BACKGROUND AND OBJECTIVES Although sickle cell disease (SCD)-related childhood mortality in the United States significantly improved in the 1990s, unclear is the trend in SCD-related mortality more recently given the continued disparities faced by this minoritized population. In this analysis, we aimed to (1) compare the overall and age-specific mortality rates from 1999 to 2009 vs 2010 to 2020 with a particular focus on the age of transition and (2) determine the most common causes of death for the US SCD population for 2010 to 2020. METHODS We analyzed publicly available data from the Centers for Disease Control and Prevention WONDER database, a compilation of national-level mortality statistics from 1979 to 2020 derived from death certificates compiled by the National Center for Health Statistics. We searched by all individuals of all ethnicities, sexes, and ages using the underlying cause of death. RESULTS The crude mortality rate for individuals with SCD for 2010 to 2020 was 1.6 per 1 000 000 individuals, which was significantly lower than the period 1999 to 2009 (crude rate 1.7 per 1 000 000, P < .0001). In addition, the mean age at mortality of those with SCD was older in 2010 to 2020 (43 years) versus 1999 to 2009 (39 years). However, there remains a significant increase in mortality rate in the 20 to 24 year age group versus 15 to 19 years (1.7 per 1 000 000 versus 0.7 per 1 000 000, P < .0001), corresponding with the age of transition from pediatric to adult centers. In addition, 39% of underlying causes of death were not caused by SCD, but rather primarily chronic conditions, including cardiovascular, cerebrovascular, malignancy, and renal disease. The study has several limitations mostly because of the imperfections of administrative data sources, including inaccuracies in diagnoses codes, risking over or undercounting. CONCLUSIONS Although the US SCD-related mortality rate continues to decrease, the age of transition to adult care is a particularly vulnerable time in the lives of this marginalized group. Innovative and expanded approaches to care are greatly needed.

中文翻译：

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镰状细胞病死亡率趋势：1979-2020 年。


背景和目标 尽管美国镰状细胞病 （SCD） 相关的儿童死亡率在 1990 年代显着改善，但鉴于这一少数族裔人群面临的持续差异，最近 SCD 相关死亡率的趋势尚不清楚。在本分析中，我们旨在 （1） 比较 1999 年至 2009 年与 2010 年至 2020 年的总体死亡率和年龄特异性死亡率，特别关注过渡年龄，以及 （2） 确定 2010 年至 2020 年美国 SCD 人群最常见的死亡原因。方法 我们分析了来自疾病控制和预防中心 WONDER 数据库的公开数据，该数据库是 1979 年至 2020 年国家级死亡率统计数据的汇编，来自国家卫生统计中心编制的死亡证明。我们使用潜在的死因检索了所有种族、性别和年龄的所有个体。结果 2010 年至 2020 年 SCD 患者的粗死亡率为每 1 000 000 人 1.6 人，显著低于 1999 年至 2009 年期间（粗死亡率 1.7/1 000 000，P < .0001）。此外，2010 年至 2020 年 （43 岁） 与 1999 年至 2009 年 （39 岁） 相比，SCD 患者的平均死亡年龄更大。然而，与 15 至 19 岁相比，20 至 24 岁年龄组的死亡率仍然显着增加（每 1 000 000 人中有 1.7 人对每 1 000 000 人中有 0.7 人，P < .0001），对应于从儿科过渡到成人中心的年龄。此外，39% 的潜在死亡原因不是由 SCD 引起的，而主要是慢性疾病，包括心血管、脑血管、恶性肿瘤和肾脏疾病。 该研究有几个局限性，主要是因为管理数据源的不完善，包括诊断代码不准确、存在高估或低估的风险。结论 尽管美国 SCD 相关死亡率持续下降，但过渡到成人护理的年龄是这一边缘化群体生活中特别脆弱的时期。非常需要创新和扩展的护理方法。