Thalassemia prevention in Bangladesh: Challenges and national policy

Bangladesh has been able to control infectious diseases and reduce child death rate over the past decade. Non-communicable diseases control (NCDC) programmes have not yet started effectively. A genetic disease thalassemia is like the tip of the iceberg which has not yet received the due importance in the government’s NCDC programme. Few NGOs like Bangladesh Thalassemia Samiti, Bangladesh Thalassemia Foundation, Bangladesh Thalassemia Protirodh Andolon are working on it with very limited resources. May 08 is the international Thalassemia Day and theme of this year’s observance is “Be Aware. Share. Care: Strengthening Education to Bridge the Thalassemia Care Gap.”

Thalassemia is a genetic blood disorder and is one of the most widespread diseases worldwide.

There are different types of thalassemia but in South East Asia Hb-E &Hb-Beta-Thalassemia are most prevalent. According to WHO reports, Bangladesh has 7% thalassemia carriers (Hb-E 4% &Hb-Beta thalassemia 3%) but, according to recent statistics, the rate has increased to 10-12% and about 1.7 million people are thalassemia carriers in 170 million population. It is assumed that every year approximately six to eight thousand thalassemic children are born in Bangladesh. The number of patients suffering from thalassemia disease Hb E/B and thalassemia major with different level of severity is estimated to be approximately 70,000 – 80,000. This high prevalence of thalassemia diseases is due to consanguineous marriage and marriage between two carriers.

Thalassemia carriers are asymptomatic and unaware of genetic problem. Carriers need no treatment or dietary restriction but their importance lies on prevention of disease by not to marry another carrier. Because when both of the couple are carriers then there is 25% (one in four birth) probability of born thalassemic baby and 50% probability to be carrier (two in four birth) but unfortunately, all the babies might be thalassemic.

 Many of thalassemia patients (E/Beta and thalassemia major) are transfusion dependent for life long, starting from the age of 6th month. They are short stature with facial deformities (Thalassemic facies), delayed or absent puberty. Some variants of thalassemia (Hydrops foetalis with hemoglobin barts) are often incompatible with life but fortunately they are rare in Bangladesh. Thalassemia patient needs medical treatment such as regular blood transfusion to keep the Hb up to target level, screening for blood born infections, Chelation programme to minimize iron overload, endocrine complications related to iron overload, control of osteoporosis and sometimes splenectomy due to hypersplenism. According to Thalassemia International federation (TIF) reports fewer than 10% of global thalassemia patient communities in the developing countries can access the optimal standard of care.

In 2017, it was estimated that the annual direct cost for treating an individual with transfusion dependent thalassemia in Italy and turkey would be approximately 30,000 and 15,000 USD respectively. In Bangladesh, about 2500 USD (Tk two lac fifty thousand only) is spent annually, much less then developed countries. But in most cases, those are out of pocket expenditures. Thus management of thalassemia patients require a significant use of public health resource (approx. 120 billion Tk annually). But, unfortunately, public hospitals & NCDC are not addressing the issue appropriately.

Many patients with thalassemia has not been diagnosed and /or appropriately treated. There is a clear need to improve community education programme as well opportunities for genetic & prenatal counseling.

Hematopoietic stem cell transplantation (HSCT) is the only curative treatment. HSCT outcomes (>80%) are best for younger individuals who have undergone rigorous medical therapy with transfusion and iron chelation to minimizes the risks of organ damage from iron toxicities. HSCT for thalassemia was first pioneered in the early 1980 and results continue to improve over time.  Bangladesh has only four Bone Marrow transplant (BMT) centers and mostly they are doing transplant for blood cancer patients. Out of total 210 bone marrow transplants done till April 2023, only two thalassemic patients have been transplanted. We should increase BMT facilities exclusively for thalassemia patients. According to a 2012 analysis, allogeneic HSCT was cost-effective compared with standard medical treatment.

Effective diagnostic and screening programme are essential for prevention of thalassemia. Carrier detection, test for thalassemia before marriage, prenatal diagnosis of thalassemic fetus before 13 weeks of pregnancy, genetic counseling and awareness are utmost important to save the nation from thalassemia curse. For thalassemia prevention Bangladesh can follow Cyprus experience. Thalassemia was a serious problem in Cyprus. Therefore, strategies for prevention and management of thalassemia had been initiated in 1976. The aim was to stop the affected newborns and provide good treatment facilities for existing thalassemia patients. In 1979, high risk families started to be screened for thalassemia. In 1980, premarital screening was made compulsory by law. After prenatal diagnosis started in 1984, affected birth rates showed a sharp decrease. Between 1991 to 2001, only five thalassemic babies have been born. No thalassemic babies have been born in the last 5 years. A great majority of thalassemia patients are over 25years (66%), living and working as the normal population.

No doubt at present, thalassemia is a serious health problem in Bangladesh. The government should formulate a policy for the prevention and treatment of thalassemia immediately. For existing thalassemia patients standard of care (medical and curative) should arrange in every public and private hospitals. Planning should be done in such a way that no thalassemia baby can be born in the next five years. The main approach for thalassemia prevention is screening programme. Initially, from 2023, a policy needs to be in place to ensure the screening programme for high-risk group i.e. young generation between ages of 18 and 30 years (all College & University students). Also premarital screening is mandated by law. Scope for prenatal diagnosis should be increased in all medical college hospitals.

We are a Nation of Heroes, we have many successes, we can win the THALASSEMIA WAR too if we have the right vision and implement it rightly.

(Professor (Dr) M. A. Khan, Adviser Thalassemia Samity Hospital. Former Head & Founder of Dept of Hematology and Bone Marrow Transplant Unit, Dhaka Medical College Hospital, Dhaka.)