Improving Sickle Cell Disease Care in Mali through Engagement with a Pan-African Sickle Cell Disease Network.

通过与泛非镰状细胞病网络的合作，改善马里的镰状细胞病护理。

基本信息

批准号：
10402805
负责人：
ALDIOUMA GUINDO
金额：
$ 16.02万
依托单位：
UNIV OF SCIENCES, TECH & TECH OF BAMAKO
依托单位国家：
美国
项目类别：
财政年份：
2021
资助国家：
美国
起止时间：
2021-05-11 至 2026-04-30
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/10402805
关键词：
Acute Address Africa Africa South of the Sahara African Age Antibiotics Antimalarials Bacterial Infections Caring Chemoprophylaxis Child Clinical Clinical Data Clinical Protocols Communicable Diseases Communities Counseling Data Data Collection Development Diagnosis Diagnostic Services Education Educational Intervention Enrollment Ensure Etiology Evidence based intervention Face Family Fetal Hemoglobin Fever Fingers Future Geography Goals Health Health Personnel Heart Diseases Hematological Disease Heterozygote Incidence Individual Infection Inherited Institutional Review Boards Kidney Diseases Life Lifting Longitudinal cohort Longitudinal cohort study Malaria Mali Measures Methods Molecular Diagnosis National Heart, Lung, and Blood Institute Neonatal Screening Observation in research Observational Study Ontology Outcome Outpatients Pain Pain management Patients Persons Population Predisposition Preventive Preventive care Process Prophylactic treatment Protocols documentation Proverb Quality of Care Registries Research Risk Safety Services Sickle Cell Sickle Cell Anemia Sickle Cell Trait Site Standardization Stroke Teleconferences Training Transfusion Vaccination Vaccines Vascular Diseases Visit acute care aged base cardiovascular disorder risk clinical care clinical practice cohort cost effectiveness high risk hydroxyurea implementation determinants implementation evaluation implementation outcomes implementation research improved infection risk instrument meetings patient population quality assurance research study response sample collection screening screening program sickling standardized care stroke risk treatment center

项目摘要

PROJECT SUMMARY Sickle cell disease (SCD) is an inherited blood disease that causes pain, susceptibility to infection, and greatly increases the risk of cardiovascular diseases such as stroke, kidney disease, and heart disease. SCD is common in Africa because individuals with sickle cell trait (i.e., heterozygotes) are protected against severe malaria. Unfortunately, there are now more than 235,000 children born with SCD in sub-Saharan Africa each year. Undiagnosed, 50-90% of these children could die before the age of five years, mostly from bacterial infections to which children with SCD are highly susceptible. Newborn screening programs across Africa need to be expanded towards universal screening for SCD so that children born with this condition can receive evidence-based interventions to manage pain, reduce the risk of infections, and slow the progression of vascular diseases. To address these challenges, a pan-African sickle cell network has been established and is supported by the NHLBI. In this proposal, we introduce a busy sickle cell treatment center based in Bamako, Mali and explain how this center establish a registry of 4,000 patients with SCD utilizing electronic clinical data capture, deliver consistent Standards of Care with ongoing quality assurance and quality improvement, and conduct observational and implementation research to address the most pressing questions facing African people with SCD. The Centre Recherche et de Lutte contre la Drepanocytose (CRLD) is comprehensive sickle cell care center established in 2010 that now serves more than 12,000 people with SCD. We continue to enroll 120-150 new patients each month. We provide diagnostic services, counseling and education, as well as preventive and acute care for people with SCD. To establish a longitudinal cohort of 4,000 patients with SCD, we will develop data capture instruments in coordination with the Consortium, establish new IRB-approved protocols, and begin enrolling patients at a rate of 500 in the first year and 1,000 each subsequent year. The descriptive clinical data and biospecimens collected on enrollment will be shared with the Consortium. We propose delivering Standards of Care that address the most pressing needs of the SCD community. These include (I) providing diagnosis, counseling, and education; (II) mitigating the risk of infectious disease by providing vaccines, antibiotics and malaria chemoprophylaxis; and (III) providing hydroxyurea, an inducer of fetal hemoglobin expression and a proven therapy for SCD, to high-risk patients. Finally, we propose observational and implementation research studies that will lead to improved care of people with SCD. These include (I) studying the impact of our counseling and education interventions; (II) describing the etiologies and consequences of fever in the outpatient setting; and (III) extending malaria chemoprophylaxis to older children. Together, we aim to improve the care and treatment of African people with SCD both now and in the future.

项目摘要镰状细胞疾病（SCD）是一种遗传性血液疾病，会引起疼痛，感染易感性，并且很大增加了心血管疾病（例如中风，肾脏疾病和心脏病）的风险。 SCD是在非洲常见，因为镰状细胞性状（即杂合子）受到保护疟疾。不幸的是，现在有超过235,000个儿童在撒哈拉以南非洲的SCD出生年。未诊断，这些孩子中有50-90％可能在五岁之前死亡，主要来自细菌患有SCD儿童的感染非常容易受到影响。非洲的新生儿筛查计划需要要扩展到SCD的普遍筛查，以使患有这种情况的孩子可以接受基于证据的干预措施，以控制疼痛，减少感染的风险并减缓血管疾病。为了应对这些挑战，已经建立了一个泛非镰状细胞网络由NHLBI的支持。在此提案中，我们引入了一个位于巴马科的繁忙的镰状细胞治疗中心，马里并解释该中心如何建立4,000名使用电子临床数据的SCD患者的注册表通过持续的质量保证和质量提高，捕获，提供一致的护理标准，以及进行观察和实施研究，以解决非洲面临的最紧迫的问题患有SCD的人。中心Recherche et de Lutte Contre la Drepanocytose（CRLD）是全面的镰状细胞护理中心成立于2010年，目前为12,000多名SCD服务提供服务。我们继续注册120-150 每个月的患者。我们提供诊断服务，咨询和教育以及预防性和预防性 SCD患者的急性护理。为了建立由4,000例SCD患者组成的纵向队列，我们将在与财团协调，建立新的IRB批准方案，并开始以一定的速度招募患者第一年的500个，随后的1,000个。描述性临床数据和生物测量在招生时收集的将与财团共享。我们建议提供标准的护理标准，以满足SCD社区最紧迫的需求。其中包括（i）提供诊断，咨询和教育；（ii）通过提供疫苗，抗生素和疟疾化学预防；（iii）提供羟基脲胎儿血红蛋白的表达和对高危患者的SCD的良好疗法。最后，我们提出了观察性和实施研究，这将改善护理患有SCD的人。其中包括（i）研究我们的咨询和教育干预措施的影响；（ii）描述在门诊环境中发烧的病因和后果；（iii）延长疟疾给大儿童的化学预防。我们共同旨在改善现在和将来患有SCD的非洲人民的护理和治疗。