessive respiratory secretions.

Nausea, Retching, Vomiting, and Feeding Intolerance

Gastrointestinal symptoms are common in children with severe NI.2729 This includes pain localized to the gut as noted in the pain section. Vomiting in these children is commonly attributed to GERD.119 Alternatively, stimulation of the emetic reflex is likely an under-reported source of symptoms in these patients.100,120 Experience and limited evidence suggests the benefit of considering sources beyond GERD. Considerations include visceral hyperalgesia, activation of the emetic reflex, and dysautonomia (Table 39-5).

TABLE 39-5 Medications for Retching, Vomiting, Feeding Intolerance in Children with NI

Activation of the emetic reflex
Medication Receptor antagonist Dose
Ondansetron (Zofran) 5HT3 0.15 mg/kg PO/IV q 8 hr prn (maximum 8 mg)
Metoclopramide (Reglan) D2—GI tract 0.1-0.2 mg/kg PO/IV q 6 hr (maximum 10 mg)
Haloperidol (Haldol) D2—CTZ, H1 & Ach 0.01-0.02 mg/kg PO q 8 hr prn (maximum 1 mg)
Promethazine (Phenergan) H1 & Ach (weak D2) 0.25-0.5 mg/kg PO/IV q 4 hr prn (maximum 25 mg)
Diphenhydramine (Benedryl) H1 0.5-1 mg/kg PO/IV q 6 hr prn (maximum 50 mg)
Cyproheptadine (Periactin) 5HT2, H1 & Ach

Ach
Visceral hyeralgesia
Medication Mechanism of action Dose
Gabapentin Thought to inhibit excitation by binding to the alpha-2-delta subunit of voltage dependent Ca ion channels in the central nervous system See Table 39-3
Nortriptyline Presynaptic reuptake inhibition in the CNS of norepinephrine and serotonin, both inhibitors of pain transmission See Table 39-3
Dysautonomia
Medication Mechanism of action Dose
Clonidine Centrally acting α2-adrenergic receptor agonist, reducing sympathetic outflow
Cyproheptadine See above See above
Gabapentin See above See above
Morphine sulfate Binds CNS opioid receptors 0.3 mg/kg PO/SL q 3-4 hr prn “autonomic storm”

Pathways that result in nausea and vomiting

An understanding of the pathways, receptors, and neurotransmitters involved in generating nausea, vomiting, and retching is critical to identifying treatment that can alleviate these symptoms.121123 The final common pathway resulting in nausea and vomiting is the vomiting center (VC) located in the medulla. The VC is triggered by numerous inputs, including the chemoreceptor trigger zone, cortical inputs, meningeal and ventricular mechanoreceptors, vestibular input, and vagal and glossopharyngeal input.

Visceral Hyperalgesia as a Source of Vomiting, Retching, and Feeding Intolerance

Visceral hyperalgesia is an altered response to visceral stimulation, resulting in a decreased activation threshold for pain in response to a stimulus, such as intraluminal pressure.124 In a case series of 14 medically fragile children with continued retching and vomiting despite maximal medical treatment and Nissen fundoplication, visceral hyperalgesia was identified in 12 children as a source of symptoms.125 Tricyclic antidepressants, gabapentin, cyproheptadine, and dicyclomine were used in various combinations depending on evaluation results, with 11 children reported as “better” or “much better” and a decrease in the mean number of retching episodes per day from 14 to 1.5. A case series of nine neurologically impaired children with symptoms indicating pain, feeding intolerance, and disrupted sleep, identified significant improvement following use of gabapentin titrated to standard doses.56 This suggests that gastrointestinal and pain symptoms may improve with medical management directed toward visceral hyperalgesia when symptoms persist despite management of the more commonly recognized gastrointestinal problems. One report speculates that repeated painful gastrointestinal experiences during infancy contributes to sensitization of visceral afferent pathways.125 It is interesting to note the higher incidence of pain in children with a gastrostomy feeding tube and taking medications for GERD.28 Children with NI have an increased frequency of such sensitizing experiences, including GERD, constipation, gastrostomy tube placement, and fundoplication.119,126

Information that suggests visceral hyperalgesia in nonverbal children with NI includes:

Retching was identified to benefit from alimemazine, a phenothiazine derivative structurally related to such medication as chlorpromazine.127 As with other phenothiazine derivatives, various properties may account for this noted benefit with retching, including antihistamine, anticholinergic, antidopinergic, and antiserotonergic. Though alimemazine is not available in the United States, cyproheptadine and other medications that block the receptors that trigger nausea and vomiting may benefit children with NI and retching.

