Improving Health Outcomes in Persons with Sickle Cell Disease

American Journal of Hematology, 2009

The American Society of Pediatric Hematology/Oncology Sickle Cell Summit brought together a broad range of constituencies to identify a unified approach to healthcare and research disparities for sickle cell disease. Recommendations included the following: (1) speak with a unified voice representing all constituencies; (2) optimize access to care from knowledgeable health care providers and create a medical home for all individuals with the disease; (3) utilize population-based surveillance to measure outcomes; (4) develop overall approaches to basic, translational, clinical, and health services research; (5) enhance the community role in advocacy, education, service, and fundraising. Taskforces were identified to effect implementation. Am. J. Hematol. 84:39-45,

American journal of preventive medicine, 2016

American Journal of Hematology, 2021

Sickle cell disease (SCD) is a genetic disorder, characterized by hemolytic anemia and vaso‐occlusive crises (VOCs). Data on the global SCD impact on quality of life (QoL) from the patient viewpoint are limited. The international Sickle Cell World Assessment Survey (SWAY) aimed to provide insights into patient‐reported impact of SCD on QoL. This cross‐sectional survey of SCD patients enrolled by healthcare professionals and advocacy groups assessed disease impact on daily life, education and work, symptoms, treatment goals, and disease management. Opinions were captured using a Likert scale of 1‐7 for some questions; 5‐7 indicated “high severity/impact.” Two thousand one hundred and forty five patients (mean age 24.7 years [standard deviation (SD) = 13.1], 39% ≤18 years, 52% female) were surveyed from 16 countries (six geographical regions). A substantial proportion of patients reported that SCD caused a high negative impact on emotions (60%) and school achievement (51%) and a reduc...

Journal of Prevention & Intervention in the Community, 2011

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2020

1. Cincinnati Children’s Comprehensive Sickle Cell Center, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA 2. King’s College Hospital, London, UK 3. INSERM U955, Université Paris-Est, EFS Créteil, Créteil, France 4. Naef K. Basile Cancer Institute, American University of Beirut Medical Center, Beirut, Lebanon 5. Study Network on Sickle Cell Disease in Central Africa (REDAC) and Medical training and support Centre (CEFA)/Centre Hospitalier Monkole, Kinshasa, Democratic Republic of the Congo 6. Sickle Cell Disease Enterprise, The Levine Cancer Institute, Atrium Health, Charlotte, North Carolina, USA 7. Sickle Cell Society, London, UK 8. Clinic of Paediatric Haematology-Oncology, University of Padova, Padova, Italy

American Journal of Preventive Medicine, 2011

Although the issue of whether sickle cell trait (SCT) is clinically benign or a signifıcant health concern has not yet been resolved, the potential health risk to affected individuals is of vital importance and represents a tremendous challenge in protecting, promoting, and improving the health of the approximately 300 million people worldwide and 3 million people in the U.S. who possess the trait. In response to a request by the Sickle Cell Disease Association of America, in December 2009, the CDC convened a meeting of partners, stakeholders, and experts to identify the gaps in public health, clinical health services, epidemiologic research, and community-based outreach strategies and to develop an agenda for future initiatives. Through facilitated discussion and presentations in four topic areas, participants discussed pertinent issues, synthesized clinical research fındings, and developed a coherent framework for establishing an agenda for future initiatives. A primary outcome of the meeting was to provide the fırst step of an iterative process to move toward agreement regarding appropriate counseling, care, and, potentially, treatment of people with SCT.

JAMA Health Forum, 2021

Although considered a rare disease with fewer than 200 000 cases annually in the US, sickle cell disease (SCD) is the most common and clinically significant inherited blood disorder in the US and worldwide. Despite the relatively high prevalence of this rare disease, there is a paucity of longitudinal data available to evaluate access to care or to identify quality metrics. OBSERVATIONS This review discusses why systematic data collection for SCD through populationwide surveillance programs can help to facilitate progress in treatment. It also explores the importance of having both a longitudinal clinical registry and a national surveillance program to improve resource utilization, clinical outcomes, and provide an equitable foundation for care. Federal funding should be appropriately allocated to establish and maintain a national SCD surveillance system supported by the Centers for Disease Control and Prevention, as well as a longitudinal registry available at recognized sickle cell centers.

Blood Science, 2020

Sickle cell disease (SCD) affects nearly 100,000 people in the United States of America and the sickle gene is present in approximately 8% of black Americans. Among Africans, the prevalence of sickle cell trait (heterozygosity) is as high as 30%. While SCD occurs among varying racial and ethnic groups, it is more commonly prevalent in individuals of African or African-American descent. This inherited blood disorder causes varying symptoms and complications among affected children and adults and early diagnosis and treatment are essential to help reduce mortality rates. Because there is no cure for SCD, management is vital to survival. Hence, there are different approaches in use to aid those living with SCD; thus, this paper provides insight into the current methods that are implemented in the management and maintenance of this disease.

2018

Introduction: Sickle cell disease is considered to be one of the commonest hereditary diseases around the world with being highly prevalent in the regions of sub-Saharan Africa, Mediterranean, Middle East, and Southeast Asia. Sickle cell disease is known to be a chronic hematological disease that leads to severe hemolysis, and is characterized by hemoglobin particles that tend to polymerize within the RBCs and cause the RBC to become in a crescent, sickle shape, which will cause hemolysis along with vasoocclusive events in different organs. Aim of the work: we tried to understand the pathogenesis, impact, diagnosis, and management of sickle cell disease in this study. Methodology: we conducted this review using a comprehensive search of MEDLINE, PubMed and EMBASE from January 1950 to March 2018. The following search terms were used: sickle cell anemia, hemoglobinopathy, genetics of sickle cell, complications of sickle cell, management of sickle cell disease Conclusion: It is important to develop therapeutic options that reduce the risk of complications of sickle cell disease. Despite all advances in treatment, sickle cell disease remains to be an important serious disease that is associated with severe morbidity and mortality, and poor quality of life. More research and clinical trials should be conducted on this disease to develop advanced treatment options that will further improve quality of life and decrease mortality and complications.

James Success Odubia, 2022