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1.
J Investig Med High Impact Case Rep ; 10: 23247096221111778, 2022.
Article in English | MEDLINE | ID: covidwho-1950962

ABSTRACT

Severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection is commonly associated with neurological complications. Patients with sickle cell disease are at increased risk of developing neurologic complications throughout their lifetimes and often have underlying cardiopulmonary comorbidities that may predispose them to poor outcomes during serious infections. In this case series, we describe 2 patients with sickle cell disease who developed devastating neurologic complications following SARS-CoV-2 infection, which ultimately led to brain edema and death. We highlight the unusual manifestations of coronavirus disease 2019 in patients with sickle cell disease and address the risk of these patients to develop catastrophic neurologic injury due to COVID-19, if not recognized promptly.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Nervous System Diseases , Anemia, Sickle Cell/complications , COVID-19/complications , Comorbidity , Humans , Nervous System Diseases/epidemiology , Nervous System Diseases/etiology , SARS-CoV-2
2.
Am J Emerg Med ; 58: 235-244, 2022 08.
Article in English | MEDLINE | ID: covidwho-1914107

ABSTRACT

INTRODUCTION: Acute chest syndrome (ACS) in sickle cell disease (SCD) is a serious condition that carries with it a high rate of morbidity and mortality. OBJECTIVE: This review highlights the pearls and pitfalls of ACS in SCD, including diagnosis and management in the emergency department (ED) based on current evidence. DISCUSSION: ACS is defined by respiratory symptoms and/or fever and a new radiodensity on chest imaging in a patient with SCD. There are a variety of inciting causes, including infectious and non-infectious etiologies. Although ACS is more common in those with homozygous SCD, clinicians should consider ACS in all SCD patients, as ACS is a leading cause of death in SCD. Patients typically present with or develop respiratory symptoms including fever, cough, chest pain, and shortness of breath, which can progress to respiratory failure requiring mechanical ventilation in 20% of adult patients. However, the initial presentation can vary. While the first line imaging modality is classically chest radiograph, lung ultrasound has demonstrated promise. Further imaging to include computed tomography may be necessary. Management focuses on analgesia, oxygen supplementation, incentive spirometry, bronchodilators, rehydration, antibiotics, consideration for transfusion, and specialist consultation. Empiric antibiotics that cover atypical pathogens are necessary along with measures to increase oxygen-carrying capacity in those with hypoxemia such as simple transfusion or exchange transfusion. CONCLUSIONS: An understanding of ACS can assist emergency clinicians in diagnosing and managing this potentially deadly disease.


Subject(s)
Acute Chest Syndrome , Anemia, Sickle Cell , Acute Chest Syndrome/diagnosis , Acute Chest Syndrome/epidemiology , Acute Chest Syndrome/etiology , Acute Disease , Adult , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Anti-Bacterial Agents , Chest Pain/etiology , Fever/etiology , Humans , Prevalence
3.
Pan Afr Med J ; 41: 332, 2022.
Article in English | MEDLINE | ID: covidwho-1912166

ABSTRACT

The main pathological effects of COVID-19 infection have been reported to occur in the lungs, with the most pronounced manifestation being reported as Adult Respiratory Distress Syndrome (ARDS) with thromboembolic phenomena. Sickle Cell Disease (SCD) is a common genetic disorder present in 2% of newborns in Ghana. The complications of SCD include Vaso-Occlusive Crisis and Acute Chest Syndrome, which primarily manifest in the lungs. The effects of SCD on the progression of COVID-19 have not been extensively and clearly documented in literature. The objective was to describe the clinical and pathological findings in three SCD patients who died of COVID-19 related complications. A complete autopsy was performed on each of the three SCD patients who were presumed to have COVID-19. Lung swabs were subsequently taken and tested for SARS-CoV-2. The differences in histopathological findings of the three cases were highlighted and correlation with clinical findings was also done. Lung histopathological findings for all three cases were consistent with Acute Respiratory Distress Syndrome (ARDS)/ Diffuse Alveolar Damage (DAD) described for infections with COVID-19 and lung swabs tested for SARS-CoV-2 by real time Reverse Transcription Polymerase Chain Reaction (rRT-PCR) were positive. Though SCD has been reported not to adversely affect an individual´s chance of worse outcome when infected with COVID-19, our findings suggest otherwise. We suggest that SCD may be an important co-morbidity that needs to be considered in COVID-19 patients and when present needs to be considered as an adverse risk for poor outcomes. Also, post-discharge anti-coagulation and monitoring should be encouraged. More autopsies are required to fully understand the pathogenesis of COVID-19 in SCD patients.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Respiratory Distress Syndrome , Adult , Aftercare , Anemia, Sickle Cell/complications , Autopsy , COVID-19/complications , Ghana/epidemiology , Hospitals, Military , Humans , Infant, Newborn , Lung/pathology , Patient Discharge , SARS-CoV-2 , United States
4.
Blood Adv ; 6(15): 4408-4412, 2022 08 09.
Article in English | MEDLINE | ID: covidwho-1910256

