Providing physiologic support to a brain-dead pregnant decedent poses complex ethical, legal, and clinical challenges. Understanding these considerations is necessary to navigate complex discussion and provide appropriate medical care. We use a theoretical case to examine these considerations and outline a path forward.

Pediatric palliative care (PPC) is an essential, evolving component of care for children with serious, complex, life-threatening, or life-limiting conditions [...]

ObjectivesTo explore inequities in prenatal consultation and parental resuscitation decisions across high-mortality conditions.Study designWe conducted a retrospective chart review of pregnant people whose liveborn neonates were diagnosed with high-mortality conditions. We examined two cohorts: periviable infants (22 0/7-24 6/7 weeks) and infants with severe congenital anomalies.ResultsA total of 194 neonates met eligibility criteria for the periviable cohort, 197 for the congenital anomaly cohort. In the periviable cohort, 94% of White vs. 81% of Black pregnant people received neonatology consultation (p = 0.009). A total of 96% of those with commercial insurance vs. 82% of those with Medicaid received consultation (p = 0.005). Half of Hispanic pregnant people did not receive neonatology consultation (p = 0.02). In the congenital anomaly cohort, pregnant people who spoke a language other than English were less likely to receive consultation (44% vs. 81%, p = 0.02).ConclusionsThis regional assessment found previously unrecognized inequities in prenatal neonatology consultation.

Objective Moral distress is psychological pain evoked by the inability to act according to ones values. Characterization of moral distress among pediatric trainees caring for patients with neurologic conditions is lacking. This study examines moral distress accordingly.Methods Cross-sectional survey at a pediatric quaternary care hospital. Trainees responded in the context of caring for children with neurologic conditions. Survey included the Measure of Moral Distress for Healthcare Professionals (MMD-HP), featuring items corresponding with moral distress causes: system, patient, team. Two items adapted from the Maslach Burnout Inventory, questions evaluating moral distress mitigation, and a free text section were featured. Responses were analyzed for all respondents and subspecialty groups.Results Participant response rate was 33% (80 of 244). Most were familiar with moral distress but recommended additional education. All experienced moral distress and associated burnout in the specified context. Patient-related situations were most distressing. No significant difference in overall MMD-HP scores was noted between subspecialty groups. Debriefing with other providers was projected to best address moral distress.Conclusions Pediatric trainees caring for patients with neurologic conditions are at risk of moral distress and associated burnout. Patient-related situations most contributed to moral distress, specifically the pursuit of life-sustaining care perceived to be futile.

Determine the impact of standardization of care and counseling on survival and morbidities of neonates born at 22–23 weeks gestation. Retrospective cohort study of 244 neonates born at 22–23 weeks gestation between 2015 and 2023 in a large healthcare system. The primary outcome was survival of neonates receiving intensive care to NICU discharge. Secondary outcomes included morbidities and resource utilization. Neonates born at 22–23 weeks received more intensive care after care standardization (OR 5.4 (95

Each year in the United States, many individuals receive a complex fetal or neonatal diagnosis. Amidst grief and processing, they are faced with making difficult and multifaceted medical decisions for the pregnancy and, at times, neonatal care. Care options often include abortion, fetal interventions, palliative and hospice care, and invasive neonatal interventions. These incredibly personal resolutions about the most appropriate care path are often based on multiple influences beyond the medical information and prognosis. External factors (geography, finances, provider bias, institutional policies, and legislation) may limit the availability of certain care options patients would prefer. This article explores how each of these external factors may limit the choices of abortion, fetal interventions, palliative and hospice care, and invasive neonatal interventions for patients facing a complex fetal or neonatal diagnosis. Further, it discusses the implications of restricted options and suggests strategies to continue to provide complete, comprehensive counseling and care.

Neonates requiring intensive care and prolonged hospitalizations often undergo procedures and therapies that predispose them to pain, agitation, and delirium. Early recognition and treatment of these symptoms can decrease morbidity and mortality. While nonpharmacologic interventions can be beneficial, they often are insufficient in managing symptoms of pain, agitation, and delirium. Familiarity with the pharmacologic concepts and therapies used to target different pathophysiological mechanisms of pain and agitation will increase the neonatal clinician's ability to effectively use and tailor these medications to each patient's needs. In this review, we summarize the pathophysiology, impact, assessment, and management options for each symptom so clinicians can individualize care and use targeted pharmacologic therapies to effectively and safely treat symptoms in various clinical scenarios. We also discuss the importance of protocol use to ensure judicious medication initiation and titration to minimize long-term neurodevelopmental effects of pain, agitation, delirium, and their associated pharmacotherapies.

