The Impact of Caring for Children With Severe Neurological Impairment on Clinicians.

Clinicians face many challenges in caring for children with severe neurological impairment (SNI). This study aimed to understand expert clinician perspectives on the personal impact of caring for children with SNI to highlight the challenges and potential solutions by underscoring the aspects of care that can be sustaining. Twenty-five clinicians participated including physicians (n = 8, 32%), social workers (n = 5, 20%), nurses/nurse practitioners (n = 5, 20%), case managers (n = 3, 12%), developmental therapists (n = 2, 8%), and other psychosocial clinicians (n = 2, 8%). Clinicians represented a variety of specialties including complex care/hospitalists (n = 10, 19%), palliative care (n = 7, 13%), and critical care (n = 6, 12%). Thematic content analysis revealed 3 major themes: (1) worries and challenges; (2) positive impact from being present; and (3) personal growth and meaning-making. Many clinicians described the ways listening, examining biases, learning about family perspectives, and normalizing the need for emotional processing helped them to understand their patients, families, and themselves more deeply.

Forming Clinician-Parent Therapeutic Alliance for Children With Severe Neurologic Impairment.

OBJECTIVES: Care for children with severe neurologic impairment (SNI) often involves complex medical decision-making where therapeutic alliance between clinicians and families is essential. Yet, existing data suggest that communication and alliance are often lacking. This study aimed to examine aspects important to developing therapeutic alliance between clinicians and parents of children with SNI. METHODS: A purposive sample of expert clinicians and parents of children with SNI completed brief demographic surveys and 1:1 semistructured interviews between July 2019 and August 2020 at a single tertiary pediatric academic center. Interviews focused on the inpatient experience and transcriptions underwent thematic analysis by a study team of qualitative researchers with expertise in palliative care and communication science. RESULTS: Twenty-five parents and 25 clinicians participated (total n = 50). Many parents were mothers (n = 17, 68%) of school-aged children with congenital/chromosomal conditions (n = 15, 65%). Clinicians represented 8 professions and 15 specialties. Responses from participants suggested 3 major themes that build and sustain therapeutic alliance including: (1) foundational factors that must exist to establish rapport; (2) structural factors that provide awareness of the parent/child experience; and (3) weathering factors that comprise the protection, security, and additional support during hard or uncertain times. Participants also shared concrete actions that promote these factors in clinical practice. CONCLUSION: Therapeutic alliance between clinicians and parents of children with SNI consists of at least 3 factors that support communication and medical decision-making. These factors are facilitated by concrete actions and practices, which enhance communication about the care for children with SNI.

From Monochromatic to Technicolor: Parent Perspectives on Challenges and Approaches to Seeing Children with Severe Neurological Impairment Holistically.

Background: Children with severe neurological impairment (SNI) commonly receive care in the hospital setting necessitating frequent interactions with clinicians. Yet, parents report that clinicians often have a limited understanding of their child's unique needs and abilities which hinders their care. Objectives: This study aimed to understand the challenges and suggested approaches parents identified to seeing their child holistically. Design: Cross-sectional qualitative study. Setting/Subjects: Parents/legal guardians of children with SNI at a tertiary pediatric academic center in the United States completed 1:1 interviews between August 2019 and February 2020. Measurements: Qualitative researchers with expertise in care for children with SNI, palliative care, and bioethics used thematic content analysis to inductively analyze data for relevant themes. Results: Twenty-five parents/legal guardians of 23 children with SNI participated. Sixty-eight percent were mothers and 24% were fathers; and 68% were white. Thirty-two percent were from other racial and/or ethnic backgrounds. Children predominantly had congenital/chromosomal (n = 15, 65%) and central nervous system static (n = 6, 26%) SNI diagnoses. Four themes emerged regarding both challenges and approaches to understanding children with SNI holistically. These included uniqueness, interdependency, complexity, and universality. Parents felt that by eliciting and incorporating their perspective on these sometimes contrasting but inherently necessary aspects of their child's care, clinicians would understand their children more fully. Conclusion: By viewing the child through the prismed lens of parents, participants described how clinicians could transition from a monochromatic to a technicolor view of their child-including the inherent contrasting needs required for their comprehensive care.

