Age, race/Hispanic origin, and COVID-19 mortality among sickle cell disease patients in the United States

The COVID-19 pandemic remains a global health challenge, with 6.7 million deaths worldwide as of January 2023. It has illuminated the health iniquities in underserved communities and populations like those with sickle cell disease (SCD). Researchers have associated the COVID-19 outcome among SCD patients in other regions of the globe. The purpose of this retrospective cross-sectional observational study was to investigate the relationship between age, race/Hispanic origin, and COVID-19 mortality among persons with SCD in the United States from January 2020 to March 2021. The Krieger ecosocial theory of disease distribution framed the study. Data were drawn from an existing Centers for Disease Control and Prevention provisional SCD death data set (N = 140). The binary logistic regression analysis result showed a statistically significant relationship between age and race/Hispanic origin and COVID-19 mortality. The variability between ages was 42.9%;race/Hispanic origin was 29.9%;and age and race/Hispanic origin were the highest, with 62.4% dying from COVID-19. Non-Hispanic Black patients were 9.6 times more likely to die overall but those aged 60+ were 17.5 times more likely to die from COVID-19 than the reference groups (0-19-year-old and other race). This study can benefit the research community, public health workers, medical professionals, and policymakers to understand better and influence policy on developing and prioritizing age- and race-tailored preventive protocols and medical care. They may minimize pain and suffering while mitigating mortality from COVID-19 and other unforeseen future pandemics within the SCD community at home and abroad and positively effect social change. (PsycInfo Database Record (c) 2023 APA, all rights reserved)

The Impact of Sickle Cell Disease on Academic Performance among Affected Students.

BACKGROUND: Sickle cell disease (SCD) is a genetic disease that is highly prevalent in Jazan Province, Saudi Arabia, and is mostly characterized by many complications such as vaso-occlusive crises (VOC), acute chest syndrome (ACS) and well-documented neurological complications. These complications may affect patients' academic performance. METHODS: An observational, cross-sectional, retrospective study was conducted in Jazan Province. General and demographic data were collected and questions about academic performance of students with SCD were answered. Both t-tests and chi-square tests, along with multiple logistic regression, were used for analysis. RESULTS: 982 participants were selected for this study with a mean age of 23 years (SD: 7). Most of the participants were female (64%). The number of participants with SCD was 339 (36%), of whom 42% were male. Students with SCD recorded lower grade point averages (GPA) and more absences compared to healthy participants. Further, about 60% of students with SCD thought they performed better than 40% of the participants without SCD during the COVID-19 pandemic when most of the educational activities were online. CONCLUSION: As has been previously reported, this study suggested that the academic performance of students with SCD is negatively affected compared to healthy individuals, and this is mostly due to complications associated with the disease. Further, students with SCD acknowledged better performance with online education, an option that should be considered to improve their academic performance. National studies on a larger population are required by health and education officials, and supportive online educational programs are warranted to enhance the academic performance of this population.

Enhanced IgG immune response to COVID-19 vaccination in patients with sickle cell disease.

Patients with sickle cell disease (SCD) are considered to be immunocompromised, yet data on the antibody response to SARS-CoV-2 vaccination in SCD is limited. We investigated anti-SARS-CoV-2 IgG titres and overall neutralizing activity in 201 adults with SCD and demographically matched non-SCD controls. Unexpectedly, patients with SCD generate a more robust and durable COVID-19 vaccine IgG response compared to matched controls, though the neutralizing activity remained similar across both cohorts. These findings suggest that patients with SCD achieve a similar antibody response following COVID-19 vaccination compared to the general population, with implications for optimal vaccination strategies for patients with SCD.

Outcomes of COVID-19 hospitalizations in patients with sickle cell disease: A nationwide analysis.

