V.M. is a 29-year-old African American married man who has sickle cell disease (SCD) marked by frequent episodes of severe pain. His anemia has been managed with multiple transfusions
Six months
ago he started showing signs of chronic renal failure. His regular medications are pentoxifylline (Trental),
oxycodone-acetaminophen (Percocet), hydroxyurea (Droxia), and folic acid. In the hematology clinic this
morning, V.M.'s hemoglobin measured 6.7 g/dL. He received 2 units of packed red blood cells (PRBCs) over
3 hours and then went home. He developed dyspnea and shortness of breath approximately 1 hour later,
and his wife called 911 . The emergency medical system crew initiated oxygen at 8 L per nasal cannula and
transported V.M. to the emergency department (ED).
What is SCD?
SCD is a group of inherited, autosomal recessive disorders characterized by the presence of an
abnormal form of hemoglobin (hemoglobin S [Hb S]) in the red blood cells (RBCs). The abnormal
hemoglobin causes the RBCs to elongate or stiffen into a sickle shape when oxygen levels are low;
repeated deoxygenation results in permanent sickling of the RBCs.
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