Sickle cell bone disease and response to intravenous bisphosphonates in children

• Published in: Osteoporosis international Vol. 33; no. 11; pp. 2397 - 2408
• Main Authors: Grimbly, C., Escagedo, P. Diaz, Jaremko, J. L., Bruce, A., Alos, N., Robinson, M. E., Konji, V. N., Page, M., Scharke, M., Simpson, E., Pastore, Y. D., Girgis, R., Alexander, R. T., Ward, L. M.
• Format: Journal Article
• Language: English
• Published: London Springer London 01.11.2022; Springer Nature B.V
• Subjects: Age; Analgesia; Avascular necrosis; Bisphosphonates; Bone diseases; Bone imaging; Bone mineral density; Children; Computed tomography; Dual energy X-ray absorptiometry; Endocrinology; Humerus; Inflammatory diseases; Intravenous administration; Magnetic resonance imaging; Medicine; Medicine & Public Health; Morbidity; Musculoskeletal diseases; Myositis; Original; Original Article; Orthopedics; Osteoporosis; Pain; Pain perception; Pediatrics; Rheumatology; Sickle cell disease; Vertebrae; Avascular necrosis; Myositis; Bisphosphonate; Bone infarcts; (4–6): children; Sickle cell disease
• ISSN: 0937-941X; 1433-2965
• DOI: 10.1007/s00198-022-06455-2

Summary Children with sickle cell disease (SCD) have the potential for extensive and early-onset bone morbidity. This study reports on the diversity of bone morbidity seen in children with SCD followed at three tertiary centers. IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications. Introduction To evaluate bone morbidity and the response to intravenous (IV) bisphosphonate therapy in children with SCD. Methods We conducted a retrospective review of patient records from 2003 to 2019 at three Canadian pediatric tertiary care centers. Radiographs, magnetic resonance images, and computed tomography scans were reviewed for the presence of avascular necrosis (AVN), bone infarcts, and myositis. IV bisphosphonates were offered for bone pain management. Bone mineral density was assessed by dual-energy X-ray absorptiometry (DXA). Results Forty-six children (20 girls, 43%) had bone morbidity at a mean age of 11.8 years (SD 3.9) including AVN of the femoral (17/46, 37%) and humeral (8/46, 17%) heads, H-shaped vertebral body deformities due to endplate infarcts (35/46, 76%), and non-vertebral body skeletal infarcts (15/46, 32%). Five children (5/26, 19%) had myositis overlying areas of AVN or bone infarcts visualized on magnetic resonance imaging. Twenty-three children (8/23 girls) received IV bisphosphonate therapy. They all reported significant or complete resolution of bone pain. There were no reports of sickle cell hemolytic crises, pain crises, or stroke attributed to IV bisphosphonate therapy. Conclusion Children with SCD have the potential for extensive and early-onset bone morbidity. In this series, IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications.