The use of projected autonomy in antenatal shared decision-making for periviable neonates: a qualitative study.

BACKGROUND: In this study, we assessed the communication strategies used by neonatologists in antenatal consultations which may influence decision-making when determining whether to provide resuscitation or comfort measures only in the care of periviable neonates. METHODS: This study employed a qualitative study design using inductive thematic discourse analysis of 'naturally occurring data' in the form of antenatal conversations around resuscitation decisions at the grey zone of viability. The study occurred between February 2017 and June 2018 on a labor and delivery unit within a large Midwestern tertiary care hospital. Participants included 25 mothers who were admitted to the study hospital with anticipated delivery in the grey zone of viability and practicing neonatologists or neonatology fellows who partnered in antenatal consultation. We used a two-stage inductive analytic process to focus on how neonatologists' discourses constructed SDM in antenatal consultations. First, we used a thematic discourse analysis to interpret the recurring patterns of meaning within the transcribed antenatal consultations, and second, we theorized the subsequent effects of these discourses on shaping the context of SDM in antenatal encounters. RESULTS: In this qualitative study, that included discourse analysis of real-time audio conversations in 25 antenatal consults, neonatologists used language that creates projected autonomy through (i) descriptions of fetal physiology (ii) development of the fetus's presence, and (iii) fetal role in decision-making. CONCLUSION: Discourse analysis of real-time audio conversations in antenatal consultations was revelatory of how various discursive patterns brought the fetus into decision-making, thus changing who is considered the key actor in SDM.

Consultation patterns before and after embedding pediatric palliative care into a pediatric hematology/oncology clinic.

INTRODUCTION: Palliative care is a critical component of pediatric oncology care. Embedded pediatric palliative care (PPC) is relatively new in pediatric hematology/oncology (PHO) and may improve access, utilization, and quality of PPC. In June 2020, the Mayo Clinic PPC service transitioned from an afternoon, physically independent clinic to an all-day clinic embedded within PHO. METHODS: Retrospective chart review was used to quantify consultation rates from PHO to PPC in 12-month study periods before and after establishment of an embedded clinic. Changes in descriptive statistics and consult patterns were calculated. Study periods were compared using either chi-square or Fisher's exact tests for categorical variables and Wilcox rank sum tests for continuous variables. RESULTS: There was an 89% increase in consultations from PHO to PPC after initiation of an embedded clinic (n = 20 vs. n = 38 per 12 months). The absolute number of completed outpatient consults increased from three (15% of visits) pre-embedment to fourteen (37%) post-embedment (p = .082). The median number of days from first oncology visit to PPC assessment was unchanged after embedment (36 vs. 47 days, p = .98). Consults for solid tumors increased from 22% (n = 4) pre-embedment to 60% (n = 18) post-embedment (p < .05). Consults for symptom management increased from 60% (n = 12) to 87% (n = 33) (p < .05). CONCLUSIONS: Embedment of PPC into a PHO workspace was associated with an increased number of total consults, outpatient consults, solid tumor consults, and consults for symptom management. Our "partial-PPO" model allowed for provision of PPC in the outpatient oncology setting in a clinic where there is not enough volume to support a full-time oncology-focused clinician team.

Risk reduction for small cell cancer of the ovary, hypercalcemic type in prepubertal patient: A clinical and bioethical perspective.

Loss of heterozygosity in the SMARCA4 gene is a hallmark feature of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), an aggressive ovarian cancer occurring in young adults and adolescents with an average age of 23 years and a median survival of less than fifteen months following diagnosis. Patients with germline pathogenic variants of SMARCA4 have a genetic predisposition to developing this aggressive ovarian cancer, a condition called rhabdoid tumor predisposition syndrome type 2 (RTPS2). Given the limited efficacy of surveillance imaging for ovarian neoplasm and the absence of an identified biomarker for the progression of this disease, asymptomatic patients who are found to possess pathogenic variants of the SMARCA4 gene following genetic testing are advised to consider risk-reducing bilateral salpingo-oophorectomy to eliminate the risk of SCCOHT. Given the reproductive impacts of this procedure, bioethical consultation must be considered when counseling patients with RTPS2, particularly for those who have not completed their desired course of family planning. In this report, we describe the bioethical considerations and outcomes for the case of a 6-year-old female with a pathogenic variant of SMARCA4 who underwent risk-reducing bilateral salpingo-oophorectomy (RRBSO). To our knowledge, this is the first time that this procedure has been reported in a prepubertal individual for cancer prevention in a patient with RTPS2.

