Outcomes of Adult Patients With Sickle Cell Disease Admitted to the ICU: A Case Series

Objective: Sickle cell disease is associated with a decreased life expectancy, half of the deaths occurring in the ICU. We aimed to describe the characteristics of sickle cell disease patients admitted to ICU and to identify early predictors of a complicated outcome, defined as the need for vital support or death. Design: Retrospective observational cohort study of sickle cell disease patients over a 6-year period. Setting: ICU of a French teaching hospital and sickle cell disease referral center. Patients: Hundred thirty-eight ICU admissions in 119 sickle cell disease patients. Interventions: None. Measurements and Main Results: ICU admission was mainly indicated for sickle cell disease-related events, especially acute chest syndrome. Mechanical ventilation, vasoactive drugs, and renal replacement therapy were administered to 25 (18%), 10 (7%), and 10 (7%) episodes, respectively. The complicated outcome group (n = 28; 20%) was characterized by a more aggressive acute disease within the 48 hours preceding ICU admission, with a higher respiratory rate, a more frequent acute kidney injury, and a more sustained drop of hemoglobin (all p < 0.01). All nine deaths (7%) were sickle cell disease related. None of the sickle cell disease baseline characteristics predicted accurately a complicated outcome. In multivariate analysis, hemoglobin less than or equal to 7.8 g/dL (odds ratio, 3.6; 95% CI, 1.1-11.9), respiratory rate more than or equal to 32 cycles/min (odds ratio, 5.6; 95% CI, 1.8-17.2), and acute kidney injury on ICU admission (odds ratio, 11.5; 95% CI, 2.5-52.6) were independently associated with a complicated outcome. Conclusions: Sickle cell disease patients are at high risk of complications when admitted to the ICU. A sustained drop of hemoglobin, acute respiratory distress, and kidney injury at admission are strong predictors of a complicated outcome.