The Effects of Patients' and Care-Givers' Knowledge, Attitude, & Practice (KAP) on Quality of Life Among Thalassemia Major Patients' in Damascus-Syrian Arab Republic
European Scientific Journal April 2018 edition Vol.14
The Effects of Patients' and Care-Givers' Knowledge, Attitude, & Practice (KAP) on Quality of Life Among Thalassemia Major Patients' in Damascus-Syrian Arab Republic
Magda El-hadi Ahmad Yousif 0 1
0 Professor, Chairperson of Primary Health Care and Health Education Center, Faculty of Medicine, University of Gezira - Sudan Moauya Al-Balal Al-Haboub, PhD Professor of Faculty of Medicine, University of Gezira - Sudan
1 Yaser Adnan Abo Jeesh, PhD Candidate Faculty of Medicine, University of Gezira - Sudan
Background: Thalassemia is the most common autosomal abnormality in Syria. Its complications have an important effect on education; time off school; sport; difference from friends/ siblings; social interactions; and stigmatization. Knowledge of factors associated with quality of life in thalassemia patients is necessary for forming appropriate clinical programs, social support, and improving treatment outcomes. Purpose: The study was to assess the effects of Patients' and Care-givers' Knowledge, Attitude, & Practice (KAP) with Quality of Life among Thalassemia Major Patients' in Syria. Methods: Cross-sectional descriptive analytical design was conducted at the national thalassemia center in Damascus. WHOQOL-BREF and a questionnaire developed by the researcher were used to measure the participants' knowledge, attitudes and practices of thalassemia. Results: Total of 238 thalassemia patients participated in the study. A statistical significance was found regarding improvement of skill and knowledge scores among caregivers and thalassemia patients before and after receiving the teaching guide from 12.52±1.77 to 14.07±1.01, t=11.447, p=0.000 and from 34.12±4.50 to 37.43±4.61, t=-8.58, p= .000 respectively. Stigmatization was significantly noticed among families caring for thalassemic patients. There were a significant differences in the mean score regarding nutritional status before and after teaching guide paired t test= 12.11, p= 0.000. A statistical significance was found in females regarding social domain p=0.04. However, mean scores for overall quality of life were better in females rather than males,
but these mean scores were statistically insignificant p>0.05. A statistical
significant difference in ferritin levels and patient’s age was found. Results
also revealed that no statistical significant differences was observed between
overall quality of life of the four domains in relation to the two treatment
groups (subcutaneous vs oral) and age groups. Conclusion: Thalassemia is a
socio-economic problem. The most efficient way to reduce risks of having
affected patients is by increasing the knowledge through training of parents
regarding the disease. Our findings highlighted that there was lack in patients’
knowledge and skills regarding the disease, but a remarkable improvement in
both knowledge and performance was found after patients received the
teaching guide, which lead to an increase in overall patient's quality of life.
The term Thalassemia was first used by Wipple and Bradford in 1932,
derived from Greek word “thlassa” meaning sea and “haemia” meaning
(Nagaraj, Umashree et al. 2011)
. Thalassemia is one of the most
common genetic blood disorders in the world, the most severe form is
betathalassemia major, which warrants regular blood transfusions at an early age,
if it not treated, the result can be early death
(El Dakhakhny, Hesham et al.
