Afranie-Sakyi Jennifer A, Randall Eldrida, Fasano Ross, McLemore Morgan L, El Rassi Fuad
Department of Internal Medicine, Morehouse School of Medicine, Atlanta, Georgia, USA.
Section of Hematology, Department of Medicine, Yale School of Medicine, New Haven, Connecticut, USA.
Eur J Haematol. 2025 Apr;114(4):663-671. doi: 10.1111/ejh.14360. Epub 2025 Jan 2.
Sickle cell disease (SCD) is the most common hemoglobinopathy in North America. The life expectancy of SCD has extended into adulthood with screenings, preventative care, and hydroxyurea. However, comorbidities arise as adults with SCD age, leading to early mortality.
We conducted a retrospective chart review of the Georgia Comprehensive Sickle Cell Center at Grady Health System, analyzing records of deceased SCD patients from 2013 to 2020.
Amongst the 72 patients analysed, majority had severe complications from SCD and at least 1 cardiovascular comorbidity. The median age of death was 44 (STD = 15.5) for all genotypes with the median age of death at 39 (STD = 14.26) for SS and Sβ0 genotypes (n = 51). There was no difference in the median age of death for patients who maintained regular clinic visits (a visit in the last 6 months prior to death) compared to those who did not. Despite hydroxyurea's known benefits in reducing SCD morbidity and mortality, less than 50% of patients had a prescription.
As new therapies are approved, their impact on SCD-related morbidity and mortality must be evaluated. Improving access to, and education about, disease-modifying therapies like hydroxyurea for both patients and clinicians is essential to improving outcomes.
镰状细胞病（SCD）是北美最常见的血红蛋白病。随着筛查、预防性护理和羟基脲的应用，SCD患者的预期寿命已延长至成年期。然而，随着成年SCD患者年龄的增长，会出现合并症，导致早期死亡。
我们对格雷迪健康系统的佐治亚综合镰状细胞中心进行了回顾性病历审查，分析了2013年至2020年期间已故SCD患者的记录。
在分析的72例患者中，大多数患者患有SCD的严重并发症和至少一种心血管合并症。所有基因型的中位死亡年龄为44岁（标准差=15.5），SS和Sβ0基因型（n=51）的中位死亡年龄为39岁（标准差=14.26）。与未定期就诊的患者相比，在死亡前最后6个月内有就诊记录的患者的中位死亡年龄没有差异。尽管羟基脲在降低SCD发病率和死亡率方面有已知的益处，但只有不到50%的患者有处方。
随着新疗法的获批，必须评估它们对SCD相关发病率和死亡率的影响。改善患者和临床医生对羟基脲等疾病修饰疗法的获取和教育，对于改善治疗结果至关重要。
