Is Sickle Cell Anemia a Neglected Tropical Disease?

Governments and philanthropic organizations collectively pour hundreds of millions of dollars annually into the prevention and treatment of a select group of life-threatening infectious diseases that includes malaria, HIV/AIDS, and tuberculosis. The merits of these high-profile programs are indisputable, yet emerging interest in traditionally neglected tropical diseases (NTDs) suggests that the lights of global awareness and medical treatment are now shining more brightly on lesserknown infections, which remain scourges for more than a billion persons worldwide [1]. More recently, noncommunicable diseases also have garnered attention and funding as causes of serious morbidity and mortality, including diabetes, cardiovascular disease, and cancer [2]. Standing awkwardly alone between these traditionally communicable and noncommunicable diseases, however, is sickle cell anemia (SCA). As a congenital but noninfectious disease with high childhood mortality, SCA is a serious health condition that represents a silent killer of children with enormous global burden. Only with improved recognition and a concerted worldwide effort can we begin to address this disparity and offer life-saving interventions to millions of affected children. Should SCA be considered an NTD? From the viewpoint of this hematologist, an NTD can be operationally defined as any serious medical affliction with the following characteristics: (1) worldwide distribution, typically affecting millions; (2) highest burden among the most impoverished and disadvantaged populations; (3) serious morbidity, diminished quality of life, and even mortality; (4) comorbidity for other life-threatening diseases; (5) relatively simple diagnostic testing; and (6) inexpensive treatment options. Although NTDs are usually infectious and communicable, perhaps those should not be strict requirements. Indeed, based on these criteria, SCA is long overdue for membership in this elite but tragic club of global medical maladies. Perhaps ironically, such a designation of SCA may allow this currently ‘‘invisible’’ lethal disease to become more noticeable as a ‘‘neglected’’ disease. How Did Sickle Cell Anemia Arise?