There are three types of iron-chelating medications– Deferoxamine injection, Deferiprone capsules/tablets and Deferasirox tablets. All three types of drugs are available in Bangladesh, but they are not given free of charge and are not readily available everywhere. These drugs are distributed free of cost in government hospitals to registered patients in India. In Algeria, thalassemia patients receive a “Chifa” card as chronic disease patients, which allows them to get free medication from pharmacies [30]. In Pakistan, the “Sehat” card provides free healthcare to the country’s underprivileged population, which is a form of health insurance for the poorest and financially weaker sections of society [31, 32]. There is no such national policy or health insurance facility in Bangladesh.

It is important to regularly monitor liver and cardiac iron concentration to prevent iron accumulation and subsequent complications. An MRI-based approach called Ferriscan is currently the most widely accepted technique for assessment of iron overload. Unfortunately, this test is available just in one or two medical centres in Bangladesh. The cost of each test is about BDT 7000 (heart) and BDT 10,000 (liver), according to BTF (Personal communication, February 2024). Besides, this test is not available in government hospitals. More affordable MRI-based techniques, such as MRI T2* and MRI R2*, also provide reliable liver and cardiac iron assessments. However, these tests are similarly limited to a few private medical centers and remain unaffordable for most, with costs ranging from BDT 10,000 to 12,000 per test. The restricted availability and high cost of these diagnostic tools continue to pose significant challenges for patients and healthcare providers in Bangladesh.

Inadequate treatment infrastructure

Treatment of thalassemia patients in Bangladesh is mainly centred in Dhaka city. According to the Directorate General of Health Services, out of 185 blood banks in Bangladesh, 116 are located in Dhaka district and the remaining 69 are located in other districts of Bangladesh. Although there are 666 hospitals and clinics in Dhaka district, there are only 68 hospitals and clinics in each district outside Dhaka [33]. District-level treatment facilities have not yet been advanced. There is a severe shortage of haematologists in most districts of the country. Even at the town level, the main means of survival, blood transfusion, has not yet been developed. Therefore, there is inequality in health services across the country.

TIF-affiliated treatment centres like Bangladesh Thalassemia Samity Hospital (BTSH), Lab One Foundation of Thalassemia, Bangladesh Thalassemia Foundation (BTF) are located in the capital city. These treatment centres offer daycare blood transfusion services, medicines and lab tests at subsidized prices for member patients. In addition, the haematology department of Dhaka Medical College Hospital and Dhaka Children’s Hospital, BSMMU are providing multidisciplinary care to thalassemia patients. However, compared to the need, these opportunities are very limited and almost non-existent outside the capital city. Organized treatment facility is available only in thalassemia treatment centres in Dhaka and Chittagong. About 68.3% of the country’s population lives in villages [8], making it difficult for them to regularly visit cities or the capital for treatment. Only financially capable families can provide long-term medical care for thalassemia patients. Most patients are deprived of adequate and standard treatment. The majority of the patients in Bangladesh die without proper treatment or without even knowing that they have the disease.

The tribal population in Bangladesh (living in tribal areas such as hill tracts) may face challenges in accessing healthcare services due to geographical remoteness, limited infrastructure, and cultural factors such as language barriers. This is an important issue to address in the context of thalassemia prevention, as the epidemiological figures among the tribal community are alarmingly different.

Monitoring and evaluating the health of thalassemia patients and its complications requires multidisciplinary treatment involving paediatricians, haematologists, transfusion medicine specialists, cardiologists, endocrinologists, gynaecologists, nutritionists, psychiatrists, dentists and strong blood bank infrastructure [34]. In a country like Bangladesh, these multidisciplinary facilities are usually unavailable in most government hospitals and private clinics. The government of Bangladesh published national guidelines on thalassemia care in 2019 for physicians [35], which is commendable, but the health system is yet to be developed to implement it.

