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17 June, 2019 00:00 00 AM
BLOOD DISEASE

Fighting against thalassemia: an ancestral curse

QUAZI ABRAR HASAN AND MD. SAJIB HOSSIAN
Fighting against thalassemia:  an ancestral curse

Globally, thalassemia is a major health problem. Every year on the 8th of May, World Thalassemia Day is celebrated with so as to increase awareness about the diseases among the general people across the world.

This year’s (2019) theme is “Universal access to quality thalassemia healthcare services: Building bridges with and for patients.”

As it is a hereditary blood disorder as the diseases are passed from parents to children through gene means if one’s family has a history of thalassemia, he or she is most likely to have an increased risk of experiencing the disease. In a similar fashion, according to health line (is a privately owned provider of health information headquartered in San Francisco, California), thalassemia is inherited, meaning that at least one of someone’s parents must be a carrier of the disease caused by either a genetic mutation or a deletion of certain key gene fragments.

The disease is one of the most dangerous and painful diseases in the world, requiring a blood transfusion after 20-25 days (India today, May 8, 2018).  Thalassemia, being an inherited blood disorder, leads the body to have an abnormal form of hemoglobin (a protein molecule in red blood cells, carrying oxygen) and the disorder results in excessive destruction of red blood cells, which leads to anemia that causes someone’s body not to have enough normal, healthy red blood cells(healthline, n.d).

Generally, in the early in life, people find themselves with   Thalassemia (whatever it is moderate or severe level) based on their having symptoms of anemia. The disease affects approximately 4.4 of every 10,000 live births across the globe, causing males and females to inherit relevant gene mutations equally as it follows an autosomal pattern of inheritance with no preference for gender (Smith, n.d.)

Thalassemia was first recognized By Cooley and Lee in 1925 as a form of severe anemia associated with splenomegaly and bone changes in children and the term thalassemia is derived from the Greek (the sea) since many of the early cases came from the Mediterranean region(SL Thein, 2011).

But this disease is not confined to the countries around the Mediterranean rather it is being widespread in the tropical and subtropical regions like the Middle East, parts of Africa, the Indian subcontinent and Southeast Asia.

It affects as many as 1 in 30 pregnancies in China, India, and Pakistan, 1 in 100 pregnancies in Thailand, and 1 in 1000 pregnancies in Northern Europe and the United States (Cole and peter, 2016).

Thalassemia may be moderate to severe level. In this case, according to Mayo Clinic(a US-based nonprofit organization committed to clinical practice, education and research), there are several types of Thalassemia that someone may experiences, depending on the number of gene mutations that he or she inherited from his/her parents coupled by which part of the hemoglobin molecule is affected by the mutations. The more mutated genes mean the more severe thalassemia (Mayo Clinic, n.d.). However, there are mainly two types of Thalassemia: alpha and Beta.

Christian Nordqvist (2018) clarified these two main types of thalassemia (alpha and beta) which are: 1) Alpha thalassemia-In alpha thalassemia, the hemoglobin does not produce enough alpha protein.  Four genes are needed in making alpha -globin protein chains, two on each chromosom16. And we get two from each parent and if one or more of these genes is missing, alpha thalassemia will result.

The severity of thalassemia depends on how many genes are faulty, or mutated (changed). one faulty gene: The patient has no symptoms. A healthy person who has a child with symptoms of thalassemia is a carrier and this type is known as alpha thalassemia minima; two faulty genes: The patient has mild anemia which is known as alpha thalassemia minor; three faulty genes: The patient has hemoglobin H disease, a type of chronic anemia, requiring regular blood transfusions throughout their life; four faulty genes: Alpha thalassemia major is the most severe form of alpha thalassemia.

It is known to cause hydrops fetalis, a serious condition in which fluid accumulates in parts of the fetus' body. A fetus with four mutated genes cannot produce normal hemoglobin and is unlikely to survive, even with blood transfusions. Alpha thalassemia is common in southern China, Southeast Asia, India, the Middle East, and Africa. 2) Beta Thalassemia, in Beta Thalassemia, someone needs two globin genes to make beta-globin chains, one from each parent.

If one or both genes are faulty, beta thalassemia will occur and the severity of beta thalassemia depends on how many genes are mutated. one faulty gene: this is called beta thalassemia minor; two faulty genes:

There may be moderate or severe symptoms and this known as thalassemia major or Cooley’s anemia. It used to be called Colley's anemia. Beta thalassemia is more common among people of Mediterranean ancestry. Prevalence is higher in North Africa, West Asia, and the Maldive Islands.

Through the symptoms of thalassemia can vary depending on the types of it. The most commons symptoms include bone deformities, especially in the face; dark urine; delayed growth and development; excessive tiredness and fatigue; yellow or pale skin; and so on (helathline, symptoms of thalassemia).

Like other parts of the global, thalassemia is the most common inherited diseases that are prevalent in children, causing severe anemia problem in Bangladesh.  According to the Bangladesh Thalassemia Foundation (a non-profit organization working on prevention and treatment of thalassemia since 2002), patients need 1-2 bags of blood every month to survive and 7 per cent of the population of the country is thalassemia carriers with every year 700 new babies are born with thalassemia.  

A recent study (on March, 2018) in this regard conducted by a team led Dr. Mohammed Sorowar Hossain, a Molecular biologist, Biomedical Research Foundation (BFT), Head of Environmental Management, Independent University of Bangladesh that took place in multiple schools and colleges in Jamalpur district of Bangladesh and it is horribly found that 10 percent of the population is a carrier of Thalassemia.  

