Division of Hematology/Oncology, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Department of Pediatrics, Warren Alpert Medical School of Brown University, Providence, Rhode Island.
Pediatrics. 2024 Dec 1;154(6). doi: 10.1542/peds.2024-067341.
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.
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.
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.
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.
尽管美国镰状细胞病（SCD）相关儿童死亡率在 20 世纪 90 年代显著下降，但由于少数族裔群体仍面临持续的不平等，最近 SCD 相关死亡率的趋势尚不清楚。在这项分析中，我们旨在：（1）比较 1999 年至 2009 年与 2010 年至 2020 年的总体和特定年龄死亡率，特别关注过渡年龄；（2）确定 2010 年至 2020 年美国 SCD 人群的最常见死因。
我们分析了疾病控制和预防中心 WONDER 数据库中的公开数据，该数据库是国家一级死亡率统计数据的汇编，源自国家卫生统计中心编制的死亡证明。我们使用根本死因对所有种族、性别和年龄的个人进行了搜索。
2010 年至 2020 年，SCD 个体的粗死亡率为每 100 万人 1.6 人，明显低于 1999 年至 2009 年期间（粗死亡率为每 100 万人 1.7 人，P<0.0001）。此外，SCD 患者的平均死亡年龄在 2010 年至 2020 年期间（43 岁）较 1999 年至 2009 年期间（39 岁）更大。然而，在 20 至 24 岁年龄组中，死亡率仍显著增加，与从儿科到成人中心的过渡年龄相对应（每 100 万人 1.7 人，而非 15 至 19 岁每 100 万人 0.7 人，P<0.0001）。此外，39%的根本死因并非由 SCD 引起，而是主要由慢性疾病引起，包括心血管疾病、脑血管疾病、恶性肿瘤和肾脏疾病。该研究存在一些局限性，主要是由于行政数据来源的不完美，包括诊断代码不准确，存在过度或低估的风险。
尽管美国 SCD 相关死亡率继续下降，但向成人护理过渡的年龄是这个边缘化群体生命中的一个特别脆弱时期。非常需要创新和扩展的护理方法。
