Thalassemia Clinic
At
DESUN there is a premier clinic for the treatment of Thalassemia which is an
inherited autosomal recessive blood disease that originated in the Mediterranean
region thousands of years ago during the early phases of human evolution. In
Thalassemia the genetic defect, which could be either be by mutation or by
deletion, results in a reduced rate of synthesis or absolute no synthesis of one
of the globin chains that make up haemoglobin. This can cause the formation of
abnormal haemoglobin molecules causing anaemia the characteristic symptom of
Thalassemia.
Thalassemias are classified according to which chain of the haemoglobin molecule
is affected. In α Thalassemias, production of the α globin chain is affected,
while in β Thalassemia production of the β globin chain is affected.
For the recessive forms of the disease both parents must be carriers in order
for a child to be affected. If both parents carry a hemoglobinopathy trait,
there is a 25% chance with each pregnancy for an affected child. Genetic
counselling and genetic testing is recommended for families that carry a
thalassemia trait. Such testing is available at DESUN for interested parties and
families.
Treatments
for thalassemia depend on the type and severity of the disorder. People who are
carriers or who have alpha or beta thalassemia trait have mild or no symptoms.
They need little or no treatment.
At DESUN Doctors use three standard treatments for moderate and severe forms of
thalassemia. These include blood transfusions, iron chelation (ke-LAY-shun)
therapy, and folic acid supplements.
But normally at DESUN transfusions of red blood cells are the main treatment for
people who have moderate or severe thalassemias. This treatment gives you
healthy red blood cells with normal haemoglobin.