As identified earlier, children with vomiting and symptoms of retching, tachycardia, sweating, or flushing indicating dysautonomia, retching, forceful vomiting, pallor, or sweating indicating activation of the emetic reflex, or pain indicating visceral hyperalgesia likely do not benefit from anti-reflux surgery.100,120 Esophagogastric disconnection is another surgical intervention that has been proposed for persistent GERD. In two case series reviewing the use of esophagogastric disconnection, morbidity (30% to 43% early complications, 41% to 43% late complications) and mortality (22% to 29%) were high.128,129 Though used for GERD, patients were also noted to have symptoms of retching, likely indicating a process distinct from GERD. It is essential that medical and symptom management be maximized before considering this surgical intervention and that any surgical intervention for GERD be considered cautiously in neurologically impaired children with retching (see Table 39-5).

Intestinal pseudo-obstruction

Intestinal pseudo-obstruction, also referred to as Ogilvie syndrome, is a clinical picture that suggests mechanical obstruction, but in the absence of any evidence of any obstruction in the intestine. Patients at risk include children with mitochondrial disorders and conditions with significant autonomic dysfunction, such as autonomic dyreflexia following spinal cord injury. Children with severe NI may be at risk for this clinical picture in the post-operative period with a clinical picture of prolonged ileus.130 Neostigmine has been reported to benefit some patients with acute intestinal pseudo-obstruction, when it fails to resolve with supportive therapy and management of contributing factors.131 It must be used with caution, given the potential side effects, including bradycardia, hypotension, increased pain, increased airway secretions and bronchial reactivity. A test dose of 0.01–0.02 mg/kg/dose intravenously can be given in the hospital to allow monitoring, titrated up to 0.08 mg/kg/dose if needed. The oral equivalent is approximately 15 mg oral to 0.5 mg intravenous. Glycopyrrolate has been suggested in conjunction with neostigmine to minimize effects of bradycardia. Children with significant recurrent episodes, as seen with mitochondrial myopathies, may benefit from as needed neostigmine once a dose has been established, though this warrants further study. Other medications that have been considered include 5-HT4 receptor agonists such as tegaserod and somatostatin analogues such as octreotide.

On further review, Tammy was experiencing daily vomiting and retching for 6 months and weekly crying for 2 months. Crying spells range from 2 hours to most of the day with crying averaging 60% of each day. Other symptoms noted at times of distress include sweating, arching, stiffening, and facial flushing. Reflective questions identified that “she has been suffering for over 6 months and is currently suffering every day.” Tammy is described as “very social but now unable to enjoy the activities she did before.” Visceral hyperalgesia and the role of the nervous system in regulating function of the intestines were discussed in detail. A symptom management plan was outlined: increase gabapentin that was being used for seizure management, initiate cyproheptadine, use morphine sulfate 0.3 mg/kg/dose by G-tube or sublingual as often as every 1 hour, and decrease formula feedings by 25%.

The primary focus of the discussion was Tammy’s comfort and the hope for improvement with these interventions. The concept of the G-tube as a life-sustaining technology was introduced with acknowledgment that it is permissible to discontinue any technology that is prolonging suffering. This was approached as an opportunity to inform without any need for a decision. There was “significant improvement” noted within 5 days, described as “the best she has looked in the past year.” This included a significant reduction in crying spells, vomiting, retching, and an increase in energy and interaction with her family. Parents were delighted; they hope for continued benefit, but are also prepared for a return of symptoms.

Persistent Feeding Intolerance

Feeding intolerance can be a source of distress for children with severe NI who are receiving fluid and nutrition through a feeding tube. As with recurrent respiratory exacerbations as a result of chronic pulmonary aspiration, the initial benefit from treatment interventions may lessen over time. At such times it can be beneficial to integrate symptom management strategies into medical care plans.