INTRODUCTION: There is a paucity of data on the outcomes of coronavirus disease 2019 (COVID-19) in patients with sickle cell disease (SCD) in the United States. We examined the outcomes of patients with COVID-19 and SCD. METHODS: We utilized the National Inpatient Sample (NIS) to identify the data of patients diagnosed with COVID-19 and SCD in 2020 using the International Classification of Disease, Tenth Revision codes. In-hospital outcomes (invasive mechanical ventilation and mortality) were compared between SCD and non-SCD groups. RESULTS: Of the 1 057 550 COVID-19 hospitalizations, 2870 (0.3%) had SCD. The median age of the SCD group was 42 (IQR: 31) vs. 66 (IQR: 23) in the non-SCD group (p < .0001). Patients with SCD were likely to be females (62.02% vs. 37.98%, p < .0001), Blacks (87.81% vs. 12.19%, p < .0001), and in the lowest income quartile (50.62% vs. 11.15%, p < .0001). There was no difference in the outcomes between the two groups. There were increased odds of invasive mechanical ventilation and in-hospital mortality in COVID-19 in Asians, Hispanics, Native Americans, and Blacks (except for in-hospital mortality) compared to Whites. CONCLUSION: In-hospital mortality and invasive mechanical ventilation outcomes in SCD are comparable to that in non-SCD patients hospitalized with COVID-19.

Side effects following COVID-19 vaccination in pediatric patients with sickle cell disease.

Vulnerable patient populations have seen decreased rates of vaccination against SARS-CoV2-19 (COVID-19) due to hesitancies and distrust, magnified by a paucity of data for certain populations. The rate of COVID-19 vaccination in children with sickle cell disease (SCD) remains low despite the risk for severe complications, resulting in continued infections and hospitalizations from COVID-19. We sought to describe vaccine reactions, including vaso-occlusive crises, emergency department visits, and hospitalizations, in children with SCD. Our findings will start to provide the necessary vaccine side effect data to inform patients, caregivers, and clinicians considering the COVID-19 primary vaccination series.

Explainable artificial intelligence (XAI) in medical decision systems (MDSSs): healthcare systems perspective

The healthcare sector is very interested in machine learning (ML) and artificial intelligence (AI). Nevertheless, applying AI applications in scientific contexts is difficult due to explainability issues. Explainable AI (XAI) has been studied as a potential remedy for the problems with current AI methods. The usage of ML with XAI may be capable of both explaining models and making judgments, in contrast to AI techniques like deep learning. Computer applications called medical decision support systems (MDSS) affect the decisions doctors make regarding certain patients at a specific moment. MDSS has played a crucial role in systems' attempts to improve patient safety and the standard of care, particularly for non-communicable illnesses. They have moreover been a crucial prerequisite for effectively utilizing electronic healthcare (EHRs) data. This chapter offers a broad overview of the application of XAI in MDSS toward various infectious diseases, summarizes recent research on the use and effects of MDSS in healthcare with regard to non-communicable diseases, and offers suggestions for users to keep in mind as these systems are incorporated into healthcare systems and utilized outside of contexts for research and development.

Impact of the SARS-CoV-2 infection in individuals with sickle cell disease: an integrative review.

Sickle cell disease is the most common hemoglobinopathy among humans. As the condition promotes susceptibility to infections, chronic inflammation, and hypercoagulability disorders, several international agencies have included individuals with this disease in the COVID-19 risk group for severe outcomes. However, available information about the subject is not properly systematized yet. This review aimed to understand and summarize the scientific knowledge about the impact of SARS-CoV-2 infection in patients with sickle cell disease. Searches were performed in the Medline, PubMed, and Virtual Health Library databases based on descriptors chosen according to the Medical Subject Headings. We analyzed studies published between 2020 and October 2022, developed with qualitative, quantitative, or mixed methodology, and written in English, Spanish, or Portuguese. The search resulted in 90 articles organized into six categories. There is disagreement in the literature about how different aspects related to sickle cell disease, such as chronic inflammation status, hypercoagulability, hemolytic anemia, use of hydroxyurea, and access to medical care interference with the clinical course of COVID-19. These topics deserve further investigation. It is evident, however, that the infection may manifest in an atypical way and act as a trigger for the development of sickle cell-specific complications, such as acute chest syndrome and vaso-occlusive crises, conditions that are associated with great morbidity and mortality. Therefore, healthcare professionals must be aware of the different forms of presentation of COVID-19 among these individuals. Specific guidelines and therapeutic protocols, as well as public policies for sickle cell individuals, must be considered. Systematic review registration: This review (https://doi.org/10.17605/OSF.IO/NH4AS) and the review protocol (https://osf.io/3y649/) are registered in the Open Science Framework platform.

The evolution of the COVID-19 pandemic in paediatric patients with sickle cell disease: From Alpha to Omicron.

