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Managing Acute Complications Of Sickle Cell Disease In Pediatric Patients

November 2016

Abstract

Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management. Patients with sickle cell trait generally have a benign course, but are also subject to serious complications. This issue provides a current review of evidence-based management of the most common acute complications of sickle cell disease seen in pediatric patients in the emergency department.

Key words: sickle cell disease, SCD, vaso-occlusive crisis, VOC, acute chest syndrome, ACS, priapism, splenic sequestration, stroke, bacteremia, febrile illness, transient red cell aplasia, sickle cell anemia, sickle cell trait, hemoglobin, pediatric, children

Points:

  • Simple vaso-occlusive crisis episodes require no laboratory testing. However, if an intravenous line is to be placed or if pain is moderate/severe, a complete blood cell count (CBC) and reticulocyte count are warranted.
  • Starting maintenance IV fluids is important; however, fluid boluses should only be given in hypotensive, unstable, or significantly dehydrated patients.
  • In patients with sickle cell disease (SCD), normal vital signs should not be interpreted as the absence of pain. Notably, drug abuse rates in individuals with SCD are not different from the general population. If there are concerns of possible addiction, focus on treating the patient's pain, then involve the patient's hematologist as well as a pain/palliative care specialist for detailed evaluation of the patient's opioid medication use.

Pearl:

  • Individuals with SCD are prone to reversible polymerization, or “sickling” of red blood cells during febrile, low-flow, low-oxygen, or acidotic states.
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