Thalassemia is obtained from the Greek word “Thalassa”
meaning “the sea” because the state was first described in populations living
near the Mediterranean Sea. Thalassemia is a genetic disorder of hemoglobin
composite indicated by a depletion in the synthesis of one or more of the
globin’s chains conduct to variation globin- chain synthesis, deformed
hemoglobin made beginning anemia (Victor et al., 1999).
is the central unit of red blood cells that carry oxygen. Its main function is
to supply the oxygen to the blood. When the production of haemoglobin is not
sufficient then oxygen is not supply properly to all the parts of body. The
decrease amount of oxygen makes the body unable to function properly.
We can define thalassemia as a genetic/inherited blood
disorder. That greatly affects our body to produce hemoglobin and red blood
cells. A person who has the disorder of thalassemia have very less quantity of
red blood cells as well as very little amount off hemoglobin. The symptoms of
thalassemia patients appear at the age of 6 months in infant. The symptoms
include chest pain, Leg cramps, Swift heartbeat, late growth etc. This disorder
has a range from mild to severe and it is classified into two major types.The two major types are called Alpha and Beta
thalassemia, depending on which part of globin chain is made in lower amounts
(Victor et al., 1999). In Alpha Thalassemia, alpha globin chain is formed by
four genes (two from each parent), two on each strand of chromosome 16. The
alpha thalassemia was initiated by
reduction in the production of alpha globin’s chains due to deletion or
mutation of one or more of the four alpha globin’s genes located on chromosome
16 (Hillman and Ault, 2002). Alpha and Beta. The Beta Thalassemia also known as
Cooley’s Anemia. Beta symptoms were appeared in the first two years of life,
the symptoms include Paleness of the skin, Poor appetite, Irritability. Alpha
Thalassemia also a severe and a very serious disease in which severe Anemia
begins even from birth.
There are more than 200 of alteration within the beta
globin gene endow worldwide to produce beta thalassemia. Unlike the deletion
that compose most of the alpha thalassemia syndromes, beta thalassemia is
inspired by mutation on chromosome 11 that affect all of beta globin
production: transcription, translation, and the reliability of the beta globin
production (Howard et al.,1996)
is hereditarily, meaning that at least one of your parents must be a transporter
of the disease. If one parent has thalassemia minor and other partner also has
thalassemia minor so in that case the child gets thalassemia major. It was
caused by a genetic transmutation or an omission of certain key gene wreckages.
is a complex group of diseases that are relatively rare in the United States
but common in South and Southeast Asia. Internationally, there are 350,000
births per year with serious hemoglobinopathies (blood disorders).
Quality of life (QOL) is a multidimensional idea
that usually have perspective of positive and negative characteristics of life. The WHO defines quality of life (QOL) as “an individual’s insight of
their situation in life in the framework of the culture and value systems in
which they live and in relation to their goal line, opportunities, morals and apprehensions.
It is a concept that affects person’s physical capability, psychosomatic
well-being, personal beliefs, social relationships and their relationship with
others in their environment.
Assessing quality of life makes it possible
to measure disease load and can be used to help to regulate risk factors that
may lead to poor quality of life among thalassemia patients.