Financial burden

About 18.7% of people in Bangladesh currently live below the poverty line [36]. Bangladesh lags far behind other South Asian countries in terms of out-of-pocket health expenditure. The financial impact of thalassemia on patients and their families is immense as patients in this country have to bear a large portion (74%) of the costs of treatment, medicines, and other health services from their own pockets [37]. In India, Pakistan, Nepal and Sri Lanka this rate is between 45 and 55%.

A TDT patient is expected to spend approximately BDT 19,000 per month to receive minimal standard treatment (Table 3). Most of the families of thalassemia patients belong to the lower and middle-income class, for whom it is quite difficult to spend this much money per month. The cost of thalassemia treatment consumes a large part of the income of low or middle-income families and sometimes the treatment cost of a family is higher than the average family income [7]. As a result, the patient as well as the whole family suffers psychologically and financially. A recent study has shown that an average thalassemia patient can spend only around BDT 2,000 per month on treatment (Table 3). The large discrepancy between estimated costs and actual expenditure results in inadequate treatment. In the absence of universal health coverage and health insurance facilities at the national level, most patients survive with inadequate treatment.

Table 3 Monthly treatment cost per patient of thalassemia

Since 2019, the Department of Social Services has been providing support to poor patients with cancer, kidney, liver cirrhosis, paralysis by stroke, congenital heart disease and thalassemia with BDT 50,000 per person per year [38]. This grant is not universal; the approval is subject to the application verification, the candidate’s eligibility and budget availability. As there is no facility for free medicine or lab tests for thalassemia in the country, this budget is inadequate given the large number of thalassemia patients and unaffordable treatment costs. Although thalassemia patients are included in the definition of disability in India, Canada and some other countries, they are not included in the Disability Act of Bangladesh yet. Therefore, patients are not eligible for disability benefits such as disability allowance, stipend, job quota, interest-free loans, or reserved seats in public transport.

Psychosocial challenges

Thalassemia patients do not usually want to disclose their disease for fear of social rejection or discrimination, as reported by a qualitative study from Iran [39]. Our study found that their fear is reasonable as our previous study in Bangladesh found that 17.3% of college students in Bangladesh do not want to be friends with thalassemia patients and 14.4% do not want to donate blood to thalassemia patients [40]. Families of thalassemia patients participate less in social events and feel socially isolated, according to a study from Thailand [41]. Similarly, our study found that more than 40% of the patients’ parents face social stigmatisation in Bangladesh [14]. Due to the lack of awareness, misinformation and superstition, thalassemia is considered a ‘blood fault’ that isolates patients’ families socially. Our unpublished (under review) study confirms that almost half (46.8%) of the mothers having children with thalassemia agree to the fact that their social life is hampered [42]. Studies in this aspect are still scarce in Bangladesh.

Physical adversity also affects the mental health of patients, but this issue remains neglected due to the preoccupation with dealing with the disease. Mental health takes a huge toll on patients as well as their families. Mothers, as the primary caregivers, suffer from anxiety (~ 58%), depression (~ 63%), and stress (~ 62%) in Bangladesh [42]. The underlying cause of social and emotional problems is their concern with the desire to live a long and quality life. As the number of adult patients increases as a result of advances in medical science, concerns about their quality of life and mental health are increasing. With increasing age, disease-related complications may also rise. Patients are always worried about the uncertainty of receiving blood regularly. Bone deformity, changes in facial structure and disrupted growth are common consequences of inadequate treatment. Because of these, some thalassemia patients—talented graduates—are refused to be hired by employers. Starting a family is also very uncertain for them. Our unpublished (under review) study found that 90.4% of mothers are concerned about their thalassemic child’s future (education, job, marriage) [42]. Adult patients are realising at some point that adequate and regular maintenance of proper treatment can lead to better survival. Patients from developed countries are now encouraged to lead a normal life, pursue higher education, work in a job, get married and have children [43]. However, the patients in Bangladesh are lagging in this regard. If adequate support is provided systematically in specialized treatment centres and if awareness among the mass can be improved, these things can become easier in patients’ lives.

