Schiltz, Brenda M

Objective: The Society of Critical Care Medicine PICU Liberation ABCDEF (A–F) Campaign Collaborative aimed to implement best practices for analgesia (A), breathing (B), comfort/consciousness (C), delirium (D), early mobilization (E), and family engagement and empowerment (F). Our post hoc study of Mayo Clinic data sought to compare the pre-implementation rounding process, which used a nursing script organized by organ systems, with a novel, liberation-focused rounding process emphasizing A–F bundle elements. Design: Secondary analysis of pre- vs. post-implementation surveys collected by independent observers during rounds. Setting: Medical/surgical PICU in a quaternary-care, academic medical center during April to July 2017. Subjects: The observed participants included PICU physicians, nurses, respiratory therapists, pharmacists, and dietitians. Interventions: None. Measurements and Main Results: Survey responses from 145 team members pre-implementation and 67 post-implementation showed improved team satisfaction, communication, and understanding of daily plans (all p < 0.05). Respondents reported an increased frequency of discussions on bundle components A–E (all p < 0.01). Observations of 41 pre-implementation and 46 post-implementation rounds showed an increase in the frequency of discussion of all components of the bundle (all p < 0.01). However, this finding was not at the expense of increased duration of individual rounds since we failed to find a difference in pre- vs. post-implementation duration per patient (480 vs. 544 s per patient, p = 0.20). Conclusions: In 2017, around the time of introducing the nurse-led PICU Liberation rounding process to our practice, we found that after implementation, there was an associated increase in discussions about bundle elements and improved communications and satisfaction in team members.

In severe pediatric acute respiratory distress syndrome, data are lacking on methods to measure and set optimal positive end-expiratory pressure. We present a 2-year-old girl with Trisomy 21 who developed severe pediatric acute respiratory distress syndrome and refractory hypoxemia from human metapneumovirus pneumonia. Esophageal manometry was utilized to measure transpulmonary pressure, and positive end-expiratory pressure was increased to 19 cm H 2 O, resulting in rapid improvement in oxygenation. Hemodynamics remained adequate without intervention. The patient improved and survived without sequelae. Our case suggests that transpulmonary pressure monitoring should be studied as an adjunct to improve outcomes in pediatric acute respiratory distress syndrome.

Abstract Background: Although the need for palliative care is gaining recognition in Southeast Asia, knowledge about how decisions are made for children near the end of life remains sparse. Objective: To explore pediatric intensivists' attitudes and practices surrounding end-of-life care in Vietnam. Methods: This is a mixed-methods study conducted at a tertiary pediatric and neonatal intensive care unit in Hanoi. Physicians and nurses completed a quantitative survey about their views on end-of-life care. A subset of these providers participated in semistructured interviews on related topics. Analysis of surveys and interviews were conducted. Results were triangulated. Results: Sixty-eight providers (33 physicians and 35 nurses) completed the quantitative survey, and 18 participated in interviews. Qualitative data revealed three overarching themes with numerous subthemes and supporting quotations. The first theme was factors influencing providers' decision-making process to escalate or withdraw treatment. Quantitative data showed that 40% of providers valued the family's ability to pay to continue life-sustaining treatment. Second, communication dynamics in decision making were highlighted; 72% of providers would be willing to override a family's wishes to withdraw life-sustaining treatment. Third, provider perceptions of death varied, with 68% regarding their patients' deaths as a personal failure. Conclusions: We elicited and documented how pediatric intensivists in Vietnam currently think about and provide end-of-life care. These findings indicate a need to strengthen palliative care training, increase family involvement in decision making, implement standardized and official do-not-resuscitate documentation, and expand pediatric hospice services at the individual, hospital, and national levels in Vietnam.

Background: The dynamin 1-like gene ( DNM1L) encodes a GTPase that mediates mitochondrial and peroxisomal fission and fusion. We report a new clinical presentation associated with a DNM1L pathogenic variant and review the literature. Results: A 13-year-old boy with mild developmental delays and paroxysmal dystonia presented acutely with multifocal myoclonic super-refractory status epilepticus. Despite sustained and aggressive treatment, seizures persisted and care was ultimately withdrawn in the context of extensive global cortical atrophy. Rapid trio-whole exome sequencing revealed a de novo heterozygous c.1207C>T (p.R403C) pathogenic variant in DNM1L. Immunofluorescence staining of fibroblast mitochondria revealed abnormally elongated and tubular morphology. Conclusions: This case highlights the diagnostic importance of rapid whole exome sequencing within a critical care setting and reveals the expanding phenotypic spectrum associated with DNM1L variants. This now includes progressive paroxysmal dystonia and adolescent-onset super-refractory myoclonic status epilepticus contributing to strikingly rapid and progressive cortical atrophy and death.