Social-emotional Functioning of Children and Adolescents With Neurofibromatosis Type 1 and Plexiform Neurofibromas: Relationships With Cognitive, Disease, and Environmental Variables

Objective This 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. Methods Psychological 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. Results Notable 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. Conclusions Cognitive, 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.