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KEY CONCEPTS

KEY CONCEPTS

  • imageSickle 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).

  • imageAlthough 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.

  • imageSickle 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.

  • imagePatients 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.

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

  • imageNeurologic 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.

  • imagePatients 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.

  • imagePain 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.

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

PATIENT CARE PROCESS

Patient Care Process for Vasooclusive Episodes

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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

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

PRECLASS ACTIVITY

Preclass Engaged Learning Activity

https://tinyurl.com/vb4g9ye

https://tinyurl.com/tel4648

Watch the short video entitled “Sickle Cell Anemia” in the DNA Learning Center. This short 1-minute video provides 3D animation of the gene mutation resulting in sickle cell disease. Then listen to the podcast in EM ...

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