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Beyond the Legislation for Sickle Cell Disease Prevention - Getting the Right Outcomes
American Journal of Internal Medicine
Volume 7, Issue 3, May 2019, Pages: 56-65
Received: Feb. 26, 2019; Accepted: Apr. 23, 2019; Published: Jun. 12, 2019
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Ogbonna Collins Nwabuko, Department of Hematology and Blood Transfusion, Federal Medical Centre, Umuahia, Abia State, Nigeria; Department of Hematology, College of Health Science, Abia State University, Aba Campus, Abia State, Nigeria; Department of Public Health Sciences, Walden University, Baltimore, USA
Ransome Eke, Department of Health Science, University of Alabama, Tuscaloosa, USA
Mwayabo Jean Claude Kazadi, Center for Disease Control Epidemic Control Project, Catholic Relief Services, Lusaka, Zambia
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This study aimed at highlighting benefits derivable from legislation for Sickle cell disease (SCD) prevention using the three levels of health legislative power (primary, secondary and tertiary). It was an evidence-based integrative review of 54 references related to SCD, epidemiology, management, and legislation for its control over the past three decades (1979-2018). Two keywords (SCD and Legislation/Policy) were used as search strategy to identify the problem, intervention, and outcome evaluation of the research questions. MeSH, Medline, CINAHL, PubMed, Cochrane Database Systematic Review, Google Scholar and African Journal Online were search databases reviewed. The expected outcomes were categorized into three levels of benefits. They were primary, secondary and tertiary categories of benefit. While the primary category would institutionalize pre-genetic counseling in the health institutions, the secondary and tertiary groups would establish screening (prenatal and newborn)/comprehensive care and curative interventions (i.e., investigational therapies and stem cell transplantation) respectively for people living with SCD. These interventions will enhance SCD surveillance, screening, auditing, health needs assessment, treatment, control, and prevention in the target audience. Conclusions The legislation for Sickle Cell Disease prevention in the target population would promote early disease detection, treatment, and prevention. We expect that with an increase in case ascertainment, the disability-adjusted life year (DALY) would reduce so also other complications. This legislation will diffuse new innovations in SCD management and research which will ultimately improve the health indices of people living with the disease globally.
Sickle Cell Disease, Legislation, Prevention, Outcome, Surveillance
To cite this article
Ogbonna Collins Nwabuko, Ransome Eke, Mwayabo Jean Claude Kazadi, Beyond the Legislation for Sickle Cell Disease Prevention - Getting the Right Outcomes, American Journal of Internal Medicine. Vol. 7, No. 3, 2019, pp. 56-65. doi: 10.11648/j.ajim.20190703.12
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Cooper R. A note on the biologic concept of race and its application in epidemiologic research. Am Heart J 1984; 108 (3 Pt 2): 715-22.
United Nations General Assembly. Recognition of sickle-cell anemia as a public health problem. United General Assembly; 2009.
World Health Organization. Management of birth defects and hemoglobin disorders. Report of a joint WHO-March of Dimes meeting. World Health Organization; Geneva, Switzerland; 2006.
World Health Organization Regional Office for Africa. Sickle cell disease prevention and control; 2015. Available at: http: //
Bosu WK. A comprehensive review of the policy and programmatic response to the chronic non-communicable disease in Ghana. Ghana Medical Journal 2012; 46 (Suppl.2): 69-78.
Modell B, Darlison M. Global epidemiology of hemoglobin disorders and derived service indicators. Bulletin of the World Health Organization 2008; 86 (6): 480-487.
Ohene-Frempong K, Oduro J, Tetteh H, Nkrumah FK. Screening newborns for sickle cell disease in Ghana. Paediatrics 2005; 121 (S120). Available at: http: //
Tshilolo L, Kafando E, Sawandogo M, Cotton F, Vertongen F, Ferster A, et al. Neonatal screening and clinical care programmes for sickle cell disorders in sub- Saharan Africa: Lessons from pilot studies. Public Health 2008; 122 (9): 933-941.
PICO Model. Available at: http: //
Whittemore R and Knafi K. The integrative review: Updated methodology. Journal of Advanced Nursing 2005; 52 (5): 546-553. Available at: http: //
Mulumba LL, Wilson L. Sickle cell disease among children in Africa: An integrative review and global recommendations. International J Afr Nursing Sciences 2015; 3: 56-64.
