Thalassemia

The silent killer of Bangladesh's children

 National Professor Dr MR Khan, Dr Shakil Ahmed

There has been a marked improvement in the health of children and status of child deaths in the last 25 years in Bangladesh. Overall average life expectancy has increased from 40 in 1970 to 60 now. To reduce the rate of child deaths the progarmmes undertaken were vaccination, diarrhoea prevention, vitamin A distribution for prevention of child blindness, prevention of respiratory tract infections and pneumonia, use of oral saline, and provision of pure water. Despite deterioration of other social and political aspects on a national scale, we have made marked improvement in the health sector. It may be surprising to learn that UNICEF State of the World Children -2003 report states that as regards the death rate of children under 5 in Bangladesh, there are 57 other countries which are behind Bangladesh including India, Pakistan, Nepal, Bhutan and Myanmar. Maldives and Bangladesh are at the same level, Sri Lanka is in a much better situation.
Statistics show that in 1976-7, 30 per cent of child deaths were from Tetanus. At present this is less than 6 per cent. For children under one the death rate was 11 per thousand in 1987-88, and in 2001 this is 51.
Common diseases in children :
1.Pneumonia 2. Diarrhoea 3. Malnutrition 4. Underweight cildren at birth. 5 Meningitis. 5.Infectious diseases -Measles, TB, Malaria.
Child deaths due to diarrhoea is far less now. But Pneumonia is still a major cause. Statistics of Dhaka Medical College Hospital's paediatrics department show that deaths from Diarrhoea are 0. 45 of total admissions whereas deaths from pneumonia is 1.66.
Owing to various medical management measures and government and non-government programmes, child death rate is falling, but some diseases are now at the top of the list. Anaemia or lack of haemoglobin is now one of the major child diseases. In our country the main cause of anemia is due to iron deficiency. Treatment for such anaemic condition is easily available, easy to manage and effective.
Another anaemic health condition is silently spreading in our country. This is Thalassaemia. Many of us are not fully aware of this disease. But those families in the grip of this disease have realised its terrible effects. This disease is caused by haemoglobin deficiencies in the white blood cell and is almost incurable. This deficiency is genetic which means hereditary.
How does the disease develop?
If the gene that creates haemoglobin is defective, then majority of the haemoglobin in the blood will be deficient and only a small portion will be deficiency-free. As a result the white blood cell with deficient haemoglobin break up before their normal life-cycle. This results in aneamia and expulsion of iron from the white blood cells. When this iron accumulates within the body, functions of various organs are adversely affected. These organs are the heart, liver, pancreas, brain, lungs etc. As a result, these organs malfunction leading to physical infirmities and adverse reactions.
On the other hand anaemic child becomes weak, physical development is retarded, puberty is delayed, studies and playing of games are affected. In sum the child cannot develop into a healthy and active adult.
To counter anaemia, the body tries to produce extra blood cells, and as a result, the face and skull bones have increased bone marrow and the child looks physically deformed.
This is called Mongoloid -Faceis in medical terminology.
Course of medical management
Blood Transfusion
In our country, to treat this disease regular blood transfusion is undertaken, Blood is essential to life, but at the same time excess iron is being deposited in the body. In many children, blood transfusion has to be undertaken once month. Nearly 40 per cent of blood in our blood-banks are used for Thalassemic patients.
