Multisystem Organ Failure

Background
Multisystem organ failure (MSOF) is a severe and life-threatening complication usually associated with a VOC and characterized by failure of the lungs, liver, and/or kidneys. MSOF may occur after several days of hospitalization and treatment for a severe VOC, often when pain is beginning to improve. In most cases, patients do not have a history of chronic organ failure. Deterioration is rapid and unexpected. It is usually associated with fever, a rapid decline in hemoglobin concentration and platelet count, and nonfocal encephalopathy. Acute respiratory failure is usually associated with development of ACS. Hepatic failure is associated with marked elevations in total and direct bilirubin, liver enzymes, and blood coagulation screening tests. Acute renal failure is associated with a rapid elevation of serum creatinine, with or without the presence of oliguria and hyperkalemia. Rapid diagnosis and treatment of MSOF is necessary to prevent death.

Summary of the Evidence
An adequate systematic review of the literature with fair sensitivity and specificity for all studies indexed by SCD terms and “multisystem organ failure” was not feasible. No systematic review was conducted, and the panel used a consensus process to develop a proposed strategy for triaging and promptly managing MSOF.

Recommendations

In people with SCD who exhibit severe deterioration during a VOC, immediately evaluate for potential MSOF.
(Consensus-Panel Expertise)
In people with SCD and respiratory failure, support respiratory status with supplemental oxygenation and mechanical ventilation when needed.
(Consensus-Panel Expertise)
Use renal replacement therapy (e.g., hemodialysis) when needed for acute renal failure.
(Consensus-Panel Expertise)
In people with SCD and MSOF, immediately initiate either simple or exchange transfusion in consultation with a sickle cell expert or hematologist.
(Consensus-Panel Expertise)