Sickle Cell Anemia Vocabulary Flashcards

Vocabulary flashcards covering the pathophysiology, clinical findings, diagnostics, nursing care, medications, and priority complications of Sickle Cell Anemia.

Sickle Cell Anemia (HbSS)

An inherited hemoglobin disorder in which abnormal hemoglobin HbS causes red blood cells to become rigid and sickle-shaped, resulting in chronic hemolytic anemia, vaso-occlusion, tissue ischemia, severe pain, and organ damage.

Hemoglobin HbS

An abnormal variant of hemoglobin produced by a genetic mutation that polymerizes when deoxygenated, causing red blood cells to become rigid, sticky, and sickle-shaped.

Hemoglobin HbF (Fetal Hemoglobin)

A form of hemoglobin that, when increased, decreases red blood cell sickling and vaso-occlusion, resulting in fewer pain crises.

Vaso-occlusion

The obstruction of blood flow caused by rigid, sticky sickled red blood cells, leading to decreased blood flow, tissue ischemia, severe pain, and organ injury.

Hemolysis (in Sickle Cell Anemia)

The premature destruction of sickled red blood cells due to their shortened lifespan, leading to chronic anemia, elevated bilirubin levels, and jaundice.

Reticulocyte Count (in Sickle Cell Anemia)

A diagnostic laboratory value that is often elevated in clients with HbSS as the bone marrow attempts to compensate for ongoing hemolysis.

Hemoglobin Electrophoresis

A diagnostic laboratory test used to identify abnormal hemoglobin (such as HbS) and assist in determining the specific hemoglobin genotype.

Hydroxyurea

A disease-modifying medication that increases fetal hemoglobin (HbF) to reduce the frequency of vaso-occlusive crises and acute chest syndrome, requiring routine CBC monitoring due to the risk of bone marrow suppression.

Acute Chest Syndrome

A priority emergency complication of sickle cell disease characterized by chest pain, fever, cough, tachypnea, dyspnea, low SpO2, and a new pulmonary infiltrate.

Functional Asplenia

The progressive impairment or loss of normal spleen function caused by repeated sickling damage, which significantly increases susceptibility to serious infections.

Stroke (in Sickle Cell Anemia)

A severe, emergency neurological complication characterized by sudden weakness, facial drooping, difficulty speaking, severe headache, confusion, seizures, or vision changes requiring immediate evaluation.

Penicillin Prophylaxis

A preventive antibiotic regimen commonly administered to young children with HbSS according to clinical guidelines to reduce the risk of infection.

Hematopoietic Stem-Cell Transplantation

A therapeutic procedure that offers a potential cure for sickle cell disease in selected patients.

Swelling of Hands and Feet

An expected clinical finding in young children with sickle cell anemia resulting from vaso-occlusion in the microcirculation of the extremities.