Cintas H¹, Bauer D², Danoff J¹, Furst G¹, Gerber L¹; ¹National Institutes of Health, Bethesda, MD, USA; ²Department of Physical Therapy and Biokinesiology, University of Southern California, Los Angeles, CA, USA. holly_cintas@nih.gov.

PURPOSE: Osteogenesis imperfecta (OI) is a heritable type 1 collagen disorder [loci 7q 2.1-2.2 and 17 q 2.1-2.1] characterized by weakness, fractures, bony malalignment, and short stature. Locomotor capability ranges from running to the need for powered locomotion. As a group, these children appear intelligent, and remarkably socially adept. It is uncertain whether this is due to advanced social skills, or their small stature makes them seem more capable than they actually are. We asked how children with OI view themselves in relation to nondisabled peers with regard to self-perception of competence. SUBJECTS: 55 children (30 girls, 25 boys) with types III (26) and IV(29) osteogenesis imperfecta, ages 4-18 years, completed annual assessments of self-perceived competence as part of an osteogenesis imperfecta research program for children birth to 18 years. METHODS AND MATERIALS: Self-perceived competence was evaluated using three standardized, self-report instruments: Harter Pictorial Scale of Perceived Competence and Social Acceptance for Young Children (age 4 - grade 2); Harter Self-Perception Profile for Children (grade 3 - age 12); and the Harter Self-Perception Profile for Adolescents (ages 13-18 years). Gross motor skill level was assessed using the Brief Assessment of Motor Function (BAMF, Cintas 1998). ANALYSES: Independent t-tests to compare reported competence of children with OI and normative values; Spearman correlation to describe significant relationships; and stepwise multiple regression to identify potential predictors of total competence RESULTS: Young children (preschool-grade 2), and adolescents with OI consistently report themselves as being less capable than nondisabled peers with respect to athletic competence (P = .02 -.001). Middle children and adolescents report themselves as being more competent than nondisabled children in domains of cognitive competence (P= .05-.001), conduct (P=. .05 - .001), and global self-worth (P=.02-.001). Significant relationships exist between motor peformance and total competence (r = .44, P < .0001). Maternal attachment predicts total competence for young children ( R² = .68, P < .001). Global self-worth predicts total competence for middle children( R² = .73, P < .001), and adolescents ( R² = .79, P< .001). CONCLUSIONS: Young children and adolescents with OI clearly perceive their physical performance limitations compared with nondisabled peers, while middle children appear unwilling to declare less athletic competence. Perceptions of higher cognitive competence, conduct, and self-worth suggest that maturing children with OI view themselves positively in these areas compared to nondisabled peers. Significant relationships exist between motor skill level and and total competence, but maternal attachment for young children, and self-worth for middle and older children are more significant predictors of total competence. FUNDING SOURCE: National Institute of Child Health and Human Development Intramural Research Program, Protocol 97-CH-0064, NIH, Bethesda, MD ; no authors have potential for material gain.

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