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Hemoglobin SS (Hb SS) refers to a specific type of sickle cell disease where an individual inherits two copies of the sickle cell gene, one from each parent. This genetic makeup leads to the production of abnormal hemoglobin (hemoglobin S), which causes red blood cells to become rigid and sickle-shaped, especially under low-oxygen conditions. As a result, Hb SS is typically associated with severe manifestations of sickle cell disease.

Patients with Hb SS often experience a range of complications, including frequent pain crises, risk of infections, acute chest syndrome, and other severe complications, which can significantly impact their quality of life and require ongoing medical management. This severe form of the disease contrasts with other sickle cell conditions, such as sickle cell trait (carrier state) or other types of sickle cell disease that may not be as severe.

Understanding the distinction between Hb SS and other conditions is crucial for the management and treatment of affected individuals, making it clear why identifying Hb SS as typically severe sickle cell disease is accurate.