After reviewing this chapter the reader should be able to:
- 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.
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 ...