2011, Safizadeh and Farahmandinia 2012, Elalfy, Farid et al. 2014)
. As other
countries of the Mediterranean region , thalassemia is the most common
autosomal disorders of the Syrian people, the number of the total registered
thalassemia patients was 8300 in year 2013 and increasing by almost 800 each
year, the average carrier rate is 5–7% of the total population
Touma, AlKeba et al. 2013)
. Regular blood transfusions and iron chelation
therapy (ICT) have significantly improved survival, reduced morbidity and
enhanced health related quality of life (HRQoL) of patients with
betathalassemia major (BTM)
(Touma, AlKeba et al. 2013, Hatami and Motamed
There has been very limited researches about the effects of thalassemia
on QoL especially for pediatric patients
(Hatami and Motamed 2014)
studies have been done to measure or to assess awareness of the Syrian people
or families with thalassemic patients regarding knowledge, attitude and
practices related to quality of life for these patients in Syria. As such more
attention is needed in issues related to knowledge and understanding of the
illness, lifelong compliance and adherence to chronic treatment regime and
attitudes of patients and family members as well as quality of interpersonal
(Jantan, Muhammad et al. 2011)
The most effective way to decrease complications of thalassemia
patients is by increasing knowledge, attitude and practice awareness of these
patients and their parents about the disease and its management, which will
result in a positive impact in their quality of life. This will improve their
performance to deal with their lifelong disease and its challenges during the
period of management (Kourorian Z et al 2014
(Dehkordi and Heydarnejad
. The obvious role of parents’ education in the prevention of
betathalassemia is evident in the study of Dehkordi and Heydarnejad 2008 which
indicated that population with poor pre marriage knowledge of the couples
about the disease resulted in 62.5% of the affected children. This problem
could have been reduced by improvement of the parents’ health education
(Dehkordi and Heydarnejad 2008)
In Syria, only one thalassemia organization is found in the capital
Damascus that provides the social support and information for the patients and
their family. Currently the Syrian Ministry of Health (SMOH) has started with
a training program for health personnel to provide good quality care and
counselling for the patients and families
(Touma, AlKeba et al. 2013)
Unfortunately, till now there are no professional psychologists who are
working in institutions caring for thalassemia to support and follow up the
Patients with β-thalassemia major require iron-chelation therapy to
avoid the complications of iron overload such as severe anemia with an
enlarged spleen, pallor, irritability, growth retardation, delayed puberty,
abdominal swelling, and jaundice
(Muncie Jr and Campbell 2009, Milat,
Wong et al. 2012)
. Other severe complications such as heart failure, cardiac
arrhythmia, liver disease, endocrine complications and infections are common
among patients with β-thalassemia and may impact negatively on patients’
quality of life (QOL)
(Borgna-Pignatti and Gamberini 2011, Liem, Gilgour et
al. 2011, Pennell, Carpenter et al. 2011, Ali, Tarawah et al. 2015)
Effective treatment of patients with β-thalassemia major consists of
regular blood transfusions, intensive administration of iron-chelating agents
such as deferoxamine (DFO) by subcutaneous infusions, or deferiprone (DFP)
and deferasirox (DFX) that are taken orally, Spleenectomy and Bone marrow
(Schrier and Angelucci 2005, Cohen 2006, Capellini, Cohen et
al. 2008, Cianciulli 2008, Vichinsky, Levine et al. 2008, Murad, Moassas et al.
. Deferoxamine (Desferal), subcutaneously or intravenously, has been
the treatment of choice
(Muncie Jr and Campbell 2009)
. Chelation therapy has
two main forms: subcutaneous slow infusion by a portable pump
(deferoxamine) and oral (deferasirox, deferiprone), whereas most patients in
Syria use classical chelation therapy using desferrioxamine and the infusion
(Al-Zir 2009, Trachtenberg, Mednick et al. 2012)
. The cost of treatment
per β-thalassemia patient is up to $1500.00 per year
(Murad, Moassas et al.
