OHSU studying drug for bone-deforming disorder

PORTLAND, Ore. - Jimmy Fox isn't typical of a person with the genetic, "brittle bone" disorder osteogenesis imperfecta (OI). He lifts weights almost daily, participates in grueling wheelchair races, chops wood and enjoys hunting in rough backcountry.

Still, Fox, who owns a busy combination grocery store-gun shop in Cloverdale, Ore., is eager to participate in an Oregon Health & Science University-led study of a drug that may help reduce the debilitating effects of OI, an inherited disorder characterized by weak bones that break easily. People with the most severe forms of OI have short stature, can suffer hundreds if not thousands of fractures in a lifetime, and are confined to a wheelchair.

"I didn't think it could do me any harm," Fox, 40, said of the study. He has a less-severe form of OI, allowing him to be more mobile - he walks on his hands when he's not in his wheelchair - and suffer fewer fractures than many others with the disorder. Fox had both of his legs amputated when he was 18 due to continued fracturing. "The study might do me some good, so I thought I might as well do it and give it a try. It will help other people."

The alternative is unacceptable to Fox, who acknowledges that his significant upper-body strength has kept many of the disorder's most debilitating effects at bay. "They told me 'If you weren't as active as you are, you would be totally screwed up by now.' To be honest, that's what really swayed me to do this."

The study, headed by Eric Orwoll, M.D., professor of medicine (endocrinology, diabetes and clinical nutrition) and director of the Bone and Mineral Unit, OHSU School of Medicine, is examining the effectiveness of the synthesized parathyroid hormone, teriparatide, in increasing bone mass and improving bone structure in adults affected by OI. Teriparatide, derived from the human parathyroid gland, is manufactured and sold by Eli Lilly and Co. under the brand name FORTEO.

FORTEO already is appr

Contact: Jonathan Modie
Oregon Health & Science University

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