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Updated: Sep 3, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Sickle cell bone disease and response to intravenous bisphosphonates in children
C Grimbly1,2, P Diaz Escagedo3, J L Jaremko4
1Department of Pediatrics, University of Alberta, 4-584 Edmonton Clinic Health Academy, 11405 - 87 Ave, Edmonton, AB, T6G 2R7, Canada. cgrimbly@ualberta.ca.
Insights
Children with sickle cell disease experience significant bone problems early in life. Intravenous bisphosphonates effectively treated bone pain without causing sickle cell complications.
Area of Science:
- Pediatric Hematology
- Pediatric Orthopedics
- Skeletal Diseases
Background:
- Sickle cell disease (SCD) frequently leads to early-onset bone morbidity in children.
- Bone complications in pediatric SCD patients can be extensive and diverse.
- Evaluating bone health and treatment responses is crucial for managing SCD.
Purpose of the Study:
- To assess the spectrum of bone morbidity in children with SCD.
- To evaluate the efficacy and safety of intravenous (IV) bisphosphonate therapy for bone pain in pediatric SCD.
- To investigate the impact of IV bisphosphonates on sickle cell disease complications.
Main Methods:
- Retrospective review of 46 children with SCD from three Canadian tertiary care centers (2003-2019).
- Radiographic, MRI, and CT imaging used to identify avascular necrosis (AVN), bone infarcts, and myositis.
- Assessment of bone mineral density via DXA and analysis of IV bisphosphonate treatment for bone pain.
Main Results:
- High prevalence of bone morbidity, including AVN (femoral/humeral heads), vertebral body deformities (H-shaped), and skeletal infarcts.
- Myositis observed in a subset of patients overlying areas of bone damage.
- IV bisphosphonate therapy in 23 children led to significant or complete resolution of bone pain without triggering sickle cell crises or stroke.
Conclusions:
- Children with SCD are susceptible to widespread and early-onset bone morbidity.
- IV bisphosphonates demonstrate effectiveness in managing bone pain associated with SCD.
- The study indicates that IV bisphosphonates are safe and do not exacerbate sickle cell disease complications.
Abstract:
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.
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