Several days after a patient has been hospitalized and treated for a vaso-occlusive crisis, with their pain, improving, they develop acute respiratory failure, liver failure +/- acute renal failure, in a life-threatening manner. It is called multisystem organ failure in sickle cell disease. It occurs in many patients with no history of chronic organ failure, with clinical deterioration so rapid and unexpected.
Multisystem organ failure is usually associated with;
-a rapid decline in haemoglobin and platelet count, and
Acute respiratory failure is often associated with acute chest syndrome. Hepatic failure is associated with marked elevations in total and direct bilirubin, liver enzymes and coagulopathy. Acute renal failure: a rapid elevation in serum creatinine, with or without oliguria and hyperkalemia.
Prompt diagnosis and treatment are mandatory if we are to prevent we are to death. Diagnosis and management require a high index of suspicion. Let’s look at the recommendations agreed upon by the panel of experts on evidence-based management of sickle cell disease.
- In people with sickle cell disease who exhibit severe deterioration during a vaso-occlusive crisis, immediately evaluate potential multisystem organ failure.
- In people with sickle cell disease, and respiratory failure, support respiratory status with supplemental oxygen and mechanical ventilation when needed.
- Use renal replacement therapy (e.g. hemodialysis) when needed for acute renal failure.
- In people with sickle cell disease and multisystem organ failure, immediately initiate either simple or exchange transfusion. Consult a sickle cell expert or haematologist first.
Take home message;
Be on the lookout for this acute complication in that patient with a vaso-occlusive crisis whose condition suddenly deteriorates when their pain was significantly improved.
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