Severe Acute Respiratory Distress Syndrome in a Patient with Sickle-Cell Anemia Requiring Veno-Venous Extracorporeal Membrane Oxygenation Therapy: Case Report and Review of the Literature

Abstract

Background: Sickle cell anemia is caused by a mutation in the beta globin gene, resulting in the production of sickle hemoglobin, which is less soluble than normal hemoglobin. The main disease features are related to hemolytic anemia and blood vessels occlusion, causing ischemia and infracts. Acute chest syndrome is one of its most dangerous manifestations, and may cause severe hypoxemia and acute respiratory failure. Conservative management is often successful, but in rare cases the syndrome may be refractory. Extracorporeal membrane oxygenation (ECMO) support may be life-saving in these extreme situations. Case Report: A 31-year-old male admitted to the intensive care unit (ICU) in our hospital due to acute chest syndrome and massive aspiration. Due to extreme hypoxemia and severe acute respiratory distress syndrome, veno-venous ECMO support was initiated with rapid improvement in both oxygenation and hemodynamic status. The patient was weaned of ECMO after 7 days. He was discharged 4 weeks later. Although initiation of ECMO in sickle cell patients is uncommon, in selected refractory cases it may be life-saving. Conclusion: Although initiation of ECMO therapy in sickle cell anemia patients is uncommon, and may be even controversial, in selected refractory cases it may be life-saving.