Sickle crisis

Management

► Early and effective treatment of crises essential (hospital).

► Rest patient and start IV fluids and O2 (patients often dehydrated through poor oral intake of fluid + excessive loss if fever).

► Start empirical antibiotic therapy (e.g. cephalosporin) if infection is suspected whilst culture results (blood, urine or sputum) are awaited.

► Analgesia usually required—e.g. intravenous opiates (diamorphine/morphine) especially when patients are first admitted to hospital. Switch later to oral medication after the initial crisis abates.

► Consider exchange blood transfusion (if neurological symptoms, stroke or visceral damage). Aim to 5 HbS to <30%.

► Exchange transfusion if PaO2 <60mm on air (►chest syndrome).

► a-adrenergic stimulators for priapism.

► Seek advice of senior haematology staff.

► Consider regular blood transfusion if crises frequent or anaemiais severe or patient has had CVA/abnormal brain scan.

► Top-up transfusion if Hb <4.5g/dL (hunt for cause).

Transfusion and splenectomy may be lifesaving in children with splenic sequestration.

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