Millicent S. Curlee

Objective: Knowledge about sexual and reproductive health is often limited among adolescents in Zambia, and this limitation may have negative health effects. Although programmes have been developed to increase such knowledge, these programmes can sometimes be seen as being in conflict with cultural values. Design: This study examined the effects of adding a novel programme involving teaching and storytelling about character (Lubuto Mentoring ) to a standard sexual and reproductive health programme (Stepping Stones), which engages with topics such as communication, assertiveness, safer sex, sexually transmitted diseases, and gender-based violence. Method: The experimental group participated in the novel curriculum, which combined Lubuto Mentoring and Stepping Stones, and the control group took part in the standard curriculum (Stepping Stones). Both treatments lasted 13 weeks. Setting: Participants were 371 Zambian young people aged 15–19 years. Results: Participants in both programmes demonstrated increases from before the intervention to after in knowledge about sexual and reproductive health, and confidence in their ability to undertake challenging behaviours pertaining to sexuality, and well-being. The increases did not differ between groups. Participants were run in three cohorts. In each cohort, one group of participants took part in then Lubuto Mentoring -enhanced programme, and the other cohort took part in Stepping Stones. Later cohorts showed larger increases than did earlier cohorts. Conclusion: Study findings suggest that training in character strengths using techniques such as storytelling as part of sexual and reproductive health programmes in Zambia is feasible without reducing overall efficacy.

Costello syndrome (CS) is a rare RASopathy that is typically associated with mild to moderate cognitive impairment. Based on detailed neuropsychological testing, this case series describes variability in the neurobehavioral presentations of three unrelated individuals with different pathogenic HRAS variants that cause CS. Results demonstrate a wide range of functioning among people with CS (extremely low to superior ranges) and describe the first cognitive testing results for an individual with HRAS c.179G>A, p.Gly60Asp. Patient‐reported social, emotional, and quality of life outcomes also are discussed. These cases highlight the potential for above average cognitive functioning, the importance of flexible neurobehavioral assessment methods, and key quality of life and mental health domains to evaluate in individuals with CS.

Background: Oral therapeutic options for plexiform neurofibromas (PNs) in individuals with neurofibromatosis type 1 (NF1) are receiving attention in clinical research. The MEK inhibitor (MEKi) Selumetinib is FDA-approved in children ages 2+ years with inoperable PNs, and shows activity in adults. Prolonged therapy with selumetinib is necessary to maintain tumor reduction. Therefore, investigating long-term adherence is vital to understand patterns of adherence over time and its impact on clinical outcomes. Mixed methods research offers rich information about adherence that can inform future intervention trials, and can assist practitioners in addressing medication adherence concerns. Methods: This mixed-method pilot study is the first examination of the feasibility of a technology-based adherence assessment method, the medication events monitoring system (MEMS^TM), among individuals with NF1-PN. Adherence was monitored in a small sample of patients (N = 12; mean age = 34.36 years; 58% male) with NF1 and PN across eighteen 28-day treatment cycles. Qualitative data were obtained from individual interviews using inductive and deductive techniques for thematic analysis. Results: The predetermined criterion was met, suggesting that using MEMS^TM is feasible despite some challenges with the caps. Depression and overall stress were significantly related to reduced adherence, although these results should be considered hypothesis-generating. Barriers to medication adherence included forgetting and the timing of doses related to eating. Facilitators included consistency, reminders, and social support. Conclusions: This study highlights patient characteristics that may be related to increased risk for nonadherence, as well as challenges with electronic pill caps that should be considered in future clinical trials for NF1-related PN. Results can inform future adherence interventions for adults with NF1 and PNs. Future research with larger samples is needed to fully explore factors related to long-term medication adherence among individuals with NF1.