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Risk Management Pitfalls For Sickle Cell Disease

  1. “I thought it was just a pain crisis.”
    Always consider non-SCD-related conditions in your differential. For example, for right lower quadrant pain, consider appendicitis, kidney stone, and gynecological causes before presuming VOC. For chest pain, consider acute coronary syndromes, pulmonary embolism, or pneumothorax before presuming acute chest syndrome.
  2. “He’s had this back pain before, so it can’t be anything dangerous.”
    Consider epidural abscess and spinal osteomyelitis in the differential of midline back pain, even when fever is absent.
  3. “The hemoglobin was low, so I gave blood. I didn’t think this would cause a stroke.”
    Never transfuse a patient simply because hemoglobin is low. Elevating the hemoglobin above baseline can cause hyperviscosity, pain, acute chest syndrome, and stroke.
  4. “I thought the patient had sickle cell trait, so I withheld pain medicines.”
    When it appears that a patient has sickle cell trait on hemoglobin electrophoresis, make sure the patient did not receive a transfusion within 90 days of having the test performed. Recent transfusion renders the electrophoresis useless for diagnostic purposes.
  5. “The patient doesn’t have SCD, so I never thought to check intraocular pressure for such minor eye trauma.”
    In cases of direct eye trauma, patients with SCD and sickle cell trait should be treated the same way. Many patients do not know that they carry the trait or will fail to mention it unless prompted. Both SCD and sickle cell trait increase the risk for catastrophic ophthalmologic complications after blunt eye trauma, even if hyphema is not apparent.
  6. “I gave the patient a prescription for iron because she was anemic. I didn’t realize this would cause liver problems.”
    Never prescribe iron for patients with SCD. These patients are usually iron-overloaded.
  7. “The creatinine was normal. How was I supposed to know the patient had kidney dysfunction?”
    Assume that all patients with SCD have some degree of renal dysfunction, even if the creatinine level is normal. Supranormal proximal tubule function creates falsely low creatinine in this patient population. Take this into consideration when prescribing NSAIDs and when ordering imaging studies with IV contrast.
  8. “I didn’t know that a few liters of normal saline could be harmful.”
    Using bolus normal saline to treat sickle cell crisis presents several problems. Excess IV fluid can result in atelectasis, which may precipitate acute chest syndrome. Large amounts of normal saline can produce a hyperchloremic metabolic acidosis, which may promote sickling.
  9. “I thought the patient was just faking because he had normal vital signs.”
    Most patients with VOC will not exhibit vital sign abnormalities.
  10. “The patient wasn’t black, and I thought SCD only occurs in people of African descent.”
    Sickle cell disease has been described in all races and should no longer be considered exclusive to black persons.

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Last Modified: 07/23/2017
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