Parents Are the Experts: A Qualitative Study of the Experiences of Parents of Children With Severe Neurological Impairment During Decision-Making.

CONTEXT: Parents of children with severe neurologic impairment (SNI) often face high-stakes medical decisions when their child is hospitalized. These decisions involve technology and/or surgery, goals of care and/or advance care planning, or transitions of care. OBJECTIVES: This study describes the experiences of parents of children with SNI during decision-making. METHODS: Eligible participants were parents facing a decision for a child with SNI admitted to acute or intensive care units at a single tertiary pediatric center. Parents completed 1:1 semi-structured interviews and brief surveys between August 2019 and February 2020. Demographic information was extracted from the child's electronic health record. A team of palliative and complex care researchers with expertise in qualitative methods used thematic content analysis to formulate results. RESULTS: 25 parents participated. The majority had children with congenital/chromosomal SNI conditions (n = 13, 65%), >5 subspecialists (n = 14, 61%), and chronic technology assistance (n = 25, 100%). 68% (n = 17) were mothers and 100% identified as being their child's primary decision-maker. Responses from parents included 3 major themes: 1) our roles and actions; 2) our stresses and challenges; and 3) our meaning and purpose. Responses highlighted the pervasiveness of parental decision-making efforts and parents' advocacy and vigilance regarding their child's needs. Despite this, parents often felt unheard and undervalued in the hospital. CONCLUSION: During hospitalizations, when parents of children with SNI often face high-stakes medical decisions, interventions are needed to support parents and ensure they feel heard and valued as they navigate their child's medical needs and system challenges.

Severe Neurological Impairment: A delphi consensus-based definition.

BACKGROUND: Severe Neurological Impairment (SNI) is a term for which there is no consistently used definition. This may hamper consistency in the reporting of research in the area and communication between professionals involved in the care of those with SNI. OBJECTIVE: We aimed to create an international, multidisciplinary, consensus-based definition of SNI. DESIGN: The Delphi method was employed to reach consensus on the definition of SNI. METHOD: An international, multi-disciplinary expert panel was recruited. The process proceeded over three rounds with feedback provided to panellists between each of them. Consensus was defined as 70% agreement. A working definition was created and, following presentation at an international meeting and consultation with parent representatives, further refined, to create a finalised definition. RESULTS: Thirty-four expert panellists commenced the process. Six items reached the threshold of consensus. The finalised definition is as follows: "Severe Neurological Impairment describes a group of disorders of the central nervous system which arise in childhood, resulting in motor impairment, cognitive impairment and medical complexity, where much assistance is required with activities of daily living. The impairment is permanent but can be progressive or static." CONCLUSION: A consensus-based definition of SNI which includes multi-disciplinary, international and parental input has been created. This should prove useful for clinical, research and resource-planning purposes.

Initial Validation of GRASP: A Differential Diagnoses Algorithm for Children With Medical Complexity and an Unknown Source of Pain.

OBJECTIVES: Identifying the source of pain for children with medical complexity (MC) is challenging. The purpose of this study was the initial validation of a tool to guide the medical evaluation for identifying the source of pain in children with MC by using differential diagnoses. The tool is entitled Guidelines for Ruling Out and Assessing Source of Pain (GRASP). METHODS: A mixed-methods approach that included expert review, focus groups, Web-based surveys, and a trial of the GRASP was used to determine validity as well as perceived clinical utility. RESULTS: Focus groups were held with 26 inpatient and outpatient clinicians. Participants consistently responded in support of the GRASP. Participants advised several suggestions for tool organization such as designing the tool as a flow diagram. Seven clinicians participated in Web-based surveys and made specific suggestions for making the GRASP more comprehensive. Six participants trialed the GRASP for 14 children with MC and pain of unknown origin. Overall, participants found that the GRASP was a clinically effective tool for guiding medical evaluation. CONCLUSIONS: These results provide preliminary evidence that the GRASP has content and face validity in evaluating the source of pain in children with MC. This tool can be used to systematically guide clinicians through a balanced approach to evaluation with a goal of determining the pain source, preventing harm, and relieving suffering without unnecessary tests.

