Giuliana Grossi

New data from an extensive literature review found that while most studies reported mild to moderate COVID-19-related disease course in patients with 

 or sickle cell trait (SCT), the literature suggests that SCD is associated with an increased risk of hospitalization and death from COVID-19, unless compared to controls with similar comorbidities and end-organ damage.

Lead investigator, Wouter Hoogenboom, PhD, Research Fellow, Department of Radiology, Albert Einstein College of Medicine, Montefiore Medical Center, explained that many of the risk factors for a poor COVID-19 outcome were the same between individuals with sickle cell disease or trait and the general population.

"And on top of that," he added "people with sickle cell also have sickle cell related symptoms, like pain crises, and acute chest syndrome, that also add to their vulnerability."

This study isn't the first one that the investigators conducted regarding the relationship between COVID-19 and sickle cell, according to Tim Duong, PhD, Professor, Vice Chair for Research Department of Radiology, Albert Einstein College of Medicine, Montefiore Medical Center.

Their previous study that examined a cohort of patients from the Bronx who had COVID-19 and sickle cell disease or trait, produced mixed conclusions.

"So, what we found as we were doing our study was that the literature on the effects of COVID-19 on sickle cell disease and trait are scarce and controversial," Duong said, "with some papers reporting positive effects, or positive outcome or better outcome, than the general population, some reported worse outcome than the general population."

With their latest study, the investigators decided to perform a critical appraisal of the literature in order to further understand and integrate the available data on the subject. 

The rest of the interview will be published on 

on Twitter so you don't miss Hoogenboom and Duong discuss the nuances of their findings. 

Videos

Investigators conducted an extensive literature review on clinical COVID-19 outcomes in patients with SCD or SCT to better navigate treatment during the pandemic. 

Wouter Hoogenboom, PhD, Tim Duong, PhD: Examining COVID-19 Outcomes in SCD, SCT

Sickle Cell Disease Association of America (SCDAA)

 has a new president and CEO. After serving on the association's board of directors for 3 years, and as the chair of the fundraising committee, Regina Hartfield assumed the position on January 14, 2022.

, Hartfield shared her priorities for 2022 as the new leader of the association. 

One of the first priorities is to increase the amount of member organizations in partnership with SCDAA across the US. Currently, SCDAA partners with 53 organizations that span 29 states, collectively serving over 500,000 individuals with 

Another pritority that Hartfield was excited to share was a clinical trial finder that SCDAA first launched in October 2021. The goal is to continue to grow number of people who have access to it and in turn, increase the amount of people participating in clinical trials. 

Finding clinical trials can be extremely difficult for people with sickle cell disease. Before SCDAA offered this clinical trial finder in a consolidated location, many people had to search through different locations or resources without a guarantee that anything would come up. 

"It's a sense of empowerment," Hartfield said. "You know, people with sickle cell disease, or their caregivers, can go on to the clinical trial finder, find out what clinical trials are out there that they're appropriate for in terms of the logistics and health situations."

Hartfield touched on the importance of education for those within the sickle cell disease community and the health care community. 

"It is very, very important for everyone in the sickle cell space to understand what those living with sickle cell disease are going through, what their needs are in terms of care, medication, timing of care, access," she said. 

After discussing recent legislative accomplishments and the expansion of SCDAA's global partnerships, Hartfield concluded the interview by expressing her gratitude.

"Every time I meet someone living with sickle cell disease, I am humbled and in awe of who they are, and what they do, and their contributions," she said. "They are unstoppable."

"We want to be equally as unstoppable as they are."

Regina Hartfield is the new CEO and president of the Sickle Cell Association of America. Listen to our interview with her to find out what's in store for 2022.

Regina Hartfield is Now the CEO and President of SCDAA

Valentina Baez-Sosa, MD, Chief Hematology-Oncology Fellow at MedStar Washington Hospital Center, discussed the findings of her study, "

Impact of Alloimmunization in Patients with Sickle Cell Disease

The new data show a higher prevalence of alloimmunization in patients with 

(25.5%) than in the previous literature (7- 59%) and the general population (2%). 

