Veno-Venous Extracorporeal Membrane Oxygenation in Adult Patients with Sickle Cell Disease and Acute Chest Syndrome: a Single-Center Experience

Hemoglobin ◽  
2020 ◽  
Vol 44 (2) ◽  
pp. 71-77
Author(s):  
Ferras Alashkar ◽  
Frank Herbstreit ◽  
Alexander Carpinteiro ◽  
Julia Baum ◽  
Asterios Tzalavras ◽  
...  
Author(s):  
Wonshill Koh ◽  
Punam Malik ◽  
Jason Whitehead ◽  
David Morales ◽  
Don Hayes

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 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.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 985-985
Author(s):  
Sindhu Devarashetty ◽  
Kimberly Le Blanc ◽  
Udhayvir Singh Grewal ◽  
Jacqueline Walton ◽  
Tabitha Jones ◽  
...  

Abstract Background and Objectives: The COVID-19 (CO19) pandemic caused by SARS-CoV-2 remains a significant issue for global health, economics, and society. Several reports have shown that African Americans (AA) have been disproportionately affected by the CO19 pandemic. Limited data have suggested that sickle cell disease (SCD) could be one of the several reasons for higher morbidity and mortality related to CO19 among AA. Recent reports have suggested higher-than-average morbidity and mortality related to CO19 among patients with SCD. We conducted a retrospective, single-institution study in adult patients with SCD who were diagnosed with CO19 infection and their outcomes. Methods: After IRB approval, we conducted a chart review of adult patients (greater than 18 years) with SCD who were diagnosed with CO19 infection between March 1st, 2020, and March 31st, 2021. We recorded demographic data including age, gender, social factors (the type of insurance, availability of primary care provider (PCP), living alone/not), clinical parameters (type of SCD, co-morbidities), outpatient management of SCD, and how CO19 infection was managed like inpatient admission and complications. In patients who were admitted or seen in the emergency department (ED), we collected additional data including vitals, labs, the severity of illness, complications, length of stay, and outcomes. Computations were performed using statistical software SAS 9.4 for Windows. Results: We found a total of 51 patients with SCD diagnosed with CO19 infection in the above period. The median age of patients was 30 years. 61% were females and 39 % were males. All of them were AA. 11.76% were living alone, 49.02% were living with family, 1.96% (1 patient) was institutionalized, and the living situation was unknown in 37.25%. Most of the patients had Medicaid Insurance (52.94%), Medicare in 33.3%, private insurance in 13.73 % and 2% were uninsured. Only 64.71% of patients had a PCP. 60% had HbSS disease, 32% had HbSC disease, 4% had HbS-beta thalassemia, one patient each had HbSS with hereditary persistence of HbF and HbS/HbD. Comorbidities and previous history included acute chest syndrome in 65.96%, avascular necrosis in 36.96%, leg ulcers in 8.7%, hypertension in 8.7%, sickle cell retinopathy in 14.57%, cerebrovascular disease in 26.19%, chronic kidney disease in 7.69%, venous thromboembolism (VTE) in 20.41%, 10.41% were on anticoagulation, history of HIV and hepatitis C infection in 6.38%. 28.21% of patients were maintained on partial exchange transfusions as an outpatient for various indications. 72.73% were on hydroxyurea, 7.5% were on crizanlizumab, 5.26% were on voxelotor and 26.83% were on iron chelation. Vitals and pertinent lab values on initial assessment were recorded and many patients had missing data. On presentation, 25.53% were febrile, 29.17% of patients were tachycardic, 31.25% were hypoxic (SpO2 < 95%), 38.46% were tachypneic, 59.18% had a body mass index (BMI) of > 24.9. Median hemoglobin and hematocrit were 8.9/27.4 g/dL. The median white blood cell count was 9490/uL and platelets were 315,000/uL. Median ferritin was 1573 ug/L. Median bilirubin and creatinine were 2.05 mg/dL and 0.86 mg/dL. The patients were further stratified based on the clinical location where CO19 infection was managed (Table 1). 39.3% were diagnosed in the outpatient setting/ED and 60.3% in the inpatient setting. Among 51 patients, 5.71% (n=2) required ICU admission and was mechanically ventilated. 17.5% received dexamethasone, 7.69% received remdesivir, 2.76% received convalescent plasma, 17.07% had infections and 47% received antibiotics. Only one patient received an exchange transfusion during admission. One patient developed a new VTE after CO19 infection. On statistical analysis, the only factor which impacted the clinical location of management was tachycardia (P=0.007). Of the 51 patients, only 3.9% (2 patients) died of complications of CO19 infection, one with hypoxic respiratory failure, disseminated intravascular coagulation, shock, and the other one with pulmonary embolism. 13% were readmitted within a month, one of them was admitted with a new pulmonary embolism and the others were admitted for acute painful episodes. Conclusion: We found a mortality rate of 3.9% in our single-center study of patients with SCD and CO19 infection. This mortality rate is lower than other published experiences in patients with SCD and CO19 infection. Figure 1 Figure 1. Disclosures Master: Blue Bird Bio: Current holder of individual stocks in a privately-held company.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4854-4854
Author(s):  
Mira T Tanenbaum ◽  
Anna Shvygina ◽  
Vaishnavi Sridhar ◽  
Jennifer E. Vaughn ◽  
Mark Joseph

