Thalassaemia : Your decision can change the world

Few years back I used to work with a NGO which used to work to prevent Thalassaemia. At that time, I had prepared some write ups to increase awareness against Thalassaemia. I thought to share that with you. The write up is mostly for India and west (data wise) but can be used for anywhere just by updating the data with that particular country. Because except the data all are scientific.

Shocking Facts:

• 8000 new Thalassaemia patients diagnosed in India annually.
• Out of which 2500 to 3000 cases are reported from West Bengal.
• Presently India houses about one lakh Thalassaemia patients where 30,000 are from West Bengal.

Thalassaemia are of two types mainly:

1. alpha Thalassaemia – due to reduced or absence of synthesis of alpha globin chain.

»a gene is located in chromosome no. 16, short arm.

2. beta  Thalassaemia – due to reduced or absence of synthesis of beta globin chain.

»b gene is located in short arm of chromosome number 11.

Highlights Of Thalassaemia:

• Beta Thalassaemia is the most common single gene disorder in our country
• 30 million people carrying the defective gene
• Carrier Frequency 3-17%
• Over 9000 Thalassaemic children born every year
• In Westbengal HbE Thalassaemia is common
• At present the no. of thalassaemic patient is more than 25,000 and carrier 10% population.
• According to W.H.O incidence = 7-8%
• Per 1000 marriage couple 1-2 are Carrier

Prevention Of Thallasseamia:

• The community control of Thalassaemia Syndrome can be achieved by :
• AWARNESS
• SCREENING
• GENETIC COUNSELLING
• PREVENTION OF BIRTH OF THALASSAEMIA MAJOR CHILD.
• The experience of other countries like Cyprus, Greece and Italy have shown that a prevention program is possible and successful control of the disease by education, screening, genetic counseling and prenatal diagnosis.

Reasons for fall in the Thalassaemia major birth-rate in Cyprus: 

• 4% decision to change marriage partner
• 20% reduction in final family size
• 76% selective abortion

TRADITIONALLY THE MANAGEMENT OF THALASSAEMIA INCLUDES:

• Blood Transfusion.
• Chelation.
• Management of Iron overload associated complication.
• Splenectomy.
• HbF manipulation.
• Stem Cell transplant.
• Gene therapy.

HAZARDS OF BLOOD TRANSFUSION:

• Transfusion reaction.
• Transmission of infection.
• Iron overload.

How to prevent:

To prevent the disease you have to know how it spreads. It is a genetic disorder  of blood. There can be two things:

• Carrier of the thalassaemia – this does not mean the person is suffering from it. S/he is perfectly normal.
• Patient of thalassaemia – This person has both the gene.

So, if you can prevent the marriage of two thallassaemia carrier persons then their children can never be patient of it. It will only happen if two carrier gets married then their children can be affected. Following picture clearly explains how thallassaemia carriers can create the patient (2nd step).

So, this is advisable to test your blood before marriage to check whether you are thallassaemia carrier or not. If you are carrier then be cautious to choose your partner. Your decision can make this a thallassaemia free world.