The Challenge We Face: Sickle Cell Disease and the COVID-19 Pandemic

The American writer, James Baldwin, wrote, “not everything that is faced can be changed, but nothing can be changed until it is faced.” This idea particularly reverberates for patients and physicians who stand at the intersection of COVID-19 and sickle cell disease (SCD). As this community braced for the impact of COVID-19, the prognostication of patients with SCD who may become infected was challenging to forecast. SCD is a hereditary hematologic condition punctuated by episodes of acute exacerbation, progressive vasculopathy, end organ damage, and shortened life span. It has often led to stigmatization of these patients in the United States as well as difficulty accessing quality care.¹ 

In light of this, numerous questions surfaced: How would the current pandemic further challenge the biopsychosocial battle that each COVID-19–infected patient would inevitably face? How would an already fractured medical system, barely able to provide quality and equality in the care of these patients, respond? Providers of SCD care, especially those treating adult patients, have contended with a lack of resources, infrastructure, and funding for decades, and have watched patients navigate a broken medical system for just as long.²  As the media frenzy around disproportionate death rates in African American and minority communities became evident within the first several weeks of COVID-19’s rampage, for the SCD community, this was just another day on the job.

In the now silent inpatient SCD units across the globe (barren without their usual census of patients) the anxiety and fear of patients with SCD echo loudly. These patients are anxious to seek care in emergency departments owing to the fear of becoming infected. Alternately, they are receiving care from an unfamiliar set of providers, who themselves are stretched thin by their new enormous workload. Patients with SCD often are now choosing to delay or avoid care altogether, stay home, and suffer in pain. The fear in this patient group is amplified and justified by the known association between respiratory infections and acute chest syndrome — a leading cause of mortality — vaso-occlusive pain exacerbation, and severe hemolysis.

Furthermore, those patients with complex SCD are often suspended delicately in a balance, carefully orchestrated by their social support system and medical care team. COVID-19 has an unquestionable ability to devastate this fragile balance by disrupting the social support and overwhelming medical care. Social distancing amidst a quarantine has resulted in the crumbling of social support systems that require proximity to loved ones. This lack of social support has driven deep divides into the landscape of these patients’ well-being. Moreover, the re-deploying of nurses, social workers, and physicians, both by hospital administrators and infection have incapacitated the health delivery system and reduced the already meager resources available to them. SCD centers across the country, already stretched dangerously thin, are experiencing new challenges in their ability to care for individuals with medically complex SCD.

Necessity drove innovation in the SCD provider community, and hordes of dedicated and committed individuals proactively sprang into action. As the wave of COVID-19 hit the SCD population, the commendable, combined efforts of ASH, the Sickle Cell Disease Association of America (SCDAA), and many others around the globe, ensured that the SCD provider community was well-equipped to manage the pandemic and well-positioned to study its effects despite limited resources. The Medical and Research Advisory Committee  of the SCDAA, led by Drs. Biree Andemariam and Lewis Hsu, collated opinions on the management of COVID-19 from SCD experts around the world, and Dr. Julie Panepinto created Secure-SCD, an SCD–COVID-19 registry with an urgency worthy of the devastating pandemic at hand. Physicians began to engage in immediate exchanges of information and discussion of challenging SCD and COVID-19 cases through an email forum created days before the first wave of sick patients hit our shores.^3-5 

Thanks to these efforts, people with SCD, especially those already connected to the health care system, have been able to receive lifesaving information and education about COVID-19, participate in webinars that address the medical and social impact of COVID-19 and the resultant isolation, and get help navigating the complex and often discordant recommendations that we are bombarded with by the media and different health care organizations. An early adoption of telehealth by many institutions, including ours, has led to the management of complications at home and a substantial decrease in emergency department visits and hospital admissions. Furthermore, existing research infrastructure has been repurposed to support clinical care and education, both in the United States and abroad. A query of Secure-SCD, as of April 25, 2020, indicates a 10 percent mortality rate among 105 patients with SCD and COVID-19. Some of these deaths occurred in individuals who previously had a mild disease course. Thankfully, the much-anticipated shortage of blood has not occurred yet, and we have been able to continue administering life-saving transfusions to our most vulnerable patients.

It is too early to assess the true impact of COVID-19 on SCD and other hemoglobinopathies. The SCD community has faced, and continues to face, many challenges. The historical context of these difficulties, that have required relentless advocacy and activism to reach minimally acceptable standards of biopsychosocial care, gives perspective to ephemeral events such as the COVID-19 pandemic. Despite this perspective, the unified SCD provider community, proactively, has helped to reduce the damage of the newest assault on the well-being of our patients. We can only hope that, as we recover from COVID-19 as a nation, the changes borne from necessity will drive us toward a more accessible and traversable health care system for patients with SCD.