Hyderabad: With the World Thalassemia Day being observed on May 9, it is essential to understand what the disorder is and how it impacts people’s health.
Thalassemia is an inherited blood disorder where the body produces an insufficient amount of hemoglobin, the protein in red blood cells that carries oxygen. The disorder could cause to anemia, a condition where there’s a lack of healthy red blood cells. Thalassemia is passed down through genes from parents to children.
World Thalassemia Day
Observed on May 9, the World Thalassemia Day is dedicated to raising awareness about the disorder amongst the general public and decision-makers. Promoting and strengthening the lifelong and difficult struggle of patients against this severe blood disease, and commemorating all the people who are no longer with us, while renewing our promise to keep fighting until the final cure for thalassemia is found.
In an interview with Siasat.com, doctors from Hyderabad shared their expertise on the disorder and called for early detection.
Addressing the impact of thalassemia on the body, Dr Suboor, a consultant oncologist and hematologist from CARE Hospital in Hyderabad said, “Thalassemia is a disorder of hemoglobin synthesis. It is an inherited condition causing reduction in production hemoglobin.”
The doctor further explained that hemoglobin has two chains including alpha and beta and any defect in the production of these chains leads to reduced production of hemoglobin, which in turn reduces production of red blood cells.
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Types
The oncologist delved into types of thalassemia classifying it into alpha thalassemia and beta thalassemia. The classification is based on the type of hemoglobin chain which is affected. Explaining how the disorder impacts the body, Dr Suboor said, “Thalassemia causes reduced hemoglobin productionin the body. This leads to inadequte supply of oxygen to the tissues, patients reflect symptoms of anemia, including fatigue, weakness and lethargy.”
Even a slight amount of exertion causes breathlessness. The oncologist added that thalassemia is further classified into major thalassemia and minor thalassemia. “Major thalassemia is reflected in early childhood, especially in the first decade itself,” the doctor said adding that the child looks lethargic, pale and blood tests reveal reduction in hemoglobin.
He further said that thalassemia minor is largely observed in adults and is asymptomatic. It is caused by blood loss or other stress, which could trigger further reduction in hemoglobin, leading to symptoms of anemia.
Treatment
Speaking of treatment for the disorder, the oncologist said that major thalassemia is caused by sever defect in the beta chains of hemoglobin. “Children as young as 4-5 years also present with thalassemia, They require frequent blood transfusions due to symptoms of anemia,” he added.
Children suffering from thalassemia experience fatigue and are unable to remain active like children their own age. “Frequent blood transfusion causes iron overload and they may need iron chelation therapy,” the doctor explained.
Commonly used medicine for iron chelation is deferoxamine along with supportive medications such as hydroxyurea to increase fetal hemoglobin levels in the child so that the body compensates for the reduction in normal hemoglobin.
For thalassemia major, bone marrow transplant is done. However, the doctor said, “A donors healthy bone marrow, transplanting it to the affected patient. The donor must not have symptoms of thalassemia or other blood disorders.”
Family members can’t be considered for a bone marrow transplant since the disorder is hereditary. In several cases bone marrow donors are hard to find, and the patients are put through blood transfusion and iron chelation therapy.
Gene therapy
Speaking of treatments for thalassemia and advancements in gene therapy, Dr K Karuna Kumar, a consultant hematologist from Yashoda Hospital, said, “India is still behind in terms of implementing these therapies for treatment. The therapies aren’t available in India, and it may take a decade for these therapies to be implemented in India. “
Currently, the research is ongoing and it may take a while for the gene therapy to be used in India for thalassemia cases.
Listing out steps that could be taken by communities and policymakers to enhance prevention and care for thalassemia patients, the doctor said, “The policymakers can help eradicate the disorder however, lack of awareness regarding the issue is a major factor for not having a policy for thalassemia.”
The doctor suggested that screening for thalassemia should be made compulsory for all pregnant women at the time of conceiving. This could help in counselling the women and a prenatal diagnosis to avoid the occurrence of thalassemia major