Novel Use of Intranasal Dexmedetomidine for Refractory Irritability in Pediatric Home Care.

Background: Dexmedetomidine is a selective alpha-2 agonist with sedative, analgesic, and anxiolytic properties used intravenously for procedural sedation and in the intensive care unit. The reported use of intranasal (IN) dexmedetomidine for symptom management in pediatric palliative care is limited. Case History: A boy with cardiofaciocutaneous syndrome and refractory irritability was supported by pediatric palliative care throughout numerous hospitalizations for goals of care discussions and pain and symptom management. Given functional and multiorgan system deterioration, he was enrolled in home hospice to optimize comfort measures at anticipated end of life. After the addition of scheduled IN dexmedetomidine for management of irritability, the boy demonstrated marked improvement in comfort and sleep. Conclusion: This case report shows the successful use of IN dexmedetomidine for management of refractory irritability with no noted adverse effects. Future studies and use of this medication will need to consider potential indications, optimal dosing, and long-term effects in the pediatric palliative care setting.

Guidance for Pediatric End-of-Life Care.

The final hours, days, and weeks in the life of a child or adolescent with serious illness are stressful for families, pediatricians, and other pediatric caregivers. This clinical report reviews essential elements of pediatric care for these patients and their families, establishing end-of-life care goals, anticipatory counseling about the dying process (expected signs or symptoms, code status, desired location of death), and engagement with palliative and hospice resources. This report also outlines postmortem tasks for the pediatric team, including staff debriefing and bereavement.

Caffeine for the Treatment of Central Apnea in Trisomy 18: A Case Study in the Novel Use of Methylxanthines in Palliative Transport.

Central apnea is a major cause of death in neonates with trisomy 18 (T18) and is likely due to immaturity of the respiratory drive, similar to the pathological mechanism in apnea of prematurity. Although caffeine has long been used for apnea of prematurity, its use has not yet been reported for infants with T18. Here we describe an infant with T18 receiving comfort-focused care and palliative transport home. Of utmost importance to her family was enabling the patient to spend time at home before her death. However, immediately before transport, she developed severe central apnea, raising concern that she may not survive the transfer. Caffeine was trialed in the hopes of reducing central apnea events. Its use was successful and facilitated safe transport, allowing the patient to uneventfully transition home, where she spent several meaningful weeks with her family before her death.

Uncertainty at the Limits of Viability: A Qualitative Study of Antenatal Consultations.

BACKGROUND AND OBJECTIVES: Antenatal consultation between a neonatologist and expectant parent(s) may determine if resuscitation is provided for or withheld from neonates born in the gray zone of viability. In this study, we sought to gain a deeper understanding of uncertainties present and neonatologists' communication strategies regarding such uncertainties in this shared decision-making. METHODS: A prospective, qualitative study using transcriptions of audio-recorded antenatal consultations between a neonatologist and expectant parent(s) was conducted. Pregnant women were eligible if anticipating delivery in the gray zone of viability (22 0/7-24 6/7 weeks' gestation). Over 18 months, 25 of 28 pregnant women approached consented to participate. Applied thematic analysis was used to inductively derive and examine conceptual themes. RESULTS: Inductive analysis of consult transcripts revealed uncertainty as a central theme. Several subthemes relating to uncertainty were also derived, including the timing of delivery, NICU course, individual characteristics (of physician, expectant parent(s), and fetus or neonate), and consequences of the decision for the expectant parent(s). Analysis revealed that uncertainty was actively managed by neonatologists through a variety of strategies, including providing more information, acknowledging the limits of medicine, acknowledging and accepting uncertainty, holding hope, and relationship building. CONCLUSIONS: Uncertainty is pervasive within the antenatal consultation for periviable neonates and likely plays a significant role in decision-making toward postnatal resuscitative efforts. Uncertainty complicated, or even paralyzed, decision-making efforts while also providing reassurance toward a positive outcome. Directions for future study should consider whether advanced communication training modulates the impact that uncertainty plays in the shared decision-making encounter.

Survival of Ventilated Extremely Premature Neonates With Severe Intraventricular Hemorrhage.