Serum ferritin is increasing as an indicative of compliance with
(Milat, Wong et al. 2012)
. Serum ferritin is the best test
to screen for iron deficiency anemia
(Muncie Jr and Campbell 2009)
Consequently, compliance with regular administration of chelation agents is a
key feature to the long-term health of thalassaemic patients
(Atkin and Ahmad
:whereas, the serum ferritin level that identifies safe levels of body iron
with respect to the development of complications of iron overload is < 2500
(Olivieri, Nathan et al. 1994, Muncie Jr and Campbell 2009)
. In a study
(Pakbaz, Fischer et al. 2005)
to investigate the factors that can
improve adherence to iron chelation therapy with DFO in patients with
thalassemia. Ninety patients with thalassemia major were assessed for
adherence to chelation therapy by using Liver Iron Concentration (LIC) and
Serum Ferritin (SF). Results showed that patient education and counseling
about the benefits of chelation therapy can play an important role in improving
adherence. Frequent measurement of liver iron concentration can be valuable
in modifying patients' belief about the medication because it can give evidence
for the effectiveness of DFO in reducing LIC and therefore lead to a better
(Pakbaz, Fischer et al. 2005)
The magnitude of thalassemia is masked by infections and nutritional
(Nagaraj, Umashree et al. 2011)
. Therefore, the thalassemia
patients should avoid food rich in iron and drink a cup of tea with every meal
because it interferes with iron absorption in the diet patient should also avoid
food rich of vitamin- C during meals
(Wonke 2001, Caro, Ward et al. 2002,
Capellini, Cohen et al. 2008)
The Quality of Life (QoL) of individuals with thalassaemia major is
influenced by the following factors: impact of the diagnosis and treatment on
family stability and family dynamics, having a chronic condition manifisting
by bone deformities, short stature, leading to poor self-image; treatment
measures like frequent hospital visits for transfusion; nightly subcutaneous
infusions; delayed or absent sexual development and impaired fertility;
complications of the disease and therapeutic interventions such as heart
disease, bone disease, diabetes, infections; and uncertainties about the future,
difficulties in long-term planning
(Telfer, Constantinidou et al. 2005,
Shaligram, Girimaji et al. 2007, Jafari, Lahsaeizadeh et al. 2008, Scalone,
Mantovani et al. 2008)
. Hence, the impact of iron chelation therapy (ICT) on
Quality of Life (QoL) will not be noticed until major complications begin to
occur in the teens or early twenties for patients with thalassaemia
Baladi et al. 2006, Ansari, Baghersalimi et al. 2014)
. The aim of this study was
to assess the effects of patients' and care-givers' knowledge, attitude, &
practice (KAP) on quality of life among β-thalassemia major patients in Syria,
and according to the research results, we are looking to develop a teaching
guide based on the previous assessment of home caregivers and patients
regarding to their needs.
Methods and design
Sample and setting
A cross-sectional descriptive analytical study was performed from 20
September 2016 to 22 December 2016, a total of 238 thalassemia patients and
their caregivers who visited the national thalassemia center in Damascus were
included in this study. The center was established in 1997 with over 1200
thalassemia patients. The diagnosis of thalassemia major is confirmed using
compete blood count and hemoglobin electrophoresis in the center. The center
provides blood transfusions, desferrioxamine and social support
Amarneh et al. 2009)
. This site was selected for the purpose of this study,
because it is considered the main center that provides a holistic care for
thalassemia patients' children/ adolescents or adults in Syria.
The inclusion criteria were, A: (1) All the subjects to be diagnosed as
beta-thalassemia major and confirmed in medical record by physician, (2)
Those receiving blood transfusion every 3-4 weeks (3) Those taking iron
chelation therapy (Desferal medication subcutaneously by using infusion
pump at home (4) All patients have to be within the three age categories I)
aged 5- to less than 12 years, II) aged 12-19 years, and III) aged =>19 years.
B: All caregivers of patients at home (next of kin) were included, one for each
patient. An official letter issued from University of Gezira and Ministry of
Health in Syria to obtain approval agreement to conduct the study, a
permission to use WHOQOL-BREF was taken, also a written consent was
obtained from each patient. Home caregivers and patients were assured that
there were no harms, risks and discomfort caused to them through
participation. The teaching guide was distributed to the patients or caregivers
through a period of data collection for initial assessment. Every patient and
caregiver was interviewed for an average of one hour to fill the data collection
formats directed towards each. All interviews were done with a patient or
home caregiver accompanying the child who was attending the center
according to his/her appointment for receiving blood transfusion and treatment
in the governmental center. The researcher assessed the patients and their
caregiver's knowledge and skills in the first interview, followed by handing of
the thalassemia guide. Two months later, a second interview was conducted to
reassess the participants.
Five tools were used in the process of data collection.
1. Validated World Health Organization Quality of Life
(WHOQOLBREF) questionnaire was used for the purpose of this study. The
WHOQOLBREF questionnaire covered four domains - physical, psychological, social
relationships and environment.
The researcher designed and developed the following structured
2. Home caregivers' knowledge about thalassemia major patient
3. Home caregiver attitudes interview sheet.
4. Physical assessment checklist.
5. Designed observational checklist was used to observe patients or their
caregivers at their homes while preparing and administering the Desferal
Demographic characteristics, disease features and complications were
analysed with mean score of QOL using chi-square, ANOVA and t-test where
appropriate. Statistical significant was considered as P-value < 0.05. The SPSS
version 20 was used to analyse the data.
