Chapter 27: Psychosocial Burden in Sickle Cell Disease

Overview

Sickle cell disease (SCD) has been described in Africa for centuries. Characteristics of the disease have become incorporated into local languages, impacting cultural understanding of the course and symptomology of SCD. For example, the prominence of sickle cell pain is the basis upon which SCD has been named in certain Ghanaian cultures. These names—chwechweechwe (“gnawing”; Ga), nuidudui (“biting”; Ewe), and ahotutuo (“body pinching”; Twi)—reflect the lived experiences of individuals affected by the disease.¹ Other names—onye kye ba (“s/he is not one who would live”; Twi)—capture the expectation of early mortality. Still others—ogbanje (“child who comes and goes”; Igbo) and sika be sa (“money will finish”; Twi)—reflect the emotional and financial impact on the family.²

SCD was first named in Western medicine following the 1910 description of “peculiar-shaped red blood cells” in a West Indian dental student experiencing anemia, pain, respiratory problems, and leg ulcers.³ The inheritance pattern, clinical course, and pathophysiologic features of this “new” disease were delineated over the next 40 years, as was the basis of the disease in an abnormality in the hemoglobin molecule.⁴ The field of molecular biology owes much to SCD as “the first molecular disease” that generated new scientific strategies and discoveries.⁵ However, the disease remained largely invisible clinically and in public awareness through the late 1940s, resulting in preventable morbidities and mortality.^6,7

After World War II, medical breakthroughs illuminated the clinical burdens of SCD (eg, pain, risk of early mortality) and approaches to clinical management, such as infection prevention.⁸ At the same time, the disease’s origin was firmly linked to the African continent, and clinical research and public awareness of SCD became fraught with racism and stigmatization.⁹ Public health efforts to prevent the disease resulted in many African Americans feeling forced to undergo sickle cell trait testing and subsequently facing discrimination in employment and in receiving health insurance.¹⁰ To activists such as the Black Panthers, the invisibility and pain experienced by individuals with SCD became an embodiment of the African American experience and a reflection of the community’s sociopolitical oppression.¹¹ The Black Panther party championed health as a human right and emphasized the inadequate funding designated for SCD care and research. Reports on funding disparities from SCD advocates, such as hematologist Robert Scott, MD, prompted the US Congress to provide funding for sickle cell trait screening and counseling, public awareness campaigns, research, and comprehensive SCD health care.⁸

During the early 1970s, the National Sickle Cell Disease Program launched at the National Heart, Lung, and Blood Institute (NHLBI), heralding major advancements in SCD care and research.¹² These advancements included the development of a newborn screen for SCD and the ultimate implementation of universal screening in every US state; providing SCD-positive infants with daily prophylactic penicillin to prevent fatal infections; educating parents about the signs of medical emergencies; and preventing ...