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Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–20212.54 MB
Published (Version of record)CC BY V4.0, Open Access
Published (Version of record)CC BY V4.0, Open
Journal article
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
The Lancet. Haematology, Vol.10(8), pp.E585-E599
08/2023
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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.
Details
- Title
- 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
- Creators
- Azalea M Thomson (Author)Theresa A McHughAssaf P OronCorey TeplyNikhil LonbergVictor Vilchis TellaLauren B WilnerKia FullerHailey HaginsRichard Gyan AboagyeMelka Biratu AboyeEman Abu-GharbiehAhmed Abu-ZaidIsaac Yeboah AddoBright Opoku AhinkorahAqeel AhmadSaif Aldeen S AlRyalatHubert AmuAleksandr Y AravkinJudie ArulappanMaha Moh'd Wahbi AtoutAshish D. BadiyeSara BagheriehMaciej BanachMorteza BanakarMainak BardhanAmadou BarrowDeriba Abera BedaneIsabela M BensenorAkshaya Srikanth BhagavathulaPankaj BhardwajPrarthna V BhardwajAjay Nagesh BhatZulfiqar A. BhuttaMariah Malak BilalagaJessica Devin BishaiSaeid BitarafArchith BoloorMuhammad Hammad ButtVijay Kumar ChattuDinh-Toi ChuOmid DadrasXiaochen DaiBardia DanaeiAnh Kim DangFitsum Wolde DemisseMeghnath DhimalDaniel DiazShirin DjalaliniaDeepa DongarwarMuhammed ElhadiMohamed A ElmonemChristopher Imokhuede EsezoborFarshid EtaeeOghenowede EyawoAdeniyi Francis FagbamigbeAli FatehizadehLisa M ForceWilliam M. GardnerKazem GhaffariParamjit Singh GillMahaveer GolechhaPouya Goleij (Contributor)Vivek Kumar Gupta (Contributor)Hamidreza HasaniTreska S Hassan (Contributor)Mohammed Bheser HassenSegun Emmanuel Ibitoye (Contributor)Adalia I Ikiroma (Contributor)Chidozie C D Iwu (Contributor)Peter Bai James (Contributor)Shubha JayaramRime Jebai (Contributor)Ravi Prakash JhaNitin JosephFarnaz KalantarHimal KandelIbraheem M KarayeWoldeteklehaymanot Dagne KassahunImteyaz A KhanShaghayegh KhanmohammadiAdnan KisaFarzad KompaniKewal KrishanIván LandiresStephen S. LimPreetam Bhalchandra MahajanSoleiman MahjoubAzeem MajeedBishnu P MarasiniHaftu Asmerom MeresaTomislav MestrovicSonica MinhasAwoke MisganawAli H. MokdadLorenzo MonastaGhulam MustafaTapas Sadasivan NairSreenivas Narasimha SwamyHasan NassereldineZuhair S NattoMuhammad NaveedBiswa Prakash NayakJean Jacques NoubiapTaylor NoyesChisom Adaobi Nri-ezediVincent Ebuka NwatahChimezie Igwegbe NzoputamOgochukwu Janet NzoputamOsaretin Christabel OkonjiAdeyinka Omoniyi OnikanMayowa O OwolabiJay PatelSiddhartha PatiShrikant PawarIonela-Roxana PetcuFrédéric B PielIbrahim QatteaMehran RahimiMosiur RahmanSalman RawafElrashdy Moustafa Mohamed RedwanNazila RezaeiBasema SaddikUmar SaeedFatemeh Saheb Sharif-AskariAbdallah M. SamyAustin E SchumacherElaheh ShakerAdithi ShettyMigbar Mekonnen SibhatJasvinder A. SinghMuhammad SulemanDev Ram SunuwarMindy D SzetoJacques JL Lukenze TamuziNathan Y TatBirhan Tsegaw TayeMohamad-Hani TemsahMuhammad UmairSahel Valadan TahbazCong WangNuwan Darshana WickramasingheArzu YigitVahit YigitIsmaeel YunusaBurhan Abdullah ZamanMoein ZangiabadianPeng ZhengSimon I. Hay (Contributor)Mohsen NaghaviChristopher J. L. Murray (Contributor)Nicholas J Kassebaum (Contributor)
- Publication Details
- The Lancet. Haematology, Vol.10(8), pp.E585-E599
- Publisher
- Elsevier Ltd
- Grant note
- Bill & Melinda Gates Foundation.
- Identifiers
- 991013125213602368
- Copyright
- © 2023 The Author(s). Published by Elsevier Ltd. Creative Commons Attribution (CC BY 4.0).
- Academic Unit
- National Centre for Naturopathic Medicine
- Language
- English
- Resource Type
- Journal article