Transfusion Therapy in Thalassemia: Efficacy and Safety

Thalassemia Major is an inherited blood disorder that poses a significant health burden in India. This disease is genetically passed to the children from their parents. Children affected with Thalassemia Major hardly live for 20 to 25 years and require frequent blood transfusions.

This condition is characterized by less haemoglobin and fewer red blood cells than normal, which causes severe anaemia. Due to this, the patients have to rely on frequent blood transfusions all their lives at a frequency of almost every 15 days. The affected children may not show symptoms up to 1-2 years of age, as they have foetal haemoglobin. However, as they grow up, the formation of haemoglobin in their bone marrow is insufficient. From the time of birth, considering the age and weight of a Thalassemia Major child, blood transfusions are given to normalize the haemoglobin levels, every month. As the age increases, the frequency of blood transfusions also increases, which can be anywhere between 15 days to 2-3 times in a month.

In order to help the Thalassemics and their families who have to bear the expenditure of blood transfusion all their lives, the National Blood Transfusion Council (NBTC) of India took the initiative to provide free blood transfusion to such patients in the year 2014. Bodies like the Indian Red Cross Society (IRCS) have taken this initiative seriously to ensure the availability of blood to affected individuals. However, patients living in urban areas have better quality of life due to ease of access to blood transfusion and related therapy. On the other hand, the lack of blood transfusion facilities and added transportation costs may pose a significant challenge for patients living in remote areas.

During blood transfusion, it is necessary for a parent/guardian to be present with the child. This further adds to the existing challenges, especially in the case of people who work on daily wages.

Blood transfusion is life saving for Thalassemics but it comes with certain negative impacts on the body. Frequent blood transfusion leads to the accumulation of iron in vital organs like the liver and heart. In order to control this, chelation therapy is required, which costs approximately Rs 8,000 to 9,000 per month.

Another challenge associated with frequent blood transfusions is the possibility of transmission of deadly blood borne infections such as HIV, Hepatitis B and Hepatitis C. Reports suggest that upto 45% of Thalassemia patients may get infections like HIV, HBV or HCV in lifetime due to transfusion of infected blood. Although the screening of blood is mandatory in India, every now and then we come across cases of Thalassemic patients getting Transfusion Transmitted Infections (TTIs) through infected blood. This highlights the limitation of serology-based blood screening assays which are mandatorily used across India.

“In order to win the battle against Thalassemia, public awareness is the key. A concentrated awareness not just about the disease but also about the importance of safer blood supply is very much required.”

In a shocking case that happened few years back in Gujarat, 32 Thalassemic children got HIV through transfusion. In another recent case in Ranchi, 7 Thalassemic children got HIV and Hepatitis C, which was traced back to infected blood given to them during transfusion. The occurrence of such ghastly incidents clearly points out that serology-based assays are unable to detect early-stage infections in asymptomatic donors, which escape detection and end up contaminating safe blood supply.

Thankfully, in order to avoid such deadly mishaps, many hospitals and blood banks in India are moving towards highly sensitive techniques to screen blood. One such technique that has found its use in the medical fraternity, for its robustness and high sensitivity is the Individual Donor Nucleic Acid Testing (ID-NAT). Many studies indicate that ID-NAT brings down the possibility of missing out an infection, as it is sensitive enough to detect early-stage infections. This is especially beneficial in cases of asymptomatic donors, which are otherwise difficult to detect using conventional techniques. This makes ID-NAT a more reliable choice for blood screening when compared to commercially available serology-based assays.

India has more than 3,000 licensed blood banks catering to the world’s second largest population. But sadly, only few hundred blood banks have adopted NAT as a routine screening test. That means only 3% of blood supply is NAT tested. Most of the blood banks that have adopted NAT are private blood banks and thus a number of Thalassemic patients receiving NAT tested blood is very less. That brings us to the conclusion that even if we accomplish the target to stop Thalassemia cases from occurring, already existing patients will be at a constant threat of contracting TTIs. Blood disorders are financially and emotionally taxing for the patients as well as their families. On top of it, a TTI or a life-long viral infection is not only traumatic for patients and their families but also for the transfusion practitioners. Other than ethical and moral issues, providing a patient with contaminated blood is a criminal offense.

Gujarat Red Cross Society started Thalassemia Prevention Control and Research Programme in the year 2004 with an aim to find out the incidence of thalassemics in Gujarat and to make the state “Thalassemia free”. Ms Anandiben Patel – Former Education Minister of Gujarat (who became Chief Minister later) issued a directive on the 18th of January 2005, to all universities in the state to make Thalassemia test compulsory in their colleges. Till date, Red Cross Society has screened more than 28 lakh individuals for Thalassemia and sickle cell anaemia under this programme. Also, Gujarat Red Cross Society has tested 1402 Thalassemia Minor and sickle cell trait couples for the prenatal diagnosis and has prevented 373 Thalassemia Major and sickle cell incidents so far.

Other than that, with the support of Government of Gujarat Health Department, Red Cross Society has taken an initiative to start “Thalassemia Major children care and support progamme”, that provides them with facilities to undergo their essential blood tests. Each Thalassemia Major child has been provided with an individual profile that contains information of all the tests carried out throughout the year. Till date, 3,723 Thalassemia Major children have undergone the required blood tests and have been benefited under this government programme.

In order to win the battle against Thalassemia, public awareness is the key. A concentrated awareness not just about the disease but also about the importance of safer blood supply is very much required.

Other than transfusion practitioners, people do not have enough knowledge about blood safety and the consequences of unsafe blood transfusions. State Governments should provide constant funding for Thalassemia prevention projects. The way cost of blood transfusions for Thalassemics are managed by the Government, the supply of safe blood must be ensured through tests like ID-NAT. Considering the magnitude of Thalassemia burden in India, wider adoption of ID-NAT can help reduce the incidence of TTIs drastically, especially among multi-transfused patients.

It is high time for the State Governments and National level policymakers to realize the importance of sensitive blood screening techniques and to come up with patient-friendly policies.

About Author: Recipient of the “Glory of Gujarat” Award, Dr Prakash Parmar is currently the General Secretary of Indian Red Cross Society, Gujarat.

Dr Parmar is also the National Coordinator for Thalassemia International Federation. In his presence as an expert panelist, Gujarat Red Cross Society received an award from the Health Minister, New Delhi, where Gujarat State was recognized as a model state for Thalassemia Prevention Programme. Dr Parmar is also a member of Thalassemia Research Committee, ICMR, Delhi.