1. University of Puget Sound
O.T. for O.I.
Evidence-Based Practice: Effective Occupational Therapy Treatments for Children with Osteogenesis Imperfecta
Michele Cheng
University of Puget Sound
Symposium, December 8, 2003

2. Overview About O.I. Evidence-Based Question Current Evidence
Implications for children with O.I., occupational therapists, and researchers
Recommendations for Best Practice

3. What is O.I.? Incidence: 1 in 20,000 live births Inherited disorder
Abnormal synthesis of type I collagen that forms the framework for bones, tendons, ligaments
No cure

4. Classifications of O.I. O.I. Types Characteristics I * mild
occasional bone fractures, minimal bone deformity, & normal stature
II *
most severe
fractures in utero lead to perinatal death
III *
severe
frequent fractures, severe deformities, triangular face, short stature

5. Classifications of O.I., continued
O.I. Types
Characteristics
IV *
moderate deformities and dwarfism
V **
 incidence of hypertrophic calluses & early ossification of interosseous membrane of forearms / legs
* Sillence, D. O. (1979)
** Glorieux, F. H. (1999)

6. Additional Impairments
Scoliosis
Laxity of ligaments
Blue sclerae
Dentinogenesis imperfecta
Loss of hearing
Hernias
Easy bruising
Excessive sweating

7. WANTED: Occupational Therapists for children with O.I.

8. Evidence-Based Practice Question:
What current evidence exists regarding effective occupational therapy treatments for children with osteogenesis imperfecta?

9. Criteria for Evidence Selection
Types of Studies
Published in the last 10 years .
Published in English.
Study Designs
Quantitative Research Designs
Qualitative Research Designs
Opinions of respected practitioners

10. “Rehabilitation Approaches to Children with O.I.: A Ten-Yr Exp”
Authors: MDs, PT
Intervention According to Functional Ability:
Posture exercises
Active ROM and strengthening
Therapeutic water activities
Developmental progression
Coordination activities
Results: Slow, continued functional improvement in most out of 25 children

11. “Craig Gets Mobile!” Authors: PTs Individual Intervention : Results:
Power Mobility Options
Aquatic Therapy
Methods for sitting & playing
Results:
Dynamic finger steering device
Long-leg sitter
 mobility & exercise opportunities

12. “Chapter 12: Osteogenesis Imperfecta”
Authors: PTs
Outcomes:
Functional independence
Play
Adjustment to school
Intervention:
Infants
Preschool aged children
School-aged children

13. “Therapeutic Strategies for O.I.”
Based on work of OTs, PTs, MD
Intervention:
Positioning & Handling
Maximize or maintain function
Education of families
Adaptive devices
Energy conservation
Joint Protection
Aquatic activities
Reduce fear of movement & trying new skills

14. “Rehabilitation & Functional Outcome in O.I.”
Author: Specialist in Pediatric Rehab
Intervention:
Prevent immobilization osteoporosis
Promote weight bearing to  bone strength
Reduce bone pain
Results:
 stamina
↓ bone pain & fatigue
 muscle strength

15. “Rehabilitation of Children & Infants with O.I.”
Author: MD
Intervention:
Water Sports
Throwing & tossing balls
Playground activity
Wheelchair aerobics
Results:
Recreational activities promote feelings of competence, fitness, well-being

16. Summary of Evidence Level III IV Reference Study Design
Level of Evidence
Binder et al. (1993)
Case-Control Study
Level
III
Paleg et al. (2002)
Case Study IV
Bleakney et al. (1995)
O.I. Chapter in P.T. Book

17. Summary of Evidence, continued
Reference
Study Design
Level of Evidence
Dollar, E. P.
Guidelines for OTs from The O.I. Foundation
Level IV
Ault, J. (1999)
Lecture at 7th International Conf. on O.I.
Gerber, L. H. (1999)
IV-V

18. Benefits for Children with O.I.
Prevention of irreversible deformities and disability
 mobility
 ADL skills
Adjustment to Environment
 Participation Level

19. Implications for Practitioners
“Little data are available to help devise a sensible, safe, and effective program for recreational activities” (Gerber, 1999).
Higher levels of current evidence to support practice in treating children with O.I. are needed.

20. Implications for Researchers
Research about surgical, medical, drug treatments suggest therapy to maintain functional ability
Level I and II evidence needed regarding effective occupational therapy treatments

21. Recommendations for Best Practice
Awareness & Judgment of Evidence
Focus on child’s individual abilities, strengths, and limitations unique to child rather than O.I. type
Multidisciplinary team
Creative Problem Solving
Listen to the children with O.I. and their families