We compare the impact of SARS-CoV-2 variants on healthcare utilization and clinical presentation in paediatric patients with sickle cell disease (SCD). One hundred and ninety-one unique patients with SCD and positive SARS-CoV-2 polymerase chain reactions were identified between March 2020 and January 2022. Hospitalizations, which accounted for 42% (N = 81) of cases, were highest during the Delta dominant era (48%) and lowest during Omicron (36%) (p = 0.285). The most common SCD-related complication was vaso-occlusive pain (37%, N = 71), which accounted for 51% of all hospital admissions (N = 41), and acute chest was highest in the Alpha variant era (N = 15). Overall, COVID-19 remained mild in clinical severity within most paediatric SCD patients.

Prevalence of SARS-CoV-2 in hemoglobinopathies is modified by age and race.

Prior literature has established a positive association between sickle cell disease and risk of contracting SARS-CoV-2. Data from a cross-sectional study evaluating COVID-19 testing devices (n = 10,567) was used to examine the association between underlying health conditions and SARS-CoV-2 infection in an urban metropolis in the southern United States. Firth's logistic regression was used to fit the model predicting SARS-CoV-2 positivity using vaccine status and different medical conditions commonly associated with COVID-19. Another model using the same method was built using SARS-CoV-2 positivity as the outcome and hemoglobinopathy presence, age (<16 Years vs. ≥16 Years), race/ethnicity and comorbidities, including hemoglobinopathy, as the factors. Our first model showed a significant association between hemoglobinopathy and SARS-CoV-2 infection (OR: 2.28, 95 % CI: (1.17,4.35), P = 0.016). However, in the second model, this association was not maintained (OR: 1.35, 95 % CI: (0.72,2.50), P = 0.344). We conclude that the association between SARS-CoV-2 positivity and presence of hemoglobinopathies like sickle cell disease is confounded by race, age, and comorbidity status. Our results illuminate previous findings by identifying underlying clinical/demographic factors that confound the reported association between hemoglobinopathies and SARS-CoV-2. These findings demonstrate how social determinants of health may influence disease manifestations more than genetics alone.

COVID-19 and Sickle Cell Disease: Two Independent Risk Factors for Venous Thromboembolism.

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has been widely documented as a multi-systemic illness and associated with an increased incidence of thromboses. Likewise, sickle cell disease (SCD) is a hematologic disease responsible for widespread effects on the vasculature and is also associated with elevated thrombotic risk. In this review, we examine the incidence rates of venous thromboembolism (VTE) in SCD and COVID-19 independently and review the mechanisms of coagulopathy associated with both diseases. We describe the possible associations and commonalities between VTE mechanisms, as both diseases cause widespread inflammation that influences each tenet of Virchow's triad. We also discuss current anticoagulation guideline recommendations for the prevention of VTE events in each of these diseases. We report on current literature to date describing rates of VTE in SCD-COVID-19 patients and outline prospective areas of research to further understand the possible synergistic influence of coagulopathy in these patients. The association between SCD and COVID-19 remains a largely under-researched area of coagulopathy in current hematology and thrombotic literature, and our report lays out potential future prospects in the field.

Strategies to meet blood demand for transfusions during the COVID-19 pandemic: lessons learnt from a large central hospital in Malawi

Background: The coronavirus disease-19 (COVID-19) pandemic affected blood banks globally. We sought to examine how COVID-19 affected blood supply and transfusion in our institution. Method(s): The study was conducted at the Queen Elizabeth Central Hospital (QECH) and the Malawi Blood Transfusion Service (MBTS) in Malawi. Data from April to November 2020, collected during the pandemic, were compared with data from the same time period in 2019 pre-COVID-19. Additionally, in-depth interviews with key personnel were conducted at both institutions. Statistical analysis was performed using Stata 15 and qualitative data were analyzed using Nvivo software. Result(s): There was a significant reduction in blood supplied to the QECH from 7,303 [2019] to 6,028 units [2020] (P<0.04). The highest reduction in blood supply was to the Adult Emergency & Trauma department (29%) while the lowest was in Obstetrics & Gynecology, and Pediatric departments (17% reduction each). This is despite that the transfusion services continued to conduct blood drives during the pandemic, and the hospital laboratory prioritized blood issuing for emergency indications. Conclusion(s): Blood supply has significantly reduced during COVID-19 pandemic in our centers. Developing plans for overcoming similar shortages in future pandemics is critical.Copyright © Annals of Blood. All rights reserved.

