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Publications (29)
Novartis, a global medicines company, and the Sickle Cell Foundation of Ghana (SCFG), an advocacy organization, have endeavored to support the implementation of global best practices in the care of people living with sickle cell disease (SCD) in Africa, and to address unmet needs relating to this condition on the continent. Beginning in 2019, a mul...
Executive summary
All over the world, people with sickle cell disease (an
inherited condition) have premature deaths and preven
table severe chronic complications, which considerably
affect their quality of life, career progression, and finan
cial status. In addition, these people are often affected by
stigmatisation or structural racism, whic...
Introduction: Sickle Cell Disease (SCD) causes significant morbidity and mortality particularly in sub-Saharan Africa (SSA) where it contributes to early childhood deaths. There is need to standardize treatment guidelines to help improve overall SCD patient health outcomes. We set out to review existing guidelines on SCD and to set minimum standard...
Hemoglobin (Hb) disorders affect nearly 7% of the world’s population. Globally, around 400,000 babies are born annually with sickle cell disease (SCD), primarily in sub-Saharan Africa where morbidity and mortality rates are high. Screening, early diagnosis, and monitoring are not widely accessible due to technical challenges and cost. We hypothesiz...
Sickle cell disease (SCD) is a common condition within sub-Saharan Africa and associated with high under-5 (U5) mortality. The American Society of Hematology instituted the Consortium on Newborn Screening in Africa (CONSA) for SCD, a seven-country network of sites to implement standardized newborn hemoglobinopathy screening and early intervention f...
Objectives
Given the fundamental role of newborn bloodspot screening (NBS) to enable prompt diagnosis and optimal clinical management of individuals with sickle cell disease (SCD), we sought to systematically assess enablers and barriers to implementation of NBS programmes for SCD in Africa using established qualitative research methods.
Setting
C...
Sickle cell disease (SCD) is the most common clinically significant hemoglobinopathy, characterized by painful episodes, anemia, high risk of infection, and other acute and chronic complications. In Africa, where the disease is most prevalent, large longitudinal data on patients and their outcomes are lacking. This article describes the experiences...
Sickle cell disease is a genetic disease with a predisposition to infections caused by encapsulated organisms, especially Streptococcus pneumoniae. Pneumococcal vaccines and prophylactic penicillin have reduced the rate of this infection and mortality in sickle cell disease. However, implementation of these interventions is limited in Africa. The o...
In an effort to explore new knowledge and to develop meaningful collaborations for improving child health, the First Pan African Workshop on Newborn Screening was convened in June 2019 in Rabat, Morocco. Participants included an informal network of newborn screening stakeholders from across Africa and global experts in newborn screening and sickle...
Sickle cell disease (SCD) is one of the most common monogenic diseases in humans with multiple phenotypic expressions that can manifest as both acute and chronic complications. Although described more than a century ago, challenges in comprehensive disease management and collaborative research on this disease are compounded by the complex molecular...
Background: It is customarily perceived that in Africa, decisions around research participation may be based not only on individual reflection but also on discussions with others. Some authors have argued that such decision making is reflective of a more traditional communitarian African worldview; one critique of such a perspective is that it is l...
Sickle Cell Disease (SCD) is an increasing global health problem and presents significant challenges to European health care systems. Newborn screening (NBS) for SCD enables early initiation of preventive measures and has contributed to a reduction in childhood mortality from SCD. Policies and methodologies for NBS vary in different countries, and...
Introduction
Sickle cell disease is highly prevalent in Africa with a significant public health burden. Nonetheless, morbidity and mortality in sickle cell disease that result from the progression of organ damage is not well understood. The Organ Damage in Sickle Cell Disease Study (ORDISS) is designed as a longitudinal cohort study to provide crit...
Advances in omics technologies alone are not a guarantee that science will translate to robust responsible innovation that is firmly grounded in societal values. This study aimed to identify best practices for Ethical, Legal, and Social Implications (ELSI) research in Africa that allows for optimal integration of community perspectives into the des...
Almost three-quarters (74%) of all the noncommunicable disease burden is found within low- and middle-income countries. In September 2014, the National Heart, Lung, and Blood Institute held a Global Health Think Tank meeting to obtain expert advice and recommendations for addressing compelling scientific questions for late stage (T4) research—resea...
Background:
In malaria-endemic countries in West Africa, sickle cell disease (SCD) contributes to childhood mortality. Historically, Liberia had regions wherein hemoglobin S and beta-thalassemia trait were mutually exclusive. Data on hemoglobinopathies in the Monrovia, the capital, are outdated and do not reflect urban migration. Updating the epid...
We previously found that blood samples collected from steady-state patients with sickle cell disease (SCD) without exposure to air contain a new type of reversibly sickled cells (RSCs) with blunt edges at a level of as high as 78%. Since partial oxygenation of once-deoxygenated sickled cells with pointy edges to near venous oxygen pressure generate...
Our understanding of genome biology, genomics, and disease, and even hu-man history, has advanced tremen-dously with the completion of the Human Genome Project. Technologi-cal advances coupled with significant cost reductions in genomic research have yielded novel insights into disease etiol-ogy, diagnosis, and therapy for some of the world's most...
Our understanding of genome biology, genomics, and disease, and even human history, has advanced tremendously with the completion of the Human Genome Project. Technological advances coupled with significant cost reductions in genomic research have yielded novel insights into disease etiology, diagnosis, and therapy for some of the world's most intr...
Introduction: Many authors have recounted the history of sickle cell disease in Africa and its first recognition in the United States Sickle-shaped red cells were first described in 1910 in the blood of a sick, anemic student from Grenada. Sickle hemoglobin (HbS) was identified in 1949 and the mechanism of inheritance of sickle cell anemia was esta...
The distortion of erythrocytes in sickle cell disease (SCD) is a direct consequence of self-assembly of deoxy-Hb S heterotetramers into low-solubility polymers. Despite the broad recognition that Hb S solubility can be used to predict the efficacy of potential antipolymerization (antisickling) therapies, currently available methods require signific...
As a mediator of neurogenic inflammation and pain, we hypothesized that levels of the neuropeptide Substance P (SP) would be elevated in patients with sickle cell disease (SCD) with vaso-occlusive pain crisis. SP is a known stimulator of tumor necrosis factor- (TNF-) release and a promoter of interleukin-8 (IL-8), which are reported to be increas...
From 1978 to 1988. The Cooperative Study of Sickle Cell Disease observed 3,765 patients with a mean follow-up of 5.3 ± 2.0 years. One thousand seventy-nine surgical procedures were conducted on 717 patients (77% sickle cell anemia [SS], 14% sickle hemoglobin C disease [SC], 5.7% Sβ0 thalassemia, 3% Sβ+ thalassemia). Sixty-nine percent had a single...