Bangalore, Karnataka, India:
Due to lack of awareness and access to quality care, thalassemia is rapidly becoming a reported major health problem Dr. Sunil Bhat, Director and Clinical Lead, Pediatric Hematology, Oncology and Blood and Bone Marrow Transplantation, Narayana Health. An opinion shared by the doctor on the occasion of International Thalassemia Day, celebrated worldwide on the 8and of May. According to the doctor, addressing the same issue would require comprehensive and equal participation of all stakeholders, including patients, families of patients, policy makers, government and health experts.
Thalassemia is a condition in which the body’s red blood cells are affected. Blood, the lifeline of our body, has three major cellular components: red blood cells, white blood cells, and platelets. Red blood cells provide energy and carry oxygen around the body. In thalassemia major, the synthesis of red blood cells is insufficient and the lifespan is also very short. The disease manifests early in childhood, mainly in the first year of life, and these patients require frequent lifelong blood transfusions (every few weeks) to stay alive.
Burden of the problem: – 80% of children with this disease are born in the Asian subcontinent and Middle Eastern countries. In India, it is estimated that 10,000 to 12,000 children are added to the pool every year. Parents, usually asymptomatic, are carriers of this disease and there is a 25% risk that this disease will affect their children. Carrier status in the Indian population ranges from
The Challenges of Blood Transfusion:- Needless to say, these patients need a fast, safe blood supply. It has been estimated that annual red blood cell requirements are around 2 million units in India. Although patient organisations, Indian Red Cross and other regional blood banks have been working tirelessly to meet this requirement, the demand is far outstripping the supply. Many families struggle month after month to make packed cells available for their children. In a country of 1.4 billion people, can we pledge today to donate blood to save the precious lives of thousands of thalassemia children.
As blood is a potential vector for transmission of infections, it is very important that the blood these patients receive is safe. Although the government of India, under the NACO scheme, mandates screening for blood-borne diseases like syphilis, hepatitis B, hepatitis C and HIV, it is still not practiced universally, in especially in small towns. Even screening for these infections by traditional methods leaves some children at risk, if blood is collected within the ‘window period’. Newer methods like NAT testing should be mandated and sponsored to minimize the risk of infection transmission during this window period.
Iron chelation: Repeated hematocrit transfusions lead to iron overload, which is deposited in important organs such as the liver, heart and endocrine glands. This iron overload is usually the cause of death in the second or third decade of life. These patients must take medication to remove this extra iron from the body, and if properly chelated, they can have a normal lifespan. Despite freely available drugs, less than 10% of patients are properly chelated. Various factors such as ignorance, poor compliance and most importantly unaffordability are the main reasons for poor chelation. Very few state governments have made these drugs available to patients, but supplies are erratic and inadequate. There is a need to have central legislation making these drugs freely and freely available to these patients.
Thalassemia care centers: Thalassemia is a multi-systemic problem and in addition to hematocrit and iron chelation, requires management by a multidisciplinary team. This team should have a hematologist, endocrinologist, cardiologist, nutritionist, nurse practitioner, etc. for holistic care of these patients. There are virtually no comprehensive thalassemia care centers and a rough estimate is that less than 5% of thalassemia patients receive care at such centers. There is an urgent need for such regional centers that can coordinate and monitor thalassemia care.
A bone marrow transplant:- Bone marrow transplantation is currently the only curative option available for this condition. Recent data shows an over 90% success rate of bone marrow transplantation in patients who have HLA-matched siblings. Very small percentage of cases undergo this procedure. Factors such as ignorance of the procedure, affordability and insufficient centers where this treatment is available are barriers to this curative treatment. It is necessary for governmental organizations, non-governmental organizations and other leaders to come together and support this curative treatment program.
Preventive measures: The need of the hour is to have an effective national program for the prevention of thalassemia, because the scale of the problem, both economic and social, is enormous. Screening for thalassemia carriers should be made compulsory and prenatal screening made free in order to detect the disease very early in pregnancy. If small countries like Cyprus have managed to eliminate this problem with an effective national thalassemia prevention program, why not us?
Need for patient advocacy groups: The best advocates for change and improvement can be the patients/families themselves. Thalassemia societies in some states have catalyzed tremendous change in thalassemia care by virtue of constant advocacy. It is necessary that these patient/family groups be formed in each city/state. These advocacy groups can identify problems and bring them to the attention of the political establishment.
In summary, the scale of the thalassemia problem is enormous, but unfortunately insufficiently addressed. The Ministry of Health needs to put it on the “priority list” and tackle the problems, of which there are many. There is also a need for medical experts and families to come together and advocate for their patients. Thanks to these collaborative efforts, our children with thalassemia will also receive the standard care they deserve!
With all the super-specialty tertiary care facilities that the medical world offers, Narayana Health is a one stop healthcare destination for everyone. Founded by Dr. Devi Shetty and based in Bengaluru, Narayana Health Group is the second largest healthcare provider in the country by number of operational beds. The first facility was established with around 225 operational beds in 2000 at NH Health City in Bengaluru. The company today operates a chain of multi-specialty tertiary and primary healthcare facilities through a network of 23 hospitals and 7 cardiac centers in India and a single overseas hospital in the Cayman Islands with nearly 5,900 operational beds. in all its centers and the potential to reach a capacity of more than 6,800 beds (For more details, visit www.narayanahealth.org)