an acute exacerbation may require steroids as part of their management; however, a taper beyond the standard 5-day course is necessary Stroke Initial Assessment The combined incidence of ischemic and hemorrhagic stroke is nearly 300-fold higher in patients with sickle cell disease compared with all children Eleven percent of all sickle cell anemia patients will suffer a stroke, with occurrence at a median age of 20 years Fatalities with ischemic strokes are less likely than with hemorrhagic stroke; however, neurologic sequelae are common with both Typical presenting symptoms and signs for ischemic stroke include focal weakness, dysarthria, aphasia, and seizure Hemorrhagic strokes present with evidence of increased intracranial pressure including severe headache, vomiting, impaired mental status, bradycardia, nuchal rigidity, coma, focal neurologic deficits, and seizure Risk factors include young age, low baseline hemoglobin concentration, and high leukocyte count Historical risk factors include previous stroke, moyamoya, cerebral aneurysms, acute chest syndrome, acute hypertension, hypertransfusion, and recent corticosteroid use Cerebral aneurysms occur with increased frequency in patients with sickle cell disease The origin of this complication, which is usually detected in teenagers or adults, remains obscure but may relate to local vessel damage The severe morbidity and high mortality associated with ruptured cerebral aneurysms require careful evaluation of patients with sickle cell disease and headaches or other neurologic findings (vertigo, syncope, nystagmus, ptosis, meningismus, or photophobia) Unfortunately, the aneurysm often escapes detection until after major, and often fatal, subarachnoid or intracerebral bleeding Management/Diagnostic Testing Use CT for initial evaluation of sickle cell patients with concern for stroke MRI can assist in early diagnosis in ischemic strokes (within to hours of symptoms) that may not be apparent on CT A 1.5 to times volume exchange transfusion should begin immediately for ischemic strokes This reduces the likelihood of further intravascular sickling and may prevent extension of cortical damage MRA should be performed in patients with identified intracranial hemorrhage to guide further diagnosis and management If an accessible aneurysm is identified and bleeding persists, surgical or interventional radiology intervention should follow radiologic confirmation Anemia Initial Assessment/H&P The baseline anemia of a patient with sickle cell disease may worsen acutely due to acute hemolysis triggered by a viral or bacterial infection or by an aplastic crisis usually in the setting of infection with parvovirus B19 halting RBC production for to days