The use of the SBS for predicting outcomes (Version 1)
Research type
Research Study
Full title
The Use of the Skeletal Burden Score (SBS) for Predicting Outcomes in Children and Adults with Fibrous Dysplasia
IRAS ID
277033
Contact name
Amaka Offiah
Contact email
Sponsor organisation
Sheffield Children's Hospital
Duration of Study in the UK
1 years, 8 months, 27 days
Research summary
Fibrous Dysplasia (FD) is a rare-non inherited genetic disease, in which healthy bone cells are replaced by fibrous tissue.FD can cause bone pain, pathological fractures, scoliosis, bone deformity and functional disabilities. While there has been considerable research on the physiology of FD and the design of treatments, less attention given to studies that observe the impact of the disease burden on patients' functional outcomes and the effect of that on the quality of life. Skeletal severity and bone pain are the most consistent predictors of impaired quality of life. Skeletal severity can be quantified using a validated quantitative scoring tool called skeletal burden score (SBS) in which a high score indicates severe skeletal involvement. There are currently no reported studies that investigate the relationship between quality of life, the type of bone pain, and clinical changes in patients with FD through follow-up study over a short period.
This is a multi-centre study that includes retrospective and prospective FD patients of all ages from the United Kingdom and Saudi Arabia. The study is aiming to evaluate the relationship between SBS and other variable FD factors (quality of life, bone pain, clinical data) by following-up all subtypes of FD patient cohort for 12 months (short-term). Given that the study population will come from the UK and Saudi Arabia, evaluating the differences in the reported QoL domains from the two countries will aid in understanding the physical, social and mental impact of the disease from countries with different cultural backgrounds.REC name
East Midlands - Leicester South Research Ethics Committee
REC reference
21/EM/0094
Date of REC Opinion
17 May 2021
REC opinion
Further Information Favourable Opinion