Sickle cell disease: a natural model of acute and chronic pain

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A 19-year-old female patient with severe sickle cell disease (SCD) presented to her hematologist with severe pain. Prior to 16 years, she reported 4 hospitalizations and 2 emergency department visits for acute pain management. Her pain significantly increased in frequency and severity between 16 and 19 years with 10 hospitalizations, and she reported almost daily pain. Her pain descriptors included radiating, burning, tingling, electric shocks, and numbness, and she reported cutaneous pain with slight skin pressure and with cold temperatures. Pain locations included chest, lower back, knees, feet, and “all over.” At 18 years, she started hydroxyurea, which induces the production of fetal hemoglobin and ameliorates some SCD complications, including acute pain. Despite a positive hematological response, significant pain persisted, severely impacting her quality of life. Her pain regimen included almost daily oxycodone, tramadol, and ibuprofen.
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