Self-management skills in a UK sample of young people with chronic liver disease.

BACKGROUND: We aimed to assess self-management skills and adherence behaviors in young people post-liver transplant and compare these with those of young people with autoimmune liver disease and other forms of chronic liver disease. METHOD: As part of our specialist multidisciplinary clinic, n = 156 young people (aged 16-25 years) completed the Liver Self-Management Questionnaire (an adaptation of the Developmentally Based Skills Checklist for adolescents post-liver transplant and modified for us across liver disease type and within the United Kingdom). Those taking medication (n = 128) also completed a service-designed questionnaire regarding adherence. The statistical significance of group differences was assessed with non-parametric analyses. RESULTS: Young people post-liver transplant were less likely to report managing their condition independently than those with autoimmune liver disease or those with other forms of chronic liver disease. They also reported higher adherence (93%) compared to those with autoimmune liver disease (77%) and those with other forms of chronic liver disease (85%). However, the vast majority of self-management and adherence behaviors were comparable between young people post-transplant and those with autoimmune liver disease/other forms of chronic liver disease. CONCLUSION: Our data are in line with existing data from US samples and also extend these findings to include those with other forms of chronic liver disease. These data highlight the importance of individualized care for young adults, regardless of condition type or healthcare setting, and of clinicians managing their expectations regarding what is considered appropriate condition management in early adulthood.

Mental Health, Cognitive, and Neuropsychiatric Needs in Children and Young People With Wilson Disease.

Wilson disease (WD) is associated with neurological, psychiatric, cognitive, and psychosocial difficulties, but there is little data regarding the nature and prevalence of these problems in children and young people (CYP). Methods: A single-center case-note review to establish the incidence and nature of these issues in CYP with WD, managed before and after multidisciplinary team (MDT) clinics, was established. Results: Out of 69 (43 males) CYP with WD, 37.8% presented with acute liver failure, 48.6% with chronic liver disease and 13.5% after family screening. Medical treatment was with penicillamine (40), trientene (18), zinc and penicillamine/trientene (11), and zinc monotherapy (2). Twenty-one underwent liver transplantation. After a median follow-up of 9.8 (IQR 6.4-16.9) years, 86% are alive. Six died posttransplantation and 7 grafts were lost. Mental health difficulties were recorded in 49.3%, particularly prevalent in the acute liver failure group (70.8%). Nonadherence was common (50.7%) and associated with greater mental health prevalence. Neurological issues were reported in 36.2% and poor cognition/attainment in 14.5%, consistent across modes of presentation. Four patients had diagnoses of autism spectrum conditions, all diagnosed pre WD. CYP seen within an MDT-clinic had more frequent documentation of all issues examined, but lower levels of late graft loss (94% versus 80%, P = 0.07). Conclusion: Our data highlight the need to offer management in WD patients especially as these aspects are underrecognized in CYP presenting with liver involvement. We aim to highlight the importance of multidisciplinary input when looking after this population beyond transition through to adult services.