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• W4366483307 abstract "Sickle cell disease (SCD) is a monogenic disorder. It is estimated that more than 0.3 million babies are born with SCD every year and more than half of the total global burden of SCD is shouldered by three countries with developing economies (Nigeria, Democratic Republic of Congo and India).1Odame I. Perspective: we need a global solution.Nature. 2014; 515: S10Crossref PubMed Scopus (20) Google Scholar Due to multi-organ involvement, life expectancy of SCD patients remains low and quality of life of the survivors is poor due to chronic anemia, pain crisis and frequent hospitalization.1Odame I. Perspective: we need a global solution.Nature. 2014; 515: S10Crossref PubMed Scopus (20) Google Scholar Such patients either need permanent cure or depend on disease-modifying agents for the rest of their lives. Unfortunately, SCD is largely confined to communities that are underprivileged in term of economy, health-seeking behavior and accessibility to public health. In all these country settings, the disease is principally managed by the public health systems. Any defined control measures of SCD is yet to be devised. SCD is managed through medicines that have shown high promises in not only enhancing life expectancy but also reducing the pain crisis and blood transfusion requirement in patient at all stages of the disease. To this extent, the golden drug ‘hydroxyurea’ in combination with prophylactic antibiotics have shown to immensely reduce under-five mortality rate,2Gardner K. Douiri A. Drasar E. et al.Survival in adults with sickle cell disease in a high-income setting.Blood. 2016; 128: 1436-1438Crossref PubMed Scopus (123) Google Scholar leading to positive impact on both longevity and quality of life in SCD patients. For example, life expectancy of SCD patients had reportedly exceeded more than 60 years and mortality rates of children with SCD remained at par to the general populations.2Gardner K. Douiri A. Drasar E. et al.Survival in adults with sickle cell disease in a high-income setting.Blood. 2016; 128: 1436-1438Crossref PubMed Scopus (123) Google Scholar Furthemore, management of SCD is also currently possible with three different recently approved medicines, i.e., Crizanlizumab, Voxelotor and l-Glutamine in patients where hydroxyurea is not effective. With high feasibility, affordability and tolerance by the patients, management of SCD by hydroxyurea has therefore become the choice of public health systems of the endemic countries. Does management alone can reduce the burden and eventually control SCD in these high endemic counties? While management is important for interim relief to the patient, it can neither provide permanent relief to the patient, nor can it reduce public health burden in long term. To this extent, the hematopoietic stem cell transplantation (HSCT) is a well-established procedure for permanent cure of SCD with a very high (>90%) survival rate.3Gluckman E. Cappelli B. Bernaudin F. et al.Sickle cell disease: an international survey of results of HLA-identical sibling hematopoietic stem cell transplantation.Blood. 2017; 129: 1548-1556Crossref PubMed Scopus (289) Google Scholar However, (i) finding of HLA-match donor, (ii) lack of awareness among treating physicians and (iii) non-availability of transplant centres in disease prevalent areas, are few challenges, and possibly therefore, HSCT is currently been performed in a meager number of SCD patients in highly endemic countries. Curative methods by gene therapy (GT), due to non-requirement of donors seems to hold promises for permanent cure of SCD. However, the risk of development of acute myeloid leukemia/myelodysplastic syndrome after GT4Jones R.J. DeBaun M.R. Leukemia after gene therapy for sickle cell disease: insertional mutagenesis, busulfan, both, or neither.Blood. 2021; 138: 942-947Crossref PubMed Scopus (38) Google Scholar puts great challenges. While the cost involved in HSCT is almost equivalent to life-long treatment, and therefore manageable by patient/public health, the high cost of GT (which can go about 90–140 times higher than the cost of HSCT in the developing countries), can be an obstacle for patients and/or public health to adopt to this procedure. To reduce the long-term burden and eventual control of SCD, preventive measures can be the best practice, as adopted in thalassemia major - another human monogenic disorder. For example, following premarital screening coupled with genetic counselling, the countries like Cyprus, Sardinia, Italy and Greece were able to significantly reduce birth of thalassemia major children.5Cousens N.E. Gaff C.L. Metcalfe S.A. Delatycki M.B. Carrier screening for beta-thalassaemia: a review of international practice.Eur J Hum Genet. 2010; 18: 1077-1083Crossref PubMed Scopus (148) Google Scholar Prevention through universal premarital and prenatal screening have proven to be rewarding in comparison to cure/treatment of SCD and other monogenetic diseases. What is the need of the hour for control of SCD in low-resource-high-burden country settings? Regular management for SCD face challenges due to inaccessible habitations, poor health seeking behavior of communities and less-trained public healthcare workers in the localities. Admittedly, these factors contribute in increase in mortality and morbidity of SCD patients. All these challenges can be mitigated by preventive strategies. Universal screening using highly-sensitive point-of-care tests in high-prevalence communities are the need of the hour. In addition, awareness generation to prevent marriages between SCD carriers under programmatic mode in community setting could prove as a ‘game changer’ in controlling SCD. While policy decisions based on feasibility and affordability of curative measures by governments of endemic countries can go a long way, reiteration on the SCD public health severity by international organizations is necessary to move forward in controlling SCD across the globe. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors." @default.
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• W4366483307 date "2023-05-01" @default.
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• W4366483307 title "Controlling sickle cell disease: The need of the hour" @default.
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