Removal of excess iron
We have now realised the poisonous effects of excess iron in the body, and this poison be extracted for relief of the organs. Extraction of iron is medically complex, painful and expensive. For this 5-6 days a week an injection (Desferroxamine) has to be taken in the skin of the belly.
For the injection, a pump has to be operated throughout the night. This pump costs 12 to 18,000 takas. The injection is also costly. There is a medicine that can be taken orally but that is not totally effective.
Bone marrow  transplantation (BMT)
This is the definitive treatment for Thalassaemia. This can completely cure the disease. This treatment is not available in Bangladesh. This costs Tk 8 to 10 lakhs abroad, and after BMT 8 to 10,000 takas of medicines have to be taken
Who can develop this disease?
This is a disease carried by the genes and comes from genes of both parents Genes from one parent will not transmit this disease. But in that case, the child is a carrier.
And if in adulthood he or she marries another carrier, there is 50 percent chance that their children will have this disease.
How many carriers are there in Bangladesh?
WHO research has shown that seven per cent of the population are carriers. For a population of 14 croes, one crore are carriers. This is a terrifying figure to contemplate.
Types of Thalassaemia.
Thalassaemia major-regular blood transfusion needed.
Thalassaemia Intermediate - Blood transfusion needed periodically.
Thalssaemia Minor - Blood transfusion not generally needed, but there is less haemoglobin in the blood of such patients. Lifestyle is normal.
What is the danger of this disease in Bangladesh?
There is no national-level statistics generally available. One research study shows that 2400 children are born every year with Thalassaemia Major which is the most dangerous form of the disease.  
The average life expectancy in the West is 25-30 years, but in Bangladesh it is 10-15 years. At the moment there are 40,000 patients with Thlassaemia Major.
These children and their parents know the fate and patients spend every day in physical pain and mental agony. Parents and guardians have to rush to hospitals and clinics every month for blood transfusion.
Besides cost of the blood, other costs include fees for cross-matching, blood -screening, cost of infusion set. Other costs include regular blood tests, price of Desferroxamine, which are huge. Guardians have leave from work and suffer mental tension during the transfusion. This is almost impossible burden for middle class or low-income families.
What should be done
Two aspects of the problem:
What the patients should do
Prevention of the disease.
For the patients :
Encourage blood donation
Import of duty-free blood containers, transfusion set etc. and manufacture of these items in Bangladesh.
The wealthy should come forward to help the less -privileged
To make provision for BMT within the country
Disease prevention
Once Cyprus had many Thlassaemia patients. This disease was prevalent in other Mediterranean countries also. Those countries are now Thalassaemia -free.
How?
Two carriers of the disease should not marry.
 This will prevent transmission of the disease. Carrier -detection tests should be made generally available. Public awareness about this must be built up through advocacy.
Affected families should get all their children examined, and also before marriage, the prospective couples should take the requisite blood tests.
Both government and non-government efforts are urgently required now for prevention and containment of this dread disease. Let us all pledge to eliminate this silent killer of children through our combined efforts and undertake an appropriate programme for our future generations.
(This is a reprint article.)

