Neurologic Diseases

Published on 09/04/2015 by admin

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Last modified 09/04/2015

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39 Neurologic Diseases

Julie Hauer, Helen Wells O’Brien

None of us is as smart as all of us.

—Japanese proverb

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 disability ³ 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:

• Static, such as hypoxic injury or structural malformations of the central nervous system,

• Progressive, including metabolic diseases and muscular dystrophies,

• Primary impairment of the nervous system, such as spinal muscular atrophy and in utero infections,

• Associated impairment, such as genetic and congenital syndromes,

• Involving the central nervous system, including metabolic diseases,

• Involving the lower motor neuron, such as muscular dystrophies, spinal muscular atrophy, and congenital myopathies.

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	Indicated by developmental label Cerebral palsy A result of an insult to the CNS Hypoxic ischemic encephalopathy In-utero infection with cytomegalovirus (CMV) Anoxic encephalopathy Associated with a specific diagnosis Genetic disorder Congenital anomaly Structural brain malformation, Mitochondrial diseases Metabolic disorders, inborn errors of metabolism

Prenatal consultation for conditions identified during pregnancy

Recurrent illness, hospitalizations, symptoms, or decline in health and function, such as respiratory exacerbations, feeding intolerance, recurrent or chronic irritability and/or pain

Consideration of the following interventions: CPAP/BiPAP, tracheotomy, tracheal diversion, anti-reflux surgery for prevention of aspiration pneumonia, esophagogastric disconnection

Intensive long-term treatment aimed at maintaining the child’s quality of life

Progressive respiratory failure

Muscular dystrophy

Spinal muscular atrophy (SMA)

At diagnosis (for conditions that progress early in life such as SMA type I)

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

Introduction of CPAP/BiPAP

Curative treatment intended but may fail

Metabolic diseases

Leukodystrophies

Mucopolysaccharidoses

Other lysosomal storage disorders

Consideration of stem cell transplant

Patients enrolling in clinical trials for new therapies such as enzyme replacement

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.

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.

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. Studies ^13,¹⁴ 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

Fig. 39-1 Prognosis and uncertainty—hope for the best, prepare for the rest.

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 success ²⁰ 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:

• Please share with us what the past few months or years have been like and what you understand about your child’s health status.

• What are you worrying about?

• What are you anticipating?

• What are your hopes for your child?

• What do you consider most important for your child as your consider decisions?

Reflective questions with parents and care providers:

• How does a typical day for your child look now compared to the start of these problems and/or symptoms?

• When did you first start worrying about these problems and/or symptoms?

• How does your child’s health compare this month to the start of these problems?

• Where would you estimate your child’s health baseline to be now compared with 2 years ago?

• Is your child’s health baseline 75%, 50%, or less than 50% of where it was? Has your child’s health declined by 25%, 50%, or more than 50%?

• How often in a month is your child sick now compared to 12 months ago? Has illness been increasing in frequency?

• What percentage of time in a typical week would you estimate that your child enjoys now compared to 12 months ago? Has this declined over time?

• Are you seeing less benefit from chronic and acute treatments over time with less return to prior baseline, longer duration with each illness, or a shorter time between each illness?

• How much reserve do you estimate for your child?

• Do you worry that your child is suffering?

• What percentage of each day or week is there suffering?

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

• What is the likelihood of the identified interventions bringing an improvement or recovery of the current health and functional baseline? To meeting the goals identified such as improving comfort and quality of life? To prolonging the suffering identified?

• How will the course look with or without the treatment available?

• Are there treatment strategies for the symptoms that are causing distress?

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:

• Define with the family the goals that will guide decisions: A decrease in distressing symptoms? A health improvement? Maintenance of health to a specific event?

• Assess the likelihood of an intervention meeting these goals.

• Know the evidence for the possible benefit and harm for the interventions available.

• Define a time period in which the intervention would be expected to meet a goal.

• Anticipate and discuss if the hoped-for benefit does not occur in this time period, if the benefit is later lost, or if increased suffering occurs. This allows end points to be anticipated before a crisis.

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:

• Children who are nonambulatory throughout life have a high incidence of osteoporosis, resulting in a high risk that chest compressions could cause fractures.

• Many children with NI, excluding such conditions as Duchenne muscular dystrophy (DMD) with associated cardiomyopathy and congenital syndromes with associated cardiac defects, experience cardiac failure as a result of respiratory failure; focusing decisions on the primary life-threatening event will guide subsequent decisions about cardiac interventions. That includes informing families that there would be no benefit to cardiac interventions when a patient is not intubated, thus eliminating the need for parents to make unnecessary decisions.

• Bagging can become a chronic intervention by default; if it is being considered when intubation will not be used it can be useful to review its historic use as a bridge to intubation.

• Some hospital forms include interventions beyond intubation, such as suctioning, nasal or oral airway, oxygen, and continuous positive end expiratory pressure (CPAP) and/or biphasic positive airway pressure (BiPAP). It is helpful to guide parents to care plans that fit with the child’s current use of oxygen and suctioning; and it can be helpful to discuss the benefit and burden of CPAP and/or BiPAP in a similar manner to intubation, that is, the difference between acute and chronic use.

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

• Neurologic, such as spasticity, seizures, and autonomic dysfunction,

• Gastrointestinal, such as vomiting, retching, constipation, and feeding intolerance,

• Respiratory, such as secretions and dyspnea.

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.

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:

• Vocalizations, such as crying and moaning

• Facial expression, including grimacing

• Consolability

• Interactivity, such as being withdrawn

• Mood

• Eating and sleeping

• Protective actions, such as guarding part of the body

• Movement

• Tone and posture, such as arching and stiffening

• Physiological measures

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