The Lancet study on sickle cell disease (SCD) presents some significant findings regarding its global impact. The study reveals that the number of people living with SCD worldwide has increased by 41.4%, from 5.46 million in 2000 to 7.74 million in 2021. This indicates a substantial rise in the prevalence of the disease.
Importantly, the study emphasizes the high contribution of SCD to overall mortality, which is not immediately apparent when each death is attributed to a single cause.
In children under the age of 5, there were 81,100 deaths attributed to SCD, ranking it as the 12th leading cause of mortality across all causes, as estimated by the Global Burden of Disease (GBD) in 2021. This ranking is considerably higher (28 places) than the cause-specific mortality attributed solely to SCD.
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% to 515 000, primarily due to population growth in the Caribbean and western and central sub-Saharan Africa the Lancet study says.
Researchers estimated 34 400 cause-specific all-age deaths globally in 2021, but total sickle cell disease mortality burden was nearly 11-times higher at 376 000.
The burden of SCD mortality is particularly pronounced in children, especially in countries with high under-5 mortality rates. The researchers caution that without comprehensive strategies to address the morbidity and mortality associated with SCD, achieving Sustainable Development Goals (SDGs) 3.1, 3.2, and 3.4, which pertain to good health and well-being, is uncertain.
The researchers employed standard Global Burden of Disease (GBD) approaches to estimate cause-specific SCD mortality using vital registration, surveillance, and verbal autopsy data.
Additionally, they aimed to estimate a more accurate account of SCD health burden by considering four types of epidemiological data: birth incidence, age-specific prevalence, with-condition mortality (total deaths), and excess mortality (excess deaths).
The study used systematic reviews, ICD-coded hospital discharge data, and insurance claims data to inform their modeling approach. DisMod-MR 2.1 was employed to triangulate between different measures, incorporating predictive covariates across age, time, and geography.
The study generated internally consistent estimates of incidence, prevalence, and mortality for different genotypes of SCD. By comparing total SCD mortality estimates with cause-specific mortality estimates, the researchers evaluated the differences in mortality burden assessment and its implications for the SDGs.
These findings underscore the substantial and often underestimated burden of SCD on global health. It highlights the urgent need for targeted efforts to improve the prevention, diagnosis, and management of SCD, especially in regions heavily affected by the disease.
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.
By addressing the challenges associated with SCD, progress can be made towards achieving better health outcomes for individuals living with the condition, particularly children.
https://doi.org/10.1016/S2352-3026(23)00118-7
