Sickle cell disease (SCD): A focus on updates in therapy
Sickle cell disease (SCD) is the most common monogenic blood disorder, affecting millions of people worldwide and approximately 100,000 Americans. Although it may be found in various areas of the world, SCD predominantly affects individuals of African or Hispanic heritage. It is caused by the inheritance of b-globin alleles that code for hemoglobin S, resulting in an amino acid substitution in hemoglobin’s b chain and clinical disease. Patients with SCD have impaired circulation, and lysis of the erythrocytes contributes to a chronic inflammatory response, causing severe pain and less efficient oxygen delivery. The hallmark clinical features of SCD are hemolytic anemia and painful vaso-occlusive crises (VOCs), which may lead to emergency department visits, hospitalization, and potentially fatal complications such as acute chest syndrome, stroke, or pneumonia. In one US study, 45% of deaths among people with SCD were related to cardiopulmonary causes, and VOCs alone have been shown to increase the risk of death by 50%. SCD may disrupt employment or school and is associated with a significant reduction in quality of life. This learning activity has been designed to bring HCPs’ knowledge of rationale behind treatment of SCD up to date and to enhance their competence and performance in the condition’s management.
Cost: Free
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Sickle cell disease (SCD) is the most common monogenic blood disorder, affecting millions of people worldwide and approximately 100,000 Americans. Although it may be found in various areas of the world, SCD predominantly affects individuals of African or Hispanic heritage. It is caused by the inheritance of b-globin alleles that code for hemoglobin S, resulting in an amino acid substitution in hemoglobin’s b chain and clinical disease. Patients with SCD have impaired circulation, and lysis of the erythrocytes contributes to a chronic inflammatory response, causing severe pain and less efficient oxygen delivery. The hallmark clinical features of SCD are hemolytic anemia and painful vaso-occlusive crises (VOCs), which may lead to emergency department visits, hospitalization, and potentially fatal complications such as acute chest syndrome, stroke, or pneumonia. In one US study, 45% of deaths among people with SCD were related to cardiopulmonary causes, and VOCs alone have been shown to increase the risk of death by 50%. SCD may disrupt employment or school and is associated with a significant reduction in quality of life. This learning activity has been designed to bring HCPs’ knowledge of rationale behind treatment of SCD up to date and to enhance their competence and performance in the condition’s management.
Learning Objectives
By the end of the session the participant will be able to:
- Recall SCD’s pathophysiology and healthcare burden.
- Describe standards of care and emerging therapies for SCD.
- Compose a treatment plan for a patient with SCD.
Target Audience
The following HCPs: Hematologists, pediatricians, and primary care physicians; physician assistants, nurse practitioners, and pharmacists who specialize in the aforementioned areas of specialty; and any other HCPs with an interest in or who may clinically encounter patients with SCD.