Inpatient management of epidermolysis bullosa: Consensus-based hands-on instructions for neonates and postneonates.

BACKGROUND: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America. OBJECTIVE: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and postneonatal period. METHODS: A modified Delphi method (expert-based input via 2 surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium centers were analyzed to determine areas of focus and formulate statements to be voted on by EB Clinical Research Consortium members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected. RESULTS: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 nonhealth care caregivers. Consensus was established on 103/119 neonatal statements and 105/122 postneonatal statements; no statements were rejected. Most recommendations applied to both age groups. LIMITATIONS: Recommendations may require adjustment based on individual patient's clinical context. CONCLUSION: Using the Delphi method, a consensus-derived resource for hospital-based health care professionals who manage patients with EB has been developed to improve the quality of inpatient care.

What Is Culture Made of? An Exploratory Study of Ethical Cultures and Provider Perspectives on the Care of Periviable Neonates.

OBJECTIVE: Studies examining intercenter variation in neonatal intensive care unit practices at the limits of fetal viability have hypothesized that institutional "culture" can be one of many factors that impact patient care. This study aimed to describe institutional culture at a single, large academic center with regard to the antenatal consultation, resuscitation, and postnatal management of periviable neonates. STUDY DESIGN: Members of six clinical groups-attending and fellow maternal-fetal medicine physicians, attending and fellow neonatal-perinatal medicine physicians, neonatal nurses, advanced practiced neonatal nurses, pediatric hospitalist physicians, and neonatal respiratory therapists-were invited to complete qualitative, semi-structured interviews. All audio recordings were transcribed. Dedoose software was used to complete team-based coding and thematic analysis. RESULTS: Twenty-two interviews were completed. Thematic analysis revealed three central themes described by participants as contributory to institutional culture: Perception, referring to factors based on individual attitudes and insights, Statements of Information, referring to factors anchored in more objective concepts such as outcomes data and institutional policy, and Dynamic Factors, referring to the relatively fluid factors of institutional culture that interact with both Perception and Statements of Information. Participants were more likely to mention factors in the Perception category (n = 430) compared with factors in the Information category (n = 225), and although the latter were described as critical components of antenatal counseling and perinatal management, the philosophy of our unit appeared to be more heavily rooted in institutional memory and individual belief systems. CONCLUSION: Our data demonstrate a personal undertone to institutional culture at the limits of viability, with an emphasis on individual attitudes and subjective interpretations of fact rather than empirical data. As the landscape of neonatology continues to change, understanding those factors that contribute to culture remains a necessary step toward deconstructing institutional belief systems and optimizing clinical care. KEY POINTS: · Institutional culture is the collective norms and attitudes that help guide organizational behavior.. · Institutional culture may be one of many factors that impact the care of periviable neonates.. · Deconstructing culture helps us better understand our clinical environment and optimize patient care..

Trial of Therapy on Trial: Inconsistent Thresholds for Discussing Withdrawal of Life-Sustaining Therapies in the Neonatal Intensive Care Unit.

OBJECTIVE: This study aimed to establish the degree of variability in thresholds for discussing withdrawal of life-sustaining therapies (WLST) in periviable infants among neonatal intensive care unit (NICU) personnel. STUDY DESIGN: A vignette-style survey was administered to NICU personnel at two urban NICUs assessing likelihood of discussing WLST or support for discussing WLST (on a scale from 1, not at all likely/supportive to 10, extremely likely/supportive) in 10 clinical scenarios. RESULTS: Response rates ranged by clinical role from 26 to 89%. Participant responses ranged from 1 to 10 in 5 out of 10 vignettes for NICU attendings, and 9 out of 10 vignettes for bedside nurses. Lower gestational age (22-23 vs. 24-25 weeks) was associated with increased likelihood to discuss WLST in some but not all scenarios. CONCLUSION: NICU personnel have widely variable criteria for discussing WLST which threatens the informed consent process surrounding resuscitation decisions in a "trial of therapy" framework. KEY POINTS: · NICU personnel have variable criteria for WLST.. · Parents have little say in whether WLST is offered.. · Disclosure of variable criteria is not routine..

Mother's Milk Donation to a Human Milk Bank During Bereavement: Circumstances Associated with Completing the Donation Process.

