Angelite Nurses: Osteogenesis Imperfecta

Osteogenesis Imperfecta

General information

An inherited disorder affecting collagen formation and resulting in pathologic fractures
Types

Osteogenesis imperfecta congenita: autosomal recessive, prognosis poor
Osteogenesis imperfecta tarda: autosomal dominant, less severe form, involvement of varying degrees

Classic picture includes soft, fragile bones; blue sclera; otosclerosis
Severity of symptoms decreases at puberty due to hormone production and child's ability to prevent injury

Medical management

Magnesium oxide supplements
Reduction and immobilization of fractures

Assessment findings

Osteogenesis imperfecta congenita

Multiple fractures at birth
Possible skeletal deformity due to intrauterine fracture
Bones of skull are soft
Occasional intracranial hemorrhage

Osteogenesis imperfecta tarda

Delayed walking, fractures, structural scoliosis as child grows
Lower limbs more frequently affected
Hypermobility of joints
Prone to dental caries

Nursing interventions

Support limbs, do not stretch.
Position with care; use blankets to aid in mobility and provide support.
Instruct parents in bathing, dressing, diapering.
Support parents; encourage expression of feelings of anger or guilt (parents may have been unjustly suspected of child abuse).

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