Sickle cell patients can present in both hemolysis (anemia, gallstones, nephropathy) and/or vaso-occlusive symptoms (acute chest, priapism, CVA, pain crisis).
Sickle cell pain crisis can be caused by a broad range of triggers, and vary broadly between patients, including heat/cold, exercise, dehydration, infection, menses and other causes. Many episodes are thought to be spontaneous. Vitals signs can frequently be normal, despite the patient suffering severe pain crisis. The NIH gold standard for sickle cell pain assessment is the patient's report of pain, and no combination of lab or clinical findings exist to determine if the patient is indeed in pain. Additionally, as the patient ages, the amount of pain between crises increases.
Evaluation of the sickle cell patient: Maintaining a high suspicion of additional medical illnesses is important. A WBC >20,000 is not typical for sickle cell.. When evaluating reticulocyte counts, <3% is indicative of an aplastic crisis, while >12% indicates rapid hemolysis. Additional labs to consider include LDH, biirubin and haptoglobin.
Pain Management: Prompt administration and rapid reassessment. If pain is not relieved with >2 doses, hospitalization is recommended for analgesia.
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