Evidence supporting the use of: Hemoglobin
For the health condition: Sickle Cell Anemia
Synopsis
Source of validity: Scientific
Rating (out of 5): 4
Hemoglobin is central to the pathophysiology and treatment of sickle cell anemia (SCA). SCA is a genetic disorder characterized by the production of abnormal hemoglobin S (HbS), which causes red blood cells to become rigid and sickle-shaped, leading to hemolytic anemia and vaso-occlusive crises. The management of sickle cell anemia sometimes involves transfusion of normal (non-sickle) red blood cells, which contain normal adult hemoglobin (HbA), to increase the proportion of healthy hemoglobin in the patient's circulation. This reduces the concentration of HbS, thereby decreasing sickling events and improving oxygen delivery. The use of hemoglobin-containing blood products is supported by decades of clinical research and is a standard of care for managing acute complications such as severe anemia, acute chest syndrome, and stroke prevention in high-risk children. In addition, therapies that increase fetal hemoglobin (HbF) production, such as hydroxyurea, are used because HbF inhibits sickling. While direct supplementation with purified hemoglobin is not a standard treatment, the manipulation of hemoglobin levels through transfusion and pharmacological induction is well-established and evidence-based. Multiple clinical guidelines and studies support the efficacy and safety of these interventions, though risks such as iron overload and alloimmunization must be managed. Overall, the use of hemoglobin in treating sickle cell anemia is scientifically validated and forms a cornerstone of modern management strategies.
