Authors: Jumana Nayef Al-Momani.
Background: Osteogensis imperfecta (IO) is a hereditary disorder characterized by bone fragility and progressive bone deformity; intravenous pamidronate had been used in the treatment of severe OI for almost 20 years, and founded to be useful in enhancing bone density and decreasing bone fracture incidence, but little studies were showed the functional outcomes after this infusion of these patients using validated measures.
Aim: This study was conducted to describe the functional outcomes of children with OI under pamidronate infusion therapy among Jordanian children.
Method: This is cross sectional study was conducted for 15 children above 3 years of their age in Queen Rania Hospitals for children / Jordan from February – July (2015), and they had been diagnosed with moderate or severe OI, the Pediatric Outcomes Data Collection Instrument (PODCI) was used in this study to evaluate the functions.
Result: There were four items measured according to the global score of pediatric outcome instrument, the upper extremities with physical function showed (mean 72.4, SD 23.61) transfer and basic mobility (mean 53.6, SD 31.09), sports with physical function (mean 49.6,SD 23.36), and pain / comfort scales (mean 37.3, SD 26.03).
Conclusion and Implication: Pamidronate was assisted for upper extremities, basic mobility, sports function and pain for OI children with total global score 53%,while basic mobility showed a significant correlation with this infusion (P< 0.05) correlation coefficient = (0.566). Further evaluation are needed to show the long term outcomes of this therapy according updated protocol measures. The quality of life of children with OI needs to be in concern for patients and their families to apply holistic and optimal health care.
View/Download pdf