What are the primary concerns and management strategies for unilateral neurological symptoms in patients with sickle cell disease during a crisis?

From the Guidelines

Unilateral neurological symptoms during sickle cell crisis require immediate medical attention as they may indicate stroke or other serious complications. The primary management strategy involves rapid assessment and neuroimaging (CT or MRI) to rule out acute stroke, followed by prompt exchange transfusion to reduce the percentage of sickled cells to below 30% 1. Initial stabilization includes oxygen therapy, IV hydration with isotonic fluids at 1.5 times maintenance rate, and pain management with opioids such as morphine (0.1-0.15 mg/kg IV every 3-4 hours) or hydromorphone. Some key considerations in the management of patients with sickle cell disease include:

• Meticulous peri-operative care to avoid factors that may precipitate sickling: dehydration; hypoxia; acidosis; hypothermia; and pain 1
• Patients are at increased risk of sickle complications (acute chest syndrome, pain, acute renal insufficiency or stroke), sepsis and venous thromboembolism in the peri-operative period 1
• The acute pain team should be notified in advance if a patient with sickle cell disease is undergoing major surgery, particularly if the patient has a history of chronic pain 1 If stroke is confirmed, exchange transfusion should be initiated within 2 hours, targeting hemoglobin S levels below 30% 1. Long-term management includes regular transfusions every 3-4 weeks to maintain hemoglobin S below 30%, hydroxyurea therapy (15-35 mg/kg/day), and consideration of chronic anticoagulation. These interventions are critical because sickle cell patients have increased risk of cerebrovascular events due to abnormal red blood cell morphology causing vascular occlusion, endothelial damage, and hypercoagulability 1. Neurological symptoms may include hemiparesis, facial droop, speech difficulties, or sensory changes, and should never be dismissed as routine crisis manifestations, as timely intervention significantly impacts neurological outcomes and reduces mortality. Recent guidelines emphasize the importance of a multidisciplinary approach to the management of patients with sickle cell disease, including input from haematology, anaesthesia, and pain teams 1.

From the Research

Unilateral Neurological Symptoms in Sickle Cell Crisis

• The primary concerns for unilateral neurological symptoms in patients with sickle cell disease during a crisis include stroke, silent cerebral ischemia, and other cerebrovascular lesions 2, 3, 4.
• Ischemic strokes are more common in younger children, while hemorrhagic strokes are more frequent in adults 2.
• The management strategies for unilateral neurological symptoms in patients with sickle cell disease include:
  • Neuroimaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) to assess the extent of parenchymal abnormalities and detect cerebrovascular lesions 2, 4.
  • Transcranial Doppler (TCD) examination to assess the risk of first stroke and detect large cerebral vessel disease 2, 4.
  • Chronic blood transfusions to reduce the risk of stroke and other neurological complications 2, 4.
  • Hydroxyurea to reduce the frequency and severity of pain crises and prevent neurological complications 5.
• Early recognition and treatment of neurological complications are crucial to improve outcomes and reduce morbidity and mortality in patients with sickle cell disease 3, 4.

Risk Factors and Prevention

• Risk factors for neurological complications in sickle cell disease include severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation 4.
• Regular blood transfusions and hydroxyurea can reduce the risk of neurological complications, but do not abolish it entirely 2, 4, 5.
• Early assessment of the risk of neurocognitive complications is important in the management of sickle cell disease 4.

Treatment Options

• Treatment options for sickle cell disease focus on managing symptoms and preventing complications, including pain management with analgesics, hydration, and blood transfusions 5, 6.
• Hydroxyurea is commonly used to reduce the frequency and severity of pain crises and prevent neurological complications 5.
• Bone marrow or stem cell transplants can cure select individuals with severe sickle cell disease 5.