The self-concept of children and adolescents with neurofibromatosis type 1.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a genetic disorder associated with cognitive deficits, learning problems, medical complications and cosmetic disfigurement. Despite the wide-ranging impact of NF1, very few studies have examined the psychosocial adjustment of individuals with NF1, and in particular, self-concept. The aims of this study were threefold: (i) to examine the self-concept of children and adolescents with NF1; (ii) to compare the self-concept of children with NF1 (NF1 only), children with NF1 and learning difficulties [NF1 + LA (low achievement)], and children with NF1 and attention deficit hyperactivity disorder (ADHD) (NF1 + ADHD); and (iii) to examine the academic self-concept of these three groups relative to objective criteria - academic achievement and teacher ratings of academic competence.
METHODS: Measures of self-concept, academic achievement and intelligence were administered to 49 children and 26 adolescents with NF1. Parents and teachers completed behavioural rating scales.
RESULTS: The majority of children and adolescents with NF1 reported positive global self-concept, with some exceptions on specific domains. Children and adolescents with NF1 reported significantly poorer self-concept for physical abilities. Adolescents also reported significantly poorer self-concept for mathematics and general self when compared with normative mean values. Clinical severity of NF1 was not a significant predictor of self-concept for physical appearance or physical abilities. Despite a significant difference between NF1 only, NF1 + LA and NF1 + ADHD groups on measures of academic achievement and teacher ratings of academic competence, there was no significant difference between the groups for academic domains of self-concept, or any other domain of self-concept. All three groups reported inflated academic self-perceptions relative to objective criteria.
CONCLUSION: Children and adolescents with NF1 are most likely to develop negative self-concepts about their physical and sporting abilities. Inflated academic self-perceptions are discussed in light of the positive illusory bias, which may serve as an adaptive or protective function.