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Updated: Aug 19, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Treatment patterns and burden of complications associated with sickle cell disease: A US retrospective claims
Deepa Manwani1, Arthur L Burnett2, Jincy Paulose3
1Albert Einstein College of Medicine The Children's Hospital at Montefiore The Bronx New York USA.
Insights
Sickle cell disease (SCD) patients experience frequent complications like kidney disease and stroke, increasing with age. Current treatments show unmet needs for managing this high patient burden.
Area of Science:
- Hematology
- Nephrology
- Neurology
Background:
- Sickle cell disease (SCD) is linked to severe complications including priapism, kidney disease, and neurological damage.
- These SCD-related complications significantly impact patient quality of life but have been historically under-researched.
Purpose of the Study:
- To analyze the incidence of key SCD complications.
- To examine SCD treatment patterns and healthcare resource utilization (HCRU).
- To assess healthcare costs associated with SCD complications in pediatric and adult patients.
Main Methods:
- Retrospective analysis of US administrative claims data from July 2013 to March 2020.
- Inclusion of 2524 pediatric and adult patients diagnosed with sickle cell disease.
- Evaluation of complication incidence, treatment modalities, HCRU, and associated costs.
Main Results:
- High incidence rates for acute kidney injury (53.1), chronic kidney disease (40.6), and stroke (39.0) per 1000 person-years.
- Complication frequency escalates with age, with acute kidney injury being significantly more common in older adults.
- Inpatient stays were the primary driver of high healthcare costs across all age groups.
Conclusions:
- Patients with sickle cell disease face a substantial burden of complications across all ages.
- The frequency of complications increases with age, highlighting age-specific risks.
- Current treatment strategies for SCD appear insufficient, indicating a need for improved management approaches.
Abstract:
Complications associated with sickle cell disease (SCD) that are highly impactful for patients but until recently have been less understood include priapism, nephropathy, and neurologic injury. We conducted a retrospective study using US administrative claims data from July 01, 2013 through March 31, 2020 to analyze incidence of these complications, SCD treatment patterns, and healthcare resource utilization (HCRU) and costs among 2524 pediatric and adult patients with SCD (mean [SD] age 43.4 [22.4] years). The most common treatments during follow-up were short-acting opioids (54.0% of patients), red blood cell transfusion (15.9%), and hydroxyurea (11.0%). SCD complications occurred frequently; in the overall population, the highest follow-up incidences per 1000 person-years were for acute kidney injury (53.1), chronic kidney disease (40.6), and stroke (39.0). Complications occurred across all age groups but increased in frequency with age; notably, acute kidney injury was 69.7 times more frequent among ages 65+ than ages 0-15 (p < 0.001). Follow-up per-patient-per-month HCRU also increased with age; however, all-cause healthcare costs were similarly high for all age groups and were driven primarily by inpatient stays. Patients with SCD across the age spectrum have a high burden of complications with the use of current treatments, suggesting unmet needs for treatment management.
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