Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021

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Abstract

BACKGROUND: Previous global analyses, with known underdiagnosis and single cause per death attribution systems, provide only a small insight into the suspected high population health effect of sickle cell disease. Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021, this study delivers a comprehensive global assessment of prevalence of sickle cell disease and mortality burden by age and sex for 204 countries and territories from 2000 to 2021. METHODS: We estimated cause-specific sickle cell disease mortality using standardised GBD approaches, in which each death is assigned to a single underlying cause, to estimate mortality rates from the International Classification of Diseases (ICD)-coded vital registration, surveillance, and verbal autopsy data. In parallel, our goal was to estimate a more accurate account of sickle cell disease health burden using four types of epidemiological data on sickle cell disease: birth incidence, age-specific prevalence, with-condition mortality (total deaths), and excess mortality (excess deaths). Systematic reviews, supplemented with ICD-coded hospital discharge and insurance claims data, informed this modelling approach. We employed DisMod-MR 2.1 to triangulate between these measures-borrowing strength from predictive covariates and across age, time, and geography-and generated internally consistent estimates of incidence, prevalence, and mortality for three distinct genotypes of sickle cell disease: homozygous sickle cell disease and severe sickle cell β-thalassaemia, sickle-haemoglobin C disease, and mild sickle cell β-thalassaemia. Summing the three models yielded final estimates of incidence at birth, prevalence by age and sex, and total sickle cell disease mortality, the latter of which was compared directly against cause-specific mortality estimates to evaluate differences in mortality burden assessment and implications for the Sustainable Development Goals (SDGs). FINDINGS: Between 2000 and 2021, national incidence rates of sickle cell disease were relatively stable, but total births of babies with sickle cell disease increased globally by 13·7% (95% uncertainty interval 11·1-16·5), to 515 000 (425 000-614 000), primarily due to population growth in the Caribbean and western and central sub-Saharan Africa. The number of people living with sickle cell disease globally increased by 41·4% (38·3-44·9), from 5·46 million (4·62-6·45) in 2000 to 7·74 million (6·51-9·2) in 2021. We estimated 34 400 (25 000-45 200) cause-specific all-age deaths globally in 2021, but total sickle cell disease mortality burden was nearly 11-times higher at 376 000 (303 000-467 000). In children younger than 5 years, there were 81 100 (58 800-108 000) deaths, ranking total sickle cell disease mortality as 12th (compared to 40th for cause-specific sickle cell disease mortality) across all causes estimated by the GBD in 2021. INTERPRETATION: Our findings show a strikingly high contribution of sickle cell disease to all-cause mortality that is not apparent when each death is assigned to only a single cause. Sickle cell disease mortality burden is highest in children, especially in countries with the greatest under-5 mortality rates. Without comprehensive strategies to address morbidity and mortality associated with sickle cell disease, attainment of SDG 3.1, 3.2, and 3.4 is uncertain. Widespread data gaps and correspondingly high uncertainty in the estimates highlight the urgent need for routine and sustained surveillance efforts, further research to assess the contribution of conditions associated with sickle cell disease, and widespread deployment of evidence-based prevention and treatment for those with sickle cell disease. FUNDING: Bill & Melinda Gates Foundation.

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The record

Venue

The Lancet Haematology

Topic

Hemoglobinopathies and Related Disorders

Field

Medicine

Canadian institutions

—

Funders

Sistema Nacional de InvestigadoresWarwick Medical SchoolNational Health and Medical Research CouncilMedical Research CouncilUniversidad Autónoma de SinaloaSecretaría Nacional de Ciencia, Tecnología e InnovaciónShahid Beheshti University of Medical SciencesUniversidad Nacional Autónoma de MéxicoUniversity of JordanUniversitetet i BergenJawaharlal Institute Of Postgraduate Medical Education and ResearchAhvaz Jundishapur University of Medical SciencesTehran University of Medical Sciences and Health ServicesSultan Qaboos UniversityShiraz University of Medical SciencesShiraz UniversityUniwersytet ŁódzkiKing Abdulaziz UniversityManipal Academy of Higher EducationIsfahan University of Medical SciencesBanaras Hindu UniversityDepartment of Sport and Recreation, Government of Western AustraliaUniversity of SydneyMinistero della SaluteSouthern Cross UniversityUniversity Grants CommissionIndian Council of Medical ResearchNational Institute for Health and Care ResearchMinistry of Health and Medical EducationUniversity of PretoriaBabol University of Medical SciencesCleveland ClinicYork UniversityScience and Technology Development FundUniversity of TorontoMacquarie UniversityImperial College LondonPhiladelphia UniversityUniversity of Technology SydneyUniversity of WarwickHøgskulen på VestlandetUniversity of Central PunjabUniversity of AberdeenUniversidade de São PauloSouth Eastern Sydney Local Health DistrictCleveland Clinic FoundationFlorida International UniversityAin Shams UniversityKing Abdulaziz City for Science and TechnologyAcademy of Scientific Research and TechnologyShaqra UniversityTulane UniversityIran University of Medical SciencesChandigarh UniversityTrường Đại học Duy TânAlfaisal UniversityKasturba Medical College, ManipalUniversity of New South WalesYale UniversityKarolinska InstitutetBill and Melinda Gates Foundation

Keywords

MedicineDiseaseEpidemiologyPopulationIncidence (geometry)Disease burdenMortality ratePediatricsAcute chest syndromeSickle cell anemiaDemographyEnvironmental healthInternal medicine

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