Thalassemia is a life threatening disease but when it combines with sickle cell anemia, it further intensifies the pain suffered by the patient by manifold. Same is the story of 9-year-old Bhupendra Ahirwar who presently faces immense pain due to Sickle Cell Thalassemia which separates him from rest of the thalassemia patient in terms of frequency of blood transfusion. Bhupendra needs blood transfusion more frequently than other patients sometimes twice a week. He received this rare disease, Sickle Cell beta-thalassemia from his parents. Normally this disease occurs when a person inherits a sickle cell trait and a beta-thalassemia trait from his parents. It results in a person having sickle-shaped RBCs and either producing a low amount of haemoglobin or none at all. In this disease, the healthy red blood cells are shaped like squishy discs. But sickle haemoglobin tends to devolve into a rigid crescent shape, deforming and damaging your red blood cells.

 

These dysfunctional red blood cells then block small blood vessels, reducing the flow of oxygen to the tissues. Dr Shweta Pathak, Bhupendra’s consultant, informed, “Due to his rare condition, Bhupendra needs particular type of blood after extended genotype testing, but unfortunately this testing is available in limited number of labs in the State. Not every lab conducts this testing. In lack of properly tested blood transfusion, Bhupendra’s body does not accept it and develop antibodies. Right now, we have changed his medication and successfully able to control his deteriorating health to a large extent. But we are planning to conduct his bonemarrow transplant as early as possible to protect his life.”

 

Bhupendra belongs to a village at Chhattisgarh border and have no proper medical facility back home, hence he has to come Jabalpur every week for blood transfusion. His paternal uncle accompanied him for blood transfusion every week and remains uncertain about the future of his nephew. “The doctors say that Bhupendra has a rare disorder in which his red blood cells are destroyed faster than they can be made. Earlier, we had to come twice a month, but later it became thrice and now its twice a week. We are not financially strong enough to bear all the costly medical expenses but somehow managing with the hope,” said Anirudha Ahirwar with deep sadness on his face.