Chronic Pancytopenia due to Centrally Mediated Hypothermia in Two Children with Severe Neurological Impairment.

We report on recurrent pancytopenia over five years in two children with severe impairment of the central nervous system. Assessment by hematology did not identify an etiology, including bone marrow biopsy in one. Both patients had sustained normalized blood cell counts following interventions to maintain or return to a temperature above 33 °C. Acute cytopenias following medically induced and environmental hypothermia have been reported. Recurrent pancytopenia due to centrally mediated hypothermia in patients with severe neurological impairment is often not recognized, putting such children at risk for unnecessary testing and transfusions. We provide a practical approach to management that is feasible for caregivers in the home setting with suggestions for monitoring.

Feeding Intolerance in Children with Severe Impairment of the Central Nervous System: Strategies for Treatment and Prevention.

Children with severe impairment of the central nervous system (CNS) experience gastrointestinal (GI) symptoms at a high rate and severity, including retching, vomiting, GI tract pain, and feeding intolerance. Commonly recognized sources of symptoms include constipation and gastroesophageal reflux disease. There is growing awareness of sources due to the impaired nervous system, including visceral hyperalgesia due to sensitization of sensory neurons in the enteric nervous system and central neuropathic pain due to alterations in the thalamus. Challenging the management of these symptoms is the lack of tests to confirm alterations in the nervous system as a cause of symptom generation, requiring empirical trials directed at such sources. It is also common to have multiple reasons for the observed symptoms, further challenging management. Recurrent emesis and GI tract pain can often be improved, though in some not completely eliminated. In some, this can progress to intractable feeding intolerance. This comprehensive review provides an evidence-based approach to care, a framework for recurrent symptoms, and language strategies when symptoms remain intractable to available interventions. This summary is intended to balance optimal management with a sensitive palliative care approach to persistent GI symptoms in children with severe impairment of the CNS.

Pain Assessment and Treatment in Children With Significant Impairment of the Central Nervous System.

Pain is a frequent and significant problem for children with impairment of the central nervous system, with the highest frequency and severity occurring in children with the greatest impairment. Despite the significance of the problem, this population remains vulnerable to underrecognition and undertreatment of pain. Barriers to treatment may include uncertainty in identifying pain along with limited experience and fear with the use of medications for pain treatment. Behavioral pain-assessment tools are reviewed in this clinical report, along with other strategies for monitoring pain after an intervention. Sources of pain in this population include acute-onset pain attributable to tissue injury or inflammation resulting in nociceptive pain, with pain then expected to resolve after treatment directed at the source. Other sources can result in chronic intermittent pain that, for many, occurs on a weekly to daily basis, commonly attributed to gastroesophageal reflux, spasticity, and hip subluxation. Most challenging are pain sources attributable to the impaired central nervous system, requiring empirical medication trials directed at causes that cannot be identified by diagnostic tests, such as central neuropathic pain. Interventions reviewed include integrative therapies and medications, such as gabapentinoids, tricyclic antidepressants, α-agonists, and opioids. This clinical report aims to address, with evidence-based guidance, the inherent challenges with the goal to improve comfort throughout life in this vulnerable group of children.

Gabapentin Use in the Neonatal Intensive Care Unit.

Gabapentin was used for the treatment of term and preterm infants with suspected visceral hyperalgesia caused by a variety of neurologic and gastrointestinal morbidities. Improved feeding tolerance and decreased irritability were seen, as well as decreased usage of opioids and benzodiazepines. Adverse events occurred with abrupt discontinuation of this medication.

Gabapentin for management of recurrent pain in 22 nonverbal children with severe neurological impairment: a retrospective analysis.