Management includes treating contributing problems such as constipation. As reviewed previously, some children benefit from an empiric trial of gabapentin or nortriptyline for visceral hyperalgesia, cyproheptadine for retching, and ondansetron, or hyoscyamine for vomiting, being mindful of which medication targets which receptor so as to avoid duplication. Because there is limited evidence to guide in such patients, one must be careful from introducing too many trial options. Other intervention strategies include an empiric trial of an elemental formula, an empiric trial of metronidazole or rifaximine for small bowel bacterial overgrowth in children with persistent diarrhea, and a trial of J-tube feedings. Experience shows that J-tube feedings often do not improve pain associated with vomiting. This may reflect irreversible changes in the nervous system with resulting autonomic dysfunction and visceral hyperalgesia.

Some of these children will have persistent problems despite using these options. At such times, some children will benefit from a decrease by 25% or more in the total amount of nutrition and fluids provided by feeding tube. Families benefit at such times from a reflection on primary goals and determining approaches that meet these goals such as feeding to comfort rather than feeding to a required amount, and withholding feedings that are resulting in pain and discomfort.

Medical Nutrition and Hydration

Forgoing medical nutrition and hydration remains one of the more difficult areas given the symbolic significance of nutrition, the myths about dehydration and starvation, and under-recognition of the complications of artificial hydration and nutrition.132 The American Academy of Pediatrics policy statement recognizes that “Life-sustaining medical treatment encompasses all interventions that may prolong the life of patients. …includes less technically demanding measures such as antibiotics, insulin, chemotherapy, and nutrition and hydration provided intravenously or by tube.”133 It is permissible to discontinue medical nutrition and hydration when it is prolonging or contributing to suffering.134137

Children with NI receiving medical nutrition and fluids through a feeding tube are vulnerable to complications as health continues to decline. This can include progressive feeding intolerance, development of edema, and increasing oral secretions. It is important to combine knowledge of reversible sources of these problems with an understanding of features that are part of progressive, irreversible decline. This can be challenging in children with NI who live chronically with impairment that is considered static and who often experience decline over a long period, increasing the likelihood of aggressive evaluation and treatment at the end of life. It is helpful to recognize that even children with static conditions experience irreversible decline in organ function, likely a result of ongoing apoptosis in the setting of baseline impairment of the nervous system. This may account for why some children with NI develop feeding intolerance later in life that is not amenable to medical interventions.

It can be helpful to recognize with parents the years of benefit provided by a feeding tube as we consider development of harm from this intervention. This is a time to inform without expecting a decision; educating on the role of feeding tubes as life-sustaining technology in the same sense as a ventilator and the benefit of considering when technology is becoming more burdensome, even when that technology has been routine and is easy to use. The previous sections are a critical part of this process, by creating a context for understanding the goals of the child and family and through knowledge of the child’s health trajectory. It is helpful to estimate for families the length of time that may pass following discontinuation of medical nutrition and hydration until death occurs, often 10 to 14 days, but can be much longer by several weeks when small amounts of fluid are used to flush a feeding tube after medications have been given.

End-of-Life Care

Preparing and supporting families through care at end of life

Preparing families can be difficult yet tremendously beneficial. Preparation includes: anticipating symptom management needs, reviewing how the child might die, discussing preferred location of death, reviewing physical changes in the child at the end of life, determining who to contact to assist throughout this process, and discussing autopsy or organ and tissue donation.

Hospice and palliative care teams are well suited to support children, parents, and siblings throughout this process. The interdisciplinary team can address the multitude of needs, such as child life specialists providing support for siblings, chaplains exploring spiritual needs in the context of a family’s faith community, and social workers addressing emotional and home care support needs. Clinicians can attend to symptoms by creating tailored care plans that use parental input, such as this example for a child with CP and progressive respiratory failure:

Bereavement

Bereavement often starts before the child’s death. Support from palliative care teams can link families to assistance from extended family, friends, or community providers, including faith communities and grief counselors, as families prepare to transition from the specialized healthcare system. Bereavement care also guides the family through the legal requirements and decisions for the care and relinquishment of the body.

Unique areas of bereavement are seen in parents of children with NI. The limited research has not found differences on measures of grief between parents of children with developmental impairment and other parents.140 Themes identified that are unique to parents of children with NI include: working through two difficult transitions, first when the child was diagnosed with NI and second when the child died; a need to justify their love of their child to others; parents’ lack of opportunity to share their grief, resulting in a socially imposed silent grief; loss of an intensive caregiver role and support group membership; conflicting feelings of sadness; and relief over prior fears about the child’s future. Bereavement is an essential part of supportive care with importance in attending to the experiences unique to families of children with NI.

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