Department of Clinical Research & Leadership, School of Medicine and Health Sciences, George Washington University, Washington, DC, USA.
Comprehensive Sickle Cell Center, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Public Health Rep. 2019 Nov/Dec;134(6):599-607. doi: 10.1177/0033354919881438. Epub 2019 Oct 10.
Sickle cell disease (SCD) is an inherited blood disorder most common among African American and Hispanic American persons. The disease can cause substantial, long-term, and costly health problems, including infections, stroke, and kidney failure, many of which can reduce life expectancy. Disparities in receiving health care among African Americans and other racial/ethnic minority groups in the United States are well known and directly related to poor outcomes associated with SCD. As an orphan disease-one that affects <200 000 persons nationwide-SCD does not receive the research funding and pharmaceutical investment directed to other orphan diseases. For example, cystic fibrosis affects fewer than half the number of persons but receives 3.5 times the funding from the National Institutes of Health and 440 times the funding from national foundations. In this review, we discuss the health inequities affecting persons with SCD, describe programs intended to improve their care, and identify actions that could be taken to further reduce these inequities, improve care, control treatment costs, and ease the burden of disease.
镰状细胞病（SCD）是一种遗传性血液疾病，在非裔美国人和西班牙裔美国人中最为常见。该疾病会导致严重、长期且昂贵的健康问题，包括感染、中风和肾衰竭等，其中许多问题会降低预期寿命。在美国，非裔美国人和其他种族/族裔少数群体在获得医疗保健方面存在明显差异，这与 SCD 相关的不良结果直接相关。作为一种孤儿病——全国范围内影响不到 20 万人——SCD 没有得到与其他孤儿病相同的研究资金和药物投资。例如，囊性纤维化影响的人数不到其一半，但却获得了美国国立卫生研究院 3.5 倍的资金和国家基金会 440 倍的资金。在这篇综述中，我们讨论了影响 SCD 患者的健康不平等问题，描述了旨在改善其护理的计划，并确定了可以采取的行动，以进一步减少这些不平等，改善护理，控制治疗成本，并减轻疾病负担。
