Assessment of End-Organ Damage Among Elderly Patients with Sickle Cell Anemia: A Single-Center Retrospective Study

• Published in: Blood 2024-11, Vol.144 (Supplement 1), p.5343-5343
• Main Authors: Kannan, Preethi, Soliman, Sara, Dambala, Nehaa, Idowu, Modupe
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2024-203820

Background: The cause of death in adulthood for patients with sickle cell anemia (SCA) is often end organ damage leading to multiorgan failure. The expected lifespan for patients is over two decades less than that of the general population. With continuing advancements in SCA disease-modifying therapies (DMTs), more patients are surpassing this threshold. However, little is known about the characteristics of patients in this unique age cohort. This retrospective study assesses the severity of end-organ damage among SCA patients living beyond the expected lifespan. Methods: This retrospective single-center study reviewed all patients ≥ 60 years old with SCA genotype HbSS treated at the UT Physicians Comprehensive Sickle Cell Center between January and June 2024. A total of 16 patients were included in this study. The most recently recorded demographic, clinical, and laboratory data were collected and analyzed using descriptive statistics. Laboratory values were compared to corresponding normal ranges published by the Centers for Disease Control and Prevention (CDC). Mean hemoglobin, total bilirubin, and reticulocyte count were used to assess SCA severity. ALT was included to assess liver function. Creatinine was used to assess kidney function. Chamber dilation, maximum tricuspid regurgitation velocity (TRV max), and ejection fraction (EF) were collected from echocardiograms to assess heart function. Five patients had a brain MRI from which significant findings were collected to assess ischemic changes. Medical history was reviewed to collect information on co-morbidities and further assess end-organ damage. Demographic Results: This study included 16 SCA patients ≥ 60 years old, of which 15 were female (94%) and one was male. The average age of patients was 67.7 ± 2.1 years. All patients included in this study were of African and/or African American ethnicity. 56% of patients had never used tobacco products, while 31% were former smokers and 13% were current smokers. 69% of patients had used at least one of three DMTs (hydroxyurea, Oxbryta, L-glutamine), while the remaining 31% managed their disease without DMT. 73% of patients on a DMT used hydroxyurea. Three patients were prescribed Oxbryta, but two discontinued due to intolerance. 25% of patients had chronic transfusions. All patients who did not use DMT had hereditary persistence of fetal hemoglobin (HPFH). Clinical Results: The average BMI for patients included in this study was 23.48 ± 2.61 kg/m2. 63%