Table 1- represents the general characteristics of patients in relation to
birth order, gender, educational level, marital status and age groups. Male
constituted 53.4%, while 46.6% were female. As regard age group, two thirds
of the sample were childhood and adolescent (72.3%). 36.6% were in the age
group of 5 to less than 12 years (20.6% male, and 16.0% female), while 35.7%
were in the age group 12 to less than 19 years (18.5% male, and 17.2% female.
Adulthood category for 19 years and above was the lowest about 27.7%
(14.3% of them male and 13.4 % were female). The majority of patients (71%)
were in school education.
*Applicable (Patients' age equal or more 16 years)
*Not applicable (Patients' age less than 16 years old)
Table 2 portrays home caregivers' characteristics. Mothers were
caregivers for patients in about two thirds of the sample (62.6%). Fathers and
patient himself were considered caregivers in 8.0%, 27.7% of cases
respectively. The majority of caregivers 75.6% were married. As regard to
educational level of caregivers, data indicated that 47.1%, 53% of caregivers
got their school education and higher education respectively. 66.4 % of
caregivers were un-employed.
Table 3 portrays about two thirds of sample (66.0 %) had insufficient
monthly income at all while 29.8 % of families had sufficient income. More
than half of the sample (58.4%) was from rural areas. Also the table depicts
great proportion of sample (65.5 %) had consanguineous parents. Less than
half of sample (44.5%) had a positive family history of thalassemia.
Knowledge about thalassaemia:
Areas of information:
Signs & symptoms
Modes of transmission
Sources of information:
Brochures & Newspaper
Table 6 Depicts great proportion of sample (59.2 %) had no difference
from peers. Nearly one third of the sample 37.1 % had physical changes such
as growth retardation, 24.7% had facial changes, 16.5 % had a weakness&
exercise intolerance, 11.3% had skin color changes and 10.3% had abdominal
changes and the majority 67.7% were upset from these differences. Regarding
to social activities, more than half of the sample 55.9% didn’t engage in group
activities and play.
Table 7 shows (39.9%) of sample had compliance with treatment.
Great proportion of the sample (61.7 %) had a capability to inject him/herself
with Desferal by using pump. Regarding reasons for not injecting Desferal
subcutaneously, third of the sample (32.5%) answered they had a lack of skills,
(22.5 %) were afraid and (45.0%) had been both lacking of skills and afraid.
Table 9 shows changes in physical structure of thalassemia patients.
Regarding to changes in physical growth retardation findings show half of
sample had normal physical growth, while quarter had changes in height and
weight. About two thirds of sample had normal facial bone, while (40%) had
different facial bone changes. More than two thirds of sample had normal
abdomen, while (28%) had an enlargement in the abdomen.
Beta-thalassemia major affects males and females equally and no sex
difference was reported
(Muncie Jr and Campbell 2009, Perisano, Marzetti et
. Findings of this study show a statistical significant was found in
females regarding to social domain (p=0.04). However, mean scores for
overall quality of life were better in females rather than males, but these mean
scores were statistically insignificant (p>0.05). These findings were consistent
with those of Dahlui and colleagues
(Dahlui, Hishamshah et al. 2009)
(Gharaibeh and Gharaibeh 2012)
regarding gender, male and female children
reported similar mean score for quality of life. Furthermore, the findings
showed that patient’s gender was not associated with HRQoL scores. These
findings were inconsistent with those reported by Seyedifar et al
Dorkoosh et al. 2016)
and Ansari et al
(Ansari, Baghersalimi et al. 2014)
found that there were significant differences in quality of life according to
gender, female patients had better quality of life than males significantly
Regarding socio-economic status, results show two thirds of families
said their monthly income was insufficient at all time. This finding was
congruent with other studies that reported nearly half of the thalassemia
families had employment and financial problems as a result of thalassemia,
presenting thalassemia as a socio-economic problem
(Zani, Di Palma et al.