ABSTRACT

Venous thromboembolism (VTE) is a life-threatening complication observed among patients with sickle cell disease (SCD) and also among those with severe COVID-19 infection. Although prior studies show that patients with SCD are at risk of severe COVID-19 illness, it remains unclear if COVID-19 infection further increases VTE risk for this population. We hypothesized that patients with SCD hospitalized for COVID-19 would have higher VTE rates than those hospitalized for other causes. Using electronic health record data from a multisite research network, TriNetX, we identified 2 groups of patients with SCD hospitalized during 2020: (1) with COVID-19 and (2) without COVID-19. We compared VTE rates using risk ratios estimated based on adjusted Poisson regression model with log link and robust error variances. Of the 281 SCD patients hospitalized with COVID-19 and 4873 SCD patients hospitalized without COVID-19 , 35 (12.46%) and 418 (8.58%) had incident VTE within 6 months of the index hospitalization respectively. After adjusting for differences in baseline characteristics, no significant differences in VTE rates within 6 months were found between the 2 groups (adjusted relative risk, 1.06 [95% confidence interval, 0.79-1.41]). These data suggest that hospitalization with COVID-19 does not further increase VTE risk in patients with SCD.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Venous Thromboembolism , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/complications , COVID-19/epidemiology , Humans , Retrospective Studies , Risk Factors , Venous Thromboembolism/complications , Venous Thromboembolism/etiology
5.
Clin Med (Lond) ; 22(3): 218-220, 2022 05.
Article in English | MEDLINE | ID: covidwho-1903880

ABSTRACT

Sickle cell disease is a common inherited disorder that is characterised by chronic haemolysis and vaso-occlusive episodes, resulting in severe pain and end-organ damage. The most frequent acute manifestation of sickle cell disease is a painful vaso-occlusive crisis, which can, in some cases, develop into a sickle chest crisis: a life-threatening complication of sickle cell disease that requires early recognition and prompt intervention to prevent progressive respiratory failure. In addition to the acute complications, patients with sickle cell disease are also at risk of a number of chronic complications that require multidisciplinary specialist input.


Subject(s)
Anemia, Sickle Cell , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , Humans
6.
Hematology ; 27(1): 742-744, 2022 Dec.
Article in English | MEDLINE | ID: covidwho-1895708

ABSTRACT

ABSTRACTPeople with sickle cell disease (SCD) are more vulnerable to hospitalization, pneumonia, and pain following COVID-19 infection. However, given the association between the inflammatory response and vaso-occlusive crises in SCD and a case report of vaso-occlusive crises following administration of the ChAdOx1 nCov-195-7/AstraZeneca vaccine, there is concern that the administration of COVID-19 vaccines in people with SCD might provoke a vaso-occlusive crisis. To address this critical gap in knowledge, we sought to examine acute care usage for vaso-occlusive crisis and frequency and severity of side effects following COVID-19 vaccination among patients at the Montefiore Sickle Cell Center for Adults. As part of regular care, patients were asked if they had received COVID-19 vaccination and any side effects were noted. Electronic medical records were reviewed for the type of vaccine, dates received, episodes of vaso-occlusive crises within seven days of a dose, and side effects noted. The risk of average hospital utilization per week in 2019 was calculated as a baseline. We found that fewer than 1 in 10 patients presented to the hospital within seven days of vaccination and that the risk of hospital utilization was similar to the average risk in a week in 2019. Of patients who reported side effects, one reported a possible case of sensorineural hearing loss otherwise no other rare side effects, including thrombosis or death, were reported.


