Evaluation

• CT scan and MRI for acute event

• Magnetic resonance angiography for asymptomatic infarction

• Transcranial Doppler to detect abnormal velocity and identify high-risk patients

• Electroencephalography if there is history of seizure

Early detection of ischemic stroke can be done with the use of transcranial Doppler ultrasonography. In the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study, screening with this method followed by chronic transfusion therapy significantly reduced the incidence of stroke.36 Screening is recommended in all patients over the age of two.

Acute Chest Syndrome

ACS will require hospitalization for appropriate management of symptoms and to avoid complications. Patients should be encouraged to use incentive spirometry at least every 2 hours. Incentive spirometry helps the patient take long, slow breaths to increase lung expansion. Appropriate management of pain is important, but analgesic-induced hypoventilation should be avoided. Patients should maintain appropriate fluid balance because overhydration can lead to pulmonary edema and respiratory distress. Infection with gram-negative, gram-positive, or atypical bacterial is common in ACS and early use of broad-spectrum antibiotics, including a macrolide, quinolone, or cephalosporin is recommended. Fat emboli, from infarction of the long bones, may lead to ACS. Oxygen therapy should be utilized in any patient presenting with respiratory distress or hypoxia. Oxygen saturations, measured by pulse oximeter, should be maintained at 92% or above. Transfusions are often indicated and patients who present

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with wheezing may require inhaled bronchodilators.

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