BACKGROUND: Severe intraventricular hemorrhage (IVH) is a leading mortality risk factor among extremely premature neonates. Because other life-threatening conditions also occur in this population, it is unclear whether severe IVH is independently associated with death. The existence and potential implications of regional variation in severe IVH-associated mortality are unknown. METHODS: We performed a retrospective cohort study of mechanically ventilated neonates born at 22 to 29 weeks' gestation who received care in 242 American NICUs between 2000 and 2014. After building groups composed of propensity score-matched and center-matched pairs, we used the Cox proportional hazards analysis to test our hypothesis that severe IVH would be associated with greater all-cause in-hospital mortality, defined as death before transfer or discharge. We also performed propensity score-matched subgroup analyses, comparing severe IVH-associated mortality among 4 geographic regions of the United States. RESULTS: In our analysis cohort, we identified 4679 patients with severe IVH. Among 2848 matched pairs, those with severe IVH were more likely to die compared with those without severe IVH (hazard ratio 2.79; 95% confidence interval 2.49-3.11). Among 1527 matched pairs still hospitalized at 30 days, severe IVH was associated with greater risk of death (hazard ratio 2.03; 95% confidence interval 1.47-2.80). Mortality associated with severe IVH varied substantially between geographic regions. CONCLUSIONS: The early diagnosis of severe IVH is independently associated with all-cause in-hospital mortality in extremely premature neonates. Regional variation in severe IVH-associated mortality suggests that shared decision-making between parents and neonatologists is strongly influenced by ultrasound-based IVH assessment and classification.

Ethical considerations of maternal-fetal intervention in a twin pregnancy discordant for anomalies.

BACKGROUND: Recent evidence suggests prenatal fetoscopic tracheal occlusion (FETO) may improve the survival and long-term morbidity of neonates with congenital diaphragmatic hernia, yet little guidance exists in the medical literature as to the ethical permissibility of performing a maternal-fetal surgical intervention in a twin pregnancy discordant for a structural abnormality. CASE: Here, we present a case of a twin pregnancy with an unaffected twin (Twin A) and a twin diagnosed with severe congenital diaphragmatic hernia (Twin B). A proposed fetoscopic tracheal occlusion (FETO) procedure may improve the likelihood of survival and postnatal outcome of Twin B; however, balloon placement may also initiate very preterm birth at 28 weeks of gestation. The Fetal Ethics Advisory Board was asked to provide guidance on the permissibility of FETO in this pregnancy. DISCUSSION: A literature review identified one brief mention of FETO in a 34-week dichorionic twin pregnancy in the medical literature, which resulted in the rupture of fetal membranes in the sac of the nonsurgical twin. Only one paper specifically addressed the question of whether it would be ethically permissible to subject a healthy twin to the risks of maternal-fetal surgery for the benefit of a compromised twin, finding that any risk to the unaffected twin would be an ethical contraindication. We offer our own analysis of moral weight and risk/benefit considerations of this proposed intervention, and present our findings on the circumstances in which it may be ethically permissible to perform a maternal-fetal intervention in a twin pregnancy. CONCLUSION: While FETO was not ethically advisable in this pregnancy, we find that in limited circumstances, certain maternal-fetal surgical interventions may be ethically permissible in a twin pregnancy discordant for a structural abnormality if the risks to the unaffected twin are minimal and the procedure would improve the likelihood of survival and postnatal outcome of a critically compromised co-twin.

Molecular epidemiology of methicillin-susceptible Staphylococcus aureus in infants in a neonatal intensive care unit.

OBJECTIVE: To investigate the molecular epidemiology of methicillin-susceptible Staphylococcus aureus (MSSA) in infants in a neonatal intensive care unit (NICU) using whole-genome sequencing. DESIGN: Investigation of MSSA epidemiology in a NICU. SETTING: Single-center, level IV NICU. METHODS: Universal S. aureus screening was done using a single swab obtained from the anterior nares, axilla, and groin area of infants in the NICU on a weekly basis. Core genome multilocus sequence type (cgMLST) analysis was performed on MSSA isolates detected over 1 year (2018-2019). RESULTS: In total, 68 MSSA-colonized infants were identified, and cgMLSTs of 67 MSSA isolates were analyzed. Overall, we identified 11 cgMLST isolate groups comprising 39 isolates (58%), with group sizes ranging from 2 to 10 isolates, and 28 isolates (42%) were unrelated to each other or any of the isolate groups. Cases of infants colonized by MSSA were scattered throughout the 1-year study period, and isolates belonging to the same cgMLST group were typically detected contemporaneously, over a few weeks or a few months. Overall, 13 infants (19.7%) developed MSSA infections: bacteremia (n = 3), wound infection (n = 5), conjunctivitis (n = 4), and cellulitis (n = 1). We detected no association between these clinically manifest infections and specific cgMLST groups. CONCLUSIONS: Although MSSA isolates in infants in a NICU showed high diversity, most were related to other isolates, albeit within small groups. cgMLST facilitates an understanding of the complex transmission dynamics of MSSA in NICUs, and these data can be used to inform better control strategies.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

Homeward Bound: A Case Series of Cross-Cultural Care at End of Life, Enhanced by Pediatric Palliative Transport.