Expensive curative treatments

Blood transfusion and iron chelation are not curative treatments for thalassemia. So far, the only conventional curative method for thalassemia is bone marrow transplantation, which is very expensive. Bone marrow transplantation for thalassemia is still to be established in Bangladesh. Dhaka Medical College Hospital, Evercare Hospital and Dhaka CMH (Combined Military Hospital) have performed bone marrow transplantations of cancer patients as well as a very small number (1.2%, 2 out of 163) of thalassemia patients that costs BDT 1.4 million on average [44]. Apart from the high cost, finding a bone marrow donor is a challenge. Transplant from matched family donor (sibling or parents) is only allowed in Bangladesh. Transplant form matched unrelated donor is currently unavailable in Bangladesh as there is no national bone marrow registry. Besides, some sibling refuses to donate marrow out of fear of complications even after proper counselling. On average, this procedure has a mortality risk of about 9%, while the success rate, i.e. the chance of having a thalassemia-free life, is only 83% [45]. Overall, the success of bone marrow transplantation depends on the patient’s age, bone marrow matching and the patient’s clinical condition.

Gene therapy has emerged as a promising treatment for beta thalassemia, offering potential cures, particularly for patients with transfusion-dependent beta-thalassemia. It was first approved by the US Food and Drug Administration (FDA) for beta thalassemia in 2022. Clinical trials demonstrated significant efficacy, with many patients achieving transfusion independence [46]. However, gene therapy is highly expensive and requires specialized medical infrastructure. This costly procedure, though available in developed countries, is unlikely to reach patients in Bangladesh soon, as its current cost exceeds BDT 300 million [47]. In addition, there are some remedial drugs, but their use needs more research. A recently approved modern injectable drug Luspatercept can retain hemoglobin in the blood. Its 25 mg price in India is around Rs. 41,000 (USD 491.50) (Corsantrum Technology- medicine exporter, personal communication, December 8, 2023). The injection has to be taken every three weeks, which means a patient weighing 50 kg may need to spend at least Rs. 92,000 (∼USD 1103) per month for this. All in all, the curative or supportive measures for thalassemia are inadequate, so prevention is the must.

Awareness of thalassemia in Bangladesh

People’s ignorance is a barrier to awareness. The people in Bangladesh know very little about this disease. Bangladesh Thalassemia Samity and the Government of Bangladesh have been trying to increase thalassemia awareness by sending short messages on mobile phones for the past few years. Yet more than half of the people (53%) had never heard of thalassemia [48]. The recent national estimate regarding this is even much higher (81.6%) [10]. And despite hearing the name, there are many misconceptions about thalassemia in people’s minds. About 21% of people do not know the disease is hereditary, many do not know exactly why the disease occurs or how to diagnose it [48].

On the other hand, our study on the parents of thalassemic children found that almost none of them (97%) had heard of thalassemia before their child was diagnosed with the disease, none of them had a premarital blood test, and most (91%) of the parents regretted not having a premarital blood test [14]. Almost half of the parents who participated in that study did not know that thalassemia is preventable (51.5%) and that carriers can lead healthy lives (54.5%). Only one-third (35.6%) of parents had heard of prenatal testing for thalassemia diagnosis. Although one-third of parents were planning to have more children in the future, but only one-quarter (24.9%) of parents wanted to have a prenatal test, while the rest relied on their fate. Parents of patients can play a significant role in creating public awareness because others can realize the severity and socioeconomic difficulties of this disease through them. However, the lack of knowledge and limited understanding about thalassemia among the parents is a hindrance. To raise awareness of thalassemia, one book targeting university students, and parents has been published where career development is linked with thalassemia [49].

