Are transition preparation consultations for adolescents with chronic conditions valuable? A mixed-methods study.

Our objective was to assess the value of transition preparation consultations (TPC) offered by the AD'venir unit (R. Debré hospital, Paris) as a new service of transitional care, from the perspective of adolescents with chronic conditions (CCs) and their referring healthcare providers (RHCPs). TPCs included a face-to-face interview with pediatricians trained in adolescent medicine, exploring the adolescent's past (CC history), present (daily life, Treatment Burden Questionnaire, family/peer relationships, school, hobbies, sexuality, drugs), and future (global life project, transition, Good2Go questionnaire). The mixed-methods design included the following: a qualitative analysis within a multidisciplinary group (clinicians/sociologists/psychologist/public health researchers) of audio-recordings of TPCs (n = 27/girls = 56%/median age = 17.7 years) and phone interviews with adolescents 2 years post-TPC (n = 26); and a quantitative analysis of the Treatment Burden and Good2Go questionnaires and the benefits perceived by RHCPs (questionnaire 6 months post-TPC). TPCs were a form of training for adult care, adolescents meeting a practitioner alone often for the first time. Naming their CC was difficult. All complained of limitations experienced in social life (diet, fatigue, laboratory/medical appointments), but not the treatment itself; most adolescents willingly talked about sexuality. Adolescents' feelings about transition were various, with poor representations of adult healthcare. Transfer was frequently unplanned. After TPCs, RHCPs modified their practices. Transition in the 2 years post-TPC was usually successful. Conclusion What is Known: • In adolescents with chronic conditions, it is advocated to personalize transition care according to the clinical and social context, pointed out as potentially impacting. • Little is known about the most effective ways to prepare patients according to their needs. What is New: • Based on a global approach to adolescent health, transition preparation consultations are delivered by specially trained physicians. • They are a feasible and valuable way to highlight facilitators and barriers to successful transition and initiate the adolescents' own vision of their future.

Error disclosure in neonatal intensive care: a multicentre, prospective, observational study.

IMPORTANCE: Surveys based on hypothetical situations suggest that health-care providers agree that disclosure of errors and adverse events to patients and families is a professional obligation but do not always disclose them. Disclosure rates and reasons for the choice have not previously been studied. OBJECTIVE: To measure the proportion of errors disclosed by neonatal intensive care unit (NICU) professionals to parents and identify motives for and barriers to disclosure. DESIGN: Prospective, observational study nested in a randomised controlled trial (Study on Preventing Adverse Events in Neonates (SEPREVEN); ClinicalTrials.gov). Event disclosure was not intended to be related to the intervention tested. SETTING: 10 NICUs in France with a 20-month follow-up, starting November 2015. PARTICIPANTS: n=1019 patients with NICU stay ≥2 days with ≥1 error. EXPOSURE: Characteristics of errors (type, severity, timing of discovery), patients and professionals, self-reported motives for disclosure and non-disclosure. MAIN OUTCOME AND MEASURES: Rate of error disclosure reported anonymously and voluntarily by physicians and nurses; perceived parental reaction to disclosure. RESULTS: Among 1822 errors concerning 1019 patients (mean gestational age: 30.8±4.5 weeks), 752 (41.3%) were disclosed. Independent risk factors for non-disclosure were nighttime discovery of error (OR 2.40; 95% CI 1.75 to 3.30), milder consequence (for moderate consequence: OR 1.85; 95% CI 0.89 to 3.86; no consequence: OR 6.49; 95% CI 2.99 to 14.11), a shorter interval between admission and error, error type and fewer beds. The most frequent reported reasons for non-disclosure were parental absence at its discovery and a perceived lack of serious consequence. CONCLUSION AND RELEVANCE: In the particular context of the SEPREVEN randomised controlled trial of NICUs, staff did not disclose the majority of errors to parents, especially in the absence of moderate consequence for the infant. TRIAL REGISTRATION NUMBER: NCT02598609.

Adult T-cell acute lymphoblastic leukemias with IL7R pathway mutations are slow-responders who do not benefit from allogeneic stem-cell transplantation.

The prognostic value of IL7-receptor pathway (IL7Rp) mutations in T-cell acute lymphoblastic leukemia (T-ALL) remains unclear. We performed a comprehensive study of 200 adult patients with T-ALL included in the GRAALL2003/2005 protocols to address the clinical significance of IL7Rp mutations. Next-generation sequencing of the IL7Rp (IL7R/JAK1/JAK3/STAT5B) revealed that IL7Rp mutations were frequent in adult T-ALL (28%) particularly in immature/early T-cell progenitor (ETP)-ALL. They were associated with mutations of NOTCH-pathway, PHF6, and PRC2 components but not with K/NRAS. IL7Rp mutated (IL7Rpmut) T-ALL were slow-responders, with a high rate of M2/M3 day-8 marrow compared with IL7Rp non-mutated (IL7RpWT) T-ALL (p = 0.002) and minimal residual disease positivity at 6-weeks (MRD1) (p = 0.008) but no difference in MRD2 positivity at 12-weeks. Despite this, no adverse prognosis was evidenced when censored for allogeneic hematopoietic stem cell transplantation (HSCT). In time-dependent analysis, HSCT did not benefit IL7Rpmut patients whereas it was of marked benefit to IL7RpWT cases. IL7Rp-mutations identify a subgroup of slow-responder T-ALLs which benefit from post-induction chemotherapy regimens but not from HSCT. Our data suggest that prior knowledge of the mutation status of IL7Rp may influence HSCT decision and help to guide therapy reduction.