Subject(s)
Anemia, Sickle Cell , COVID-19 Vaccines , COVID-19 , Pain , Adult , Anemia, Sickle Cell/complications , COVID-19/complications , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Humans , Pain/etiology , Peripheral Vascular Diseases/etiology , Vaccination/adverse effects
7.
Paediatr Int Child Health ; 42(1): 1-4, 2022 Feb.
Article in English | MEDLINE | ID: covidwho-1890707

ABSTRACT

ABBREVIATIONS: COVID-19: Coronavirus disease 2019; HIC: high-income countries; IBD: inflammatory bowel disease; LMIC: low- and middle-income countries; PUCAL: paediatric ulcerative colitis activity index; SCD: sickle cell disease; UC: ulcerative colitis.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Colitis, Ulcerative , Inflammatory Bowel Diseases , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/complications , Child , Colitis, Ulcerative/complications , Colitis, Ulcerative/epidemiology , Humans , Morbidity
8.
Transfusion ; 62(7): 1446-1451, 2022 07.
Article in English | MEDLINE | ID: covidwho-1874476

ABSTRACT

BACKGROUND: Hyperhemolysis syndrome (HHS) is a severe delayed hemolytic transfusion reaction seen in sickle cell disease (SCD) patients, characterized by destruction of donor and recipient RBCs. It results in a drop in hemoglobin to below pretransfusion levels and frequently reticulocytopenia. CASE REPORT: We report a case of a man in his thirties with SCD with a recent hospitalization 2 weeks prior for COVID-19. His red cell antibody history included anti-Fy(a) and warm autoantibody. At that time, he was given 2 units of RBC and discharged with a hemoglobin of 10.2 g/dl. He returned to the hospital approximately 1.5 weeks later with hemoglobin 6.0 g/dl and symptoms concerning for acute chest syndrome. Pretransfusion testing now showed 4+ pan-agglutinin in both gel-based and tube-based testing. Alloadsorption identified an anti-N and a strong cold agglutinin. Three least incompatible units were transfused to this patient over several days, with evidence of hemolysis. Further reference lab work revealed anti-Fya , anti-Fyb , anti-Lea , anti-Leb , and an anti-KN system antibody. The patient's hemoglobin nadired at 4.4 g/dl. The patient was treated with a single dose of tocilizumab, his hemoglobin stabilized, and he was discharged. DISCUSSION: We present a case of HHS proximate to recent SARS-CoV-2 infection with multiple allo and autoantibodies identified. Information on the relationship between SARS-CoV-2 infection and HHS is limited; however, it is possible that inflammation related to COVID-19 could predispose to HHS. Tocilizumab is an approved treatment for COVID-19. Additionally, tocilizumab appears to be a promising treatment option for patients with HHS.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , Antibodies, Monoclonal, Humanized , COVID-19/complications , COVID-19/drug therapy , COVID-19/therapy , Erythrocyte Transfusion/adverse effects , Hemoglobins , Hemolysis , Humans , Isoantibodies , Male , SARS-CoV-2
12.
Am J Ophthalmol ; 236: 241-248, 2022 04.
Article in English | MEDLINE | ID: covidwho-1757062

ABSTRACT

PURPOSE: To determine the feasibility and accuracy of nonmydriatic ultra-widefield (UWF) fundus photographs taken in a hematology clinic setting for screening of sickle cell retinopathy (SCR) DESIGN: Prospective cohort study. METHODS: This single-site study took place at the Johns Hopkins Sickle Cell Center for Adults and the Wilmer Eye Institute. The study population was 90 eyes of 46 consecutive adults with sickle cell disease (SCD). Bilateral nonmydriatic fundus photos taken by clinic personnel during the participants' routine hematology appointment were graded by 2 masked retina specialists at the Wilmer Eye Institute for the presence of nonproliferative SCR (NPSR) and proliferative sickle retinopathy (PSR). A third retina specialist adjudicated in cases of grader disagreement. All participants underwent the standard dilated fundus examination (DFE) within 2 years of acquisition of UWF photographs. The main outcome measure was the sensitivity and specificity of nonmydriatic UWF images for the detection of NPSR and PSR RESULTS: PSR was noted in 19 of 90 eyes that underwent DFE and in 9 of 67 gradable UWF images. Interrater agreement between the 2 graders was moderate, with κ = 0.65 (range 0.43-0.87) for PSR. For gradable UWF photos, the sensitivity and specificity of detecting SCR using the nonmydriatic photos compared with the DFE were 85.2% and 62.5% for NPSR, respectively, and 69.2% and 100% for detection of PSR, respectively. One participant required ophthalmic therapy in both eyes for active sea-fan neovascularization. CONCLUSIONS: UWF imaging shows utility in screening for SCR and may help identify patients with PSR who require a DFE and who may benefit from treatment.


