Arji Emmanuel Emenike, Eze Ujunwa Justina, Ezenwaka Gloria Oluchukwu, Kennedy Neil
School of Medicine, Dentistry and Biomedical Science, Queen's University Belfast, Belfast, UK.
Department of Family Medicine, WellSpan Good Samaritan Hospital, Lebanon, PA, USA.
SAGE Open Med. 2023 Sep 15;11:20503121231197866. doi: 10.1177/20503121231197866. eCollection 2023.
Sickle cell disease is a lifelong illness affecting millions of people globally, but predominantly burdensome in sub-Saharan Africa, where most affected children do not live to adulthood, despite available evidence-based interventions that reduce the disease burden in high-income countries.
We reviewed studies evaluating evidence-based interventions that decrease sickle cell disease-related morbidity and mortality among children living in sub-Saharan Africa. We used the Joanna Briggs scoping review methodological framework and grouped identified evidence-based interventions into preventative pharmacotherapeutic agents, newborn screening and comprehensive healthcare, disease-modifying agents, nutritional supplementation, systemic treatment, supportive agents and patient/carer/population education.
We included 36 studies: 18 randomized controlled trials, 11 observational studies, 5 before-and-after studies and 2 economic evaluation studies, with most of the studies performed in West African countries. Included studies suggest evidence-based interventions effectively to reduce the common morbidities associated with sickle cell disease such as stroke, vaso-occlusive crisis, acute chest syndrome, severe anaemia and malaria infection. Evidence-based interventions also improve survival among study participants. Specifically, our review shows hydroxyurea increases haemoglobin and foetal haemoglobin levels, a finding with practical implications given the challenges with blood transfusion in this setting. The feasibility of implementing individual interventions is hampered by challenges such as affordability, accessibility and the availability of financial and human resources.
Our review suggests that regular use of low-dose hydroxyurea therapy, sulphadoxine-pyrimethamine chemoprophylaxis, L-arginine and Omega-3 fatty acid supplementation and establishment of specialist stand-alone sickle cell clinics could reduce the sickle cell disease-associated morbidity and mortality in sub-Saharan Africa countries.
镰状细胞病是一种影响全球数百万人的终身疾病，但在撒哈拉以南非洲地区负担尤为沉重，尽管有循证干预措施可减轻高收入国家的疾病负担，但大多数受影响的儿童仍无法活到成年。
我们回顾了评估循证干预措施的研究，这些措施可降低撒哈拉以南非洲地区儿童镰状细胞病相关的发病率和死亡率。我们使用了乔安娜·布里格斯范围综述方法框架，并将已确定的循证干预措施分为预防性药物治疗剂、新生儿筛查和综合医疗保健、疾病改善剂、营养补充、全身治疗、支持性药物以及患者/护理人员/人群教育。
我们纳入了36项研究：18项随机对照试验、11项观察性研究、5项前后对照研究和2项经济评估研究，其中大多数研究在西非国家进行。纳入的研究表明，循证干预措施能有效降低与镰状细胞病相关的常见发病率，如中风、血管闭塞性危机、急性胸综合征、严重贫血和疟疾感染。循证干预措施还可提高研究参与者的生存率。具体而言，我们的综述表明羟基脲可提高血红蛋白和胎儿血红蛋白水平，鉴于在此环境下输血存在挑战，这一发现具有实际意义。实施个体干预措施的可行性受到诸如可负担性、可及性以及资金和人力资源可用性等挑战的阻碍。
我们的综述表明，定期使用低剂量羟基脲疗法、磺胺多辛 - 乙胺嘧啶化学预防、补充L - 精氨酸和ω - 3脂肪酸以及建立独立的镰状细胞专科诊所，可降低撒哈拉以南非洲国家镰状细胞病相关的发病率和死亡率。
