Effect of intravenous pamidronate therapy on everyday activities in children with osteogenesis imperfecta.

AIM: To evaluate the effect of intravenous pamidronate therapy on everyday activities, well-being, skeletal pain and bone density in children with osteogenesis imperfecta (OI).

METHODS

In a prospective observational study, monthly intravenous pamidronate infusions were given to 43 children (aged 4 months-16 years) with different severity and form of OI. Outcome measures included the Pediatric Evaluation of Disability Inventory (PEDI), a score of well-being, registration of days with pain and bone density measured by using dual energy X-ray absorptiometry (DXA). Data were collected before and after the first year of treatment.

RESULTS

Self-care and mobility measured by PEDI increased significantly in both the Functional Skill scale and in the Caregiver Assistance scale. The youngest children improved more than the older ones according to the PEDI. The days with skeletal pain were reduced and both well-being and bone density increased.

CONCLUSIONS

Intravenous treatment with bisphosphonates influenced health status, according to the International Classification of Functioning, Disability and Health (ICF). This group of 43 children experienced beneficial effects on everyday activities, skeletal pain, well-being and bone density.