Ten tips for managing critically ill patients with sickle cell disease

Abstract

Sickle cell disease (SCD), one of the most common monogenic disorders in the world, is caused by a mutation of hemoglobin (typically β6Glu > Val). When deoxygenated, sickle hemoglobin tends to polymerize within the red blood cells, causing their deformation and rigidity (sickling), with hemolysis and vascular obstruction leading to vaso-occlusive pain crisis. Vaso-occlusive crisis will be experienced by nearly all individuals with SCD during their lifetime. There is a complex network of associated cellular changes (e.g., cell adhesion, endothelial activation, ischemia-reperfusion, and hemolysis-driven scavenging of nitric oxide), leading to a multisystem vascular disease involving progressive chronic organ damage and acute complications that may require intensive care unit admission (Fig. 1). We herein review some tips to manage critically ill patients with SCD.

Nationwide retrospective study of critically ill adults with sickle cell disease in France

“…As previously reported, a high respiratory rate and impaired creatinine clearance at ICU admission were associated with adverse outcomes 16 , 19 , 20 . A high haemoglobin level was also associated with adverse outcomes, whereas previous studies showed that a low haemoglobin level predicted a poor prognosis 13 , 16 , 21 . This discrepancy may be related to the administration of blood support before ICU admission in some patients in our study.…”

“…In other studies, ACS was the reason for ICU admission in 30% to 70% of patients 7 , 8 , 13 , 15 , 16 . This wide range can be explained by differences in types of ICUs, study populations, and varying proportions of medical and surgical admissions.…”

Focus on improved patient management

“…In 2017, Joly et al described, very precisely, the potential therapeutic targets for TTP patient management in three steps: (1) restoring the processing of the ultra-large VWF multimers by administrating plasma and therefore active ADAMTS13; (2) eliminating potential anti-ADMATS13 autoantibodies, using plasma exchange, steroids, and monoclonal antibodies targeting the CD20 antigen; and (3) targeting large VWF multimers, using dedicated therapies such as caplacizumab (a nanobody directed against the VWF A1 domain), which specifically blocks interactions between VWF and platelet GPIbα [2,3]. Similarly, Mekontso Dessap et al discussed the principles of managing critically ill patients with sickle cell disease, one of the most common monogenic disorders [4]. They described multiple mechanisms for the lung injury that occurs as a consequence of necrosis-driven fat emboli, pulmonary artery thrombosis, and infection in patients with functional asplenia.…”

“…They described multiple mechanisms for the lung injury that occurs as a consequence of necrosis-driven fat emboli, pulmonary artery thrombosis, and infection in patients with functional asplenia. Additionally, they discussed results from the most recent trial in this field and the risk/benefit ratio of blood transfusion in patients receiving frequent alloimmunization [4]. The authors also stressed the need for systematic search (and treatment) of in situ pulmonary artery thrombosis while keeping a high degree of suspicion regarding cor pulmonale that may be found in 80% of patients developing acute respiratory distress syndrome features [4].…”

“…3 Faculté de Médecine, Université Paris-Diderot, Sorbonne-Paris-Cité, Paris, France. 4 ECSTRA Team, Biostatistics and Clinical Epidemiology, UMR 1153 (Center of Epidemiology and Biostatistics Sorbonne Paris Cité, CRESS), INSERM, Paris, France.…”