Key Takeaways

✅ Veno-occlusive crisis (VOC), characterized by severe, acute pain, often in the extremities, joints, chest, and lower back, is the most common reason for patients with sickle cell disease to seek care in the emergency room and to be admitted to hospital.

✅ VOC is caused by microvascular occlusion by irreversibly sickled red cells, leading to downstream tissue ischemia and ischemia-reperfusion injury, especially in the bone and bone marrow.

✅ VOC is a diagnosis of exclusion. It is important to rule out other causes of acute pain.

✅ Patients with VOC may have a normal physical exam, and their labs may be normal (relative to baseline) or show a reduction in hemoglobin (Hb) and/or accentuation of hemolytic markers relative to baseline. They may have leukocytosis, and thrombocytosis or thrombocytopenia.

✅ Individuals with more than three hospitalizations for a VOC in a year are at an increased risk of early death.

✅ Treatment includes rapid use of analgesics, administration of supplemental oxygen if arterial oxygen saturation is < 95%, hydration (with IV fluids if oral intake not possible) and incentive spirometry to reduce the incidence of acute chest syndrome.

✅ Patients with VOC should not be routinely transfused unless there are other indications for transfusion.

✅ VOC typically resolves after several days.