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Osteogenesis imperfecta in childhood: perceived competence in relation to impairment and disability

R H Engelbert1, V A Gulmans, C S Uiterwaal

  • 1Department of Pediatric Physical Therapy, University Medical Center, Wilhelmina Children's Hospital, Utrecht, The Netherlands. R.Engelbert@wkz.azu.nl

Archives of Physical Medicine and Rehabilitation
|July 7, 2001
PubMed

Insights

Children with osteogenesis imperfecta (OI) show varying physical impairments but maintain positive self-perceptions. This study found no significant differences in perceived competence across different OI types, despite physical differences.

Area of Science:

  • Pediatric Orthopedics
  • Child Psychology
  • Genetics

Background:

  • Osteogenesis imperfecta (OI) is a genetic disorder characterized by brittle bones.
  • Children with OI experience a range of physical impairments affecting mobility and strength.
  • Understanding their psychological well-being, specifically perceived competence, is crucial for holistic care.

Purpose of the Study:

  • To assess the perceived competence of children with different types of osteogenesis imperfecta (OI).
  • To explore the relationship between perceived competence and physical impairment parameters in children with OI.

Main Methods:

  • A cross-sectional study was conducted with 40 children diagnosed with OI (Types I, III, and IV).
  • Evaluations included joint range of motion, muscle strength, functional skills (self-care, mobility, social function), ambulation, and perceived competence using the Harter Self-Perception Profile for Children.
  • Data were collected at a national referral center for OI treatment.

Main Results:

  • Children with Type III OI exhibited severe decreases in joint range of motion and muscle strength.
  • Type IV OI also showed decreased joint range of motion and muscle strength, particularly in lower extremities.
  • Despite physical impairments, children across all OI types reported generally positive perceived competence, with notable exceptions in athletic performance (Type I) and romance (Type III).
  • No significant correlations were found between physical parameters (joint ROM, muscle strength, functional skills) and perceived competence subscales.

Conclusions:

  • Physical impairments in children with OI correlate with disease severity and differ significantly across OI types.
  • Overall perceived competence in children with OI remains largely positive and does not significantly vary between different types of the disorder.
  • These findings highlight the resilience of children with OI in maintaining a positive self-view despite physical challenges.
Abstract

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