sickle cell vaso-occlusive crisis

Etiology

• sickle cell disease

Pathology

• precipitation of hemoglobin SS under low oxygen tension resulting in loss of erythrocyte structure & deformability
• microvascular occlusion & infarction
• overactivity of SLC12A4 may contribute to erythrocyte dehydration

Clinical manifestations

• vaso-occlusive episodes or "painful crises"
  • may be very painful & debilitating for the patient
  • there are no objective findings to confirm the presence of pain

Management

• analgesia for painful crises:
  • non-steroidal anti-inflammatory agents (NSAIDs)
  • acetaminophen
  • parenteral opioids for severe pain
    • morphine, oxycodone agents of choice
    • therapeutic management of the pain is a frequent source of conflict between the physician & patient or patient's family
  • agents used in combination with analgesics
    • antihistamines: diphenhydramine, hydroxyzine
    • tricyclic antidepressants
    • phenothiazines for nausea (ondansetron might be an option for nausea)
  • ref states scheduled morphine is treatment of choice citing ref
• general management principles include:
  • hydration with 3-4 L of oral fluid/day
  • intravenous fluids for dehydration or acute illness
  • avoid iatrogenic fluid overload in patients with renal, cardiac or hepatic insufficiency
  • infusion centers with better outcomes than emergency departments for managing uncomplicated crises
  • incentive spirometry to avoid acute chest syndrome
• nitric oxide of no benefit

More general terms

• complication
• sign/symptom

Additional terms

• sickle cell (hemoglobin SS) disease