Certified Pediatric Hematology Oncology Nurse (CPHON) Practice Exam 2025 - Free CPHON Practice Questions and Study Guide

Hb Sbeta0 Thalassemia is classified as usually severe due to the significant reduction or absence of beta globin chains, which leads to a clinical presentation characterized by severe anemia and associated complications. The combination of sickle hemoglobin (Hb S) and the absence of beta globin results in aggressive sickling of red blood cells, leading to vaso-occlusive crises, splenic sequestration, and increased risk of infections.

Patients with Hb Sbeta0 Thalassemia typically exhibit symptoms such as jaundice, fatigue, and growth impairment due to the severity of the anemia. Chronic hemolysis further exacerbates their condition, making regular transfusions and extensive medical management necessary to maintain hemoglobin levels and prevent complications.

In contrast, other classifications such as usually mild or moderate do not accurately represent the debilitating nature of Hb Sbeta0 Thalassemia, and the carrier state does not involve the same symptomatic and clinical challenges faced by those with the condition. Therefore, the classification of Hb Sbeta0 Thalassemia as usually severe underscores the critical need for comprehensive care and intervention in these patients.