BACKGROUND: Children with severe impairment of the central nervous system (CNS) have a high incidence of distressing symptoms, with many experiencing frequent recurrent pain episodes. OBJECTIVE: The study objective was to describe presenting pain behaviors, daily dose, and response to gabapentin for the management of frequent recurrent pain in this population. METHODS: A retrospective analysis was performed with data from 22 children with severe impairment of the CNS residing at a long-term care facility, treated with gabapentin for recurrent pain behaviors. Response was considered significant if the frequency and severity of symptoms decreased by more than 50% as assessed by nursing staff. RESULTS: Pain behaviors commonly reported included facial grimacing, crying, or moaning. Intermittent increase in muscle tone was identified in 86% (n=19). Gastrointestinal (GI) symptoms occurred in 64% (n=14), including pain localized to the GI tract and vomiting. All were assessed for nociceptive pain sources, many with repeated testing. Most were on medications for spasticity (n=20, 91%) and gastroesophageal reflux disease (GERD) (n=22, 100%) prior to gabapentin use. Of the 22 treated with gabapentin, 21 (91%) had a significant decrease in symptoms. No serious adverse events occurred. The mean gabapentin dose for children five years of age or less (n=11) was 50 mg/kg/day (95% CI 45-56) compared to children older than 11 years (n=11) with a mean dose of 36 mg/kg/day (95% CI 34-38). CONCLUSIONS: Gabapentin appears to be an effective treatment for children with severe impairment of the CNS and recurrent pain behaviors, including intermittent changes in muscle tone. Dosing information can guide treatment trials and future prospective studies.

Treating dyspnea with morphine sulfate in nonverbal children with neurological impairment.

Children with severe neurological impairment (NI) are at risk for recurrent respiratory illness with risk for associated distressing respiratory symptoms as respiratory exacerbations become more frequent. Evidence for treating dyspnea in adults with severe pulmonary disease offers interventions for consideration, including morphine sulfate. This case series of four individuals with severe NI reviews the benefit from morphine for respiratory distress. Information includes descriptors of distress, starting dose, and dose increases. This is the first report to review the use of morphine for the treatment of dyspnea in nonverbal children with severe impairment of the central nervous system.

Supportive and palliative care of children with metabolic and neurological diseases.

PURPOSE OF REVIEW: To review the role of pediatric palliative care (PPC) for children with metabolic and neurological diseases. RECENT FINDINGS: There is a growing body of literature in PPC, though it remains limited for children with metabolic and neurological diseases. Evidence indicates the benefit of PPC. Utilization of PPC programmes can facilitate communication, ensure that families are better informed, improve certainty with decisions, enhance positive emotions, result in fewer invasive interventions at the end of life, and have an impact on location of death. Barriers to utilization of PPC include concern about taking away hope and uncertainty about prognosis. Challenging areas for children with metabolic and neurological diseases include the identification of distressing symptoms and prognostic uncertainty. This article aims to review literature relevant to this group of children, as well as provide a framework when considering specific palliative care needs. SUMMARY: PPC for children with metabolic and neurological diseases can lessen a child's physical discomfort and enhance parental certainty with decision-making. These areas along with other needs throughout the illness trajectory and bereavement are being increasingly met by the growing availability of PPC programmes.

Treatment with gabapentin associated with resolution of apnea in two infants with neurologic impairment.

BACKGROUND: Infants with neurologic impairment (NI) as a result of injury to the central nervous system have a significant rate of pain. Problems associated with pain in children with NI include increased tone. This secondary hypertonia may contribute to apnea as a result of alterations in airway tone and chest wall movement. Infants are at greatest risk given their smaller airways, lower functional residual capacity, and the high rate of unrecognized pain at this age. DISCUSSION: We report two cases of infants with NI, identified to have significant improvement in apnea following empiric treatment with gabapentin for presumed central pain and/or visceral hyperalgesia. This correlation between pain and apnea, with resolution in apnea following effective treatment of these presumed sources, has not been previously reported in the literature. CONCLUSION: Infants with NI and apnea should have careful pain assessment and treatment, when considering other causes and interventions for apnea.