Subject(s)
Anemia, Sickle Cell , Diabetic Retinopathy , Hematology , Retinal Diseases , Adult , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/diagnosis , Diabetic Retinopathy/diagnosis , Fundus Oculi , Humans , Photography/methods , Prospective Studies , Retinal Diseases/diagnosis , Retinal Diseases/etiology
13.
J Investig Med ; 70(5): 1243-1246, 2022 06.
Article in English | MEDLINE | ID: covidwho-1736086

ABSTRACT

Continued investigation of comorbid conditions that increase the mortality rate of COVID-19 is necessary to provide the best care for those affected. This continued push to find answers is even more important for populations with COVID-19 comorbidities that are historically under-researched. We performed a retrospective analysis of 30 patients with sickle cell disease (SCD) who tested positive for the COVID-19 virus. An analysis of each patient's history of SCD complications, hydroxyurea usage, comorbidities, and several other factors was performed to identify the trends that will allow the practitioners to better predict the outcomes of patients with SCD before and during hospitalization for COVID-19. Through these analyses, we found that patients receiving hydroxyurea before COVID-19 infection and patients with SCD-type HbSC had significantly milder COVID-19 disease courses than those not receiving hydroxyurea or with SCD-type HbSS. A history of acute chest syndrome (ACS), a complication seen in patients with SCD, appeared to be associated with a more severe COVID-19 disease course. By creating systems to better interpret what makes a patient with SCD at high risk for a poor prognosis, practitioners are better equipped to make data-supported recommendations for prevention, risk, and treatment. These recommendations should include beginning or maintaining hydroxyurea usage in all qualifying patients with SCD, advising patients with a history of ACS to take extra precautions to prevent initial COVID-19 infection, and initiating close monitoring in the hospital for patients with HbSS and a history of ACS.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/drug therapy , COVID-19/complications , Humans , Hydroxyurea/therapeutic use , Protective Factors , Retrospective Studies
14.
J Gynecol Obstet Hum Reprod ; 51(3): 102328, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-1654804

ABSTRACT

INTRODUCTION: The effect of coronavirus disease (COVID-19) on pregnancy outcome in women with sickle cell disease (SCD) is unknown. OBJECTIVES: To analyze the severity of the SARS-CoV-2 infection in pregnant women with SCD and its impact on pregnancy. METHODS: This retrospective cohort study included SCD pregnant women tested positive for COVID-19 between March 2020 - February 2021. The primary endpoint was the severity of the COVID-19 infection. Secondary endpoints were pregnancy complications and fetal outcomes. RESULTS: During the study period among 82 pregnant women with SCD, 8 have presented symptoms suggestive of COVID-19 and were tested positive. A common mild clinical presentation was observed in 6 women (75%), one woman was asymptomatic and one required oxygen. The latter was admitted to the Intensive Care Unit and a cesarean section was performed in the context of an ongoing vaso-occlusive crisis and acute chest syndrome together with incidental preeclampsia. Labor was induced in another patient who developed a vaso-occlusive crisis after COVID-19 remission. Fetal outcomes were good with an average Apgar score of 10 and normal umbilical blood pH at birth. Two newborns were small-for-gestational-age as expected on the ultrasound follow-up before occurrence of COVID-19. CONCLUSION: COVID-19 infection in our population of pregnant women with SCD had typical presentation and rarely triggered a sickle cell crisis or other complications. Fetal outcomes were good and did not seem to be directly influenced by the SARS-CoV-2 virus. Further studies are required to confirm these observations as compared to the population of women with SCD without COVID-19 infection.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Pregnancy Complications, Infectious , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Cesarean Section , Female , Humans , Infant, Newborn , Pregnancy , Pregnancy Complications, Infectious/diagnosis , Pregnancy Outcome/epidemiology , Pregnant Women , Retrospective Studies , SARS-CoV-2
15.
Pediatr Pulmonol ; 57(4): 1096-1099, 2022 04.
Article in English | MEDLINE | ID: covidwho-1650861

ABSTRACT

Children with sickle cell disease (SCD) are at increased risk for severe illness due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We describe the successful native lung recovery of a child with SCD referred for lung transplant (LTx) evaluation who was on prolonged veno-venous extracorporeal membrane oxygenation (VV-ECMO). He initially presented with acute chest syndrome complicated by SARS-CoV-2 infection that ultimately required dual-lumen, single bicaval VV-ECMO cannulation for respiratory support. Despite the increased risk of hemolysis and thrombosis from SCD and SARS-CoV-2 infection, he was successfully supported on VV-ECMO for 71 days without complications leading to native lung recovery with meticulous management of his SCD therapy. This report provides new insight on our approach to VV-ECMO support in a child with SCD and SARS-CoV-2 infection. With a successful outcome, the patient has returned home but still on mechanical ventilation with LTx still an option if he is not eventually liberated from invasive respiratory support.


