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.