Social-emotional functioning of children and adolescents with neurofibromatosis type 1 and plexiform neurofibromas: Relationships with cognitive, disease, and environmental variables

ObjectiveThis descriptive cross-sectional study aimed to determine how cognitive, disease, and environmental variables relate to social-emotional functioning in youth with NF1 and plexiform neurofibromas.MethodsPsychological assessments were administered to 53 children (mean age 12.4 years); parents and teachers completed the Behavior Assessment System for Children - Second Edition (BASC-2). Disease severity was quantified by nurse-practitioner ratings and tumor burden, and parents completed a life events checklist to indicate environmental stressors.ResultsNotable proportions of children scored in the at-risk/clinically significant ranges on several parent and teacher BASC-2 subscales including Somatization, Attention Problems, Depression, and Withdrawal. Combinations of cognitive, disease, and environmental variables predicted scores on parent BASC-2 Internalizing Problems, Behavior Symptoms Index, and Adaptive Skills composites.ConclusionsCognitive, disease, and environmental variables relate to social-emotional outcomes in children with NF1. These youth may benefit from interventions targeting social skills, cognitive functioning, and adaptive ways of coping with NF1-related pain.