osteogenesis imperfecta

• brittle bone disease
• spectrum of disease from I-VIII, a rare genetic disorder that affects the formation and strength of bones
• have bones that fracture easily, often with little or no obvious cause, most kids with this disorder can reach full potential
• Dentinogenesis imperfect may also occur
• nursing assessment: family history of osteogenesis imperfect, pattern of fractures, screening associated with routine care and handling of the newborn, eyes may have bleeding, purple, or grey tint, abnormalities of the primary teeth, joint hyper mobility with active ROM
• lab results may include a skin biopsy(reveals abnormalities in type 1 collagen) or DNA testing(locate the genetic mutation)
• rule out abise for fractures under 1 year
• nursing management: handle child carefully, teach family to avoid trauma
• promote fin motor skills and independence in ADL's
• injury prevention: never push or pull an arm or leg, do not bend limb into awkward position, lift baby carefully, do not lift from the armpits, provide supported positioning, if fracture suspected, handle the limb minimally