Prevalence of Sickle Cell Disease in Bahia, Brazil: Overcoming the Myth of Rarity as a Local Expression of Global Health Inequalities
1
1 Institute of Collective Health Federal University of Bahia, Salvador, Brazil
2
2 Florida University of Science and Theology, Florida, United States
3
3 State Health Secretariat of Bahia, Saint Anthony of Jesus, Brazil
4
5 Association of Parents and Friends of Exceptional Children APAE, Salvador, Brazil
5
4 Rilza Valentim State Reference Center for People with Sickle Cell Disease and Hemoba Foundation of Bahia, Salvador, Brazil
Popul. Med. 2026;8(Supplement Supplement 1):A2762
ABSTRACT
BACKGROUND:
Sickle cell disease (SCD) is a prevalent genetic condition worldwide, particularly among individuals of African descent. The historical and institutional marginalization of populations affected by SCD has contributed to its epidemiological invisibility, hindering progress in treatment and policy development.
METHODS:
A cross-sectional, ecological, and time-series study was conducted on the prevalence of SCD in Bahia from 2010 to 2022. Data from six major health information systems were integrated through linkage and deduplication: Mortality Information System, Notifiable Diseases Information System, Live Birth Information System, Ambulatory Information System, Hospital Information System, and the National Neonatal Screening Program. Segmented regression (joinpoint) and Prais-Winsten regression were applied to estimate the annual percentage change (APC) and perform temporal decomposition (STL).
RESULTS:
In 2022, the prevalence of SCD was 81.57 per 100,000 people, which is above the threshold for rare diseases defined by the WHO. The prevalence increased during the study period (APC = 2.29%; 95% CI: 1.52–3.07), while potential years of life lost remained stable. The highest prevalence rates were concentrated in East and South Bahia.
CONCLUSIONS:
Bahia, the fourth most populous state in Brazil, has one of the highest SCD prevalence rates in the world outside of Africa. Institutional racism and limited data quality impact the surveillance of the disease. Strategies such as active screening, expanding specialized services, consolidating interoperability between health information systems, and implementing policies to combat institutional racism could improve healthcare and reduce inequalities in the black population.
Sickle cell disease (SCD) is a prevalent genetic condition worldwide, particularly among individuals of African descent. The historical and institutional marginalization of populations affected by SCD has contributed to its epidemiological invisibility, hindering progress in treatment and policy development.
METHODS:
A cross-sectional, ecological, and time-series study was conducted on the prevalence of SCD in Bahia from 2010 to 2022. Data from six major health information systems were integrated through linkage and deduplication: Mortality Information System, Notifiable Diseases Information System, Live Birth Information System, Ambulatory Information System, Hospital Information System, and the National Neonatal Screening Program. Segmented regression (joinpoint) and Prais-Winsten regression were applied to estimate the annual percentage change (APC) and perform temporal decomposition (STL).
RESULTS:
In 2022, the prevalence of SCD was 81.57 per 100,000 people, which is above the threshold for rare diseases defined by the WHO. The prevalence increased during the study period (APC = 2.29%; 95% CI: 1.52–3.07), while potential years of life lost remained stable. The highest prevalence rates were concentrated in East and South Bahia.
CONCLUSIONS:
Bahia, the fourth most populous state in Brazil, has one of the highest SCD prevalence rates in the world outside of Africa. Institutional racism and limited data quality impact the surveillance of the disease. Strategies such as active screening, expanding specialized services, consolidating interoperability between health information systems, and implementing policies to combat institutional racism could improve healthcare and reduce inequalities in the black population.
| eISSN: | 2654-1459 |
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