Introduction of Variables
Thalassemia came from a Greek word “Thalassa “the sea”
first described by Mediterranean Sea. Thalassemia is a genetic blood disorder
indicated by a reduction in the blend of one or more of the globin’s chains manipulations
of variation globin- chain synthesis, deformed hemoglobin made beginning anemia
(Victor et al., 1999).
is the central unit of red blood cells that carries oxygen. Its main function
is to supply the oxygen to the blood. The decrease amount of oxygen makes the
body unable to function properly. When the production of haemoglobin is not
sufficient then oxygen is not supply properly to all the parts of body. 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 thalassemia appears at the age of 6 months in newborn. The prominent symptoms
of thalassemia include trunk pain, Leg contractions, Swift heartbeat, late
growth etc. The disorder has a range from minor to severe and it is characterized
into two major types.
The two major types are Alpha and Beta thalassemia,
depending on which part of globin chain is made in lower amounts (Victor et
al., 1999). In Alpha Thalassemia globin chain is formed by four genes, two on
each strand of chromosome 16. (Hillman and Ault, 2002). The Beta Thalassemia is
also known as Cooley’s Anemia. Beta symptoms were give the impression in the
first two years of life, it contains Sallowness of the skin, Poor hunger, Prickliness.
Alpha Thalassemia also a severe and a very serious disease in which severe
Anemia begins even from birth.
There are more than 200 of amendment within the beta
globin gene endow worldwide to produce beta thalassemia. Unlike the deletion
that compose most of the alpha thalassemia conditions, 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. It was caused by a genetic transmutation or an omission of
certain key gene wreckages. If one
parent has thalassemia minor and other partner also has thalassemia minor so in
that case the child gets thalassemia major.
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 is a multidimensional idea that
have perspective of positive and negative characteristics of life.
According to world health organization Quality of life is defined as An
Invidia’s insight about their cultural system and values system in which they
lived and individual’s relation to their morals, opportunities and fears,
worries’ it is a concept that effects individual’s all perspective such as
Physical, emotional, psychological, personal relationships, social and their
relationship to their environment. To
assess quality of life makes it possible to measure disease load and can be
used to help to reduce the risk factors that may leads to poor quality of life
among thalassemia patients.
is long-lasting disease that boons serious medical and psychological problems.
Thalassemia effects on physical health that lead to physical distortion, growth
delay, and delayed sexual maturity. It also effected physical appearance such
as bone malformations and short physique, and poor self-image. Some severe
medical problems also occur due to thalassemia such as heart failure, cardiac
arrhythmia, liver disease, endocrine hitches, and infections these are very
common in thalassemia patients. The problems that were stated do not only
affect patients’ physical functioning but also effects patients demonstrative
functioning, common functioning and conservatory functioning, leading to decreased
health-related quality of life (HRQOL) of the patients. (Acta Haematol.2002).
Thalassemia is a significant health problem, placing an
unlimited emotional, psychological and economic load on millions of people all
over the World (Panos, 2005; Riewpaiboo et al. 2010). Current information assumes
that around 7% of the World’s population was a transporter of a hemoglobin
disorder and that 3,00,000-5,00,000 children are born each year with the serious
homozygous complaint of these diseases. 80% patients of thalassemia major were suffering from
psychiatric disorders such as anxiety and depression. Anxiety and Depression
are the most common disorders among thalassemia patients.
Once study states that thalassemia patient’s quality
of life is lower as compared to older patient. Once study on thalassemia
patients showed that around 10,000 new born are brought each year with severe
form of thalassemia. Mednick and its colleges organization conduct a research
and explains that 32% patients suffer from anxiety and 11% patients from
depression. Unprocessed thalassemia major leads to failure of heart and liver complications
these forms of difficulties effected normal functioning of patient. Thalassemia
patients needs blood transfusion treatment after every 10 to 15 days. Lifelong
blood transfusion leads to iron over load which can be treated or prevent early
death or create a failure to organ. Patients with Thalassemia major need blood transfusion
to improve their quality of life to survive. (Mednick,2012).
One such study often mentioned
overprotection and overdependency responses by parents. The thalassemia
patients are so weak so they are totally dependent on some other person such as
mother, father, siblings and other family member for their work. So, in that
case they want others and its management tend to prolong dependence of the
patient on family care and demonstrative support. Parental anxiety may lead to restraint
activities of the child or teenagers, incompatible with the tendency toward self-sufficiency
(Minerva Pediatr,2002). Most studies inclined
to highlight the psychosocial side of coping with thalassemia, but clearly the disorder
itself and its treatment have a major effect on Quality of Life. A recent study
on adults with thalassaemia suggests that treatment and cultural differences
did not have a major effect on the Quality of life in Cypriot thalassaemia
patients (Telfer P et Collegues,2005).
to Messina and its colleagues, in their study about the young adults of thalassemia
patients initiate that the self-image was characterized by poor feeling and helplessness;
the quality of life presented that the emotional role and social function were significantly
low in all the areas; that thalassemia patients showed a personality categorized
by somatization, depression and obsessive-compulsive traits (Messina et
were very insufficient practical data that describe the psychological
experience of adult patients, up to 50 years. In accumulation, there were few
studies that have included in the evaluation some projective method credibly
due to the difficulty of applying psychological and compound analysis of
personality in these patients.
This study aims to determine
the problem of illness associated with the patients of thalassemia and to gain
knowledge about the aspects associated with quality of life among patients of thalassemia
and it is essential in evolving more suitable clinical, counselling, and social
support programs to improve treatment outcomes of these patients. This study
also aims to examined factors associated with QOL among children and
adolescents with thalassemia in Sialkot. The purpose of this
study was to explore whether patients with thalassemia have specific pattern