Sickle Cell Disease (SCD) Bone Pain Study

A prospective study to determine how low bone mineral density and/or vertebral compression fractures associate with pain in adults with sickle cell disease

The investigators hypothesize that adults with sickle cell disease (SCD) and low bone density and/or vertebral compression fractures on a dual X-ray absorptiometry (DXA) scan (adjusted for age, sex, SCD genotype, relevant labs, presence of osteonecrosis, and SCD-modifying therapies) will report more severe pain than those with normal bone density or no vertebral fractures. The Adult Sickle Cell Quality of Life Measurement System (ASCQ-Me) is a validated patient-reported outcome measure of physical, mental, and social health in adults with SCD. This cross-sectional observational study involves obtaining a baseline DXA scan, vertebral fracture analysis (VFA) and pain assessment using ASCQ-Me pain impact scores. The investigators plan to recruit 50 adults with SCD followed at University of California Davis Medical Center between Nov 2022- Oct 2023, and anticipate enrolling 4-6 adults with SCD per month. The study endpoints are listed below: To determine the association between bone density Z-scores and ASCQ-Me pain impact scores in a prospective cohort of adults with SCD To study the association between Spine Deformity Index scores (SDI, a proxy for vertebral fracture analysis) and ASCQ-Me pain impact scores in a prospective cohort of adults with SCD To assess the correlation between baseline hematological and biochemical laboratory parameters (including bone biomarkers), bone density, and/or vertebral fractures in a prospective cohort of adults with SCD The investigators' goal is to complete primary data analysis by Mar 2024. As an exploratory endpoint, 1cc of serum and 5cc of urine will be collected from each study participant once (at baseline), after an overnight fast, for bone biomarker analyses.

Sickle Cell Disease, Sickle Cell Anemia, Low Bone Density, Osteoporosis, Osteopenia, Vertebral Fracture, Vertebral Compression, Osteonecrosis, Ischemic Necrosis, Avascular Necrosis

Prospective cohort of 50 adults with sickle cell disease (SCD) undergoing research DXA scan to assess bone mineral density and thoracolumbar morphometry for vertebral fracture analysis

Obtain thoracolumbar morphometry in DXA scan, then determine presence and severity of vertebral compression fractures by VFA

Calculate patient-reported total pain scores to determine the pain phenotype of each study participant

Patient-reported outcome measure of pain level in the past 7 days. The ASCQ-Me pain scale ranges from 0-100, with a standardized sickle cell disease population mean of 50 (standard deviation=10), where lower scores signify worse disease impact.

Inclusion Criteria: Age 18-80 years with SCD (any genotype, confirmed by hemoglobin electrophoresis or high performance liquid chromatography) Ability to provide written informed consent Ability to lay on a DXA scanner Negative urine pregnancy test for women of childbearing potential at study entry Exclusion Criteria: Pregnant women Adults unable to consent Individuals who are not yet adults (infants, children, teenagers) Prisoners Hospitalizations (any cause) within 2 weeks of study entry