Abstract

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.

Highlights

  • Sickle cell disease (SCD) is a group of hemoglobin disorders in which abnormal beta-globin gene, known as sickle cell gene, is inherited either in a homozygous (SS) or heterozygous state (i.e., SC, SD, Sβ) [1]

  • This review focuses on a comprehensive strategy on SCD prevention at an organized all-inclusive pattern

  • Databases introduced in the initial search included MeSH, Medline, CINAHL, PubMed, Cochrane Database Systematic Review, Google Scholar, and African Journal Online using keywords: ‘'Sickle Cell Disease (SCD)'', ‘'Sickle Cell Anaemia (SCA)'', SCD/Sickle cell anemia (SCA) Prevention, Control, Screening, Treatment and Legislations'' cross-listed with keywords ‘'Adult'' and ‘'Children''

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Summary

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 other complications. This legislation will diffuse new innovations in SCD management and research which will improve the health indices of people living with the disease globally

Introduction
Epidemiology
Health Legislation and SCD
Prevention of SCD Using the Three Levels of Health Legislative Power
The Primary Preventive Step
Implementation of Legislation
Newborn Screening
Public Health Awareness Campaign on SCD
Establishment of Database System
Increase in the Burden of Malaria
SCD and Other Hemoglobinopathies Screening Tests
Findings
Conclusion
Full Text
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