Written by Dr. Md. Hafizur Rahman
Dhaka, the capital of Bangldesh, being also considered to be the capital of thalassaemia in Bangladesh, sees some 5,000-6,000 children born with the condition every year who need regular blood transfusion for survival. Every year, May 8 is observed as World Thalassaemia Day across the globe. The main goal of the observation of the day is to raise awareness about the disease and other fatal diseases, making the world a better place to live on.
Haemoglobin disorders or haemoglobinopathies are a group of conditions affecting human blood – more specifically an important substance or protein called haemoglobin contained in the red blood cells, hence the name haemoglobin disorders or haemoglobinopathies. Simply, the condition is called thalassaemia which originated from two Greek words (thalassa, the sea + haima, blood).
Haemoglobin disorders, including thalassaemia are an international concern. It is estimated that 7% of the world population are carriers of a severe haemoglobin disorder and 300,000-500,000 children are born with a severe haemoglobin disorder each year. Research advances in the clinical care of thalassaemia have managed to transform thalassaemia from a fatal disease of childhood that it once was into a chronic, yet well-managed disease, increasing patients’ survival rate and improving their quality of life.
Bangladesh, considered to be the mostly densely populated country in the world with about 180 million populations, sees some 5,000-6,000 children born with the condition every year. However, the disease is still overlooked when we talk about the burden of the disease in the country. Among them, 50% die before the age of 20 due to poverty and lack of treatment. Reportedly, about 10-15% population of Bangladesh being carriers and over 70,000 live patients in national pool.
Recently DGHS, GoB has taken several key initiatives with its main stakeholders to create awareness among general population, control programme at the national level and also prevention with adequate and effective treatment those who are suffering with thalassaemia.
International Thalassaemia Day
In time-honoured tradition, the Thalassaemia International Federation (TIF) is organising many diverse activities for the International Thalassaemia Day. Celebrated on the 8th of May every year, these activities aim to sensitise the decision and policy makers and the health and patient committees at large on thalassaemia: How to prevent, treat and cure in a patient-centred manner.
• 2018 – “Thalassaemia past, present and future: Documenting progress and patients’ needs worldwide”
• 2017 – “Get connected: Share knowledge and experience and fight for a better tomorrow in thalassaemia”
• 2016 – “Access to safe and effective drugs in thalassaemia”
• 2015 – “Enhancing partnership towards patient-centred health systems: good health adds life to years!”
• 2014 – “Economic Recession: Observe – Joint Forces – Safeguard Health”
• 2013 – “The right for quality health care of every patient with Thalassaemia: major and beyond”
• 2012 – “Patients Rights Revisited”
• 2011 – “Equal Chance to Life”
For the global thalassaemia family, the 8th of May constitutes a very special day as it is dedicated to both commemorate the thalassaemia patients who are no longer with us but are always close in our heart and to celebrate all those patients who are alive and fighting everyday for their right to a better quality of life.
On this special day, every year, TIF focuses on a different theme that intervenes with the quality and appropriate treatment of patients and impacts their quality of life.
Activities Done on World Thalassemia Day
This deadly disease thalassaemia has the capability to affect people of almost all age groups. This causes children to be at risks of acquiring this disease. World Thalassemia Day aims in reducing the transfer of the genes by conducting pre-marriage tests that stop the genes from spreading. Here are some of the most important activities carried over by this disease.
• Increase awareness about Thalassemia to the general public.
• Preventive measures must be developed which are feasible and can be easily implanted.
• Motivate people to donate blood. The youth are specifically targeted for this and help people realize that donating blood is actually good for their health.
• Help doctors and hospitals with facilities that can improve the treatment methods of thalassemia.
• Pre-marriage tests should be done to combat the spread of the genes to the child.
• World Thalassemia Day aids to reduce the death rate caused by Thalassemia. It also aims to help improve the facilities in more risk areas like Saudi Arabia.
How can you celebrate World Thalassemia Day?
You play an important role making World Thalassemia Day 2017 a success story. There are a lot of activities and seminars organized by the Thalassemia International Federation which is a non-profit organization. There is a list of things that you could do with little or no effort on this day.
