Parents' Views on Autopsy, Organ Donation, and Research Donation After Neonatal Death.

Importance: Parents who experience neonatal loss have the option to participate in autopsy, organ donation, and research donation. However, clinicians are uncomfortable discussing autopsy and may not be aware of research and organ donation opportunities. Objective: To capture the perspectives of parents who had experienced neonatal loss about autopsy, organ donation, and research donation. Design, Setting, and Participants: This qualitative study used virtual focus groups with parents who attended a local bereavement support group in the US. Participants were recruited from Helping After Neonatal Death, a support group with a local chapter. Participants self-selected from an email request if they met the following criteria: aged 18 years or older, English speaking, at least 6 months elapsed since neonatal death, and access to a video conference device with internet. Focus groups took place between April and September 2021. The recorded sessions were analyzed using a grounded theory-informed approach by the research team that included parents with experience of neonatal loss. Data were analyzed from December 2021 through December 2022. Results: A total of 14 mothers engaged in the focus group; 9 (75%) were aged 30 to 39 years, and 8 (66%) were White. The mothers were overall well educated. The first main theme grew from the lived experience of neonatal loss, specifically the importance of offering all parents the option to donate, rather than prejudging who would or would not be interested. Parents of neonates who die have few opportunities to parent that child and make loving decisions for them. Participants emphasized that the conversation about autopsy, organ donation, and research donation, albeit difficult, can offer a meaningful parenting experience. A second main theme that emerged related to how organ or tissue donation could provide additional meaning to a child's life. These choices contributed to building a legacy to honor their child's memory, which also helped with grief and coping with their loss. A third theme included recommendations to clinicians and health systems for improving communication, including written information for parents and communication training for health care professionals. Conclusions and Relevance: In this qualitative study, parents who experienced neonatal loss endorsed the importance of offering parents the choice of autopsy, organ donation, or research donation with skillful and empathetic communication. They provided practical recommendations to improve communication and empower families.

Teaching Death Disclosure: A Mixed-Method Comparison of Resident Self-Assessment and Standardized Patient Assessment.

OBJECTIVE: To characterize how pediatric resident self-evaluation compares to standardized patient evaluations in simulated child death disclosure scenarios. METHODS: This was a prospective, observational, mixed-methods study in which 18 second-year pediatric residents delivered the news of a death of a child to a trained standardized patient (SP) couple. The SPs evaluated residents via a quantitative global rating (1-3 scale) and via qualitative comments. Following the training, the residents completed self-assessments consisting of a global rating, qualitative comments, and their confidence related to 5 death disclosure skills. RESULTS: Agreement between SPs and resident ratings was poor; resident scores were compared to each of their 2 SP evaluators yielding Kappa coefficients of -0.23 (95% confidence interval = -0.60 to -0.07) and -0.30 (95% confidence interval = -0.70 to -0.04). Residents uniformly rated themselves as less capable in their communication skills than SPs did. Residents reported significant increases in their confidence in discussing autopsy and organ donation. Major themes determined from the qualitative comments from SPs included nonverbal communication, verbal communication, attunement to parents, and management of next steps. Residents' comments mirrored these themes with the exception of the absence of nonverbal communication. CONCLUSIONS: Pediatric residents underestimated their abilities in a self-assessment of their performance in a SP death disclosure scenario, demonstrating the importance of external feedback, particularly from SPs themselves. Based on SP feedback, future death disclosure trainings should emphasize nonverbal communication skills and specific behaviors that convey effective attunement to families.

The neurocognitive impact of Fabry disease on pediatric patients.

Fabry disease (FD) is an X-linked lysosomal storage disorder that results in progressive multisystemic organ complications. Several studies have examined neurocognitive impairments in adults; however, there is a paucity of research examining neurocognitive functioning in children with FD. This is the first exploratory study to examine the neurocognitive functioning of pediatric patients with FD and to evaluate the effects of enzyme replacement therapy (ERT) on neurocognitive functioning within this population. Families attending a national conference with at least one child with FD and one parent affected by FD comprised the sample (n = 48; 24 pediatric patients, 24 parents). Pediatric participants (10 males, 14 females) between the ages of 6 and 18 years and their parent(s) were involved in the study. Data from a demographic questionnaire and two neurocognitive self-report and parent-report measures were analyzed. Parent reports of neurocognitive functioning were also compared to a sample of children with and without head injury and to a sample of children who had undergone liver transplant (LT). Children with FD had poorer cognitive and executive functioning than healthy peers, and were comparable to children with head injury and LT. In addition, children using ERT had higher scores on measures of overall cognitive functioning, as well as fewer problems with attention/working memory and executive functioning. Results of this study suggest that children with FD may exhibit poorer cognitive and executive functioning relative to healthy peers. The use of ERT may mitigate the negative impact of FD on neurocognitive functioning in pediatric patients.

Inborn errors of metabolism: psychosocial challenges and proposed family systems model of intervention.

Inborn errors of metabolism result in psychosocial crises that challenge individual and familial modes of functioning across the life cycle. Increased stress, mood disorders, interpersonal challenges, decreased quality of life, and grief reactions are all common for patients and their families. To effectively care for these patients, a holistic approach to their care, which incorporates their social context, is essential. Patients and their families need support as they focus on immediate practical demands, grieve over illness-related losses, and reorient future expectations. A family systems based model provides a flexible and individualized approach to care that allows for optimal psychosocial adjustment throughout the disease process.