Subject(s)
Acute Chest Syndrome , Anemia, Sickle Cell , COVID-19 , Extracorporeal Membrane Oxygenation , Acute Chest Syndrome/complications , Acute Chest Syndrome/therapy , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/therapy , COVID-19/complications , COVID-19/therapy , Child , Extracorporeal Membrane Oxygenation/adverse effects , Humans , Male , SARS-CoV-2
16.
Public Health Rep ; 137(2): 234-238, 2022.
Article in English | MEDLINE | ID: covidwho-1643029

ABSTRACT

Sickle cell disease (SCD) is associated with increased risk of poor health outcomes from respiratory infections, including COVID-19 illness. We used US death data to investigate changes in SCD-related mortality before and during the COVID-19 pandemic. We estimated annual age- and quarter-adjusted SCD-related mortality rates for 2014-2020. We estimated the number of excess deaths in 2020 compared with 2019 using the standardized mortality ratio (SMR). We found 1023 SCD-related deaths reported in the United States during 2020, of which 86 (8.4%) were associated with COVID-19. SCD-related deaths, both associated and not associated with COVID-19, occurred most frequently among adults aged 25-59 years. The SCD-related mortality rate changed <5% year to year from 2014 to 2019 but increased 12% in 2020; the sharpest increase was among adults aged ≥60 years. The SMR comparing 2020 with 2019 was 1.12 (95% CI, 1.06-1.19). Overall, 113 (95% CI, 54-166) excess SCD-related deaths occurred in 2020.


Subject(s)
Anemia, Sickle Cell/mortality , COVID-19/epidemiology , Adolescent , Adult , Age Distribution , Anemia, Sickle Cell/complications , COVID-19/complications , Child , Child, Preschool , Humans , Infant , Middle Aged , Mortality/trends , Race Factors , SARS-CoV-2 , Time Factors , United States/epidemiology
17.
J Pediatr Hematol Oncol ; 44(1): e134-e137, 2022 Jan 01.
Article in English | MEDLINE | ID: covidwho-1632085

ABSTRACT

To this day, there are limited data about the effects and management of coronavirus disease infection in pediatric patients with sickle cell disease. We present the management and successful clinical course of an 8-year-old female with homozygous sickle cell disease (SS) and severe acute chest syndrome secondary to coronavirus disease 2019 infection, complicated by cortical vein thrombosis.


Subject(s)
Anemia, Sickle Cell/complications , COVID-19/complications , Systemic Inflammatory Response Syndrome/complications , Anemia, Sickle Cell/pathology , Anemia, Sickle Cell/therapy , Anti-Bacterial Agents/therapeutic use , Azithromycin/therapeutic use , COVID-19/pathology , COVID-19/therapy , Ceftriaxone/therapeutic use , Child , Erythrocyte Transfusion , Female , Humans , Intensive Care Units , Systemic Inflammatory Response Syndrome/pathology , Systemic Inflammatory Response Syndrome/therapy
18.
Pediatr Infect Dis J ; 41(3): e104-e105, 2022 03 01.
Article in English | MEDLINE | ID: covidwho-1592757

ABSTRACT

Multisystem inflammatory syndrome in children (MIS-C) is a serious complication that is observed most commonly in pediatric patients following severe acute respiratory syndrome coronavirus 2 infections. However, the mechanism and predictors of disease are poorly understood. There are no prior reports of MIS-C among patients who have been fully vaccinated, and only a single case of MIS in an adult patient who had received his second shot just 4 days prior to symptom onset. Here, we present an adolescent with sickle cell disease who was fully vaccinated against severe acute respiratory syndrome coronavirus 2 and had no prior history of known or suspected infection, who presented in shock and was ultimately diagnosed with MIS-C. This case highlights the importance of clinical suspicion for MIS-C even when patients are fully vaccinated.


Subject(s)
Anemia, Sickle Cell/complications , COVID-19 Vaccines/adverse effects , COVID-19/complications , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/etiology , Vaccines, Synthetic/adverse effects , /adverse effects , Adolescent , COVID-19/diagnosis , COVID-19/drug therapy , COVID-19/etiology , COVID-19/prevention & control , Diagnosis, Differential , Drug Therapy, Combination , Female , Glucocorticoids/therapeutic use , Humans , Immunoglobulins, Intravenous/therapeutic use , Methylprednisolone/therapeutic use , Pandemics , SARS-CoV-2 , Systemic Inflammatory Response Syndrome/drug therapy
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