Thalassemia prevention strategy and care in the context of Bangladesh: future perspectivesPreventing the birth of children with thalassemia

The sole aim of the Thalassemia Prevention Action Plan is to prevent the birth of children affected by the disease. There are two ways to achieve this: i) Preventing marriage between two thalassemia carriers, after knowing whether or not someone is a carrier through a blood test (Hemoglobin Electrophoresis) before marriage, known as premarital screening. Carriers carry the thalassemia gene silently and do not show any symptoms. The Hemoglobin Electrophoresis test is enough to be done once in a lifetime and only this test can diagnose thalassemia carriers. (ii) In the case of two carrier parents, the affected child’s birth can be prevented by screening the fetus for thalassemia, known as prenatal screening. Its purpose is to legally carry out an abortion on the advice of a doctor if the unborn child is diagnosed with thalassemia disease. Thus, theoretically, thalassemia is 100% preventable.

Why is the thalassemia prevention strategy different from other diseases?

Thalassemia is usually confined to malaria-prone areas of the world and does not spread like an infectious disease. The issue of thalassemia is localized in the global thalassemia belt (thalassemia-prone regions). Thalassemia is neglected in international research because thalassemia is not a major health concern in developed countries. Therefore, the lack of international funding and cooperation appears to be the major setback to tackling this preventable disease. Funding and patronage of international health agencies and major NGOs are at the heart of Bangladesh’s success in children’s vaccination and preventing various infectious diseases (such as diarrhoea, tuberculosis and worms). Despite the higher prevalence of thalassemia compared to some infectious diseases, thalassemia is not well studied. Consequently, its economic and social impact remains unknown.

A study in Sri Lanka found that 5% of the country’s health budget is required to manage thalassemia patients [16]. Another study conducted in Israel showed that prevention is at least four times less expensive than treatment of thalassemia [50]. A study in Iran also found that the cost of treating a thalassemia patient per month is more than four times the cost of preventing the birth of one thalassemia child through screening [51]. Hence prevention is the most effective strategy to reduce the burden of thalassemia in a country like Bangladesh.

Why is it challenging to implement the prevention model of other countries in Bangladesh?

Prevention programs are mainly focused on mandatory premarital screening and prenatal screening, as well as optional abortion which is a sensitive religious issue in conservative Muslim countries. Thalassemia-prone countries such as Cyprus, Greece, and Italy have succeeded in reducing the birth rate of thalassemia children by implementing simple prevention strategies such as premarital blood tests to detect carriers and prenatal fetal testing to prevent the birth of thalassemia-affected children. For example, the successful implementation of the prevention program has been possible in Cyprus due to the small population of the country and the support from the developed countries of Europe. However, studies in Muslim-majority countries such as Saudi Arabia and Iran have shown that despite mandatory premarital blood tests and genetic counselling, many carrier-to-carrier marriages could not be prevented (Table 4).

Table 4 Lessons learned from thalassemia prevention strategies around the world

Among Muslim countries, Iran has been emphasizing the implementation of thalassemia prevention measures for 20 years [52]. As part of the program, Iran has legalized abortion for fetuses with thalassemia and initiated free prenatal blood tests and prenatal screening facilities. Despite such efforts, Iran has not achieved the expected success in eradicating thalassemia. A closer look reveals that there is less real awareness of thalassemia in Iran. The same scenario exists in Saudi Arabia. Even though blood tests before marriage are mandatory, more than half of college students (including married couples) in Saudi Arabia have never heard of thalassemia. Even though the bride and groom were carriers of thalassemia, they continued the marriage in 90% of cases [53]. Since therapeutic abortion is a religiously sensitive and controversial issue, prenatal screening in other Middle Eastern countries, including Saudi Arabia, is not included in the thalassemia prevention strategy.

From the lessons learned from other countries, it appears that thalassemia prevention programs fail to achieve the desired success due to the lack of awareness. Compared to the countries that have implemented thalassemia prevention programs, Bangladesh has a huge population (more than 160 million) and limited healthcare resources i.e. screening and treatment centres. Most importantly, most people in the country are not aware of this disease.

