Sickle Cell Crisis

Your Path

  • Sickle cell crisis


  • Autosomal dominant genetic disorder leading to sickling and hemolysis
  • Precipitants include hypoxemia, hypovolemia, infection, other --> sickling--> veno-occlusive disease--> end organ ischemia

Risk Factors

  • African descent
  • Family hx
  • Sx worse if air quality poor


  • Recurrent bouts of pain during crises due to veno-occlusive disease
  • Variable severity based on genotype--> degree of sickling
  • Acute chest syndrome: Sob/CP from ischemia, infection, fat embolism, hemolysis
  • Acute priapism, abdominal pain (ischemia)
  • Chronic sx: multi-site pain, leg ulcers, sepsis from fxnl asplenia, joint pain from avascular necrosis, stroke, biliary colic or cholecystitis from bilirubin gall stones; sob from restrictive lung dz, plum htn

Physical Exam Findings

  • Fever, tachypnea, tachycardia, hypoxia, wheezing