Nwabuko OC, Nwamoh UN, Chikezie JA. A Retrospective Review of Ebola Outbreak in West Africa: Social Determinants and Control Measures. Epidemiology: Open Access 2018; 8: 360. doi: 10.4172/2161-1165.1000360.
Center for Disease Control and Prevention - Global health reports, Nigeria. 2012. Available at https: // > nigeria > wha t> scd.
Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anemia in children under five, 2010-2050: Modelling based on demographics, excess mortality, and interventions. PLoS Med 2013; 10: e1001484.
Gabriel A and Przybylski J. Sickle-cell anemia: A Look at Global Haplotype Distribution. Nature Education 2010; 3 (3): 2.
Aygun B and Odame I. A global perspective on the sickle-cell disease. Pediatric Blood & Cancer 2012; 59 (2): 386-390. DOI: 10.1002/pbc.24175.
Piel FB1, Patil AP, Howes RE, Nyangiri OA, Gething PW, Williams TN, Weatherall DJ, Hay SI. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010; 1: 104. DOI.10.1038/ncomms1104.
Akinyanju OO. A profile of sickle cell disease in Nigeria. Annals of the New York Academy of science 1989; 565: 126-136.
Nwabuko OC and Okoh DA. Hemoglobinopathy- the old and new eras in a south-eastern Nigeria tertiary health center. Blood 2015 126 (23): 4577. Available at: http: //
Brousseau DC, Panepinto, JA, Nimmer, M and Hoffmann, RG. The number of people with the sickle-cell disease in the United States: national and state estimates. Published online 21 October 2009 in Wiley InterScience ( doi: 10.1002/ajh.21570
Makani J, Cox SE, Soka D, Komba AN, Oruo J, Mwamtemi H, et al. Mortality in sickle cell anemia in Africa: A prospective cohort study in Tanzania. PLoS ONE 2011; 6 (2): e14699.
Nwabuko OC, Alozie E, Chikezie K, Chukwuonye II, Ejele OA. Socio-demographic overview of sickle cell disease seen in Abia State: a single institution experience over seven years. National Society of Haematology and Blood Transfusion NSHBT 2018 Abstract 025.
World Health Organization Commission on the Social Determinants of Health. Closing the gap in a generation: Health equity through action on the social determinants of health. Executive summary. World Health Organization; 2008. Available at
Bezruchka S. From womb to tomb: The influence of early childhood on adult health [Audio podcast] 2005. Available at
Hunter D. Leading for Health and Wellbeing: the need for a new paradigm. Journal of Public Health 2009; 31 (2): 202-204.
Central Intelligence Agency. (n.d.). The world factbook. Available at
Max R. Life expectancy. Published online at OurWorldInData. Available at: https: //
Country Comparison: Life Expectancy at Birth. Available at: https: //
Country Comparison: Infant Mortality Rate, Available at: https: // factbook/rankorder/2091rank.html.
World Health Organization. Health systems in sub-Saharan Africa: What is their status and role in meeting the health millennium development goals? World Health Organization; 2012. Available at: http: //
Ogun GO, Ebili H, Kotila TR. Autopsy findings and pattern of mortality in Nigeria sickle cell disease patients. The Pan African medical journal 2014, 18: 30. Doi: 10.11604/pamj.2014.18.30.4043.
Lanzkron S, Carroll P, Haywood C. Mortality rates and age at death from sickle cell disease: U. S., 1979-2005. Public Health Rep 2013; 128 (2): 110-116. doi: 10.1177/003335491312800206.
Public Health Agency of Canada; 2014. Available at
The Upstream Doctors; 2017. Available at:
Herbes-Sommers C, Smith LM. Health in America [Video excerpt]. In L. Adelman, Unnatural causes: Episode 1 – In sickness and wealth. United States: Public Broadcasting Service. 2008. Available at: http: //
World Health Organization. Health Promotion. World Health Organization; 2018 [online] Available at: http: // [Accessed 24 May 2018].
Nietert PJ, Silverstein MD, and Abboud MR. Sickle Cell Anaemia. Pharmacoeconomics 2002; 20 (6): 357-66. DOI. 10.2165/00019053-200220060-00001.
