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.

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 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

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.

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:

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.

Evaluation

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

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