Premarital blood tests and fetal testing facilities are also available in Bangladesh but on a limited scale. Policymakers are giving special emphasis on mandatory blood tests before marriage to prevent thalassemia. However, the Hemoglobin Electrophoresis test before marriage is not mandatory currently in Bangladesh, not even free. Aspirants have to take this test on their own initiative and with their own pocket money, which is not done due to a lack of awareness. Again, if genetic counselling is not given to both spouses in addition to the blood test, it may not prevent marriage between at-risk couples. For those carrier couples who cannot back out of marriage due to mutual emotions or family pressure, prenatal diagnosis can be a good option. According to Islamic fatwa, abortion within a certain period of pregnancy (120 days) is permissible considering the health of the mother and the physical anomalies of the fetus [54]. However, as the issue is sensitive, it needs extensive awareness to establish it in a Muslim-majority country like Bangladesh.

High school-centric intervention should be the cornerstone of the thalassemia prevention strategy

It has been demonstrated by earlier research conducted in other countries that screening and teaching about thalassaemia should be directed at high school students (age 16 and up) to prevent the birth of affected children [55, 56]. According to the Bangladesh Bureau of Statistics, girls are married on average at the age of 19 in rural areas and at the age of 20 in urban areas [8]. Due to the success of various government programs (including stipends) for the expansion of women’s education, more than 80% of females study up to the secondary level [57].

High school student-centred awareness programs, screening, genetic counselling, and district-based medical care facilities should be key to the Thalassemia Prevention Program in Bangladesh (Fig. 2). A long-term action plan based on the country’s established system (in health and education) and untapped resources in the community should be implemented step by step to succeed in the prevention of and clinical management of thalassemia.

Fig. 2

Thalassemia Prevention Action Plan in the Context of Bangladesh

Teachers are highly respected in society and students at the high school level are generally obedient to their teachers. Therefore, teachers can play a key role in creating effective awareness about the dangers and prevention of this disease through textbooks and classroom discussions. The cost of this prevention strategy will be much lower. Media-based awareness alone is less likely to be effective in the context of Bangladesh, as it is a different kind of disease, where social issues are involved.

Various school-based health interventions and projects in Bangladesh have already been quite successful; such as hygiene education [58], menstrual awareness [59], mental health [60], deworming [61] etc. The reasons for these successes are the facility of using the existing institutional system of the school, the special respect of teachers in the society and the importance of their words to the general public and the high enrollment rate of boys and girls at the school level. Such a school-based project on the prevention of thalassemia should be planned.

Incidentally, our study on 1578 college students found that about 67% of students had not heard of thalassemia before although the government’s Directorate General of Health sent text messages to all mobile phone users about thalassemia as part of awareness initiatives around a month before our survey [40]. Of those who heard about thalassemia, 82% were studying science, 16% arts, and 22% commerce. The ninth-grade biology course in the science department sheds some light on thalassemia, which is why they heard the name. Only 33% of secondary school students and 21% of higher secondary students in the country study science, which is mainly city-centric [57]. On the other hand, we already discussed that most people who know the name of the disease have a misconception about thalassemia. The prevention program should be targeted at the large share of the country’s population who live in rural areas (about 68.5% according to the latest census).

Many people, even after learning about the disease, may delay screening for carrier diagnosis. Regular motivation and supervision of class teachers can be effective in implementing awareness in screening. To create long-term awareness, the topic of thalassemia needs to be included in the syllabus of all departments of ninth and tenth classes (science, arts, commerce). The government has formulated ‘National Strategy Paper 2017-30’ for adolescent health and other services. The continuity of this work is important. The issue of thalassemia awareness can be included in this program.