Each year, many neonates are born with genetic diagnoses that carry a range of prognoses. As the types and availability of genetic testing have expanded, neonatal intensive care units (NICUs) have served as “launching points” for their clinical application. Broad genetic testing has both improved diagnostic precision and expanded uncertainty. Genetic information may be explicitly uncertain, as in the case of a variant of unknown significance (VUS). But it is also frequently uncertain whether/how the information relates to a patient’s phenotype or what it may mean for a child’s future. Even without ambiguity in the diagnosis or prognosis, the significance within a clinical and familial context may be less certain. Applying the information to clinical care is complex and may engender confusion among clinicians and families as readily as it offers guidance. Since genetic testing results can impact management and, at times, end-of-life decisions, misunderstanding and misapplication of genetic results pose a significant risk. We describe a hypothetical case of an infant with congenital hypotonia and respiratory failure. The family, after discussions with the care team about medically appropriate care paths, is navigating goals of care and considering tracheostomy placement for chronic mechanical ventilation. They consent to rapid genome sequencing in hopes of better understanding the etiology and severity of the neuromuscular condition. We explore three possible scenarios following different genomic results. With each, we discuss how the results may impact decision-making about the best plan of care. We propose a framework for navigating discussions about genetic testing results with families of critically ill children. We illustrate the importance of a multidisciplinary approach with collaboration between neonatology, genetics, and palliative care. By employing the strengths of each subspecialty, providers can manage the inherent uncertainty in genetic testing results, help determine the meaning of the results to the family in the context of their child’s medical care, and enhance the care and support of critically ill neonates and their families.

Technological advancements before and after delivery have greatly altered the counseling of pregnant patients facing a fetal diagnosis of severe oligohydramnios or anhydramnios secondary to congenital anomalies of the kidneys and urinary tract. Once considered a nearly uniformly lethal abnormality, long-term survival may now be possible secondary to prenatal innovations aimed at restoring the amniotic fluid volume and the availability of more advanced neonatal dialysis techniques. However, these available therapies are far from perfect. The procedures are onerous for pregnant patients without a guarantee of success, and families must prepare themselves for the complex life-long medical care that will be necessary for surviving individuals. Multidisciplinary counseling is imperative to help pregnant individuals understand the complexity of these conditions and assist them in exercising their right to informed decision-making. Moreover, as with any developing field of medicine, providers must contend with ethical questions related to the treatment options, including questions regarding patient-hood, distributive justice, and the blurred lines between research, innovation, and standard care. These ethical questions are best addressed in a multidisciplinary fashion with consideration of multiple points of view from various subspecialties. Only by seeing the entirety of the picture can we hope to best counsel patients about these highly complex situations and help navigate the most appropriate care path.

Delirium often goes unrecognized in neonates and children because of lack of experience in evaluating behavior and cognition, insufficient awareness of the prevalence, and nondistinctive symptoms in this population. Although there are increasing reports of the presence of delirium in neonates, there are few data to guide the pharmacologic treatment in this population. In this retrospective single-center case series, we present our experience using quetiapine to treat delirium in 9 medically complex neonates. Based on an extensive literature review, expert opinion, and institutional experience, we propose an approach for monitoring and treating delirium in neonates and infants.

ObjectiveThe objective of this study was to identify factors birthing parents consider related to potential resuscitation of a periviable infant.Study designBirthing parents who received a prenatal consult from a newborn intensive care unit provider between 22.0 and 24.6 weeks gestational age were eligible to participate in a semi-structured interview focused on their periviable decision making. Interview transcripts were coded and analyzed using thematic content analysis.ResultQualitative analysis shows that birthing parents attribute their decision to a balance between vitality and suffering, with the balance point influenced by various elements. While parents described the choice they made, none reported that the information they received during the prenatal consult had a significant impact.ConclusionThis study highlights the minimal impact that information given during a periviable consult has on parental decision making. Information from this study can be used to develop an improved model of perinatal consultation.

