In this online, self-learning activity: 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 (ED) visits, hospitalization, and potentially fatal complications. 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 also imposes other significant health burdens on patients: it is associated with a significant reduction in quality of life, malnutrition, the development of mental health disorders like depression and anxiety, loss of work or school days, frequent antibiotic use leading to higher rates of antimicrobial resistance, acute chest syndrome, and stroke.
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- Adolescent Medicine
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- Endocrinology, Diabetes & Metabolism
- Gastroenterology
- General Internal Medicine
- General Pediatrics
- Hematology
- Medical Oncology
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- Pediatric Infectious Diseases
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- Pulmonary Disease
- Rheumatology
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- Cardio-Vascular
- Cross-Specialty
- Emergency & Critical Care
- GI & Liver
- Infectious Disease
- Internal Medicine
- Medical Subspecialties
- Neurology
- Oncology & Hematology
- Pediatrics
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