TREATMENT FOR OSTEOGENESIS IMPERFECTA:
The goals of therapy are to reduce fracture rates, prevent deformities, minimize pain, and maximize functional abilities. Treatment requires a multidisciplinary approach. Many of the treatment options that can be used are listed below:
- Maintain a healthy lifestyle
- Exercises as tolerated
- Avoid smoking
- Treat bone infections when they occur
- Bisphosphonates (are a class of drugs that are used to treat and prevent the loss of bone mass and are commonly used to treat osteoporosis)
- Calcium and Vitamin D
- Pain medication
- Care for broken bones: surgery, casting, or splinting
- Spinal fusions for scoliosis
- Surgery can include management of fractures or implantation of metal rods through the long bones to strengthen them
- Telescoping rods that grow along with the child are available
- Occupational & physical therapy to increase muscle strength
- Hydrotherapy ( pool therapy). This is used for strengthening because it has a lower risk for injury
- Custom splints and braces to support the fragile bones
- Support cushions to improve posture and change pressure points
- Customized wheelchairs
- Assessment for the need for assistive devices for ambulation
- Assessment for the need for adaptive equipment for ADLs
Case example:
Indria’s initial exam: Diagnosis is Osteogenesis Imperfecta, however, her exact type was not determined due to the lack of clinic testing available at the missions hospitals. Her clinical exam showed she had a right humeral fracture. Her mother reported that no trauma had occurred. X-Rays confirmed the humeral fracture and revealed a hairline fracture in her left arm and multiple fractures in her left leg. Indria had been wearing a leg brace with an elevated shoe due to the shortened long bones in her leg. She had been experiencing pain in her left lower extremity for years. Notice that Indria does not have tinted sclera.
Indria also has an undescended left scapula
Without the walking brace, Indria’s leg showed a bone shortening deformity and was very painful to move.
A custom elbow splint was made for Indria’s left arm hairline fracture for support. She can wear this splint as needed for pain and comfort.
Indria had surgery for her right arm. The Pediatric surgeon implanted a rod through the center of her humerus to realign the fracture and then placed her in a long arm cast. “Rodding” is the placement of an intramedullary rod into the internal cavity of a long bone under X-Ray guidance. “Rodding” is used for fractures that won’t heal or for moderate to severe OI cases. “Rodding” is preferred over plates for the treatment of OI.
While recovering, Indria asked the Occupational therapist to make a special splint for her left leg that would be comfortable for her to wear when she is in bed.
Indria was able to go home with less pain and very happy with her new pink leg splint.