For most families, the preferred location of death for their child is home, yet most children still die in the hospital. Many children with life-threatening and life-limiting illness are medically dependent on technology, and palliative transport can serve as a bridge from the intensive care unit to the family's home to achieve family-centered goals of care. Palliative transport may also present an opportunity to prioritize cultural care and rituals at end of life which cannot be provided in the hospital. We describe a case series of pediatric patients from communities espousing markedly diverse cross-cultural values and limited financial resources. Specific cultural considerations at end of life for these children included optimizing the presence of the shared community or tribe, the centrality of healing rituals, and varied attitudes toward withdrawal of life-sustaining medical treatment. By addressing each of these components, we were able to coordinate palliative transport to enhance cross-cultural care and meaning at end of life for children with life-limiting illness.

Infant male with TARP syndrome: Review of clinical features, prognosis, and commonalities with previously reported patients.

TARP syndrome (talipes equinovarus, atrial septal defect, Robin sequence, and persistent left superior vena cava) is a rare X-linked condition. As more patients are identified through genetic testing, it is increasingly clear that the original TARP acronym does not fully describe the complete phenotypic spectrum of this syndrome. The presented patient had genetically confirmed TARP syndrome and demonstrated new findings of hydronephrosis and hemodynamically significant hypertrophic obstructive cardiomyopathy. The patient also had physical findings common with previously reported individuals with TARP syndrome in the literature but not described by the TARP acronym. These features include central nervous system dysfunction, renal abnormalities, cardiac lesions other than atrial septal defect or persistent left superior vena cava, and distal limb defects other than talipes equinovarus. By adding to the known spectrum of the TARP phenotype, this report will aid clinicians as they care for patients with this rare condition.

Outcomes of early inhaled nitric oxide use in premature African American neonates.

OBJECTIVE: Meta-analysis of individual-patient clinical trial data suggests that inhaled nitric oxide (iNO) improves respiratory outcomes in premature African American neonates. We hypothesized that early iNO therapy would be associated with lower mortality and less chronic lung disease (CLD) in extremely premature African American neonates. STUDY DESIGN: We conducted a retrospective cohort study of propensity score- and race-matched neonates 22-29 weeks gestation who were mechanically ventilated for treatment of respiratory distress and associated pulmonary hypertension (RDS + PPHN). We evaluated the association of iNO within 7 days of life with in-hospital mortality and CLD, using Cox proportional hazards regression and logistic regression, respectively. RESULT: Among 178 matched pairs of African American patients, iNO was not associated with lower mortality (HR = 0.94, 95% CI 0.69-1.30) or less CLD (OR = 0.94, 95% CI 0.47-1.87). CONCLUSIONS: Early, off-label iNO use is not associated with improved outcomes in premature African American neonates with RDS + PPHN.

Attitudes of paediatric and obstetric specialists towards prenatal surgery for lethal and non-lethal conditions.

BACKGROUND: While prenatal surgery historically was performed exclusively for lethal conditions, today intrauterine surgery is also performed to decrease postnatal disabilities for non-lethal conditions. We sought to describe physicians' attitudes about prenatal surgery for lethal and non-lethal conditions and to elucidate characteristics associated with these attitudes. METHODS: Survey of 1200 paediatric surgeons, neonatologists and maternal-fetal medicine specialists (MFMs). RESULTS: Of 1176 eligible physicians, 670 (57%) responded (range by specialty, 54%-60%). In the setting of a lethal condition for which prenatal surgery would likely result in the child surviving with a severe disability, most respondents either disagreed (59%) or strongly disagreed (19%) that they would recommend the surgery. Male physicians were twice as likely to recommend surgery for the lethal condition, as were physicians who believe that abortion is morally wrong (OR 1.75; 95%CI 1.0 to 3.05). Older physicians were less likely to recommend surgery (OR 0.57; 95%CI 0.36 to 0.88). For non-lethal conditions, most respondents agreed (66% somewhat, 4% strongly) that they would recommend prenatal surgery, even if the surgery increases the risk of prematurity or fetal death. Compared with MFMs, surgeons were less likely to recommend such surgery, as were physicians not affiliated with a fetal centre, and physicians who were religious (ORs range from 0.45 to 0.64). CONCLUSION: Physician's attitudes about prenatal surgery relate to physicians' beliefs about disability as well as demographic, cultural and religious characteristics. Given the variety of views, parents are likely to receive different recommendations from their doctors about the preferable treatment choice.

