Revisiting medical neglect concerns in children with life-threatening complex chronic conditions.

BACKGROUND/OBJECTIVES: Life-Threatening Complex Chronic Conditions (LT-CCCs) increase the complexity of medical neglect concerns. Clinicians' perspectives are central in concerns for medical neglect, yet little is currently known regarding clinicians' understanding of and approach to these situations. We explored how clinicians who care for children with LT-CCCs understand medical neglect. METHODS: We conducted a semi-structured qualitative interview study with 20 clinicians of varying disciplines from critical, palliative, and complex care services about medical neglect in children with LT-CCCs. We used inductive thematic analysis to generate themes. RESULTS: Three primary themes emerged: Relationship between family and medical community, family overwhelmed with medical demands, and insufficient support. Taken together these themes suggest that concerns for medical neglect are directly related to clinician perception of family inability to meet medical needs. CONCLUSION: Clinicians report that concerns for medical neglect in children with LT-CCCs often arise from a mismatch of medical expectations and the perception of familial ability to provide said medical care. Given the complex and delicate medical and psychosocial environments of care for children with LT-CCCs, these medical neglect concerns are more accurately described as Medical Insufficiency, a new term. By reframing this entity, we can reframe the dialogue surrounding this issue, and reconsider approaches to studying, preventing, and resolving it.

Health Care Providers' Perspectives on COVID-19 and Medical Neglect in Children with Life-Threatening Complex Chronic Conditions.

Purpose: Little is known regarding medical neglect in children with Life-Threatening Complex Chronic Conditions (LT-CCCs). We examined the impact of COVID-19 on concern for medical neglect in this population. Methods: Qualitative interview study of multi-disciplinary health care providers (HCPs) from critical care, palliative care, and complex care services on the topic of medical neglect in children with LT-CCCs. We used inductive thematic analysis to generate themes. Findings presented herein are derived from a sub-analysis of the larger study that focused specifically on discussion of COVID-19 by HCPs. Results: 9 of the 20 HCPs interviewed mentioned COVID-19 as influencing situations of potential medical neglect. These 9 represent all disciplines and teams. Interviewees reported COVID-19 increased burden on parents and likelihood of medical neglect due to: 1) Familial distancing from medical and social support and, 2) Changes to medical care delivery that impaired the medical community's ability to engage and support families. Conclusions: The COVID-19 pandemic has exposed the fragility of the medical and social systems that supports families of children with LT-CCCs. These findings are consistent with previous literature that suggest that the COVID-19 pandemic has increased the risk for child maltreatment. It additionally highlights the vulnerability of this patient population.

Factors Affecting Recruitment and Participation of Bereaved Parents in Research: A Brief Report.

CONTEXT: Inclusion of bereaved parents in survey-based research is essential to improving end-of-life care for children and their families. However, racial and ethnic minorities are vastly underrepresented in these studies. OBJECTIVES: Examine which child demographic characteristics are associated with parental participation in survey-based research. METHODS: Post-hoc secondary analysis of the Survey of Caring for Children with Complex Chronic Conditions. Demographic data were extracted from children's medical records for all eligible parents. Using logistic regression, associations were calculated between child demographics and level of parental study participation (enrolled and completed survey (full participation), enrolled but did not complete survey (incomplete participation), and declined participation). RESULTS: Two hundred nine children's parents were eligible for analysis, 114 (55%) fully participated, 36 (17%) incompletely participated, and 59 (28%) declined participation. Compared to those who fully participated, parents of non-white children were more likely to incompletely participate (OR 3.01, 95% CI 1.2-7.4), while those with public insurance only were more likely to decline participation (OR 2.1, 95% CI 1.08-4.03). Parents of children who had a documented limitation to resuscitation order were more likely to fully participate in the study (reference group) than incompletely participate (OR 0.42, 95% CI 0.2-0.91), while parents of children who had subspecialty palliative care involvement were less likely to decline participation in the study (OR 0.5, 95% CI 0.26-0.97). CONCLUSIONS: To ensure optimal end-of-life care for children, all parental voices should be heard. Recruitment of racial/ethnic minorities in bereaved parent survey-based studies can be improved by focusing on specific factors that are associated with parental survey completion.

Genetic prion disease: Experience of a rapidly progressive dementia center in the United States and a review of the literature.

Although prion diseases are generally thought to present as rapidly progressive dementias with survival of only a few months, the phenotypic spectrum for genetic prion diseases (gPrDs) is much broader. The majority have a rapid decline with short survival, but many patients with gPrDs present as slowly progressive ataxic or parkinsonian disorders with progression over a few to several years. A few very rare mutations even present as neuropsychiatric disorders, sometimes with systemic symptoms such as gastrointestinal disorders and neuropathy, progressing over years to decades. gPrDs are caused by mutations in the prion protein gene (PRNP), and have been historically classified based on their clinicopathological features as genetic Jakob-Creutzfeldt disease (gJCD), Gerstmann-Sträussler-Scheinker (GSS), or Fatal Familial Insomnia (FFI). Mutations in PRNP can be missense, nonsense, and octapeptide repeat insertions or a deletion, and present with diverse clinical features, sensitivities of ancillary testing, and neuropathological findings. We present the UCSF gPrD cohort, including 129 symptomatic patients referred to and/or seen at UCSF between 2001 and 2016, and compare the clinical features of the gPrDs from 22 mutations identified in our cohort with data from the literature, as well as perform a literature review on most other mutations not represented in our cohort. E200K is the most common mutation worldwide, is associated with gJCD, and was the most common in the UCSF cohort. Among the GSS-associated mutations, P102L is the most commonly reported and was also the most common at UCSF. We also had several octapeptide repeat insertions (OPRI), a rare nonsense mutation (Q160X), and three novel mutations (K194E, E200G, and A224V) in our UCSF cohort. © 2016 Wiley Periodicals, Inc.