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What is the treatment of Type I Osteogenesis Imperfecta?

By  ,  National Institute of Health
Jan 12, 2013
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Managing and Treating Type I OI in Children


The cornerstones of treatment for children with Type I OI are fracture management, healthy diet, therapy to regain strength and mobility after fractures or surgery, and an ongoing program of safe exercise and activity to develop muscle control and build strength. Recognizing that prolonged immobilization can weaken muscles and bones, many orthopaedists prefer short-term casting for fractures, followed as soon as possible by a splint or brace that can be removed for appropriate exercise.


For all children who have had a fracture, physical therapy, including water exercise, will reduce the effects of immobilization. This is recommended for even the most mildly affected children. Inactivity and inappropriate diet should not be permitted to cause obesity, because excess weight adds stress on the bones and tends to limit activity even more. Developing healthy lifestyle habits is an important part of managing OI.


Rodding surgery (in which metal rods are inserted into the long bones) is a standard treatment for children with OI in two situations:

  • to set a particularly bad fracture
  • to straighten and strengthen a bone that is bowed (curved) enough to break repeatedly.

Many children with Type I OI have minimal bone deformity and do not require rodding surgery unless they have a particularly bad fracture. However, some children with Type I OI do have problems with repeated fractures and increasing deformity of a long bone. In such cases, rodding surgery may be appropriate.


Some infants with mild OI have delays in gross motor skills, such as pulling to a stand, crawling, or walking. These delays may be caused by fractures, low muscle strength, loose joints, or a child’s fear of movement because of previous fractures. Physical and occupational therapy are recommended as soon as such delays are noticed. Therapists can instruct parents in the best ways to hold or position their children, and to encourage children to learn new skills. Most infants with OI will qualify for their state’s early intervention program, which provides therapy and other services free of charge.


Older children with Type I OI also will benefit from physical and occupational therapy to maximize strength and function. In addition to therapy, regular exercise geared toward children’s individual interests helps them to socialize with their peers, develop bone and muscle strength, and maintain a healthy weight. Water therapy and swimming are particularly good exercises for children with OI, as the gravity-free environment reduces fracture risk. Many children with Type I OI swim, dance, ride regular or adapted bikes, and participate in other recreational and competitive activities.


For long-term health and comfort, it is important to develop the habit of protecting the spine. Back pain or compression fractures of the spine can be minimized both by avoiding activities that jolt or twist the spine and by using proper techniques for lifting, reaching, or sitting. It is also wise to protect the joints. Hyperlaxity (being double-jointed) can be amusing in childhood, but it can lead to painful arthritis in adulthood. A child’s doctor or therapist can advise the family on safe exercises.

 

 

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