A Humbling Case of Survival in the Intensive Care Unit

Abstract Introduction: Sickle cell anemia (SCA) is the most common inherited blood disorder in Canada. Patients with SCA have a propensity for profound multiorgan failure and, when admitted to intensive care units, may have limited access to hemoglobinopathy experts. While the effects of critical injury and organ damage in this population can be considerable, SCA exhibits unique pathophysiology with the possibility of hypoxic preconditioning contributing to survival.Case presentation: A 45-year-old female with known hemoglobin (Hb) SS type SCA and RBC autoantibodies (anti-Jsb, anti-C, anti-E) presents to hospital with one-week history of diarrhea, vomiting and progressive weakness. Initial bloodwork showed new worsened anemia (Hb 3.2 g/dL) from her baseline (7.0 g/dL). The patient is admitted to hospital with a plan for transfusion of suitably matched red blood cells with pre-emptive administration of corticosteroids and intravenous immunoglobulin.Within 24 hours of hospital presentation, the patient has rapid clinical deterioration including altered mental status, hypoxemic respiratory failure, and subsequent cardiac arrest. After 5 minutes of advanced cardiovascular life support, endotracheal intubation, and the transfusion of one unit of emergency blood, return of spontaneous circulation is achieved and the patient is transferred to the intensive care unit (ICU). She receives two additional units of “least incompatible” blood to a transfusion target of 5.0 g/dL, without developing hemolysis. While initial chest imaging was unremarkable, she develops fulminant bilateral pulmonary infiltrates with copious volume of golden endotracheal secretions, consistent with acute chest syndrome. Despite standard treatment for acute respiratory distress syndrome, her oxygen saturation remains under 35% with a maximum PaO2 of 40 mmHg. This degree of hypoxemia persists for over 16 hours, accompanied by post-arrest cardiogenic shock, and multiorgan failure. Given the lack of phenotypically compatible blood, usual management of sickle cell crisis, including aggressive blood transfusions and red cell exchange, were not feasible. She was deemed not a candidate for extracorporeal membrane oxygenation for this same reason. Despite early concerns for ischemic/hypoxic encephalopathy, the patient realized complete neurologic recovery following cardiac arrest. By day 7 in hospital, she was transferred from the ICU to a general medical ward with preserved function of all major organ systems.Discussion: This case illustrates remarkable physiologic adaptability and presumably also the concept of hypoxic pre-conditioning. It highlights our tendencies to extrapolate expected outcomes based on common ICU presentations in less common diseases. SCA is not common in the ICU and as such, assumptions on disease trajectory should be made with caution.

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