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Saving Lives in Sickle Cell Disease: Why ER Visits and Provider Awareness Are Key


Not all chronic diseases receive equal attention. Funding, visibility, and treatment are influenced by factors ranging from pop culture and publicity to taboo and stigma. Sickle cell disease (SCD) and cystic fibrosis (CF), for example, are two frequently compared diseases because they share clinical similarities, but the two illnesses impact different patient populations and receive vastly different levels of public attention. This comparison highlights the pervasiveness of racial disparities in the health sciences.

Both are inherited diseases that affect quality of life, require a large volume of care, and lead to a lower life expectancy. CF is much less common than SCD; it affects mainly White Americans at a rate of about 1 in 3,200, while SCD affects primarily Black Americans at a rate of about 1 in 365. Nevertheless, CF receives roughly 10 times the research funding per patient. This leads to more advances and treatment — for instance, the FDA has approved CF medications at a rate of about 4:1 compared with medications for SCD. These differences are largely due to racism, which impacts not only the care patients experience, but the media attention for these diseases, philanthropic and funding efforts, and personal biases by those in positions of power and influence.

SCD affects people of all races globally, and emerged as a protective trait against severe malaria. In today’s United States, it is found primarily among Black Americans, due to the country’s history of chattel slavery and the transatlantic slave trade. SCD causes painful clotting events that can affect the heart, lungs, and eyes, and can be life threatening. Patients with the disorder require comprehensive lifelong health management to navigate its symptoms, impacts, and risks.

The most common cause of hospitalization and death amongst people with SCD is acute chest syndrome (ACS), a complication that occurs when blood flow to the lungs becomes blocked, or when a lung infection grows. People with ACS develop pain, cough, fever, and fatigue, and must be diagnosed and treated as early as possible. Barriers in access to care and racial stigma can affect the timeliness of treatment received by people with SCD and ACS. One recent study found that one third of SCD patients reported having a negative experience when they visited the emergency department (ED), including having severe pain or of feeling there was a lack of empathy for their pain. Half reported that negative past ED experiences influenced their care-seeking decisions. These and other factors accumulate, leading to significant gaps in care that increase the risk of serious complications and suffering in SCD patients. 

To improve our understanding of how SCD patients present, members of our Black Dragons Affinity Group looked at the journeys of SCD patients in the 30 days before they were diagnosed with ACS. Using Komodo’s Prism software and Patient Journey feature, we sought to identify key red-flag symptoms leading up to a diagnosis. Here is what we found: 

The most common "red flag" symptoms of ACS were chest pain and fever.
In the 30 days prior to experiencing ACS, our data indicates 17% of SCD patients were reported to have experienced chest pain and 10% were reported to have experienced fever. Other symptoms included cough, fast-paced breathing, shortness of breath or grunting, and wheezing. Because symptoms are often coded less consistently and diligently compared to diagnoses and treatments, it’s likely these symptoms are under-documented in the data seen here. 

About 17% of SCD patients diagnosed with ACS received inpatient or emergency care in the 30 days prior to experiencing ACS.
These encounters may represent opportunities for intervention, when SCD patients present with emerging symptoms of ACS. This also stresses the need for positive experiences of care in the emergency department for SCD patients, who have a high need for emergency services and may experience worse outcomes due to delays in care. These encounters also present an opportunity for future research to identify important moments in the patient journey, to further understand the onset of ACS.

Most SCD patients with ACS developed the pulmonary complication several months to years after their SCD diagnosis. 
We found that 1% of people with SCD experienced ACS within 90 days of their SCD diagnosis, and 3% experienced it within one year. This more than doubled by two years from diagnosis, when 7% of patients had experienced ACS. This tells us there is typically a delay in onset of ACS after a SCD diagnosis, and that risk may increase progressively, making it important for providers to monitor patients for symptoms of ACS over time.

One step in fighting stigma for SCD patients who present with ACS is training emergency room providers to ask about SCD and to know the red-flag symptoms of ACS. While the social construct of race should not determine care or outcomes, and while SCD should not be categorized as a “Black disease,” racial bias training and awareness of risk profiles in patients of color can help combat stigma and improve the likelihood of a patient receiving a comprehensive assessment and having a positive emergency room experience. 

Komodo Health’s mission is to reduce the burden of disease. We cannot achieve our mission as long as health disparities continue to persist for people of color. As we work with the industry to achieve health equity, real-world, real-time data and AI offer a newfound capacity to identify and measure disparities in health, care, and outcomes that may previously have been obfuscated. At Komodo, patient journey analyses and highly linked, Census-level race and ethnicity data allow us to better understand the experiences of patients of color, and pinpoint moments in the patient journey where targeted intervention is needed.

For all patients, early diagnosis saves lives. To improve outcomes and experiences for SCD patients, we must work to break down all forms of barriers, especially those barriers erected by racism — implicit, explicit, systemic, and social.

Learn how late diagnosis and delayed treatment result in worse outcomes for Black patients with colorectal cancer and kidney disease, in Komodo Health’s analyses done in partnership with BLKHLTH.

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