Carriers of thalassemia are not sick; they live a healthy life in most of the cases. However, carriers are also thought to be ill due to misinformation and socially negative perceptions about the disease, which was found in our previous research. Nearly two-thirds (64.5%) of the college students who had heard of thalassaemia were not aware of the fact that thalassaemia carriers are essentially as healthy as non-carriers [40]. Therefore, if genetic counselling is not included in the program after identifying the carriers, adverse situations will be created in the community. School teachers can play a role in genetic counselling under the supervision of experts.

Building treatment centres for patients

In 2018, the Ministry of Health undertook free prenatal testing of thalassemia carrier-risk couples and a national registry of thalassemia patients [62]. The target was set to eliminate thalassemia from Bangladesh by 2028. But there is still no initiative in this regard and there is no national-level data on patient mortality, age-wise distribution and financial burden. BTSH and BTF are two well-known thalassemia treatment centres located in Dhaka, each of them has about 4500 registered patients. But these are only centre-based internal registrations. These must become national registration programs with government support. Registration of all thalassemia patients at the national level will facilitate the participation of thalassemia patients in all fields, including employment. A health card or insurance should be arranged for Thalassemia patients like Pakistan’s “Sehat Card” or Algeria’s “Chifa Card”.

Several discussions are organized on International Thalassemia Day every year in Bangladesh as well, but these discussions are mostly limited to preventive measures. It is time to focus on improving the quality of life of existing patients along with prevention. Patients need to be assured of proper medical care, blood transfusion and the chelation of excess iron deposited in the body should be addressed. Thalassemia patients are also benefiting from medical advances, reducing their mortality and increasing their quality of life. Earlier, Thalassemia patients used to die at a very young age due to lack of adequate treatment. With increased awareness, many patients can now lead fairly normal lives with regular treatment, but they need more support to continue this fight. Thalassemia is still thought by many to be a disease of children. Due to the increase in the average life expectancy of patients, there are many elderly patients now, they should not be forgotten, and their participation in all aspects of society and their normal life should be ensured.

According to the Directorate General of Health Services, the government will soon set up 8 thalassemia treatment centers across the country, two DNA test labs in Dhaka and Chittagong, and provide free blood, transfusion equipment and iron chelation drugs to patients [35]. Speedy implementation of these announcements is needed. Given our economic condition and limited resources, it seems reasonable to build at least one district-based patient support system where trained doctors, healthcare providers, safe blood and adequate medicines at affordable prices should be ensured. National guidelines for the treatment of thalassemia [35] should be ensured to reach the hands of doctors treating thalassemia patients in all parts of the country and training should be provided for them. A national expert committee can be formed to fill the shortage of hematologists at the district level, under whose supervision pediatricians can provide medical services in the districts.

Although a thalassemia carrier screening center could not be set up in every district in the first phase, a pipeline can be set up to allow blood samples to be tested at a National Reference Lab in Dhaka. There is a serious crisis of blood in the country, especially at the community level. Modernization of existing blood banks for blood preservation along with the creation of a community-based blood donor database can be helpful in this regard. Joint efforts of private and public will accelerate the blood collection initiative and also create awareness about thalassemia. Emphasis should be given to developing a step-by-step implementation of thalassemia screening, genetic counselling and treatment centers at district hospitals or medical colleges.

Limitations

This review is limited by the reliance on peer-reviewed and English-language sources, potentially excluding relevant studies in other languages or formats. The data mainly focuses on specific populations, which may not fully reflect the diversity across all regions of Bangladesh. The lack of longitudinal studies on clinical outcomes and prevention program effectiveness restricts long-term impact assessment. There is also insufficient data on healthcare infrastructure, particularly on the distribution of treatment centers and specialized clinics. While social and cultural barriers are acknowledged, the review does not deeply explore region-specific factors or provide a comprehensive sociocultural analysis. Additionally, the focus on prevention strategies leaves less emphasis on the ongoing treatment and management challenges for existing patients, especially in rural areas. Lastly, while international models are referenced, their direct applicability to Bangladesh’s unique context is not fully examined.