BACKGROUND: Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Despite being the leading cause of death in adult patients with SCD, current recommendations for treatment of ACS remain largely supportive, consisting of pain management, aggressive fluid resuscitation, respiratory support, and transfusion therapy. Despite these measures, it is not uncommon for patients to require intubation due to progression to acute respiratory distress syndrome (ARDS). Recently, there have been a number of case reports that have successfully utilized extracorporeal membrane oxygenation (ECMO) for the management of ACS in those patients who fail to respond to conventional therapy [Kuo et al., 2013, Sewaralthahab et al, 2018]. However, the use of ECMO in this patient population remains uncommon, and further evaluation of this intervention is needed. This case report details an unsuccessful attempt at the use of ECMO in the case of ARDS secondary to ACS, in an attempt to identify critical pitfalls. CASE REPORT: A 32-year-old African-American female with HbSS disease on hydroxyurea therapy was transferred from an outside hospital following 3 days of respiratory decompensation. Prior to arrival, patient coded once at the outside hospital and once on transfer. Veno-arterial ECMO was initiated with improving oxygen saturation and volume status with continuous renal replacement therapy. To maintain an ECMO-specific goal hemoglobin level of 10 g/dL, 1:1 manual exchange transfusions were performed due to an inability to access equipment for automated RBC exchange. Once stable enough for CT, patient was found to have gray-white inversion suggestive of irreversible severe brain damage. Following another 28 days of supportive care with no neurologic improvement, the family decided to withdraw care, and the patient expired. CONCLUSION: While unsuccessful, this patient's case revealed a need for defining parameters regarding the initiation of ECMO in SCD patients with severe ACS. A review of previously-published literature has shown that the use of ECMO for the management of ARDS in adults is more efficacious than conventional ventilation support [Peek et al., 2009]. In patients with SCD, this improvement in efficacy is not readily reproduced, likely due to unique challenges presented by the pathophysiology of the disease. Notably, patients with SCD face additional risks of venous thromboembolism and strokes while on prolonged bed rest due to a baseline prothrombotic state [Sewaralthahab et al., 2018]. A systematic review of available case reports is needed to develop a protocol for the management of severe ACS that takes SCD-specific risks into account. The present report also makes a case for the training of providers in the early recognition of severe ACS in SCD patients. SCD remains largely undertreated in the United States, likely due to a complex interplay of patient, physician, and institutional factors. Had this patient been transferred immediately to a facility better equipped to provide a higher level of care, her condition could have arguably taken a different course. Despite the aforementioned challenges, ECMO remains a feasible option for the management of severe ACS in patients with SCD, and efforts should be made to standardize current treatment protocols. REFERENCES: Kuo KW, Cornell TT, Shanley TP, Odetola FO, and Annich GM. The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease. Perfusion. 2013 September; 28(5): 424-432. Peek GJ, Mugford M, Tiruvoipati R, Wilson A, Allen E, Thalanany M, et al. Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial. The Lancet. 2009 October; 374(9698): 1351-1363. Sewaralthahab SS, Menaker J, Law JY. Successful use of veno-venous extracorporeal membrane oxygenation in an adult patient with sickle cell anemia and severe acute chest syndrome. Hemoglobin. 2018 42(1): 65-67. Disclosures No relevant conflicts of interest to declare.


eJHaem ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 608-614 ◽  
Author(s):  
Preethi Ramachandran ◽  
Abhilash Perisetti ◽  
Balachandar Kathirvelu ◽  
Mahesh Gajendran ◽  
Snigdha Ghanta ◽  
...  

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