Thalassemia

The silent killer of Bangladeshs children

National Professor Dr MR Khan, Dr Shakil Ahmed There has been a marked improvement in the health of children and status of child deaths in the last 25 years in Bangladesh. Overall average life expectancy has increased from 40 in 1970 to 60 now. To reduce the rate of child deaths the progarmmes undertaken were vaccination, diarrhoea prevention, vitamin A distribution for prevention of child blindness, prevention of respiratory tract infections and pneumonia, use of oral saline, and provision of pure water. Despite deterioration of other social and political aspects on a national scale, we have made marked improvement in the health sector. It may be surprising to learn that UNICEF State of the World Children -2003 report states that as regards the death rate of children under 5 in Bangladesh, there are 57 other countries which are behind Bangladesh including India, Pakistan, Nepal, Bhutan and Myanmar. Maldives and Bangladesh are at the same level, Sri Lanka is in a much better situation. Statistics show that in 1976-7, 30 per cent of child deaths were from Tetanus. At present this is less than 6 per cent. For children under one the death rate was 11 per thousand in 1987-88, and in 2001 this is 51. Common diseases in children : 1.Pneumonia 2. Diarrhoea 3. Malnutrition 4. Underweight cildren at birth. 5 Meningitis. 5.Infectious diseases -Measles, TB, Malaria. Child deaths due to diarrhoea is far less now. But Pneumonia is still a major cause. Statistics of Dhaka Medical College Hospitals paediatrics department show that deaths from Diarrhoea are 0. 45 of total admissions whereas deaths from pneumonia is 1.66. Owing to various medical management measures and government and non-government programmes, child death rate is falling, but some diseases are now at the top of the list. Anaemia or lack of haemoglobin is now one of the major child diseases. In our country the main cause of anemia is due to iron deficiency. Treatment for such anaemic condition is easily available, easy to manage and effective. Another anaemic health condition is silently spreading in our country. This is Thalassaemia. Many of us are not fully aware of this disease. But those families in the grip of this disease have realised its terrible effects. This disease is caused by haemoglobin deficiencies in the white blood cell and is almost incurable. This deficiency is genetic which means hereditary. How does the disease develop? If the gene that creates haemoglobin is defective, then majority of the haemoglobin in the blood will be deficient and only a small portion will be deficiency-free. As a result the white blood cell with deficient haemoglobin break up before their normal life-cycle. This results in aneamia and expulsion of iron from the white blood cells. When this iron accumulates within the body, functions of various organs are adversely affected. These organs are the heart, liver, pancreas, brain, lungs etc. As a result, these organs malfunction leading to physical infirmities and adverse reactions. On the other hand anaemic child becomes weak, physical development is retarded, puberty is delayed, studies and playing of games are affected. In sum the child cannot develop into a healthy and active adult. To counter anaemia, the body tries to produce extra blood cells, and as a result, the face and skull bones have increased bone marrow and the child looks physically deformed. This is called Mongoloid -Faceis in medical terminology. Course of medical management Blood Transfusion In our country, to treat this disease regular blood transfusion is undertaken, Blood is essential to life, but at the same time excess iron is being deposited in the body. In many children, blood transfusion has to be undertaken once month. Nearly 40 per cent of blood in our blood-banks are used for Thalassemic patients. Removal of excess iron We have now realised the poisonous effects of excess iron in the body, and this poison be extracted for relief of the organs. Extraction of iron is medically complex, painful and expensive. For this 5-6 days a week an injection (Desferroxamine) has to be taken in the skin of the belly. For the injection, a pump has to be operated throughout the night. This pump costs 12 to 18,000 takas. The injection is also costly. There is a medicine that can be taken orally but that is not totally effective. Bone marrow transplantation (BMT) This is the definitive treatment for Thalassaemia. This can completely cure the disease. This treatment is not available in Bangladesh. This costs Tk 8 to 10 lakhs abroad, and after BMT 8 to 10,000 takas of medicines have to be taken Who can develop this disease? This is a disease carried by the genes and comes from genes of both parents Genes from one parent will not transmit this disease. But in that case, the child is a carrier. And if in adulthood he or she marries another carrier, there is 50 percent chance that their children will have this disease. How many carriers are there in Bangladesh? WHO research has shown that seven per cent of the population are carriers. For a population of 14 croes, one crore are carriers. This is a terrifying figure to contemplate. Types of Thalassaemia. Thalassaemia major-regular blood transfusion needed. Thalassaemia Intermediate - Blood transfusion needed periodically. Thalssaemia Minor - Blood transfusion not generally needed, but there is less haemoglobin in the blood of such patients. Lifestyle is normal. What is the danger of this disease in Bangladesh? There is no national-level statistics generally available. One research study shows that 2400 children are born every year with Thalassaemia Major which is the most dangerous form of the disease. The average life expectancy in the West is 25-30 years, but in Bangladesh it is 10-15 years. At the moment there are 40,000 patients with Thlassaemia Major. These children and their parents know the fate and patients spend every day in physical pain and mental agony. Parents and guardians have to rush to hospitals and clinics every month for blood transfusion. Besides cost of the blood, other costs include fees for cross-matching, blood -screening, cost of infusion set. Other costs include regular blood tests, price of Desferroxamine, which are huge. Guardians have leave from work and suffer mental tension during the transfusion. This is almost impossible burden for middle class or low-income families. What should be done Two aspects of the problem: What the patients should do Prevention of the disease. For the patients : Encourage blood donation Import of duty-free blood containers, transfusion set etc. and manufacture of these items in Bangladesh. The wealthy should come forward to help the less -privileged To make provision for BMT within the country Disease prevention Once Cyprus had many Thlassaemia patients. This disease was prevalent in other Mediterranean countries also. Those countries are now Thalassaemia -free. How? Two carriers of the disease should not marry. This will prevent transmission of the disease. Carrier -detection tests should be made generally available. Public awareness about this must be built up through advocacy. Affected families should get all their children examined, and also before marriage, the prospective couples should take the requisite blood tests. Both government and non-government efforts are urgently required now for prevention and containment of this dread disease. Let us all pledge to eliminate this silent killer of children through our combined efforts and undertake an appropriate programme for our future generations. (This is a reprint article.)