Weighing the Social and Ethical Considerations of Maternal-Fetal Surgery.

OBJECTIVES: The ethics of maternal-fetal surgery involves weighing the importance of potential benefits, risks, and other consequences involving the pregnant woman, fetus, and other family members. We assessed clinicians' ratings of the importance of 9 considerations relevant to maternal-fetal surgery. METHODS: This study was a discrete choice experiment contained within a 2015 national mail-based survey of 1200 neonatologists, pediatric surgeons, and maternal-fetal medicine physicians, with latent class analysis subsequently used to identify groups of physicians with similar ratings. RESULTS: Of 1176 eligible participants, 660 (56%) completed the discrete choice experiment. The highest-ranked consideration was of neonatal benefits, which was followed by consideration of the risk of maternal complications. By using latent class analysis, we identified 4 attitudinal groups with similar patterns of prioritization: "fetocentric" (n = 232), risk-sensitive (n = 197), maternal autonomy (n = 167), and family impact and social support (n = 64). Neonatologists were more likely to be in the fetocentric group, whereas surgeons were more likely to be in the risk-sensitive group, and maternal-fetal medicine physicians made up the largest percentage of the family impact and social support group. CONCLUSIONS: Physicians vary in how they weigh the importance of social and ethical considerations regarding maternal-fetal surgery. Understanding these differences may help prevent or mitigate disagreements or tensions that may arise in the management of these patients.

Emergency Video Telemedicine Consultation for Newborn Resuscitations: The Mayo Clinic Experience.

OBJECTIVE: To describe the Mayo Clinic experience with emergency video telemedicine consultations for high-risk newborn deliveries. PATIENTS AND METHODS: From March 26, 2013, through December 31, 2015, the Division of Neonatal Medicine offered newborn telemedicine consultations to 6 health system sites. A wireless tablet running secure video conferencing software was used by the local care teams. Descriptive data were collected on all consultations. After each telemedicine consult, a survey was sent to the neonatologist and referring provider to assess the technology, teamwork, and user satisfaction. RESULTS: During the study, neonatologists conducted 84 telemedicine consultations, and 64 surveys were completed. Prematurity was the most frequent indication for consultation (n=32), followed by respiratory distress (n=15) and need for advanced resuscitation (n=14). After the consult, nearly one-third of the infants were able to remain in the local hospital. User assessment of the technology revealed that audio and video quality were poor or unusable in 16 (25%) and 12 (18.8%) of cases, respectively. Providers failed to establish a video connection in 8 consults (9.5%). Despite technical issues, providers responded positively to multiple questions assessing teamwork (86.0% [n=37 of 43] to 100.0% [n=17 of 17] positive responses per question). In 93.3% (n=14 of 15) of surveyed cases, the local provider agreed that the telemedicine consult improved patient safety, quality of care, or both. CONCLUSION: Telemedicine consultation for neonatal resuscitation improves patient access to neonatology expertise and prevents unnecessary transfers to a higher level of care. A highly reliable technology infrastructure that provides high-quality audio and video should be considered for any emergency telemedicine service.

Pediatric Palliative Care in the Intensive Care Unit.

The chronicity of illness that afflicts children in Pediatric Palliative Care and the medical technology that has improved their lifespan and quality of life make prognostication extremely difficult. The uncertainty of prognostication and the available medical technologies make both the neonatal intensive care unit and the pediatric intensive care unit locations where many children will receive Pediatric Palliative Care. Health care providers in the neonatal intensive care unit and pediatric intensive care unit should integrate fundamental Pediatric Palliative Care principles into their everyday practice.

Two infants, same prognosis, different parental preferences.

A central principle of justice is that similar cases should be decided in similar ways. In pediatrics, however, there are cases in which 2 infants have similar diagnoses and prognoses, but their parents request different treatments. In this Ethics Rounds, we present such a situation that occurred in a single NICU. Three physician-ethicists analyze the issues.