An unexpected full neurological recovery after cardiac arrest in a sickle cell anemia patient with bilateral cervical carotid artery disease

Sickle cell anemia (SCA) is a hereditary hemoglobinopathy caused by homozygosity for sickle hemoglobin (Hb S). Hb S is caused by a single mutation in the -globin gene (HBB, glu6val) resulting in the presence of valine instead of glutamic acid in the sixth position of the -globin chain.1,2 Hb S can form polymers when deoxygenated leading to vaso-occlusion and tissue ischemia which may result in severe pain of the chest, back, abdomen, or extremities.2,3 Acute chest syndrome (ACS) is an acute illness characterized by fever and/or respiratory symptoms, accompanied by new pulmonary infiltrates on a chest X-ray. 4–10 It is the leading cause of hospitalization and intensive care unit admission,4,6 as well as the most common cause of death in adult patients 8,9 with SCA even among those on hydroxyurea (HU) therapy. Factors such as infection, fat embolism, iatrogenic fluid overload, hypoventilation from pain or narcotic analgesics, and