Development and Evaluation of an Information Management and Communication System for Population-wide Point-of-Care Infant Sickle Cell Disease Screening.

项目摘要 尽管超过75%的镰状细胞病（SCD）儿童出生在撒哈拉以南地区， 至5岁以下死亡率，并导致终身衰弱，以证据为基础的战略，以控制SCD并不广泛 在该地区实施。通过普遍婴儿筛查早期发现SCD是SCD控制的支柱。尽管 在撒哈拉以南非洲，由于缺乏筛查， 信息管理和通信系统（SIMCS）阻碍了标准化、系统化、协调化， 全国性的SCD筛查计划拟议研究的长期目标是开发SCD SIMCS， 在撒哈拉以南非洲地区实现普遍的SCD筛查。目的是测试和优化定制SCD SIMCS应用程序和数字网络，以促进SCD筛查，然后评估其对SCD筛查的影响， 乌干达SCD儿童的护理和临床结果。核心假设是SCD SIMCS将 促进在乌干达城市和农村的卫生中心进行准确和协调的POC SCD筛查。的 基本原理是在乌干达现有的全国数字和卫生基础设施上建立一个定制的SCD SIMCS， 在全国范围内的卫生中心标准化使用负担得起的HemoTypeSCTM POC检测。核心假设将 通过追求两个具体目标进行测试：1）为新型SCD开发和评估四模块≥3G手机应用程序 SIMCS（R21阶段）; 2）评价SCD SIMCS对儿童筛查和护理以及结局的影响 SCD（R33相位）。我们将使用软件设计和重组的创新组合来实现这些目标 SCD筛查工作流程。其中包括组装与iOS和Android兼容的现成软件 操作系统，以可靠，准确，方便地捕获，解释，传输和检索/回放信息， 患者ID、测试结果、显著临床事件和教育。新的筛选工作流程预计将 显著降低成本，增加SCD筛查和护理的可及性。这项研究意义重大，因为 它将确定如何在全国范围内大规模使用POC SCD筛查测定。它还将使协调 循证护理和初级和专家提供者之间的护理连续性，以及患者的纵向 终身-控制这种终身疾病的一个关键方面。SCD SIMCS还将促进真实的时间数据 研究管理和SCD控制政策。这项研究的预期直接成果是SCD SIMCS 在乌干达的数字和卫生基础设施上发挥最佳作用，并展示其对获得 SCD筛查和护理以及SCD儿童的临床结局。预期的长期结果是， SIMCS将在乌干达和其他撒哈拉以南非洲国家的卫生系统中得到采用、整合和推广， 特别是那些HemoTypeSCTM已经被采用为SCD筛查的国家标准的国家。如果 有效，SCD SIMCS将产生重要的积极影响，因为它将降低SCD筛查的成本， 筛查服务和循证护理更接近撒哈拉以南地区大多数儿童居住的农村社区， 非洲生存，并最终使数百万儿童免于可预防的残疾死亡。

Project Summary Although over 75% of children with sickle cell disease (SCD) are born in sub-Sahara where the disease highly contributes to under-5 mortality and causes life-long debilitation, evidence-based strategies to control SCD are not widely implemented in this region. Early detection of SCD by universal infant screening is a pillar of SCD control. Despite the affordability and move to adopt point-of-care (POC) SCD screening assays in sub-Sahara Africa, the absence of screening information management and communication systems (SIMCS) impedes standardized, systematic, coordinated, nationwide SCD screening programs. The long-term goal of the proposed research is to develop a SCD SIMCS that will enable universal SCD screening in the sub-Sahara African setting. The objective is to test and optimize a custom SCD SIMCS app and digital network to facilitate SCD screening and then evaluate its impact on access to SCD screening and care and on clinical outcomes of children with SCD in Uganda. The central hypothesis is that the SCD SIMCS will facilitate accurate and coordinated POC SCD screening that is accessible at health centers in urban and rural Uganda. The rationale is to build a custom SCD SIMCS on existing nationwide digital and health infrastructure in Uganda to standardize use of the affordable HemoTypeSCTM POC assay at health centers nationwide. The central hypothesis will be tested by pursuing two specific aims: 1) Develop and evaluate a four-module ≥3G cell phone app for a novel SCD SIMCS (R21 Phase); 2) Evaluate the impact of the SCD SIMCS on access to screening and care and outcomes of children with SCD (R33 Phase). We will pursue these aims using an innovative combination of software design and re-organization of SCD screening workflows. These include assembly of off-the-shelf software that is compatible with iOS and Android operating systems to reliably, accurately, and handily capture, interpret, transmit, and retrieve/playback information for patient’s IDs, test results, salient clinical events, and education. The novel screening workflows are expected to dramatically reduce the cost and increase access to SCD screening and care. The proposed research is significant, because it will determine how to use POC SCD screening assays on a large nationwide scale. It will also enable coordination of evidence-based care and continuity of care between primary and specialist providers and longitudinally over the patient’s lifetime – a critical aspect in controlling this life-long disease. The SCD SIMCS will also facilitate real time data management for research and policy for SCD control. The expected immediate outcome of this research is a SCD SIMCS that optimally functions on the digital and health infrastructure in Uganda and demonstration of its impact on access to SCD screening and care and on clinical outcomes of children with SCD. The expected long-term outcome is that the SCD SIMCS will be adopted, integrated, and scaled-up in the health systems of Uganda and other sub-Sahara Africa countries, particularly those where the HemoTypeSCTM has already been adopted as the national standard of SCD screening. If effective, the SCD SIMCS will have an important positive impact because it will reduce the cost of SCD screening, take screening services and evidence-based care closer to rural communities where the majority of children in sub-Sahara Africa live, and, ultimately, save millions of children from preventable and disability death.