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HIGH-YIELD FACTS

  • Sickle cell disease (SCD) is a chronic hemolytic anemia that is most common among African Americans, but may occur in children of any ethnic background. Patients with a single abnormal gene for HbS have sickle cell trait and remain essentially asymptomatic.

  • Acute vasoocclusive crisis (VOC) events (painful crisis events) are the most common complication of SCD and are the most frequent cause of emergency department visits.

  • A complete blood count and reticulocyte count should be obtained every time a patient with SCD presents to the emergency department (ED).

  • Patients with SCD presenting with a new infiltrate on chest radiograph, and chest pain, fever, and/or respiratory symptoms have acute chest syndrome (AChS). Therapy for AChS consists of antibiotics, pain control, respiratory support, and possibly transfusion. All children with AChS should be admitted to the hospital.

  • A blood culture should be obtained and parenteral antibiotic given to every patient with SCD and fever due to the risk of sepsis from encapsulated bacteria, especially Streptococcus pneumoniae.

  • Splenic sequestration crisis occurs when red blood cells (RBCs) become entrapped in the spleen, resulting in a rapidly enlarging spleen and a sudden drop in hemoglobin. The mainstay of therapy is blood transfusion.

  • Stroke occurs in 11% of patients with SCD under 20 years of age. Patients with signs and symptoms concerning for stroke should have neuroimaging performed (preferably magnetic resonance imaging [MRI] and magnetic resonance angiography [MRA]) and consultation with a hematologist as soon as possible.

Patients with sickle cell disease (SCD) experience a number of complications that are likely to bring them to the emergency department (ED). Hemoglobin S (HbS) is a variant resulting from a single nucleotide mutation in the sixth codon of the β-globin gene leading to the substitution of hydrophobic valine for the normal hydrophilic glutamic acid. SCD occurs when an individual is homozygous for HbS or is a compound heterozygote for HbS and another interacting β-globin variant. The most common combination of hemoglobins leading to SCD are HbSS (sickle cell anemia), HbSC (hemoglobin SC) disease, and HbS-β thalassemia (either β0 or β+). Although there is wide variability in individual severity of illness, patients with HbSS or HbSβ0 (no hemoglobin A production) have the greatest severity of disease.1,2 The other double heterozygous states, such as HbSC and HbSβ+ thalassemia, are typically less seriously affected.1 Patients with HbS/HPFH (hereditary persistence of HbF) and HbS/HbE show mild to no symptoms.1 Approximately 1 in 12 African Americans carry the allele, and 1 in 500 African Americans born has the disease. HbS also occurs in people of Mediterranean, South Asian, and Middle Eastern descent.1

Patients with a single abnormal gene for HbS have sickle cell trait. The concentration of HbS is typically 40%, and the large percentage of normal hemoglobin allows the patients to remain asymptomatic except under the most severe hypoxic stress. Sickle trait should be considered a ...

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