From the Guidelines
The initial workup and management for a patient with sickle cell disease experiencing a stroke requires immediate neuroimaging and supportive care, with a focus on reducing HbS levels to below 30% and increasing total hemoglobin to 10-12 g/dL through simple or exchange blood transfusion, as recommended by the most recent guidelines 1.
Initial Assessment and Management
The patient should undergo emergent non-contrast CT scan of the brain to differentiate between ischemic and hemorrhagic stroke, followed by MRI/MRA for detailed evaluation. Immediate management includes stabilization of airway, breathing, and circulation, with supplemental oxygen to maintain saturation above 95%.
- Hydration with IV fluids at 1.5 times maintenance rate is essential, avoiding hypotonic solutions.
- Pain management should be provided if the patient has concurrent vaso-occlusive crisis.
- Neurological assessment should be performed frequently using standardized stroke scales.
Transfusion Therapy
Simple or exchange blood transfusion should be initiated promptly to reduce HbS levels to below 30% while increasing total hemoglobin to 10-12 g/dL. For exchange transfusion, a central venous catheter is typically placed, and red cell exchange is performed at a volume of approximately 1-1.5 times the patient's blood volume, as supported by previous studies 1.
Long-term Management
After stabilization, patients require long-term transfusion therapy (typically every 3-4 weeks) to maintain HbS levels below 30% for at least 5 years to prevent recurrence, as stroke recurrence risk is high in these patients 1. This aggressive approach is necessary because sickle cell disease causes chronic endothelial damage and hypercoagulability, making these patients particularly vulnerable to cerebrovascular complications. Thrombolytic therapy with tPA is generally avoided in sickle cell patients with stroke due to increased bleeding risk.
From the Research
Initial Workup for Sickle Cell Crisis Stroke
- The initial workup for a patient with sickle cell disease experiencing a stroke should include a thorough evaluation of the patient's risk factors for stroke, such as severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation 2.
- Imaging studies such as magnetic resonance imaging (MRI) or computed tomography (CT) scans should be performed to confirm the diagnosis of stroke and to identify any underlying vasculopathy 3.
- Transcranial Doppler or magnetic resonance angiography may be used to diagnose vasculopathy 2.
Laboratory Tests
- Complete blood count (CBC) and reticulocyte count may not be necessary in the evaluation of acute vasoocclusive sickle-cell crisis, as they do not appear to be useful in predicting the need for admission 4.
- However, recent studies suggest that reticulocyte and erythrocyte parameters, such as reticulocyte count, immature reticulocyte count, and medium fluorescence reticulocytes, may be useful in predicting the development of vaso-occlusive crisis in patients with sickle cell disease 5.
Management
- Regular blood transfusion may reduce the risk of neurological complications in children with sickle cell disease and either previous overt stroke or silent cerebral infarction or abnormal transcranial Doppler 2.
- Hydroxyurea or other management strategies may also be considered, although there are relatively few data on their use in this context 2.
- Secondary prevention and curative measures, such as stroke management and prevention of recurrence, should also be considered 6.