An increase in alloimmunization was associated with an increased number of exchanges but not with simple transfusions. Independent from iron overload, alloimmunization was also associated with increased end organ damage.

The benefits of red blood cell transfusion therapy are prominent among the sickle cell disease population. Baez-Sosa explained that it can increase the oxygen saturation levels of a patient by 1-6%, allowing more oxygen delivery to the tissue. 

"However," she said, "transfusion therapy can lead to the development of antibodies to the donor's red cell antigens, or alloimmunization. And this has serious complications including the delay hemolytic transfusion reaction, hyper hemolysis, and worsening vaso-occlusive episodes (VOE), and even life threatening anemia."

"So, patients with sickle cell disease are among one of the most frequently alloimmunized transfused population," Baez-Sosa continued, "probably because of the polymorphism difference in immunogenic, red blood cell antigens between the predominantly white general blood donors and patients of predominantly African descent."

In preparation for this study, Baez-Sosa said her team found only a few case series that showed a proposed pathway involving red blood cell alloimmunization and decreased survival in patients with sickle cell disease. 

"Putting the long term consequences of iron overload aside," she said, "there is no long term data regarding the clinical consequences, multiorgan damage, and increased mortality in sickle cell patients comparing alloimmunized to non-alloimmunized."

With results indicating a higher prevalence of alloimmunization among these patients, the complications associated with alloimmunization and sickle cell disease become more prevalent as well. 

"And that is why we believe that strategies to decrease alloimmunization are needed to prevent these complications," Baez-Sosa said. 

Baez-Sosa explains why further investigation of alloimmunization in patients with sickle cell disease is crucial.

Valentina Baez-Sosa, MD: New Data on Alloimmunization in Patients with SCD

Unfortunately, on December 31, 2021, the 

, Jr, PhD—a warrior, a researcher, and a friend. 

was an assistant professor in the Division of Hematology at the Johns Hopkins School of Medicine, part of the core faculty at the Johns Hopkins Berman Institute of Bioethics, as well as the Welch Center for Prevention, Epidemiology, and Clinical Research.

As an individual living with sickle cell disease, Haywood focused his studies and career on understanding the variety of obstacles that present to people with the disease, in hopes of improving the quality of life for everyone in the community. 

Mary Catherine Beach, MD, MPH, worked alongside him as a Professor of Medicine, Johns Hopkins School of Medicine, Core Faculty, Johns Hopkins Berman Institute of Bioethics, and a Professor, Department of Health, Behavior and Society, Johns Hopkins Bloomberg School of Public Health. 

Beach shared that Haywood was committed to speaking to patient advocacy groups, no matter how much travel was involved. 

"He was selfless in the way he prioritized his life," Beach said. "He would really try to meet every request that was made of him from patient advocacy groups across the country to plenary presentations."

For his dissertation, Haywood presented 1 of the first studies to thoroughly examine the construct of trust among sickle cell disease patients and clinicians, and that was only the start for him.

Beach and Haywood became research partners early in his career. They collaborated on studies that evaluated respect and communication dynamics between patient and clinician, specifically in regards to those with sickle cell disease. 

Early on, they conducted a systematic review together to examine the quality of care for patients with sickle cell disease. An abundance of studies all revealed the same thing: clinicians had negative attitudes toward the patients, who would experience incredible amounts of pain and distress. 

"(We) came to the conclusion at some point, you know, a few years ago that we don't need more studies demonstrating that the quality of care for people with sickle cell disease is inadequate," Beach explained. 

"We don't need to demonstrate that this is a problem anymore," she continued. "We need to fix it."

Haywood experienced the extreme pain and medical complications that came with sickle cell disease throughout his life. 

In spite of dealing with the adversity of the disease, Beach emphasized how insightful, smart, thoughtful, and important his work was.

"He persevered through all of that," she said, "to bring the message and do the work that he thought would help other people with sickle cell disease."

Beach shares about Haywood's life, research, and impact on the sickle cell disease community after working alongside him at Johns Hopkins School of Medicine.

Giuliana Grossi

Articles