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.
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 |
Recurrent illness, hospitalizations, symptoms, or decline in health and function, such as respiratory exacerbations, feeding intolerance, recurrent or chronic irritability and/or pain
| |
| Intensive long-term treatment aimed at maintaining the child’s quality of life |
Consideration of gastrostomy feeding tube placement. This is an opportunity to identify goals of care that will then be relevant to future decision making such as use of mechanical ventilation
| |
| 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.
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.
Leo is a 6-year-old boy with cerebral palsy and intellectual impairment as a result of hypoxic-ischemic encephalopathy. He is nonverbal and nonambulatory, and receives fluid and nutrition by a gastrostomy feeding tube. Associated problems include neuromuscular scoliosis and recurrent pulmonary illnesses. He has experienced an increased frequency of respiratory exacerbations during the past 6 months with two recent hospitalizations. His daily chronic treatment includes: nebulizer treatments with a daily inhaled steroid, and albuterol and atrovent as needed; chest physiotherapy and vest for bronchial drainage; and supplemental home oxygen as needed. He is cared for lovingly by his adoptive parents and his devoted home care nurses. They have always anticipated that Leo would not live into adulthood. They celebrate their time together and describe Leo as a boy who enjoys cuddling, music, being around his siblings and being part of family activities.
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.7–12 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.
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.
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.
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.
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.
Care in the school
Children with NI routinely attend school as assured by the Individuals with Disabilities Education Act (IDEA). Along with educational and socialization opportunities, this has meant that school systems may have children with chronic healthcare needs. For children with a significant compromise to baseline health, this creates the challenge of determining what interventions and monitoring is possible in the school. For children in the community with Do Not Attempt Resuscitation (DNAR) or Allow Natural Death orders, it is necessary to determine state requirements and school policy. This includes meeting with school nurses, teachers, and administrators to develop a plan of care in the event of a life-threatening event. Members of the palliative care team, such as social workers and child life specialists, can work with school systems to assist with the needs of the other children in the classroom. This may involve preparing students prior to a child’s return to school if changes have occurred, or assisting following the death of a child.
Practical considerations: guardianship and long-term financial plans
Financial issues are a practical concern for families of children with NI. This may be a result of reduced work hours or discontinuing work to stay home and provide care for their child. In addition, overall expenses are often greater for families of children with chronic health care needs. Many families will benefit from assistance with the legal and financial areas of special-needs trusts and guardianship. Government programs can cover some basic medical needs, but for families creating a fund in their child’s name for long-term needs, the child will lose eligibility if there is more than $2000 in assets. A special-needs trust is the process that provides financial protection of assets for lifetime care needs and preserves eligibility for Medicaid, Social Security income, and other need-based benefit programs for those with disabilities. For any individual older than 18 years of age unable to participate in decisionmaking, legal guardianship is the court process of documenting the legal decision maker.
Symptom Management
Children with NI experience pain more frequently than the general pediatric population.26–29 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: 31–33
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.
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.
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).37–49 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.
BOX 39-1 Pain Assessment Tools for Non-Verbal Neurologically Impaired Children
The Non-Communicating Children’s Pain Checklist-Revised (NCCPC-R)40,41
30-item standardized pain assessment tool for children with severe cognitive impairment.
Validated to be related to pain intensity ratings provided by caregivers, consistent over time, sensitive and specific to pain, and effective for different levels of cognitive impairment.
Score of 7/90 or above provided 0.84 sensitivity and 0.77 specificity for classifying moderate-severe pain
Paediatric Pain Profile (PPP)42,43
Sensitivity (1.0) and specificity (0.91) optimized at a cut-off of 14/60
Available to download from the web following registration at www.ppprofile.org.uk.
Evaluation
This section discusses the sources of pain in neurologically impaired children.
Nocioceptive: Tissue Injury and Inflammation
Commonly recognized pain sources in children with NI include acute sources, such as fracture, urinary tract infection, or pancreatitis;51 and chronic sources, such as gastroesophageal reflex (GER), constipation, feeding difficulties from delayed gut motility, positioning, spasticity, hip pain, or dental pain. (Box 39-2).
BOX 39-2 Etiology of Pain/Irritability in Nonverbal Neurologically Impaired Children
Head, Eyes, Ears, Nose, Throat (HEENT)
Acute otitis media, pharyngitis, sinusitis, dental abscess and/or gingival inflammation, corneal abrasion, glaucoma, ventriculoperitoneal shunt malfunction
Abdomen
Gastrointestinal: gastroesophageal reflux disease, gastritis and/or gastric ulcer, peptic ulcer disease, food allergy, appendicitis, intussusception, constipation, delayed and/or impaired motility, rectal fissure, visceral hyperalgesia
Liver and/or gallbladder: hepatitis, cholecystitis
Renal: urinary tract infection, nephrolithiasis, neuropathic bladder, obstructive uropathy
Genitourinary: inguinal hernia, testicular torsion, ovarian torsion and/or cyst, menstrual cramps
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
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