Late PAMM-Like Lesions in a Patient with HIV Retinopathy

This report describes a case of a newly diagnosed 49-year-old HIV patient, who presented with decreased visual acuity and retinal lesions characterized by ischemia at the level of the deep retinal capillary plexus, documented with optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and visual fields testing. These lesions closely resembled the morphologic and clinical characteristics of late paracentral acute middle maculopathy. The presence of these lesions suggests that HIV microangiopathy can potentially affect both superficial and deep retinal capillary plexuses.

A perspective on the sickle cell disease international COVID-19 registry.

To understand the risks and outcomes of COVID-19 in the sickle cell disease (SCD) population, our team established a rapid reporting registry to collect data on the course of COVID-19 illness in individuals with SCD. The registry includes cases reported voluntarily by providers. All data are collected through an online case report form available at covidsicklecell.org. The registry helped to recognize patients with SCD as a population at risk of severe COVID-19 illness and to identify comorbidities that put them at higher risk. In this report, we present data on 1045 reported COVID-19 cases based during a two-year long data collection period. Data include 590 (56.5%) children and 455 (43.5%) adults; 51.2% of total population were female. Most individuals (63.1%) had HbSS genotype. Majority of individuals experienced mild symptoms (62.2% of children, 55.6% of adults). We also present a perspective on setting up the registry and experiences through its growth.

Impact of COVID-19 infection among indian sickle cell disease patients.

Background: Sickle cell disease (SCD) is one of the most common hemoglobinopathy disorders and is widely prevalent in India, especially in the tribal population. SCD patients are prone to develop recurrent respiratory infections and related complications owing to the microvascular occlusion and impaired immunological response. Objectives: We aimed to determine the prevalence and impact of COVID-19 in SCD patients from India. Methodology: We conducted a cross-sectional study in Chandrapur district of Maharashtra, India, between August and October 2021. After taking informed consent, details of 300 SCD patients' demographic data, history of COVID-19 testing, infection, symptoms related to COVID-19 in the past 1 year, hospitalization, complications, mortality, COVID-19 vaccination, and side effects were recorded. Results: We found that 93 (31%) of SCD patients had influenza-like symptoms during the COVID-19 pandemic with symptoms of fever (81.72%), cough (35.48%), sore throat (18.27%), headache (15.05%), and breathlessness (7.52%). A total of 13 (4.33%) SCD among 300 SCD were tested as COVID positive. Majority of them were mild cases and the 1st dose of COVID-19 vaccine was received by 47 (29.37%) of SCD patients and 10 (6.02%) of the patient had received second dose of vaccine. Conclusion: Low incidence of COVID-19 and milder disease spectrum in our study cohort suggests that there is no increased risk of COVID-19 mortality and morbidity in SCD patients compared to general population. However, the reason for low COVID vaccination in our study could be due to the fear of complications of COVID vaccine.

Avascular Necrosis of the Hip Triggered by COVID-19 Infection in a Patient with Sickle Cell Disease: A Case Report.

Introduction: The COVID-19 pandemic has affected many people around the world with its primary and secondary consequences. The use of high-dose steroids in its treatment brought along steroid-related femoral head avascular necrosis (AVN) complication. Case Presentation: We present a case of bilaterally developed femoral head AVN following COVID-19 infection in a patient with sickle cell disease (SCD), without the history of steroid use. Conclusion: With this case report, we aimed to raise awareness that COVID-19 infection may trigger AVN of the hip joint in SCD patients.

Renal Amyloid-Associated (AA) Amyloidosis in a Sickle Cell Patient: A Case Report and Literature Review.

Renal amyloid-associated (AA) amyloidosis is a rare occurrence in sickle cell disease (SCD). Very little literature is available on renal AA amyloidosis in sickle cell disease. Nephrotic range proteinuria is associated with higher mortality among patients with SCD. We present a case of a young reproductive-age African American woman who presented with massive nephrotic range proteinuria. Other more common causes of AA amyloidosis such as immunologic and infectious etiologies were ruled out by history, physical examination, radiologic investigation, and serology. Renal biopsy showed mesangial expansion with Congo red-positive material. Staining for immunoglobulins was negative. Electron microscopy showed nonbranching fibrils. These findings were consistent with AA amyloidosis. This case report adds to the rare findings of renal AA amyloidosis in sickle cell disease. The patient refused any intervention to decrease her Glomerular Filtration Rate (GFR) in the hopes of potentially reversing the disabling proteinuria. We report sickle cell disease presenting with nephrotic syndrome secondary to AA amyloid.

