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

Abstract

Acute chest syndrome (ACS) in adult patients with sickle cell disease represents a leading cause of death. It is characterized by a new density on chest X-ray accompanied by fever and/or respiratory symptoms. Currently, 49 adult patients with sickle cell disease are registered at our department. By now, 12 patients (24.5%) suffered from ACS and two patients showed multiple/recurrent (>2) episodes. Death in one patient was related to acute respiratory failure secondary to ACS. In three patients with ACS, invasive mechanical ventilation and subsequent veno-venous extracorporeal membrane oxygenation (VV-ECMO) was mandatory. Veno-venous ECMO was applied within 24 hours upon arrival to the intensive care unit (ICU). All patients were treated aggressively for ACS including exchange transfusions [packed red blood cell (pRBC) units 5–16] maintaining a Hb S threshold of <30.0% in addition to broad-spectrum antibiotics, resulting in a successful outcome following decannulation from VV-ECMO (49 hours, 251 hours, 30 min., and 98 hours, respectively). Limited information is presently available on the use of VV-ECMO in adult patients with sickle cell disease in the context of acute respiratory failure secondary to ACS. The adequate timing of the decision to place ECMO in critically ill adults with sickle cell disease, incapable of being treated by conventional mechanical ventilation secondary to very severe vaso-occlusive crisis (VOC), might further reduce mortality rates while treating the underlying condition.

Keywords:

• Acute chest syndrome (ACS)
• sickle cell disease
• extracorporeal membrane oxygenation (ECMO)

Acknowledgments

These authors contributed the following: F. Alashkar performed the research, designed the research study, analyzed the data, and wrote the paper; F. Herbstreit, A. Carpinteiro, J. Baum, A. Tzalavras, C. Aramayo-Singelmann, C. Vance, V. Lenz, E. Gulbins, D. Reinhardt, D. W. Beelen, U. Dührsen, and A. Röth analyzed the data. M. Koldehoff and T. Liebregts performed the research, designed the research study, analyzed the data, and wrote the paper. M. Koldehoff and T. Liebregts contributed equally to this project and should be considered last coauthors. All authors read and approved the final manuscript. F. Alashkar was supported as a Clinician Scientist within the University Medicine Essen Academy (UMEA) program, funded by the German Research Foundation (DFG) [grant FU356/12-1] and the Faculty of Medicine, University of Duisburg-Essen, Essen, Germany.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.