Chapter 120: Sickle Cell Disease

KEY CONCEPTS

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

• 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.

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

• Patients with sickle cell disease are at risk for infection. Prophylaxis against pneumococcal infection reduces death during childhood in children with sickle cell anemia or hemoglobin SS.

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

• Neurologic complications caused by vasoocclusion 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.

• 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.

• 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.

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

Patient Care Process for Vasooclusive Episodes

Collect

• Patient characteristics (eg, age, sex, SCD genotypes)

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

• Immunization history

• Social history (eg, tobacco use)

• Pain diary

• Objective data

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

  • Labs: CBC, Basic chemistry,

  • Additional labs or imaging per presenting symptoms (see Table 120-2)

Assess

• Hemodynamic stability

• Pain scale

• Adherence to home medication

• Sign or symptoms associated with sickle cell acute complications (see Table 120-2)

Plan^*

• Fluid

• Pain management (see Table 120-6)

• Initiate antibiotics if febrile

• Oxygen

Implement

• Provide education on current pain regimen

• Develop individualized plan for pain management

• Evaluate if initiation of hydroxyurea or glutamine

• Schedule follow-up

Follow-Up: Monitor and Evaluation

• Ongoing evaluation of pain level

• Monitor for side effects of pain medication and initiate supportive care if needed

Preclass Engaged Learning Activity

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