This was the case probably due to the lack of awareness of the diseases among the mass people of the study area  given that Jamalpur  is one of the most poverty-stricken districts in Bangladesh with Poverty headcount rate (HCR) > 60 (Updated poverty maps of Bangladesh) coupled with low literacy rate (only 47 percent) (population housing census, 2011).

Thalassemia tends to pass from parents to children and is costly and chronic diseases.  The most common treatment for thalassemia is regular blood transfusions that continue for life.   One the other hand, the only cure so far available for the disease is a bone marrow transplant which is expensive that most family cannot afford and also requires a matched sibling donor that is tough to find and is available in only 1-2% families (Bangladesh thalassemia foundation).

 However, this is a  good news regarding the cure  for the disease as Dr. Rabi Hanna, M.D., a specialist in pediatric hematology, oncology, and blood and bone marrow transplantation at Cleveland Clinic in the United States, says a series of recent breakthroughs in gene therapy means a cure for thalassemia is now almost within reach:  gene therapy is presenting several new possibilities, treating and even curing the disease by altering the pieces of genetic code that cause it ( source: open access government, May 8, 2018.  Is 2018 the year we will see a cure for Thalassemia?)

Considering the poor socio-economic landscape of the country, a major section of the people in the society can afford the financial burden of this expensive disease due to high-cost and a lack of readily available medicine including deferasirox, deferiprone, Desferrioxamine , Ferriprox, etc.  On the other hand, inadequate supply of blood for transfusion purposive is one of the major constraints in dealing with the diseases.  

And pathetically, it is seen that it is the parents who suffer the most along with the child with thalassemia. When a father runs for a bag of blood under the scorching sun and a mother wails as she holds her child and watches him die, it is a vicarious feeling and difficult to empathize.

In order to gain the upper hand in the diseases what is the most needed initiative is building up awareness about the dire consequences and promoting the practice of premarital screening regarding this.

The pre-marriage genetic screening will identify both male and female whether they are carriers of the disease or not through an electrophoresis screening and which in turn, will assure their safer future before getting married.  

Otherwise, if the numbers of carriers are still unidentified, there are higher possibilities of two carriers are getting married and conceiving a thalassemia child and which ultimately would lead to a generation with a good number of thalassemia patients. In this aspect, pre-marital screening to detect the carriers of the diseases should be made like several other rituals of marriage.  

However, if there is no awareness or any sort of prevention for the carriers from getting married and conceiving with a child, the country might be dealing with signification portion of the upcoming generation with thalassemia diseases.

It is worth mentioning that few students of Independent University of Bangladesh, Department of Environmental Science and Management (SESM) took part in this revolutionary movement of Thalassemia Awareness Campaign inter-university.  And as part of awareness, they have all gone through an electrophoresis screening test to know if they are carriers or not.

In Bangladesh, there are two specialized hospital dealing with thalassemia in Bangladesh thalassemia foundation and Bangladesh thalassemia hospital and apart from these two, there are thalassemia Centre in   some public and private hospitals in the country.  

Lastly, as a part of ensuring affordable cost of dealing with thalassemia diseases and universal access to quality health services and thereby stopping the spread of this disease and having a healthy generation that has been envisioned in the country’s Agenda 2030:  Sustainable Development Goals (GOAL 3: Good Health and Well-being), the government should strengthen its efforts by allocating funds in ensuring sufficient medicine and blood, testing lab and technology  in the country’s existing public medical hospitals dealing with thalassemia disease, whereas initiatives should be taken ,on an  urgent basis, in installing bone marrow transplantation center among these hospitals where it has not been installed yet.

Besides the government efforts, educational institutions, donor agencies, and social media should come forward to fight against this disease as a part of their respective responsibilities towards the society and humanity in the following ways:

 Teachers and Students from Life science department of the different Universities and also  teachers and students from   different  medical colleges and universities  in Bangladesh should engage in Awareness Campaign against Thalassemia disease following the model of Department of Environmental Science and Management (SESM), Independent University of Bangladesh;

International Donor agencies should cooperate government with  grant in aid whilst taking an  initiative to educate the mass people  that is the considered most necessary instruments in order to prevent the disease;

Different social media should be active to build awareness among the mass people regarding the disease.

Quazi Abrar Hasan, an undergrad student of Environmental Science, Independent University, Bangladesh   and Md. Sajib Hossain, an ex-graduate student (Environmental Economics) of Dhaka School of Economics, Bangladesh.    To reach at:   sajibeconbd@gmail.com; abrar.hasan94@gmail.com

 

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Editor : M. Shamsur Rahman

Published by the Editor on behalf of Independent Publications Limited at Media Printers, 446/H, Tejgaon I/A, Dhaka-1215.
Editorial, News & Commercial Offices : Beximco Media Complex, 149-150 Tejgaon I/A, Dhaka-1208, Bangladesh. GPO Box No. 934, Dhaka-1000.

Editor : M. Shamsur Rahman
Published by the Editor on behalf of Independent Publications Limited at Media Printers, 446/H, Tejgaon I/A, Dhaka-1215.
Editorial, News & Commercial Offices : Beximco Media Complex, 149-150 Tejgaon I/A, Dhaka-1208, Bangladesh. GPO Box No. 934, Dhaka-1000.

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