• You can make use of the internet and the social media and start sharing and posting your opinion on World Thalassemia Day.
• Educate people around you including your family and friends and help them understand the seriousness of this disorder.
• You can visit a school nearby and help young children get to know about the importance of good health. This small act of kindness is second to none.
• Visit a few thalassaemia patients and help them to improve their quality of life. You can make them understand to get over this disease.
For the global thalassaemia community, May 8th is a very special day as it is dedicated to both commemorate the thalassaemia patients who are no longer with us and to celebrate all those patients who are fighting every day to improve their lives and achieve their dreams. It also provides a focal point around which to build awareness within the community, especially those from higher risk communities, who may be unaware that they carry the altered gene. Our aim was to spread awareness of Thalassaemia & haemoglobinopathies and encourage people to talk to their family doctor about having a simple blood test. We will also hope to meet patients and families and listen to your ideas and feedback.
Thalassemia is a type of Disease by which people celebrate it on the 8th May to provide the people to get the knowledge about the Thalassemia Disease. It is very necessary for the people to do the Thalassemia Disease test to remove the Thalassemia Disease or to get every kind of prevention from many of the treatment. Thalassaemia is used to make the people to suffer from the Red Blood Cells Disorder. It is the Disease which makes the Red Blood Cells to be affected in a bad way. It also affects the haemoglobin in the blood. It is used to celebrate to make a better awareness amongst the people of the Thalassemia Disease.
Government should develop much more effective as well as lot of preventive measures for controlling the Thalassemia Disease. Government should encourage every people to provide the Blood Donations for the Young generations to make the people cure or prevent from the Thalassemia Disease. People should be motivated to do the pre-marriage test for getting diagnose for the Thalassemia Disease and also protect the Child to get inherit from one person to another.
Thalassemia Day is used to make every Society, Country, State as well as Whole World to become free from the Thalassemia Disease and other Diseases which are inheritable. To make the thalassemia patients to get to live normal life like a Healthy person. Government should make much more organizations to encourage and motivate the people to improve the facilities and services of the health care in the areas which are vulnerable. World Thalassemia Day is celebrated to decrease the death rates of the people in the world due to the thalassemia.
Government should be able to improve the people’s Health to make them to live better without the disease of thalassemia to them. World Thalassemia Day is also used to celebrate in many of the colleges, schools, public areas, various institutes of the education etc. There are many events organized by the government where many people like many patients and their family members are used to participate in the thalassemia event to get them provide every information about the thalassemia disease. TIF which is also known as the Thalassemia International Federation which is a type of Non-Governmental Organization as well as Non-Profitable Organization which makes the people to celebrate the Thalassemia Day by organizing it in a better way in every country.
Many organizations such as World Health Organization as well as many other organizations fully concentrates on the basic rights of the Thalassemia Patients to motivate more patients of the thalassaemia. There are many types of activities are done on the Thalassemia Day such as subjects of the health topics by the government to make the people to understand the importance of the prevention from the thalassemia disease. Many people also discuss about the thalassemia disease patients to get them every services and facilities of the health care. There are many types of banners as well as posters are stuck on the walls and many other places to get the people aware about the thalassaemia disease.
Thalassaemia being a genetic disease should be stopped from spreading at any cost. This not only affects you but also affects your child. Make sure that you get to the nearest check-up clinic and get diagnosed for thalassaemia. It is never too late to start getting treated for this disorder. If you begin treatment earlier, the effects can be minimized. Join hands with us and let us march towards a healthier future by reducing and eventually eradicating Thalassaemia.
The Thalassaemia International Federation (TIF) is a non-profit, non-governmental organisation founded in 1986 by a small group of patients and parents representing mainly National Thalassaemia Associations in Cyprus, Greece, UK, USA and Italy; that is, countries who first recognised thalassaemia as an important public health issue and who began to promote and implement the first national programmes for thalassaemia control, including prevention and clinical management. TIF was officially registered under Cyprus Company Law in 1987, and since 1996 it has worked in official relations with the World Health Organisation (WHO).
