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Thalassemia What is Thalassemia? Haemoglobin
is an essential element that is found in red blood cells. It plays the
vital function of transporting oxygen through the cells to the body. Thalassemia
involves decreased and defective production of haemoglobin or red blood
cells (in which case it takes the form of anaemia). Another form
of Thalassemia includes the destruction of red blood cells; a condition
known as microcytosis. In its mildest form, Thalassemia results
in anaemia, while the severest form of Thalassemia could require a lifetime
of regular blood transfusion.
Thalassemia Minor Many people have the Thalassemia
gene in them, but it is dormant. Such people are said to have Thalassemia
Minor. People with Thalassemia Minor are silent carriers of the Thalassemia
trait. They are not affected in any way by the trait and go on to lead
perfectly normal lives. The trait may be passed from generation to generation,
without carriers being aware that they possess the trait.
Thalassemia Major All Thalassemia Major patients require
frequent blood transfusions. Some patients may require a transfusion every
3 weeks. The patients are infused with fresh blood with a normal haemoglobin
level, so as to carry out the function of providing oxygen to the body.
However, this leads to an excess of iron as the body is unable to break
it down and naturally eliminate it. Thus, the patient would also require
undergoing a more painful method of treatment, which involves attaching
a needle to an area under the skin almost everyday for twelve hours, to
draw out the iron with the help of a drug and a pump.
Psychological Counselling The shock that the family unit receives
as a whole upon hearing that their child suffers from a major disease can
be very traumatic. In addition, a person with Thalassemia requires frequent
medical care and attention as Thalassemia can lead to many secondary disturbances.
Regular check-ups, constant hospitalisation, dealing with possible diabetes,
osteoporosis, jaundice, cardiac problems- are all a part of the illness,
and can lead to extreme feelings of guilt, anxiety and depression for the
parents. Children too, when they enter adolescence, will have to come to
terms with life or death. Thus, psychological counselling is strongly recommended
for the patient and the parents.
Here are some important facts at a glace:
Annual screenings for Hepatitis need
to be undertaken. Children above the age of 10 should start thyroid testing,
diabetes testing (oral glucose tolerance), pubertal development (girls
age 12, boys age 14). At 15 years biannual bone density evaluations begin.
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