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Mobility challenges and solutions for fibrodysplasia ossificans progressiva
C Levy1, T F Berner, P S Sandhu
1Department of Physical Medicine and Rehabilitation, The Ohio State University Medical Center, Columbus, USA.
Insights
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder causing progressive bone formation in soft tissues. This case study highlights adaptive equipment and physiatric interventions enabling an individual with advanced FOP to maintain employment and independence.
Area of Science:
- Medical Genetics
- Rehabilitation Medicine
- Skeletal Dysplasias
Background:
- Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive heterotopic ossification of soft tissues.
- Ectopic bone formation typically begins in early childhood, primarily affecting axial musculature and progressing to ligaments, tendons, and joint capsules.
Observation:
- This report details a 30-year-old woman with advanced FOP, presenting with a fused spine, fixed pelvis, and severe joint contractures in all limbs.
- The patient experienced profound mobility limitations, rendering her unable to stand or sit independently.
Findings:
- A modified power wheelchair and custom footwear were successfully implemented, enabling the patient to continue her employment as a preschool teacher.
- Creative physiatric interventions were crucial in adapting to the patient's functional limitations.
Implications:
- This case underscores the importance of innovative assistive technologies and tailored rehabilitation strategies for individuals with severe FOP.
- Such interventions can significantly enhance quality of life, promote independence, and facilitate continued participation in meaningful activities for those with rare genetic disorders.
Abstract:
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive soft tissue ossification. Although signs may be present at birth, the first appearance of ectopic bone typically occurs in early childhood. The primary target is the axial musculature. Eventually ectopic bone also occurs in ligaments, fascia, aponeurosis, tendons, and joint capsules of the appendicular skeleton with a proximal to distal predilection. As the disease advances, mobility becomes restricted, and affected individuals are typically limited to bed or chair by their early 30s. This report describes a 30-year-old woman with advanced FOP. She had a fused spine and a fixed pelvis, with hips and knees locked in flexion and feet in plantarflexion. Her upper limb mobility was similarly restricted. She was not able to stand upright or sit independently. The modification of a commercially available power wheelchair that allowed the patient to maintain her employment as a preschool teacher and custom shoes are described. Creative physiatric intervention is essential to liberate human potential for people with FOP.
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