Osteogenesis imperfecta medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
At present there is no cure for OI. Treatments are aimed at increasing overall bone strength to prevent fracture and maintain mobility.
There have been many clinical trials done with the drug, Fosamax, a drug used to treat women experiencing brittleness of bones due to osteoporosis. More success was seen in the pill form versus the IV form, but success was still seen. The FDA will not approve Fosamax as a treatment for OI because long term effects of the drug have not been studied.
Bone infections are treated as and when they occur with the appropriate antibiotics and antiseptics.
Physiotherapy
Physiotherapy used to strengthen muscles and improve motility in a gentle manner, while minimizing the risk of fracture. This often involves hydrotherapyand the use of support cushions to improve posture. Individuals are encouraged to change positions regularly throughout the day in order to balance the muscles which are being used and the bones which are under pressure.
Children often develop a fear of trying new ways of moving due to movement being associated with pain. This can make physiotherapy difficult to administer to young children.
Physical aids
With adaptive equipment such as crutches, splints, grabbing arms, and/or modifications to the home many individuals with OI can obtain a significant degree of autonomy.
Bisphosphonates
Bisphosphonates (BPs), particularly those containing nitrogen, are being increasingly administered to increase bone mass and reduce the incidence of fracture. BPs can be dosed orally (e.g. alendronate) or by intravenous injection/infusion (e.g. pamidronate,[1] zoledronic acid).
BP therapy is being used increasingly for the treatment of OI. It has proven efficiency in reducing fracture rates in children,[2] however only a trend towards decreased fracture was seen in a small randomized study in adults.[3] While decreasing fracture rates, there is some concern that prolonged BP treatment may delay the healing of OI fractures, although this has not been conclusively demonstrated.
Pamidronate is an approved treatment for osteogenesis imperfecta in Canada, but not yet in the United States. Marketed under the brand nameAredia®, Pamidronate is usually administered as an intravenous infusion, lasting about three hours. The therapy is repeated every three to six months, and lasts for the life of the patient. Common side effects include bone pain, low calcium levels, nausea, and dizziness.
References
- ↑ Glorieux FH, Bishop NJ, Plotkin H, Chabot G, Lanoue G, Travers R (1998). "Cyclic administration of pamidronate in children with severe osteogenesis imperfecta". N. Engl. J. Med. 339 (14): 947–52. PMID 9753709.
- ↑ DiMeglio LA, Peacock M (2006). "Two-year clinical trial of oral alendronate versus intravenous pamidronate in children with osteogenesis imperfecta". J. Bone Miner. Res. 21 (1): 132–40. doi:10.1359/JBMR.051006. PMID 16355282.
- ↑ Chevrel G, Schott AM, Fontanges E; et al. (2006). "Effects of oral alendronate on BMD in adult patients with osteogenesis imperfecta: a 3-year randomized placebo-controlled trial". J. Bone Miner. Res. 21 (2): 300–6. doi:10.1359/JBMR.051015. PMID 16418786.