ObjectiveTo evaluate whether racial and socioeconomic inequities in pediatric palliative care utilization extend to children with high-intensity neurologic impairment (HI-NI), which is a chronic neurological diagnosis resulting in substantial functional morbidity and mortality.Study designWe conducted a retrospective study of patients with HI-NI who received primary care services at a tertiary care center from 2014 through 2019. HI-NI diagnoses that warranted a palliative care referral were identified by consensus of a multidisciplinary team. The outcome was referral to palliative care. The primary exposure was race, categorized as Black or non-Black to represent the impact of anti-Black racism. Additional exposures included ethnicity (Hispanic/non-Hispanic) and insurance status (Medicaid/non-Medicaid). Descriptive statistics, bivariate analyses, and multivariable logistic regression models were performed to assess associations between exposures and palliative care referral.ResultsA total of 801 patients with HI-NI were included; 7.5% received a palliative referral. There were no differences in gestational age, sex, or ethnicity between patients who received a referral and those who did not. In multivariable analysis, adjusting for ethnicity, sex, gestational age, and presence of complex chronic conditions, Black children (aOR 0.47, 95% CI 0.26, 0.84) and children with Medicaid insurance (aOR 0.40, 95% CI 0.23, 0.70) each had significantly lower odds of palliative referral compared with their non-Black and non-Medicaid-insured peers, respectively.ConclusionsWe identified inequities in pediatric palliative care referral among children with HI-NI by race and insurance status. Future work is needed to develop interventions, with families, aimed at promoting more equitable, antiracist systems of palliative care.

As the field of fetal-neonatal neurology has expanded over the past 2 decades with increasingly complex diagnoses, multidisciplinary collaboration with many subspecialties including genetics, neonatology, obstetrics, maternal fetal medicine, surgical sub-specialties, cardiology, radiology, palliative care, and ethics has needed to evolve to strive to offer optimal patient care. While comprehensive care delivery with an inter-disciplinary approach is preferred, there are often barriers based on numerous health disparities especially in resource limited settings. Even in the context of comprehensive care, diagnostic and prognostic uncertainty lead to challenges for providers during fetal neurology consultations. We present a case that highlights advantages of a comprehensive multi-disciplinary team in caring for the medical and social challenges of patients faced with a fetal neurologic diagnosis. Inter-disciplinary training focusing on maternal, fetal, neonatal, and childhood neurodevelopmental course and collaboration among the numerous stakeholders that contribute to fetal neurology practice is needed to provide optimal counseling and care for families faced with a fetal neurological diagnosis.

Objective: To determine levels of moral distress in a pediatric unit caring for patients with tracheostomy/ventilator dependence. Hypothesis: Moral distress will be significant in a dedicated pediatric trach/vent unit. Methods: The Moral Distress Survey-Revised (MDS-R) is a 21-question survey measuring moral distress in pediatrics. The MDS-R was anonymously distributed to MD/DOs, advanced practice practitioners (APPs), RNs and RTs in a unit caring for tracheostomy/ventilator dependent patients. Descriptive statistics, bivariate and multivariate analysis were performed. Results: Response rate was 48% (61/127). Mean MDS-R score was 83 (range 43-119), which is comparable to reported levels in the pediatric intensive care unit. APPs had the highest median rate of moral distress (112, IQR 72-138), while MD/DOs had the lowest median score (49, IQR 43-77). RNs and RTs had MDS-R scores between these two groups (Medians of 91 and 84 respectively). Conclusions: Moral distress levels in a unit caring for long term tracheostomy and ventilator dependent patients are high, comparable to levels in pediatric ICUs. APPs had higher levels of distress compared to other groups. This may be attributable to the constant stressors of being the primary provider for complex patients, especially in a high-volume inpatient setting.

Palliative care is the total care of a patient with a life-limiting illness regardless of the disease trajectory or treatment options chosen. There is a special focus on pain/symptom management, communication, quality of life, family support, and grief support. Neonatal-perinatal palliative care is the unique care provided to neonates and women pregnant with a fetus with a life-limiting diagnosis. Up to 3% of pregnancies are complicated by a life-limiting diagnosis and roughly one-third of deaths in children's hospitals occur in neonatal intensive care units (NICUs). As a result, many babies and families benefit from palliative care. There is a special role for palliative care when the likelihood of long-term survival is minimal or when there is a low likelihood of survival without severe morbidity. Decisions around the most appropriate treatment plan are often based around views of acceptable quality of life. While palliative care is essential in the NICU, it is often overlooked, and there are many barriers to it being considered or implemented in suitable cases. However, palliative care can readily be integrated into the management of neonates with life-limiting illnesses and can be provided concurrently with cure-oriented or life-extending care. With the support of the care team, families can begin to grieve, plan, make meaningful memories, redefine hope, and make value-driven medical decisions for their baby that align with their goals and values. When a child is imminently dying, the end-of-life care provided ensures the baby is comfortable and aids the family in the grieving process.

