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Publication Title : Current Trends in the Management of Sickle Cell Anaemia
Author(s) : Abubakar Umar Musa
Abstract : Abstract Background: Sickle cell anaemia (SCA) is the most frequent monogenic disorder affecting more than 50 million persons worlwide with the largest health and socio-economic burden felt in the sub-saharan Africa particularly Nigeria. Methods: Standard textbooks of haematology were consulted while online searches for relevant materials using specific keywords were conducted on PubMed and Google Scholar databases. Results: Despite the current indepth understanding of the pathophysiology of SCA, majority of patients living with this disease are yet to have a sigh of relief. In developing countries where most patients with SCA abound, there is dearth of health care resources and the political will to tackle the menace of the disease. While most of the treatment modalities available address the downward sequelae of SCA, stem cell transplantation and gene therapy that offer cure for the disease are not yet affordable and accessible for most patients needing them. Additionally, the advent of COVID-19 pandemic has further exposed the challenges and the inequities encountered by SCA patients especially in the sub saharan Africa. During the pandemic, SCA patients could not access routine care due to lockdown, fear of contagion and redeployment of medical specialists involved in such care to the COVID-19 response. Furthermore, shortage of blood donors led to lack of optimal blood transfusion services for this patients and added to the increased morbidity and mortality of SCA patients during the pandemic. Conclusion: While researches on antisickling agents, gene therapy and stem cell transplantation are on-going, there is the urgent need to put more emphasis on provision of basic management modalities for SCA such as health education, premarital counselling and screening, newborn screening and early intervention, routine folic acid supplementation, antimalarial and penicilline prophylaxis, use of hydroxyurea and availability of safe blood for transfusion. Keywords: sickle cell anaemia, hydroxyurea, stem cell transplantation, gene therapy
DOI : 10.51658/ABMS.202122.10
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