Through the years, TIF has established collaborations and networks with a number of other official health orientated agencies and associations, and patient orientated organizations at the national, regional and international level. Today, TIF has evolved into an umbrella federation, with 118 member associations, in 57 countries of the world, safeguarding the rights of patients to quality health and other care.
Global Epidemiology of Thalassaemia
• Population: 170-180 million
• Carrier frequency: In Dhaka city, Beta thalassaemia carrier is 4.1%, HbE is 6.1 and thee is higher carrier frequency of HbE in tribal population in hill tract area reaching upto 41.7% and in Dinajpur district, HB E carrier frequency is 16-17%.
• Expected birth of thalassaemia patient each year = 15-16000/year with extreme phenotypic variability from transfusion dependent thalassaemia (TDT) to transfusion independent thalassaemia so called NTDT.
• Number of TDT patient: 2000-3000 (Beta Thalassaemia +E-Beta Thalassaemia) in each year added to national thalassaemia pool.
• Population: 19 million
• Carrier frequency: 2.2%
• Expected birth of thalassaemia patient each year = 62/year
• Number of TDT patient: 2000 (Beta Thalassaemia) + 1000 (E-Beta Thalassaemia)
• About 40% of patients are Hb E/Beta thalassaemia which costs about 5% of annual national health budget.
• Population: 0.4 million
• Carrier frequency: 16-18%
• Expected birth of thalassaemia patient each year = 28
• Number of TDT patient: 2000 (Beta Thalassaemia) + 1000 (E-Beta Thalassaemia)
(If we consider for same carrier frequency, 28X16/0.35 = 12,800 patients will be added in BD every year (new patient))
• Total population = 1300 million
• Number of new patients with thalassaemia = 10,000 in each year
• Overall Carrier frequency = 19%
• Number of new patient each year = 5000 – 6000
• Number of affected patient (Total) = 100,000 with symptomatic disease require regular BT.
• Population: 7.4 million
• Carrier frequency: 4,5% for alpha and 3-6% for beta thalassaemia
• Expected birth of thalassaemia patient each year = 28
• Total Number of TDT patient: 330 (Beta Thalassaemia) + 1000 (E-Beta Thalassaemia)
(If we consider for same carrier frequency in BD, then 7500 patients will be added in every year (new patient) in thalassaemia pool.)
Carrier frequency = 19%
• Population: 70 million
• Carrier frequency: 1/10000 (same is also in other European countries)
• Number of TDT patient: 7000
(If we consider for same carrier frequency, 17000 patients will be added in BD every year (new patient))
Beta thalassaemia & HbE/Beta Thalassaemia
• Carrier frequency: (1.5% of the global population)
• Total population of carrier = 80 to 90 million
• Number of new cases each year (all): 300,000
• Number of diseased patients (BTM) = 68,000 (TDT); symptomatic require regular blood transfusion.
• Total number patient affected with E/Beta thalassaemia 100,000. Among them, 20,000 are present with severe forms of thalassaemia that is designated now TDT and 50% of them are severe form of TDT accounting 30-50% of beta thalassaemia.
• In Bangladesh, the frequency of E/Beta is 5-6 times higher than beta thalassaemia major among them 60-70% are NTDT (non-transfusion dependant thalassaemia).
• 23,000 children are born with transfusion-dependent β-thalassemia major each year in Southeast Asia.
• Low- or middle-income countries of the tropical belt –sub-Saharan Africa, Mediterranean region and the Middle East, to South and Southeast Asia.
• Countries with high frequency of Hb E are: India, Bangladesh, Thailand, Laos, and Cambodia
• Hereditary persistence of fetal hemoglobin (HPFH) mostly found in Greeks and Blacks.
• α-Thalassemia is the most common inherited disorder of haemoglobin.
• Around 5% of the world’s population being carriers
• An approximate of 1,000,000 patients affected with the various α-thalassemia syndromes worldwide.
• Globally, more than 5,000 children are born each year with the severe form of α-thalassemia, α-thalassemia major or hemoglobin Bart’s hydrops fetalis.
• The annual number of births for the NTDT form of α-thalassemia, α-thalassemia intermedia or hemoglobin H disease, is approximately 10,000.