1995, Aydinok, Erermis et al. 2005, Khurana, Katyal et al. 2006, Gharaibeh
and Mater 2009, Ayoub, Radi et al. 2013)
. A further study carried out by
Prasomsuk et al. reported that mothers caring for children with thalassemia
major in Thailand had financial difficulty due to loss of income and increased
expenses related to treatment, transportation and living costs during their
children's admission to the hospital was the main cause of financial burden for
these mothers. Not surprisingly, the burden became greater when the children
(Prasomsuk, Jetsrisuparp et al. 2007)
In our study, parents are consanguineous in 65.5% of patients' families.
Consanguineous marriage is a social phenomenon among Syrians, it is higher
in urban than rural areas 30.3% and 39.8% respectively
(Othman and Saadat
. In the present study, 23.1% of the sample had knowledge deficit
regarding causes, prevention, signs and symptoms, transmission, treatment
and complications of thalassemia. Many other studies
(Mrayyan, Al-Omary et
al. , Hanl, Han et al. 1992, Seyam and Assemi 2010, Miri‐Moghaddam, Zadeh‐
Vakili et al. 2011, Shrivastava, Sinha et al. 2011, Al Hajeri and Al Arrayed
2012, Ishaq, Hasnain Abid et al. 2012, Kourorian, Azarkeivan et al. 2014)
reported there was a lack of information and knowledge through the
thalassemia patients and families regarding the signs and symptoms, causes
and treatment of this disorder.
Health professionals are facing many problems in giving care to
thalassemia patients. The main problem is parents’ lack of awareness about the
disease. Medical staff can help to increase knowledge of families’ having
patients with thalassemia disorder
(Dehkordi and Heydarnejad 2008, Patricia
. In this study, caregivers’ overall knowledge score ranged from 16
– 45 out of 50 regarding thalassemia major and its management before
teaching guide with a mean score M±SD (34.12±4.50), while after teaching
guide ranged from 21-47 with a mean score M±SD (37.43±4.61). Overall, this
study found a statistical significant relationship among caregivers and
thalassemia patients in six out of seven areas of knowledge that where assessed
before and after distributing the teaching guide. Results of this study are
parallel with other studies
(Romeo, Di Gregorio et al. 1997, Yang, Chen et al.
2001, Heydarnejad and Hasanpour-Dehkordi 2008, Wong, George et al. 2011)
that found the education of parents regarding thalassemia could have
significant positive effect on increasing their knowledge.
As results revealed in our study, there were significant differences in
the mean scores regarding nutritional status before and after teaching guide
(paired t test= 12.49, p= 0.000). This could be explained that nutritional
information included in the guide had an influence on their knowledge score
that will reflect on nutritional behavior of thalassemia patients later on during
their daily life.
Findings of this present study show no significant difference among
three independent groups of caregivers regarding overall attitude score of four
categories included in the questionnaire (F ratio = 0.926; P= 0.398). Through
examining of each attitude-item separately, one item (q25) was statistically
significant among the independent groups (F ratio =3.216, p= 0.042). This
item “question 25: the family would not like others to know their affected
children particularly if the family has a history for thalassemia”. These
findings are agreement with other studies
(Zahed and BOU‐DAMES 1997,
Alkuraya and Kilani 2001, Zahed, Nabulsi et al. 2002, Kourorian, Azarkeivan
et al. 2014, Basu 2015)
who found that the majority of their study sample has
a positive attitude regarding thalassemia.
In Syria Al-Zir 2009 reported that carriers may decide to undergo
genetic testing and counseling to assess potential risks to their children,
including prenatal testing, so we have given emphasis to health education. We
believe that these measures will improve the quality of our life
Also in Syria, Murad 2014 reported in his study conducted among Syrian
families to evaluate the prenatal diagnosis (PND) of β-thalassemia and sickle
cell anemia results pointing to a successful future for PND of β-thalassemia
and sickle cell anemia in Syria, using a rapid and accurate molecular method
(Murad, Moassas et al. 2014)
. Also in Syria Kyriacou 2000 mentioned that
their findings provide a sound foundation on which to base a preventive
program for thalassemia and we believe that the data that we present will
facilitate the improvement of medical services such as carrier screening,
genetic counseling, and prenatal diagnosis. Furthermore a detailed knowledge
of the molecular pathology of β-thalassemia will strongly improve the prenatal
diagnosis services in Syria
(Kyriacou, Quobaili et al. 2000)
Thalassemia was commonly seen by the community as a stigma for the
patients and their families, who were often isolated
(Bush, Mandel et al. 1998)
This led most parents to be very secretive about the illness, and to avoid
discussion of the subject with friends and relatives. The ill children were
literally hidden away, did not attend school, and were not allowed to play with
(Bush, Mandel et al. 1998, Caocci, Efficace et al. 2012)
. In this
present study, stigmatization was significantly noticed among families caring
for thalassemia patients. This result was congruent with Gharaibeh et al
(Gharaibeh, Amarneh et al. 2009)
(Wong, George et al. 2011)
that stigmatization had many risks on thalassemia patients such as decreasing
the opportunity for social interactions with increasing the chance for isolation
and its negative impact in increasing the psychological burden, especially on
adolescent patients aged (13-18) years old.