Diagnosis of Bone Marrow Necrosis following Severe Vaso-Occlusive Crisis in Patient with Compound Heterozygous Sickle Cell Disease

Introduction: Bone marrow necrosis is a rare entity that can develop in context of a sickle cell disease vaso-occlusive crisis. Its physiopathology is related to an endothelial dysfunction taking place in bone marrow microvasculature. Case Presentation: A 30-year-old patient with history of compound heterozygous sickle cell disease was admitted following SARS-CoV-2 infection with fever and diarrhea. After initial favorable evolution, he developed a severe vaso-occlusive crisis with intense hemolysis and multi-organ ischemic complications. Patient then developed high fever and hypoxemia. With the suspicion of acute thoracic syndrome, a red blood cell exchange was performed. Respiratory symptoms ceased but patient persisted febrile with very high levels of acute phase reactants, persistent pancytopenia, and leucoerythroblastic reaction. An infectious cause was ruled out. Afterward, bone marrow aspiration and bone marrow biopsy showed a picture of bone marrow necrosis, which is an extremely rare complication of vaso-occlusive crisis but, paradoxically, more frequent in milder heterozygote cases of sickle cell disease. Ultimately, large deposits of complement membrane attack complex (particles C5b-9) were demonstrated after incubation of laboratory endothelial cells with activated plasma from the patient. Discussion: The clinical presentation and findings are consistent with a case of bone marrow necrosis. In this setting, the demonstration of complement as a potential cause of the endothelial dysfunction mimics the pattern of atypical hemolytic uremic syndrome and other microangiopathic anemias. This dysregulation may be a potential therapeutic target for new complement activation blockers. [ FROM AUTHOR] Copyright of Transfusion Medicine & Hemotherapy is the property of Karger AG and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full . (Copyright applies to all s.)

Adenotonsillectomy in high risk patients: Hematologic abnormalities and COVID-19 considerations.

Adenotonsillectomy is one of the most common surgical procedures performed by otolaryngologists. It is vital that surgeons are aware of the risks in performing this surgery especially during the COVID-19 pandemic and in children with hematologic disorders. In this review we describe common hematologic disorders often noted in pediatric patients undergoing this procedure, as well as proper screening and management of these patients. In addition, we also address the impact of the COVID-19 pandemic and some measures to help mitigate the risks of this procedure during this time.

Emergency Management of Priapism

Pathophysiology To have an erection, the smooth muscle of the cavernosal arteries and tissue must be relaxed, resulting in increased non-ischemic inflow and decreased venous outflow. The sickling of red blood cells can decrease venous outflow in the corpora cavernosa during an erection leading to low-flow priapism in men with sickle cell disease. Doppler ultrasound can be utilized to observe if blood flow is low or absent, but corporal aspiration is the gold standard. Adjunctive laboratory evaluation should be performed based on the clinical concern for blood dyscrasia or other conditions affecting clinical decision-making.

Redox stress in COVID-19: Implications for hematologic disorders.

COVID-19 is the respiratory illness caused by the beta coronavirus SARS-CoV-2. COVID-19 is complicated by an increased risk for adverse thrombotic events that promote organ failure and death. While the mechanism of action for SARS-CoV-2 is still being understood, how SARS-CoV-2 infection impacts the redox environment in hematologic conditions is unclear. In this review, the redox mechanisms contributing to SARS-CoV-2 infection, coagulopathy and inflammation are briefly discussed. Specifically, sources of oxidant generation by hematopoietic and non-hematopoietic cells are identified with special emphasis on leukocytes, platelets, red cells, and endothelial cells. Furthermore, reactive cysteines in SARS-CoV-2 are also discussed with respect to oxidative cysteine modification and current therapeutic implications. Lastly, sickle cell disease will be discussed as a hematologic disorder with a pre-existing prothrombotic redox condition that complicates treatment strategies for COVID-19. An understanding of the redox mechanism may identify potential targets for COVID-19-mediated thrombosis in hematologic disorders.