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  • Image not available.Sickle cell disease is an inherited disorder caused by a defect in the gene for hemoglobin. Patients can have one defective gene (sickle cell trait) or two defective genes (sickle cell disease).
  • Image not available. Although sickle cell disease usually occurs in persons of African ancestry, other ethnic groups can be affected. Different mutation variants can result in variation in clinical manifestations.
  • Image not available. Sickle cell disease involves multiple organ systems. Usual clinical signs and symptoms include anemia, pain crisis, hepatosplenomegaly, and pulmonary diseases. Sickle cell disease can be identified by routine neonatal screening programs. Early diagnosis allows early comprehensive care.
  • Image not available. Patients with sickle cell disease are at risk for infection. Prophylaxis against pneumococcal infection reduces death during childhood.
  • Image not available.Hydroxyurea has been shown to decrease the incidence of painful crises, but patients treated with hydroxyurea should be carefully monitored.
  • Image not available. Neurologic complications caused by vasoocclusion can lead to stroke. Chronic transfusion therapy programs have been shown to be beneficial in decreasing the occurrence of stroke in children with sickle cell disease.
  • Image not available. Patients with fever greater than 38.5°C (101.3°F) should be evaluated, and appropriate antibiotics should include coverage for encapsulated organisms, especially pneumococcal organisms.
  • Image not available. Pain episodes can usually be managed at home. Hospitalized patients usually require parenteral analgesics. Analgesic options include opioids, nonsteroidal antiinflammatory agents, and acetaminophen. The patient characteristics and the severity of the crisis should determine the choice of agent and regimen.
  • Image not available. Patients with sickle disease should be followed regularly for healthcare maintenance issues and monitored for changes in organ functions

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After reviewing this chapter the reader should be able to:

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  • 1. Describe the hemoglobin abnormality in sickle cell disease.
  • 2. Discuss the pathophysiology of sickle cell disease.
  • 3. Discuss the role of newborn screening in sickle cell disease.
  • 4. List the clinical presentations of sickle cell disease.
  • 5. Describe the characteristics of sickle cell crisis and complications.
  • 6. Recommend the appropriate immunization schedule and recognize patients who are not up-to-date on immunization.
  • 7. Recognize patients who require penicillin prophylaxis.
  • 8. Discuss different fetal hemoglobin inducers available and explain the rationale of using HbF inducers and discuss the differences of
  • 9. Determine the appropriate hydroxyurea regimen and monitoring requirements.
  • 10. Discuss the role of transplantation in sickle cell disease.
  • 11. Discuss the indications, risks, and benefits of transfusions.
  • 12. Select the appropriate empiric antibiotics for patients with sickle cell disease presenting with fever.
  • 13. Formulate plans for management of acute chest syndrome, priapism, and sickle cell crises.
  • 14. Design a pain management regimen for patients presenting with pain crisis.
  • 15. Discuss the pharmacoeconomic impact of sickle cell disease.

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Image not available. Sickle cell syndromes, which can be divided into sickle cell disease (SCD) and sickle cell trait (SCT), are a group of hereditary disorders characterized by the presence of sickle cell hemoglobin (HbS) in red blood cells. SCT is the heterozygous inheritance of one normal cell and one sickle cell hemoglobin (HbAS) gene. Individuals with SCT are usually asymptomatic. SCD can be of homozygous ...

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