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  • image Sickle cell disease is an inherited disorder caused by a defect in the gene for β-globin, a component of hemoglobin. It is considered to be a qualitative hemoglobinopathy. Patients can have one defective gene (sickle cell trait) or two defective genes (sickle cell disease).

  • image Although sickle cell disease usually occurs in persons of African ancestry, other ethnic groups can be affected. Multiple mutation variants are responsible for differences in clinical manifestations.

  • image Sickle cell disease involves multiple organ systems. Usual clinical signs and symptoms include anemia, pain, splenomegaly, and pulmonary symptoms. Sickle cell disease is diagnosed through routine newborn screening programs available in all 50 states. Early diagnosis allows early preventive and comprehensive care.

  • image Patients with sickle cell disease are at risk for infection. Prophylaxis against pneumococcal infection reduces death during childhood.

  • image Hydroxyurea decreases the risk of painful episodes, but patients treated with hydroxyurea require careful monitoring.

  • image Neurologic complications caused by vasoocclusion and hemolysis can lead to stroke. Screening with transcranial Doppler ultrasound to identify children at risk accompanied by chronic transfusion therapy programs can decrease the risk of overt and silent stroke in children with sickle cell disease.

  • image Patients with fever greater than 38.5°C (101.3°F) should be evaluated, and appropriate antibiotics administered immediately, including coverage for encapsulated organisms, especially pneumococcal organisms.

  • image Pain episodes can often be managed at home. Hospitalized patients require parenteral analgesics. Analgesic options include opioids, nonsteroidal anti-inflammatory agents, and acetaminophen. The patient characteristics and the severity of the pain should determine the choice of agent and regimen.

  • image Patients with sickle cell disease should be followed regularly for healthcare maintenance issues and monitored for changes in organ function.


Patient Care Process for Vasoocclusive Episodes



  • Patient characteristics (eg, age, sex, sickle cell disease genotypes)

  • Patient medical history (include organ function and psychosocial issues)

  • Immunization history

  • Social history (eg, tobacco use)

  • Pain diary

  • Medication use

  • Blood transfusion history

  • Objective data

    • Vital signs: blood pressure, heart rate, respiratory rate, height, weight, O2 saturation

    • Labs: complete blood count (CBC), reticulocytes, basic chemistry, lactate dehydrogenase, ferritin, urine analysis, hemoglobin fractionation

    • Additional labs or imaging per presenting symptoms (see Table 124-2)


  • Hemodynamic stability

  • Pain scale

  • Adherence to home medication

  • Sign or symptom associated with sickle cell acute complications (see Table 124-2)


  • Fluid

  • Pain management (see Table 124-6)

  • Initiate antibiotics if febrile

  • Oxygen


  • Provide education on current pain regimen

  • Develop individualized plan for pain management

  • Evaluate for initiation of hydroxyurea, L-glutamine, voxelotor, crizanlizumab

  • Schedule follow-up

Follow-Up: Monitor and Evaluation

  • Ongoing evaluation of pain level

  • Monitor for adverse drug reactions of pain medication and initiate supportive care if needed

*Collaborate with patients, caregivers, and other healthcare professionals.



Watch the short video entitled “Sickle Cell Anemia” in the DNA Learning Center. This short 1-minute video ...

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