Sickle Cell Issue Editorial

Abstract

The year 2010 marks the 100th anniversary of the first case of sickle cell disease (SCD) reported in the medical literature: James B. Herrick’s landmark article, “Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia,” appeared in the November 1910 issue of the Archives of Internal Medicine. In recognition of this significant event, the National Medical Association is dedicating the entire November issue of its journal to this important disorder, which continues to wreak havoc on the lives of SCD patients and their families. Having learned much during the past 100 years, medical researchers and practitioners have developed many helpful interventions. The continuing toll in human suffering highlights the fact that much more remains to be done. It should be noted that 1910 merely marks the year Western medicine recognized the disease—not the year SCD made its appearance on this planet. As pointed out in both Savitt’s and Ballas’ articles in this issue, people in West Africa knew of the disease, though by different names, and suffered from it for centuries before 1910. Permit us editorial prerogative to speculate for a moment about the relationship between the African slave trade and SCD in West Africa. In the many recorded accounts of the slave trade along the west coast of Africa from Senegambia to present-day Angola, there were 2 methods of “warehousing” slaves for trans-Atlantic shipment after they had been captured/collected from inland: (1) fort trade (in fixed fortifications), which was conducted on land such as at the famous Cape Coast Castle on the Gold Coast (present-day Ghana), in which European traders sold their slaves to ship captains; and (2) boat trade, which took place where there were no forts and slaves were ferried out to a ship via canoes or longboats and traded on the main deck of a slave ship. This was also referred to as the black trade because it was controlled largely by African merchants from the traditional trading states. In either venue, captives often lived in horrible conditions for many weeks until the ocean-going slave ship had acquired a full cargo and could begin its journey across the ocean. Even at anchor, there was little ventilation, a restricted water supply, and a meager subsistence diet. Although many prisoners fell victim—both before and during the Middle Passage—to disease, starvation, and depression/psychoses, it is also likely that a small number of these newly enslaved males and females, not noticed as sickly or weak, were sufferers of SCD and died in sickle cell crisis while confined in their contained and unventilated spaces characterized by increased carbon dioxide and reduced oxygen content. Those healthy captives who were unknowing carriers of the sickle cell gene took that gene with them to North and South Americas where, if they mated with another carrier, created offspring with SCD. Much progress has been made in the scientific understanding and management of SCD since its discovery. One of the latest and most promising advances has been the development of hematopoietic stem cell transplantation. Though it offers a cure and has been used successfully in a small number of patients, this treatment is associated with significant morbidity and mortality and requires further refinement. We would like to pay homage to Dr Roland B. Scott, director of the Comprehensive Sickle Cell Center at Howard University, Washington, DC, for many years, who was instrumental in bringing this disease to the attention of the entire nation in the early 1970s. One product of his efforts was the creation of 10 National Institutes of Health– mandated comprehensive sickle cell centers across the country that were in existence for more than 30 years. Sadly, this mandate expired, although many of the original centers continue to maintain centers of excellence in SCD with institutional, governmental, and private funding. Until there is a cure for SCD, perhaps Congress and the National Institutes of Health should revisit this decision. In our special sickle cell issue of the JNMA, we present historical perspectives on the disease and its discovery as well as reports of scientific advances and new work in diagnosis, management, and psychosocial concerns. We hope that these articles will provide readers with a sense of the progress made in managing SCD patients over the last 100 years and, perhaps, a glimpse into the future as it might unfold for both patients and providers.