Managing Sickle Cell Disease and Thalassemia with blood transfusion
Posted on 2024-04-06 By : Dr. Gaurav Kharya
Blood transfusion therapy is a crucial treatment for patients grappling with genetic blood disorders such as sickle cell disease and thalassemia. Blood transfusions offers relief from the symptoms and help manage the disease. But the transfusions have limitations and drawbacks. So, it's important to weigh the advantages of blood transfusions against its potential drawbacks to make an informed decision.
SCD and thalassemia, both are genetic disorders that affect the haemoglobin, the protein responsible for carrying oxygen in red blood cells. In SCD, the haemoglobin molecules form abnormal shapes, causing the red blood cells to become rigid and assume a sickle-like form. Thalassemia, on the other hand, involves an abnormal production of haemoglobin, resulting in fewer healthy red blood cells.
Thus, blood transfusions serves as a critical intervention for patients with these disorders as transfused red blood cells can provide normal haemoglobin, improving oxygen supply throughout the body. It helps cells to function and grow normally. However, patients often require frequent transfusions, which may lead to iron overload. To prevent excessive iron from damaging the organs, some additional treatment are required to manage excess iron levels.
Patients suffering from sickle cell disease often complain of pain. The sickle-shaped cells can cause stress in blood vessels. Regular transfusions can reduce the frequency and severity of pain crises in sickle cell patients, improving their overall quality of life.
Besides, transfusions help prevent complications such as stroke in sickle cell patients and anaemia in thalassemia patients.
Blood transfusions support normal growth and development in children with thalassemia, reducing the impact of the disease on their physical well-being.
However, managing transfusion-related complications, such as infections or allergic reactions, requires close monitoring and medical intervention.
With frequent blood transfusion, there is always a risk of transfusion-transmitted infections and alloimmunization (development of antibodies against donor blood).
Besides, frequent transfusions contribute to iron accumulation in the body. Chelation therapy helps remove excess iron, but again its effectiveness requires careful monitoring and adherence.
Most importantly, regular transfusions require efficient scheduling and accessibility to healthcare facilities. While blood transfusion offers indispensable benefits, addressing the potential drawbacks is crucial for ensuring the overall well-being of patients. Continued research, technological advancements, and a holistic approach to patient care are essential to optimise the effectiveness of blood transfusion therapy in improving the lives of individuals with these genetic blood disorders.