Old children and adolescents with thalassemia major have difficulty to
develop positive body image. Caregivers reported that (40.8%) of their
children were different from peers. Such feeling is reported by many authors
(Angastiniotis and Hadjiminas 1981, Politis 1998, Angastiniotis 2002,
Aydinok, Erermis et al. 2005, Abetz, Baladi et al. 2006, Cao and Kan 2013)
Differences in their appearance as facial aspect, growth retardation and skin
color made them the subject of sidelong looks and curiosity
Pozzan et al. 1986, Nagaraj, Umashree et al. 2011)
. In the present study, the
common causes of such feeling were physical changes as growth retardation,
facial changes, weakness and exercise intolerance, skin color and abdominal
enlargement. Therefore, this study revealed that (67.7%) of children and
young adults were disturbed from these differences as reported by caregivers.
The vast majority of thalassemia patients told their parents about negative
feelings coming from others. The findings of this study were similar to Abetz
et al. who reported that the impact of iron chelation therapy on patients with
thalassemia is high
(Abetz, Baladi et al. 2006, Payne, Rofail et al. 2008)
with Beratis 1993 who found that thalassemia patients demonstrated a
significantly greater frequency of disturbed behavior towards relatives and
friends than the control subjects (Beratis 1993).
The results of this study showed that vast majority of patients attach
much importance to their appearance. It was also found that (84.5%) of
thalassemia patients have special interests and hobbies, but unfortunately
(55.9%) of the patients did not engage in group activities and play. This is
because of exercise intolerance and feeling of shamed face as mentioned by
them. Practicing sport was one of the burdens that affects patients with
thalassemia, as thalassemia leads to low hemoglobin level, fatigue and general
(Politis, Di Palma et al. 1990, Bush, Mandel et al. 1998, Gharaibeh,
Amarneh et al. 2009)
. In our research sample over quarter of thalassemia
patients had enlarged spleen.
Dental caries is common among patient with thalassemia major
Wahadni, Taani et al. 2002, Schrier 2002)
. This level of disease may be
explained on the basis of chronic nature of thalassemia. The higher caries
prevalence can be also attributed to the poor oral hygiene, improper dietary
habits, lack of dental knowledge, and poor motivation of these patients. In the
present study, results revealed that about third of thalassemia patients
infrequently or didn't perform oral hygiene respectively. The vast majority of
the patients used toothpaste and brush to clean their teeth daily. Therefore,
parents are more concerned with serious physical problems, paying lesser
attention to the dental ailments, and only seek dental care when the child is in
pain. This poor oral hygiene might be changed after giving teaching that
emphasis to educate patients regarding the effects of thalassemia on teeth
(Mehdizadeh, Mehdizadeh et al. 2008)
Results reveal statistical significant differences observed among three
groups of thalassemic patients (F ratio= 6.46, p=0.002) regarding serum
ferritin, this is parallel with study conducted with Tuysuz
(Senol, Tiftik et al.
2016, Tuysuz and Tayfun 2017)
who found that the mean serum ferritin levels
were significantly higher than the normal values and high ferritin level had a
negative impact on HRQoL. The present study showed that the mean score of
ferritin of adolescent group was higher than other groups. These findings are
in agreement with previous study
that found rejection of
treatment by adolescents and young adults is a recognized problem, whereas
these findings are inconsistent with Safizadeh et al
who found no statistical differences in all aspect of
quality of life with serum ferritin level.
