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      Knowledge and Attitudes of Blood Donors Toward Sickle Cell Anemia in Ibadan

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          Abstract

          Background

          Sickle cell anemia (SCA) is a common condition of public health concern in Nigeria. Different therapeutic approaches have been developed to manage SCA, including blood transfusion. In a bid to develop a sustainable solution to the blood supply pool, the SmileBuilders Initiative organizes a blood donation drive (Donate-A-Pint Project) quarterly in Ibadan, Nigeria. This study aimed to assess the awareness, knowledge, and attitudes of blood donors in Ibadan, Nigeria, towards SCA.

          Methods

          A descriptive cross-sectional study was conducted at the University College Hospital Blood Bank in Ibadan over a period of three months. A validated questionnaire, based on similar studies in Nigeria, was used to collect data from blood donors. The SPSS version 23 was used for data analysis, and the chi-square test of independence assessed associations between variables, with a significance level of 0.05.

          Results

          Among 205 donors, 68.8% were male, and 42.4% were in the age category of ≥25 years. Most donors (60.0%) were university students. While 12.3% had never heard of SCA, 79.5% were aware of their genotype. Regarding the intrauterine diagnosis of hemoglobin SS (HbSS), 20.0% would allow pregnancy, 31.7% would abort, and 48.3% were undecided. Most respondents (73.2%) had good knowledge of SCA (mean score = 7.0/10.0). If partners were found to have SCA after marriage, 42.9% were undecided. Knowledge of SCA was significantly associated with sex (p = 0.017) and education level (p = 0.001).

          Conclusion

          Blood donors in Ibadan have good knowledge, awareness, and attitudes toward SCA. However, more health education would benefit the population.

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          Most cited references24

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          Sickle-cell disease.

          Sickle-cell disease is one of the most common severe monogenic disorders in the world. Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to the pathophysiology of this disease, although the importance of chronic anaemia, haemolysis, and vasculopathy has been established. Clinical management is basic and few treatments have a robust evidence base. One of the main problems of sickle-cell disease in children is the development of cerebrovascular disease and cognitive impairment, and the role of blood transfusion and hydroxycarbamide for prevention of these complications is starting to be understood. Recurrent episodes of vaso-occlusion and inflammation result in progressive damage to most organs, including the brain, kidneys, lungs, bones, and cardiovascular system, which becomes apparent with increasing age. Most people with sickle-cell disease live in Africa, where little is known about this disease; however, we do know that the disorder follows a more severe clinical course in Africa than for the rest of the world and that infectious diseases have a role in causing this increased severity of sickle-cell disease. More work is needed to develop effective treatments that specifically target pathophysiological changes and clinical complications of sickle-cell disease. Copyright © 2010 Elsevier Ltd. All rights reserved.
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            Gene Therapy in a Patient with Sickle Cell Disease.

            Sickle cell disease results from a homozygous missense mutation in the β-globin gene that causes polymerization of hemoglobin S. Gene therapy for patients with this disorder is complicated by the complex cellular abnormalities and challenges in achieving effective, persistent inhibition of polymerization of hemoglobin S. We describe our first patient treated with lentiviral vector-mediated addition of an antisickling β-globin gene into autologous hematopoietic stem cells. Adverse events were consistent with busulfan conditioning. Fifteen months after treatment, the level of therapeutic antisickling β-globin remained high (approximately 50% of β-like-globin chains) without recurrence of sickle crises and with correction of the biologic hallmarks of the disease. (Funded by Bluebird Bio and others; HGB-205 ClinicalTrials.gov number, NCT02151526 .).
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              Crises in Sickle Cell Disease.

              In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still responsible for high morbidity and early mortality. While most patients initially seek care in the acute setting for a seemingly uncomplicated pain episode (pain crisis or vaso-occlusive crisis), this initial event is the primary risk factor for potentially life-threatening complications. The pathophysiological basis of these illnesses is end-organ ischemia and infarction combined with the downstream effects of hemolysis that results from red blood cell sickling. These pathological changes can occur acutely and lead to a dramatic clinical presentation, but are frequently superimposed over a milieu of chronic vasculopathy, immune dysregulation, and decreased functional reserve. In the lungs, acute chest syndrome is a particularly ominous lung injury syndrome with a complex pathogenesis and potentially devastating sequelae, but all organ systems can be affected. It is, therefore, critical to understand the SCD patients' susceptibility to acute complications and their risk factors so that they can be recognized promptly and managed effectively. Blood transfusions remain the mainstay of therapy for all severe acute crises. Recommendations and indications for the safest and most efficient implementation of transfusion strategies in the critical care setting are therefore presented and discussed, together with their pitfalls and potential future therapeutic alternatives. In particular, the importance of extended phenotypic red blood cell matching cannot be overemphasized, due to the high prevalence of severe complications from red cell alloimmunization in SCD.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                25 September 2024
                September 2024
                : 16
                : 9
                : e70199
                Affiliations
                [1 ] Urology, Luton and Dunstable University Hospital, Luton, GBR
                [2 ] Urology, University College Hospital, Ibadan, NGA
                [3 ] Medicine and Surgery, Faculty of Clinical Sciences, College of Medicine, University of Ibadan, Ibadan, NGA
                [4 ] Medicine and Surgery, Zenith Medical and Kidney Centre, Abuja, NGA
                [5 ] Biomedical Laboratory Science, Faculty of Basic Medical Sciences, College of Medicine, University of Ibadan, Ibadan, NGA
                [6 ] Medicine and Surgery, UNIOSUN Teaching Hospital, Osogbo, NGA
                [7 ] Urology, University Hospitals Birmingham, Birmingham, GBR
                Author notes
                Article
                10.7759/cureus.70199
                11509437
                39463608
                f47340a6-425a-4162-8b4b-4069e85fb4ca
                Copyright © 2024, Amusat et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 25 September 2024
                Categories
                Genetics
                Hematology

                blood,donor,hbss,sca,sickle cell anemia
                blood, donor, hbss, sca, sickle cell anemia

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