Kuznik A, Habib AG, Munube D, Lamorde M. New-born screening and prophylactic interventions for sickle cell disease in 47 countries in sub-Saharan Africa: a cost-effective analysis. BMC Health Services Research 2016; 16: 304. DOI 10.1186/s12913-016-1572-6.
Kanter J. Point-of-Care Testing in Sickle Cell Disease. In: Inusa BPD (eds). Sickle Cell Disease – Pain and Common Chronic Complications, 2016. Available at https: //doi/10.5772/64862.
Yenilmez ED and Tuli A. New Perspectives in Prenatal Diagnosis of Sickle Cell Anemia. In: Inusa BPD (eds). Sickle Cell Disease – Pain and Common Chronic Complications, 2016. Available at https: //doi/10.5572/64646.
Mpalampa L, Ndugwa CM, Ddungu H, and Idro R. (2012). Fetal hemoglobin and disease severity in sickle cell anemia patients in Kampala, Uganda. BMC blood disorders 2012; 12: 11. doi: 10.1186/1471-2326-12-11.
Sepulveda C, Marlin A, Yoshida T, Ulrich A. Palliative care: the World Health Organization's global perspective. J Pain Symptoms Manage 2002; 24: 91-96.
Estepp JH. Voxelotor (GBT440), a first-in-class hemoglobin oxygen-affinity modulator, has promising and reassuring preclinical and clinical data. American Journal of Hematology 2018; 93: 3. Available at https: //
Ribeil J, Hacein-Bey-Abina S, Payen E, et al. Update from Hgb-205 Phase ½ Clinical Study of Lentiglobin Gene Therapy: Sustained Clinical Benefit in severe Hemoglobinopathies. Blood 2018; 128 (22): 2311. Available at http: //
Nwabuko OC, Nnoli MA, Okoh DA, Chukwuonye II. Taming the tide of HIV and TTI Scourge in sub-Saharan Africa using autologous blood transfusion. Hematology and Leukemia Herbert Open Access Journals 2013; 1 (7). Available at
Endari (L-glutamine) for Sickle cell disease. Available at
Isgro A, Paciaroni K, Gaziev J, et al. Haemopoietic stem cell transplantation in Nigerian sickle cell anemia children patients. Niger Med J. 2015; 56 (3): 175-179.
Makani J, Ofori-Aquah S, Nnodu OE, Treadwell M, Royal C, and Ohene-Frempong K. Sickle cell disease and H3Africa: enhancing genomic research on cardiovascular disease in African patients. Cardiovascular J Afr. 2015; 26 (2H3Africa Suppl): S50-S55.
Nwabuko OC, Okoh DA, Iyalla C, Omunakwe H. Prevalence of sickle cell disease among pregnant women in a tertiary health center in south-south Nigeria. Sub-Saharan Afr J Med 2016; 3: 132-6. DOI: 10.4103/2384-5147.190843.
How Race Plays an Ugly Role in the Drastic Underfunding of Sickle Cell Research and Advocacy. Available at: http: //
Green L, Kreuter M. Health program planning: An educational and ecological approach. 4th ed. New York: McGraw-Hill; 2005.
Parker J C, Thorson E editors. Health communication in the news media landscape. New York, NY: Springer Publishing Company; 2009.
Nwabuko OC, Igbigbi E, Ejele OA. Promoting Public Health Campaign on Awareness and Screening for Multiple myeloma in Nigeria. Archives of Hematology and Blood Diseases 2018; 1 (2): 15-20.
Text-S. 2465-115th Congress (2017-2018): Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention, and Treatment Act of 2018/ Available at > senate-bill.
Fleming AF, Storey J, Molineaux L, Iroko EA, Attai ED. Abnormal hemoglobins in the Sudan Savanna of Nigeria. I. Prevalence of hemoglobins and relationships between sickle cell trait, malaria, and survival. Ann Trop Med Parasitol 1979; 73: 161-72.
Komba AN, Makani J, Sadarangani M, Ajala-Agbo T, Berkley JA, Newton CR, et al. Malaria as a cause of morbidity and mortality in children with homozygous sickle cell disease on the coast of Kenya. Clinical Infectious Disease, 2009; 49 (2): 216-222. DOI: 10.1086/599834.
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