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Thalassemia
What is thalassemia?
Thalassemia is a genetic disease caused by transmission of two defective
genes from both parents to the offspring. If both parents carry the gene,
there is 25% possibility of a child born with the severe form of the
disease, where only regular blood transfusion can sustain life.
Whilst, regular transfusion can sustain life, the complications resulting
from prolonged transfusion are not compatible with life beyond the second
decade unless blood is safe and iron overload resulting from transfusion
is taken care of.
Thalassemia in India
In a country like India, where over 40 million people carry the
gene for thalassemia major and over 10,000 children are born each year
with the severe form of thalassemia, 80-90% fail to survive beyond the age
of 10.
Between 2,000 and 6,000 children are born each year in the eastern and
north-eastern part of India where no such treatment is available. The
internationally acclaimed centre in Vellore in southern India has
performed over 200 Bone Marrow Transplants (BMTs) in thalassemia, but only
5% of these patients originated from the eastern and north-eastern region
in India.
Treatment for Thalassemia
Thalassemia can be treated by early diagnosis and prompt intervention in
the form of regular and life-long blood transfusion every 2-3 weeks. The
major problems arising from regular blood transfusion are:
• acquiring serious infections from blood products
• excess of iron from blood which gets deposited in heart and liver
• antibodies produced in response to repeated transfusions resulting in
severe
and life-threatening reactions to blood products.
In western countries, 90% of the children reach adulthood with advanced
supportive therapy. This is in the form of:
• Improved and safe blood products
• Availability of adequate and appropriate blood products
• Administration of medicines to take care of excess iron in the body
using
chelating agents such as desferioxamine (desferral).
In India:
• 50% of children become infected with viruses transmitted through
infected
blood.
• Less than 10% of children can afford sustained treatment with Desferral.
• 80-90% children die by the age of 10 years either due to lack of
treatment or
one of its complications.
Can Thalassemia be cured?
Yes there is a cure for this condition. Guido Lucarelli pioneered the use
of bone marrow transplantation in this condition and documented that if
done early without too much damage caused by iron overload in the liver,
94% of the children can be cured by this procedure. If thalassemia is
treated later, the survival rate drops to 60%.
WHAT DO WE OFFER:
• COMPREHENSIVE WORK-UP ON FIRST VISIT
• PROVIDE A LONG-TERM TREATMENT PLAN
• FOLLOW-UP
• BMT COUNSELLING
• DEDICATED DAY-UNIT FOR BLOOD TRANSFUSION
• WORK-UP FOR PATIENTS WHO HAVE DEVELOPED TRANSFUSION
PROBLEMS
• ONLY CENTRE IN EASTERN INDIA TO PROVIDE LEUCOCYTE-DEPLETED
BLOOD TO REDUCE ALLO-IMMUNISATION.
• OFFER BMT TO SUITABLE CANDIDATES FROM SEPTEMBER 2009
• THALASSEMIA SCREENING FOR FAMILY AND CHILDREN
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