Building partnerships to target sickle cell anemia in Africa

Sickle cell anemia (SCA), the most common hemoglobinopathy worldwide, is an important and under-recognized contributor to the mortality of children under age 5 in low- and middle-income countries (LMIC) 1. Survival of children with SCA throughout high-income countries has improved dramatically over the last four decades, largely attributable to implementation of newborn screening, early initiation of prophylactic penicillin, use of pneumococcal and Haemophilus influenzae vaccines, and parental education 2. In that period, local newborn screening initiatives and programs for clinical care have been piloted in a variety of sub-Saharan Africa countries and in India 3. Unfortunately, many of these have not yet grown into robust national programs. Little research has been dedicated to the effective implementation of sustainable sickle cell management and education programs in resource-limited settings, especially where the prevalence of SCA is so high. The inexpensive, life-saving interventions outlined above have not yet been universally implemented in many LMIC. Though it is widely understood that SCA is common and associated with high mortality in LMICs, much of the incidence and mortality data is based upon seminal public health studies that are now over 40 years old 4. In this issue of the American Journal of Hematology, McGann and colleagues demonstrate that neonatal screening for SCA and subsequent follow-up and treatment is feasible in a LMIC, Angola 5. They successfully obtained testing for a majority of infants born in five medical facilities in the capital, defining the local epidemiology of the disease. They designed and implemented a clinical program to provide care for newly diagnosed patients. Importantly, they integrated local human resources throughout the process. This article highlights a critical role for north-south partnerships to launch public health and research initiatives in LMICs. The authors illustrate the effectiveness of a three-way public-private partnership including academia, government, and the private sector. This partnership combined specific medical expertise, local ownership, as well as much needed funding. Local support is critical, as evidenced by the decision of the Ministry of Health to make screening compulsory. This is a major public health statement to the importance of SCA. The contribution of hematologist expertise to facilitate training and optimization of equipment was crucial. Of course, financial resources, a major impediment in many settings, are essential. Similar models have been used successfully for sickle cell research elsewhere in sub-Saharan Africa, but many more are needed 6, 7. The success of this three-way partnership should encourage similar partnerships in the future. The Angola team has modeled the components of a national sickle cell program as outlined by the World Health Assembly, including: early diagnosis, preventative care, awareness, and training 8. Through early diagnosis and initiating preventative care, they were able to bring infant mortality in the sickle cell population on par with national data. Equally important is developing a program where the medications and tools required to provide care are available. While a 56% rate of return should be considered a success, it highlights the critical role of raising awareness amongst the population and the medical community to encourage all parents to seek test results. Demonstrating feasibility in remote areas is vital. In order to minimize fatalities, community–based health care providers will need updated education and training. Not only is the burden of SCA high currently, but it is likely to increase. As efforts targeted at decreasing death from communicable causes of early childhood mortality continue to have impact, the population of infants and children with SCA will grow, and they will remain at high risk for premature death. The authors have demonstrated what can be accomplished through a focused intervention with a well-structured partnership, and have broadly opened the door to what is yet to be known. There are many important questions about SCA in Africa that have yet to be answered, many including the role of environmental and socioeconomic factors that may contribute to the poor survival statistics for these children, best practices for preventative management locally, and the role of existing and emerging therapies for SCA in LMICs. With several regional and national programs now underway, patients are surviving infancy and the need for the medical community to address these issues is now a reality.