Background: Bereaved mothers describe positive experiences donating breast milk and negative experiences when not informed of opportunities to donate. Predictors of whether mothers donate milk are unknown, impairing efforts to optimize support in completing donation. Objective: To define circumstances associated with completing mother's milk (MM) donation during bereavement. Methods: A retrospective cohort study included dyads of bereaved mothers and their deceased children if a child's death occurred on-site at a quaternary care children's hospital during 2016-2020, the child had documentation of MM availability, and age at death <24 months. The primary outcome was the completion of MM donation to the milk bank. Multivariate logistic regression measured associations between clinical variables and odds of completion. Results: Of 124 deceased children with documented MM exposure, 34 mothers (28%) of 35 of those children completed MM donation, donating a mean of 13.7 liters (SD 16.8). The child's race/ethnicity documented in the medical record was White for 25 (71%), Black/African American (AA) for 1 (3%), Asian for 1 (3%), and Hispanic/Latino for 8 (23%). Referenced to mothers of White children, being a mother of an AA [OR 0.05 (95% CI: 0.01-0.43)] or Asian [0.08 (0.01-0.75)] child was associated with lower odds of donation. Referenced to mothers delivering full term (≥37 weeks'), mothers delivering <34 weeks showed higher odds [5.0 (1.5-17.5)] of donation. Conclusion: Relatively few bereaved mothers of children with indicators of MM exposure completed donation. The results suggest an opportunity to ensure bereaved mothers are uniformly informed and supported in donating.

Navigating the post-Dobbs landscape: ethical considerations from a perinatal perspective.

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.

Variability of Care Practices for Extremely Early Deliveries.

OBJECTIVES: Assess temporal changes, intercenter variability, and birthing person (BP) factors relating to interventions for extremely early deliveries. METHODS: Retrospective study of BPs and newborns delivered from 22-24 completed weeks at 13 US centers from 2011-2020. Rates of neonatology consultation, antenatal corticosteroids, cesarean delivery, live birth, attempted resuscitation (AR), and survival were assessed by epoch, center, and gestational age. RESULTS: 2028 BPs delivering 2327 newborns were included. Rates increased in epoch 2-at 22 weeks: neonatology consultation (37.6 vs 64.3%, P < .001), corticosteroids (11.4 vs 29.5%, P < .001), live birth (66.2 vs 78.6%, P < .001), AR (20.1 vs 36.9%, P < .001), overall survival (3.0 vs 8.9%, P = .005); and at 23 weeks: neonatology consultation (73.0 vs 80.5%, P = .02), corticosteroids (63.7 vs 83.7%, P < .001), cesarean delivery (28.0 vs 44.7%, P < .001), live birth (88.1 vs 95.1%, P < .001), AR (67.7 vs 85.2%, P < .001), survival (28.8 vs 41.6%, P < .001). Over time, intercenter variability increased at 22 weeks for corticosteroids (interquartile range 18.0 vs 42.0, P = .014) and decreased at 23 for neonatology consultation (interquartile range 23.0 vs 5.2, P = .045). In BP-level multivariate analysis, AR was associated with increasing gestational age and birth weight, Black BP race, previous premature delivery, and delivery center. CONCLUSIONS: Intervention rates for extremely early newborns increased and intercenter variability changed over time. In BP-level analysis, factors significantly associated with AR included Black BP race, previous premature delivery, and center.

The role of palliative care in critical congenital heart disease.

Patients with critical congenital heart disease are exposed to significant lifetime morbidity and mortality. Prenatal diagnosis can provide opportunities for anticipatory co-management of patients between palliative subspecialists and the cardiac care team. The benefits of palliative care include support for longitudinal decision-making and avoidance of interventions not consistent with family goals. Effectively counseling families requires an up-to-date understanding of outcomes and knowledge of provider biases. Patient-proxy reported quality of life (QOL) is highly variable in this population and healthcare providers need to be aware of limitations in their own subjective assessment of QOL.

Neonatologist training in communication and palliative care.

Neonatologists receive highly varied and largely inadequate training to acquire and maintain communication and palliative care skills. Neonatology fellows often need to give distressing news to families and frequently face unique communication challenges. While several approaches to teaching these skills exist, practice opportunities through simulation and role play will likely provide the most effective learning.