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Interventions needed to reduce global burden of sickle cell anemia
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Unified and consistent intervention strategies on a supranational level are necessary to reduce sickle cell anemia mortality in children aged younger than 5 years, according to researchers.
Incidence of sickle cell anemia is expected to increase as a result of improved survival and population movements from high-prevalence to low-prevalence nations.
Frederic B. Piel, PhD, of the evolutionary ecology of infectious disease group at the University of Oxford, and colleagues from the University of Oxford and Imperial College in the United Kingdom and the KEMRI/Wellcome Trust Research Programme in Kenya, aimed to estimate trends in the future number of newborns with sickle cell anemia and how interventions might impact survival among children with the disease.
“Our quantitative approach confirms that the global burden of sickle cell anemia is increasing and highlights the need to develop specific national policies for appropriate public health planning, particularly in low- and middle-income countries,” Piel told HemOnc Today.
The authors projected that the number of newborns with sickle cell anemia will increase from 305,800 in 2010 to 404,200 in 2050. The largest projected increases are expected to occur in Nigeria —from 91,000 newborns with the disease in 2010 to an estimated 140,800 by 2050 — and the Democratic Republic of the Congo, where incidence is projected to increase from 39,700 to 44,700. In India, incidence is expected to decrease from 44,400 newborns in 2010 to 33,900 in 2050.
The implementation of strategies such as prenatal diagnosis, penicillin prophylaxis and vaccination in 2015 could yield significant mortality reductions among affected children aged younger than 5 years, according to Piel and colleagues. They estimated that 5.3 million lives could be prolonged by 2050 if these strategies are successfully employed.
The implementation of large-scale universal screening could save the lives of as many as 9.8 million newborns with sickle cell anemia around the world in that same time period, researchers projected. Eighty-five percent of those deaths could be prevented in sub-Saharan Africa, according to the researchers.
“Policy makers must set priorities in an environment of multiple burdens, unfinished agendas, competing discourses, and the voices of interest groups, a process that has been described as a chaos of purposes and accidents,” Edward Fottrell, PhD, MPH, and David Osrin, PhD, both of the Institute for Global Health at UCL Institute of Child Health in London, wrote in an accompanying editorial. “In an environment of Realpolitik, the generation of estimates of burden is important for advocacy. Characteristically, investigators working in an important public health field that has not received global attention lay down the strategic epidemiology, as Piel and colleagues are doing, demonstrating that lack of progress will hinder efforts to attain targets such as those of the Millennium Development Goals.”
The researchers suggested that the priority is to identify births of infants with sickle cell anemia, but those births could be avoided through prenatal diagnosis and genetic counseling, Fottrell and Osrin wrote.
“Routine newborn screening remains costly — but is likely to become less so — and may miss infants born at home,” they wrote. “Penicillin prophylaxis and pneumococcal immunization are possible in most health care systems. The most beneficial approach involves comprehensive care: family education, routine immunization, malaria prevention, nutrition and hydration, prophylactic antibiotics, folic acid supplements, transfusion when required, support groups for children and their families, protocols for the management of acute events by health workers and — most importantly — regular follow-up. Human resources for health need to be well trained, and the medicines required need to be affordable and available, including the pain relief required by many people with [sickle-cell disease].”
For more information:
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Piel FB. PLoS Med. 2013;doi:10.1371/journal.pmed.1001484.
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Fottrell E. PLoS Med. 2013;doi:10.1371/journal.pmed.1001483.
Perspective
Back to TopAlexis A. Thompson, MD, MPH
Estimation of the number of current newborns with sickle cell anemia worldwide, and the anticipated increase over the next 4 decades, should provide additional impetus to develop and expand initiatives that can potentially saves lives and reduce suffering. Assuming a relatively stable sickle gene frequency over this period and using projections for birth rates and population densities, there are opportunities in countries such as Nigeria, the Democratic Republic of Congo and India — which accounted for more than half of newborns with sickle cell in 2010 — for both research and resource allocation for disease diagnosis and basic health interventions.
Differences in existing public health infrastructure in low- and middle-income countries — as well as less predictable factors such as political instability, migration and environmental changes — may impact the magnitude of improvement in excess mortality to varying degrees, and some interventions will take much longer to fully implement. Improvements can also be realized to lesser degrees in higher-income countries. These new data provide an important framework to increase awareness of sickle cell, and the need for increased engagement by hematologists with public health agencies regarding priorities for policies and funding.
Perspective
Back to TopElliott Vichinsky, MD
This is a landmark study that estimates trends in the global numbers of newborns with sickle cell disease and the impact of specific health interventions on survival. This is the first study to quantify the magnitude of increased births based on existing epidemiologic data and demographic projections. This information is critical to determining international public health policy. The quantitative changes in sickle cell disease is based on the increased predictions of sickle cell birth rates and the lives saved from the availability of basic medical care (newborn screening, penicillin prophylaxis and vaccinations). The number of newborns with sickle cell disease globally will increase from 305,000 in 2010 to 404,000 in 2050. The implementation of basic health interventions for sickle cell anemia could save the lives of up to 9.8 million newborns, particularly those born in sub-Saharan Africa, in the next 30 years.
This study has a number of recognized limitations. It focuses only on newborns, there is little analysis of phenotypic outcomes affected by genetic and environmental factors, and it does not analyze the impact of consanguineous marriages. It also lacks data on the costs and likelihood on implementing basic medical treatment in low- and middle-income countries. However, this is a seminal study that has the potential to change the lives of millions of people if world health organizations and countries implement adequate policy and infrastructure to address this enormous global public health burden.
Perspective
Back to Top
Clinton H. Joiner
The world public health community is awakening to the significance of hemoglobin disorders. Piel and colleagues have synthesized a global picture of the incidence of sickle cell disease using an innovative model incorporating local screening data, birth rates and other demographics. They measure the burden of disease by the number of newborns that could potentially be saved from early mortality by newborn screening and infection prophylaxis. The numbers are striking, mounting to millions of lives by 2050. The costs of screening programs and the measures necessary to prevent death in infants with sickle cell disease are substantial, especially for countries struggling to provide basic health services.
Mary Frederickson
Newborn screening and preventative measures will certainly save lives, but the burden of sickle cell disease will not soon be lifted. It will shift to older age groups and place expanding demands on fragile economies, as Piel and colleagues point out, as infants who would have died become children and adults. The quantitative impact of this shift on the health care systems, economies and societies of affected countries is unclear and difficult to predict. However, it is clear that simply importing western-style management of sickle cell disease — requiring transfusions, sophisticated imaging, intensive medical and surgical management, and laboratory-guided drug therapies — will not be a viable option, at least in the decades ahead. Innovative approaches to therapy, grounded in local realities, must be developed so that increased longevity for children with sickle cell disease does not impose new burdens on their families and their societies.
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