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

Alashkar, Ferras; Herbstreit, Frank; Carpinteiro, Alexander; Baum, Julia; Tzalavras, Asterios; Aramayo-Singelmann, Carmen; Vance, Colin; Lenz, Veronika; Gulbins, Erich; Reinhardt, Dirk; Beelen, Dietrich; Röth, Alexander; Koldehoff, Michael; Liebregts, Tobias

doi:10.1080/03630269.2020.1745827

Cited by 5 publications

(3 citation statements)

References 17 publications

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“…Publicizing these potential complications in carriers may help healthcare providers to include sickling complications in their differential diagnosis when assessing individuals who are from ethnicities with a high prevalence of sickle cell trait. This may facilitate prompt recognition of at‐risk individuals and the provision of individualized care for instance: anticoagulation, 3,4 oxygen therapy, 59 blood transfusion, 57,58 red blood cell exchange transfusion, 57,71 or extracorporeal membrane oxygenation 72‐74 when necessary. Individuals who have sickle cell trait and are aware of their HbS genotype could be encouraged to self‐identify when presenting to healthcare providers for COVID‐19 assessment.…”

Section: Covid‐19 Pneumonia In Scd: Potential Relevance To Sickle Celmentioning

confidence: 99%

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Kehinde

Osundiji

2020

European J of Haematology

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Following the emergence of Coronavirus Disease 2019 (COVID-19) in Wuhan (China) around December 2019, 1 the infection has become a pandemic, decimating over three hundred thousand globally. 2 The severity of COVID-19 cases reported hitherto has varied greatly from asymptomatic to severe pneumonia and thromboembolism, accompanied by overt respiratory failure with subsequent mortality. 3-5 As the COVID-19 pandemic continues to evolve, potential risk factors that may predispose individuals to fatal outcomes are increasingly becoming topical. There are ongoing epidemiological investigations and discussions of potentially at-risk populations and ethnicities. 6,7 An improved understanding of the risk factors as well as clinical course for severe COVID-19 may shed some light on novel personalized approaches to optimize clinical care and outcomes. Emerging trends in the United States suggest possibly higher mortality rates of COVID-19 among African Americans, 8,9 although epidemiological study data with adequate denominator 10 across board is pending. There is no doubt that socioeconomic determinants of health play crucial roles in health inequities and disease trajectory. 11 Nevertheless, interplay of genetic and environmental factors contributes to overall clinical outcomes in a substantial burden of human diseases. 12-17 Sickle cell disease (SCD) disproportionately affects Black/ African Americans in the United States as well as forebearers from sub-Saharan Africa, the Western Hemisphere (South America, the Caribbean, and Central America), and some Mediterranean countries. 18-20 Increasing evidence suggests that COVID-19 pneumonia can cause acute chest syndrome (ACS), a potentially life-threatening complication in SCD. 21-23 These observations raise questions on the potential contributions of SCD-related complications, for instance

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Section: Covid‐19 Pneumonia In Scd: Potential Relevance To Sickle Celmentioning

confidence: 99%

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Kehinde

Osundiji

2020

European J of Haematology

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“…10 While trends in the use of or outcomes of ECMO in patients with ACS specifically are not known, there have been a few reports in adults of successful ECMO therapy for ACS. 5,[11][12][13][14][15][16][17][18][19][20][21][22][23] However, in one case series of 22 subjects, mortality was 73% 5 (Table 1). This discrepancy is likely explained in part by publication bias, although could also reflect uncertainties in appropriate ECMO candidacy.…”

Section: Discussionmentioning

confidence: 97%

“…Additionally, advances in ECMO technology have allowed support of sicker patients for longer ECMO durations 10 . While trends in the use of or outcomes of ECMO in patients with ACS specifically are not known, there have been a few reports in adults of successful ECMO therapy for ACS 5,11–23 . However, in one case series of 22 subjects, mortality was 73% 5 (Table 1).…”

Section: Discussionmentioning

confidence: 99%

Acute chest syndrome from sickle cell disease successfully supported with veno‐venous extracorporeal membrane oxygenation

Grotberg,

Sullivan,

McDonald

et al. 2024

Artificial Organs

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Sickle cell disease (SCD) affects ~100 000 people in the United States and carries significant morbidity. 1 Under physiologic stresses, such as infection or hypoxemia, red blood cells (RBC) sickle causing vaso-occlusive crisis affecting bones and other organs. In the lung, the pulmonary capillaries are occluded by sickled RBCs (or fat emboli) resulting in a clinical syndrome of fever, pleurisy, hypoxemia, tachypnea, and lung infiltrates called acute chest syndrome (ACS). 2 Roughly, half of patients with SCD will develop ACS and many require intensive care unit (ICU) level care. 3 In severe cases, invasive mechanical ventilation (IMV) is necessary due to secondary lung injury and worsened hypoxemia in a feed-forward cycle (Figure 1). Treatment is supportive including supplemental oxygen, fluids, antibiotics, lung protective ventilation (when intubated), and reduction of sickled hemoglobin (HbS)% via simple RBC transfusion or exchange transfusion. 1,2 Rarely, ACS may progress to severe acute respiratory distress syndrome (ARDS). 4 There are limited data on the use of ECMO for refractory ARDS due to ACS, but those available suggest a high associated mortality. 5 We present a case of severe ARDS secondary to ACS successfully supported with veno-venous (V-V) ECMO. | CASEA 25-year-old male with SCD presented to the emergency department with 4 days of fever, cough, and pleurisy. He displayed hypoxemia (O 2 15 L/min), leukocytosis (15.8 × 10 9 /L), acute-on-chronic anemia (Hb 6.3 g/dL, baseline of 9 g/dL), and thrombocytopenia (93 × 10 9 /L). Percentage of HbS was 77%. A computed tomography scan of the chest demonstrated right lower lobe consolidation. He received antibiotics (vancomycin, cefepime, and azithromycin) and RBC transfusion. However, his respiratory failure rapidly progressed requiring IMV on hospital day (HD) 1. He was transferred to our center for emergent RBC exchange transfusion.Despite improvement in HbS percentage (22.7%), he developed worsening hypoxemia and bilateral infiltrates consistent with severe ARDS, and received paralysis, proning, and inhaled epoprostenol. On HD6, after a bedside positive end-expiratory pressure (PEEP) titration, ventilator settings were as follows: volume assist/control, tidal volume 4.7 mL/ kg ideal body weight, respiratory rate 32, PEEP 10 cmH 2 O, and fraction of inspired oxygen (FiO 2 ) 100%. Plateau pressure was 34 cmH 2 O, driving pressure was 24 cmH 2 O, and static compliance was 12.5 mL/cmH 2 O. Arterial blood gas measured pH of 7.38, carbon dioxide tension (PaCO 2 ) of

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High risk and low prevalence diseases: Acute chest syndrome in sickle cell disease

Koehl¹,

Koyfman²,

Hayes³

et al. 2022

The American Journal of Emergency Medicine

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

Cited by 5 publications

References 17 publications

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Sickle cell trait and the potential risk of severe coronavirus disease 2019—A mini‐review

Acute chest syndrome from sickle cell disease successfully supported with veno‐venous extracorporeal membrane oxygenation

High risk and low prevalence diseases: Acute chest syndrome in sickle cell disease

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