Restrictive abortion laws have impacts reaching far beyond the immediate sphere of reproductive health, with cascading effects on clinical and ethical aspects of neonatal care, as well as perinatal palliative care. These laws have the potential to alter how families and clinicians navigate prenatal and postnatal medical decisions after a complex fetal diagnosis is made. We present a hypothetical case to explore the nexus of abortion care and perinatal care of fetuses and infants with life-limiting conditions. We will highlight the potential impacts of limited abortion access on families anticipating the birth of these infants. We will also examine the legally and morally fraught gray zone of gestational viability where both abortion and resuscitation of live-born infants can potentially occur, per parental discretion. These scenarios are inexorably impacted by the rapidly changing legal landscape in the U.S., and highlight difficult ethical dilemmas which clinicians may increasingly need to navigate.

Fetal neurology is a rapidly evolving and expanding field. Discussions about diagnosis, prognosis, treatment options, and goals of care often begin in the antenatal period. However, there are inherent challenges to fetal counseling of neurological diagnoses due to limitations of fetal imaging, prognostic uncertainty, and variability in neurodevelopmental outcomes. In the midst of uncertainty, families are challenged with preparing a care plan for their baby while simultaneously experiencing profound grief. The paradigms of perinatal palliative care can assist with the grieving process and help frame diagnostic testing and complex decision-making within the context of a family's spiritual, cultural, and social belief system. This ultimately leads to a shared decision-making process and value driven medical care. While perinatal palliative care programs have expanded, many families faced with such diagnoses never meet with a palliative care team prior to delivery. Moreover, there is significant variability in the availability of palliative care services throughout the country. Using an illustrative vignette of a patient with a prenatally diagnosed encephalocele, this review aims to provide a basic framework of perinatal palliative care for fetal neurology diagnoses that emphasizes 1) importance of clear, consistent, and transparent communication among all subspecialists and families, 2) creation of a palliative care birth plan, 3) importance of consistent care providers and longitudinal points of contact prenatally and post-delivery, 4) close communication between the prenatal and post-natal providers to allow for optimal continuity of care, and 5) recognize that information, care plans, and goals of care often evolve over time.

EDITORIAL article Front. Pediatr., 06 November 2023Sec. Neonatology Volume 11 - 2023 | https://doi.org/10.3389/fped.2023.1307584

Ryanodine receptor type 1-related disorder (RYR1-RD) is the most common subgroup of congenital myopathies with a wide phenotypic spectrum ranging from mild hypotonia to lethal fetal akinesia. Genetic testing for myopathies is imperative as the diagnosis informs counseling regarding prognosis and recurrence risk, treatment options, monitoring, and clinical management. However, diagnostic challenges exist as current options are limited to clinical suspicion prompting testing including: single gene sequencing or familial variant testing, multi-gene panels, exome, genome sequencing, and invasive testing including muscle biopsy. The timing of diagnosis is of great importance due to the association of RYR1-RD with malignant hyperthermia (MH). MH is a hypermetabolic crisis that occurs secondary to excessive calcium release in muscles, leading to systemic effects that can progress to shock and death if unrecognized. Given the association of MH with pathogenic variants in RYR1, a diagnosis of RYR1-RD necessitates an awareness of medical team to avoid potentially triggering agents. We describe a case of a unique fetal presentation with bilateral diaphragmatic eventrations who had respiratory failure, dysmorphic facial features, and profound global hypotonia in the neonatal period. The diagnosis was made at several months of age, had direct implications on her clinical care related to anticipated need to long-term ventilator support, and ultimately death secondary an arrhythmia as a result of suspected MH. Our report reinforces the importance of having high suspicion for a genetic syndrome and pursuing early, rapid exome or genome sequencing as first line testing in critically ill neonatal intensive care unit patients and further evaluating the pathogenicity of a variant of uncertain significance in the setting of a myopathic phenotype.