Abstract
The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries. The host of quantitative evidence documenting these changes has not been assembled at the global level. The purpose of this study is to estimate trends in the future number of newborns with SCA and the number of lives that could be saved in under-five children with SCA by the implementation of different levels of health interventions. First, we calculated projected numbers of newborns with SCA for each 5-y interval between 2010 and 2050 by combining estimates of national SCA frequencies with projected demographic data. We then accounted for under-five mortality (U5m) projections and tested different levels of excess mortality for children with SCA, reflecting the benefits of implementing specific health interventions for under-five patients in 2015, to assess the number of lives that could be saved with appropriate health care services. The estimated number of newborns with SCA globally will increase from 305,800 (confidence interval [CI]: 238,400-398,800) in 2010 to 404,200 (CI: 242,500-657,600) in 2050. It is likely that Nigeria (2010: 91,000 newborns with SCA [CI: 77,900-106,100]; 2050: 140,800 [CI: 95,500-200,600]) and the Democratic Republic of the Congo (2010: 39,700 [CI: 32,600-48,800]; 2050: 44,700 [CI: 27,100-70,500]) will remain the countries most in need of policies for the prevention and management of SCA. We predict a decrease in the annual number of newborns with SCA in India (2010: 44,400 [CI: 33,700-59,100]; 2050: 33,900 [CI: 15,900-64,700]). The implementation of basic health interventions (e.g., prenatal diagnosis, penicillin prophylaxis, and vaccination) for SCA in 2015, leading to significant reductions in excess mortality among under-five children with SCA, could, by 2050, prolong the lives of 5,302,900 [CI: 3,174,800-6,699,100] newborns with SCA. Similarly, large-scale universal screening could save the lives of up to 9,806,000 (CI: 6,745,800-14,232,700) newborns with SCA globally, 85% (CI: 81%-88%) of whom will be born in sub-Saharan Africa. The study findings are limited by the uncertainty in the estimates and the assumptions around mortality reductions associated with interventions. Our quantitative approach confirms that the global burden of SCA is increasing, and highlights the need to develop specific national policies for appropriate public health planning, particularly in low- and middle-income countries. Further empirical collaborative epidemiological studies are vital to assess current and future health care needs, especially in Nigeria, the Democratic Republic of the Congo, and India.
Highlights
While considerable efforts are currently being invested into reducing the global burden of infectious diseases, malaria, tuberculosis, and HIV [1,2], the burden of birth defects has largely been neglected [3,4,5]
Our quantitative approach confirms that the global burden of sickle cell anaemia (SCA) is increasing, and highlights the need to develop specific national policies for appropriate public health planning, in low- and middle-income countries
Sickle cell disorders include SCA and co-inherited haemoglobin S and haemoglobin C (HbSC disease) or b-thalassaemia (HbS/bthalassaemia), the present study focuses exclusively on SCA, the most severe and most common globally, accounting for an estimated 83% of all newborns with sickle cell disorders [8]
Summary
While considerable efforts are currently being invested into reducing the global burden of infectious diseases, malaria, tuberculosis, and HIV [1,2], the burden of birth defects has largely been neglected [3,4,5]. Sickle cell disorders include SCA and co-inherited haemoglobin S and haemoglobin C (HbSC disease) or b-thalassaemia (HbS/bthalassaemia), the present study focuses exclusively on SCA, the most severe and most common globally, accounting for an estimated 83% of all newborns with sickle cell disorders [8]. SCA arises when a baby inherits the gene for sickle hemoglobin (HbS), a structural variant of normal adult hemoglobin (HbA, the protein in the disc-shaped red blood cells that carry oxygen round the body), from both its parents. Sickle cells can block blood vessels in the limbs and organs and have a shorter lifespan than normal red blood cells, which causes anemia Together, these changes can cause acute pain and organ damage, and can increase the risk of severe infections. SCA can be prevented by prenatal diagnosis and managed by interventions such as the provision of antibiotics and vaccination to prevent infections
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