A 15-year-old girl is brought to the emergency department due to pain in her arms, legs, and lower back. The patient has sickle cell disease and in the past has been hospitalized for pain crises and pneumococcal sepsis. Her current symptoms began this morning, but she is out of her medications, including oxycodone, hydroxyurea, and penicillin. Temperature is 37.6 C (99.7 F), blood pressure is 158/88 mm Hg, pulse is 110/min, and respirations are 24/min. Pulse oximetry is 99% on room air. The patient is alert but anxious. Cardiac examination shows a flow murmur. The lungs are clear to auscultation. The abdomen is soft with no organomegaly. The legs and back are moderately tender to palpation. Morphine is given for pain control, and 1 hour afterward the patient is suddenly unable to move her left side. Examination shows 1/5 strength in the left upper and lower extremities and 5/5 strength in the right upper and lower extremities. Sensation is also diminished on the left side. Fingerstick glucose is 110 mg/dL. CT scan of the head shows no acute pathology. Which of the following is the best next step in management of this patient?

Question

A 15-year-old girl is brought to the emergency department due to pain in her arms, legs, and lower back.  The patient has sickle cell disease and in the past has been hospitalized for pain crises and pneumococcal sepsis.  Her current symptoms began this morning, but she is out of her medications, including oxycodone, hydroxyurea, and penicillin.  Temperature is 37.6 C (99.7 F), blood pressure is 158/88 mm Hg, pulse is 110/min, and respirations are 24/min.  Pulse oximetry is 99% on room air.  The patient is alert but anxious.  Cardiac examination shows a flow murmur.  The lungs are clear to auscultation.  The abdomen is soft with no organomegaly.  The legs and back are moderately tender to palpation.  Morphine is given for pain control, and 1 hour afterward the patient is suddenly unable to move her left side.  Examination shows 1/5 strength in the left upper and lower extremities and 5/5 strength in the right upper and lower extremities.  Sensation is also diminished on the left side.  Fingerstick glucose is 110 mg/dL.  CT scan of the head shows no acute pathology.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec25c8_d15d_e04b_bc81_e1ccc27567c1_MD0003_00 This child with sickle cell disease (SCD) has sudden-onset unilateral weakness without intracranial hemorrhage, findings concerning for an ischemic stroke. SCD increases the risk of ischemic stroke due to intimal hyperplasia and stenosis of large arteries, as well as occlusion of vasculature by adherent, stiff, sickled red blood cells. Vascular and oxidative injury leads to recruitment of inflammatory cells and activation of the coagulation cascade. Precipitating factors may include acute drops in oxygenation or hemoglobin (often seen in pain crises). This patient is at increased risk of SCD complications due to not taking hydroxyurea, which decreases frequency of pain crises by increasing fetal hemoglobin. Treatment of stroke in SCD patients is exchange transfusion (ie, slowly removing the patient's blood and replacing it with allogeneic blood), which lowers the relative percentage of sickled cells compared to healthy red blood cells. By decreasing RBC sickling, exchange transfusion increases the oxygen-carrying capacity of the blood and improves hyperviscosity, limiting further occlusion. Exchange transfusion also decreases the risk of stroke recurrence. Simple transfusion (ie, packed red blood cell transfusion) should be performed when exchange transfusion is not available. (Choice A) Antiplatelet therapy is an appropriate treatment for many non-SCD patients with ischemic stroke when stroke is due to atherosclerosis, small vessel disease, or thromboembolism. However, stroke in SCD patients has a different etiology (ie, alteration in cerebral hemodynamics due to sickled red blood cells); antiplatelet therapy has not been shown to improve stroke outcomes or prevent recurrent stroke in SCD, unlike exchange transfusion. (Choice B) Due to impaired splenic function, SCD increases the risk of life-threatening infections caused by encapsulated bacteria (eg, Streptococcus pneumoniae). However, acute neurologic deficits in an afebrile patient make infection less likely and blood cultures and antibiotics unnecessary. (Choice D) Intravenous heparin, which can treat deep venous thrombosis and pulmonary embolism, is not typically used in acute ischemic stroke management due to risk of intracranial hemorrhage. (Choice E) Naloxone reverses the effects of opioid overdose, which typically presents as a decreased level of consciousness and respiratory depression; tachypnea, present in this patient, would not be seen. In addition, acute, unilateral weakness is not associated with opioid overdose. Educational objective: Exchange transfusion is the treatment of ischemic stroke in patients with sickle cell disease. Replacing sickled cells with healthy red blood cells increases oxygen-carrying capacity and improves hyperviscosity, limiting further occlusion. References: Therapeutic red cell exchange transfusion as an adjuvant therapy for management of sickle cell crisis in adults. (http://www.ncbi.nlm.nih.gov/pubmed/29962749) How I treat and manage strokes in sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/25824688)

A 15-Year-Old Girl Is Brought to the Emergency Department Due