When Actions Speak Louder Than Words — Racism and Sickle Cell Disease

“SCD [sickle cell disease] is a life-threatening, inherited blood disorder, affecting more than 100,000 Americans. Painful vaso-occlusive crises, the hallmark of SCD, result in substantial suffering and lead to associated stigma. Without adequate treatment, SCD affects all organs and is associated with decreased quality of life and a shortened life span. Among the dozens of conditions that are screened for in state newborn-screening programs, SCD is the most commonly detected condition, regardless of ethnicity. It is thus important to recognize SCD as a common and important medical condition among Americans, and not “just Black Americans.”

[..] Although SCD is a global disorder affecting people of all races, in the United States, as a direct result of the transatlantic slave trade, nearly all patients with SCD are Black. This fact would be mere medical trivia if we did not live in such a highly racialized society. Unfortunately, the social construct of race in America requires the majority of patients with SCD not only to face the consequences of a serious health condition, but also to navigate a society in which the color of their skin is often an unfair disadvantage.

[..] Although SCD was first described more than 100 years ago, the development of disease-modifying therapies has stagnated because of inadequate research funding, attributable at least in part to structural racism. Cystic fibrosis is comparable to SCD as an inherited, progressive, life-threatening disease associated with decreased quality of life and shortened life span, but it primarily affects White Americans. Cystic fibrosis affects one third fewer Americans than SCD but receives 7 to 11 times the research funding per patient, which results in disparate rates of development of medications: currently, the Food and Drug Administration has approved 4 medications for SCD and 15 for cystic fibrosis. We need to analyze the role of race and racism in the persistence of such discordant resource allocation. Federal funders, lawmakers, advocates, and health care leaders need to work together to reverse the impact of years of structural racism on SCD funding, research, and policy decisions.

[..] The term “sickler” — a word laced with racist overtones — is often used ignorantly to describe and depersonalize patients with SCD. Deplorably, especially in the midst of the opioid crisis, patients with SCD are often described as drug seekers and accused of feigning their pain, which results in inadequate treatment and more suffering. Because of the challenges in receiving adequate care and the stress associated with perceived racial stigma, many patients choose to avoid care altogether, further increasing the risk of life-threatening complications. It is unacceptable for the medical community to tolerate this racist mistreatment of patients with SCD in our emergency departments and hospitals.

[..] Racism is deeply ingrained in our society and culture. It informs unconscious judgments and implicit biases, even in well-intentioned clinicians, that can negatively affect the evaluation and care of patients and can be exacerbated in stress-filled emergency departments, hurried clinics, and busy wards. All health care providers must be open-minded and self-aware as they care for patients with SCD, recognizing their own implicit biases and making a conscious effort to treat patients equitably, regardless of race. They should be required to regularly examine their own racial biases in a supportive environment that helps reduce the impact of these biases on their behavior. Outpatient providers who develop long-term relationships with patients with SCD must be willing to have open, honest, and explicit conversations about race and the impact of racism on patients’ lives. Finally, inequitable treatment of patients based on race should be reported and addressed as urgently as are other hospital safety events.”

Full editorial, Power-Hays A and McGann PT. New England Journal of Medicine 2020.11.12