A 19‐year‐old woman with a history of sickle cell disease (HgbSC) presented to the hospital with an acute pain crisis. Initial vital signs were stable with normal oxygen saturation. Examination was unremarkable. The patient was admitted and placed on a morphine PCA pump. On hospital day 2 the patient became uncooperative and refused further diagnostic testing. She appeared confused, frequently complaining of pain but did not use the PCA pump. Ativan was added for sedation. On hospital day 3. the patient was either sleeping or moaning in pain, unable to give further history. Later in the day the patient was noted to urinate on the floor and exhibit regressive behavior. The patient's mother stated that morphine sometimes made her daughter disoriented. The morphine was held, but on hospital day 4, the patient was found lying sideways in bed, groaning, and unresponsive. A lumbar puncture performed at the bedside was negative. The patient soon developed worsening tachypnea (RR = 45‐50), hypoxia (room air O2 saturation = 68%), and tachycardia (pulse = 160, sinus tachycardia) requiring intubation. Chest X‐ray revealed bilateral air‐space opacities consistent with acute chest syndrome. An MRI of the brain revealed multiple patchy foci in the deep and subcortical white matter of the frontal lobe consistent with early subacute vessel infarcts. The sickle cell consultants recommended exchange transfusion to maintain a Hgb = 10. The patient remained intubated in the MICU and after several days of receiving exchange transfusions was extubated and transferred to the medical floor, where she made a full recovery.
Cerebral infarction is the most common neurological complication in sickle cell hemoglobinopathy. As sickle red blood cells become deoxygenated, they increase blood viscosity, resulting in sludging in the microvascular circulation of the brain. Red cell exchange transfusion removes sickle cells that may participate in new vaso‐occlusive events, reduces hemolytic complications, and provides added oxygen‐carrying capacity while decreasing blood viscosity. Indications for red cell exchange include acute infarctive stroke, acute chest, and multiorgan failure syndrome. Initially the patient's behavioral changes were thought to be secondary to morphine. If her frontal lobe stroke symptoms had been promptly diagnosed, exchange transfusion would have started sooner, most likely preventing the acute chest syndrome that followed. Patients with sickle cell disease are often withdrawn and minimally interactive during a pain crisis. In those instances, daily assessment of neurologic function is essential for early detection and prompt recognition of stroke symptoms.
Stroke is the most common neurologic complication in sickle cell disease. Early recognition of stroke symptoms and red cell exchange transfusion improves outcomes.
N. Winawer, none; A. Jabbar, none; A. Von, none.