In the present study, results revealed that 115 out of 238 (48.3%) of the
sample used Desferal subcutaneously, while (51.7%) used oral chelation
(Exjade). Through revising the 16 steps of administering Desferal S.C that
were included in the teaching guide for both caregivers and patients, results
reveal fourteen steps out of sixteen steps were improved by comparing the
percentage of each before and after distributing the teaching guide. Statistical
significant differences were found in performing all these steps before and
after distributing the guide through comparing the overall performance
(12.52±1.77 compared to 14.07±1.01). (Paired t test = 11.477, p= 0.000). The
result is supported by a study carried by Abu- Salim who reported that skill
score of administrating Desferal S.C was improved after a self- care program
for children with thalassemia (Abu-Salim L 1999). In addition, chelation
therapy indeed is difficult to accept because it is a daily reminder of one’s own
(Roy and Chatterjee 2007)
. It is usually performed in the evening when
patients seek greater reassurance to be able to sleep peacefully. They may
therefore complain of difficulty getting to sleep and resting well. There is no
doubt that compliance rate can be improved by health education of both
thalassemic patients and their caregivers. This fact is supported by numerous
studies (Tsiantis, Anastasopoulos et al. 1990, Politis 1998, Atkin and Ahmad
2000, Hoch, Göbel et al. 2000, Angastiniotis 2002)
In this study, overall quality of life score ranged from 21.7 to 99.1, with
a mean score M±SD (62.3±18.0 compared to 67.5±16.4) of adolescent and
adult group respectively. These results are comparable to those described by
(Burckhardt and Anderson 2003, Mann‐Jiles and Morris 2009, Kaheni,
Yaghobian et al. 2013, Senol, Tiftik et al. 2016)
who reported that QoL has
low to moderate correlation with physical health status.
No statistical significant differences were noticed between the two age
groups regarding 24 items of the quality of life. However, mean scores in
physical health, psychological, social relationships and environmental
domains insignificantly better in adult rather than adolescent groups (p>0.05).
Through comparing the overall quality of life scores between the two age
groups by taking into account all four domains together, results also reveal no
statistical significant differences between the adolescent and adult group. This
might indicate that the adult group adapted relatively well physically and
psychologically to the burden of chronic illness and treatments. . Findings of
this study are in agreement with a study conducted by
(Amani, Fathi et al.
who reported that the quality of life among the patients with thalassemia
in Ardabil was very good, and
(Ali, Tarawah et al. 2015)
who found in his
study conducted in KSA that the quality of life in thalassemia patients was
similar to the control group. Findings of this study are in agreement with
Kaheni et al
(Kaheni, Yaghobian et al. 2013)
, Gharaibeh et al
and Cheuk et al
(Cheuk, Mok et al. 2008)
that stated better
quality of life with the passage of time. On other hand, our findings were
inconsistent with other studies
(Cheuk, Mok et al. 2008, Ansari, Baghersalimi
et al. 2014)
reported that the QOL in all 6 dimensions was lower in patients
compared to the controls.
Furthermore, the findings showed that patient’s age was not associated
with HRQoL scores. These findings were parallel with
Gharaibeh 2012, Haghpanah, Nasirabadi et al. 2013, Ali, Tarawah et al. 2015,
Amani, Fathi et al. 2015)
who found there were no significant differences
between age and quality of life among their study sample. On other hand our
findings were inconsistent with those reported by Dahlui and colleagues
(Dahlui, Hishamshah et al. 2009)
who found there were significant differences
in quality of life according to age, quality of life was lowered as age increased
and with Dhirar et al
(Dhirar, Khandekar et al. 2016)
who reported that the
total quality of life scores were better for boys as compared to girls but the
difference was not statically significant (p= 0.131).
No statistical significant differences were found between the two
treatment groups (subcutaneous vs oral) regarding 24 items of the quality of
life. However, mean scores for each independent domain were better in
subcutaneous rather than oral group, but these mean scores were statistically
insignificant (p>0.05). Through comparing the overall quality of life scores
between the two groups, results also reveal that no statistical significant
differences were found between the subcutaneous and oral group respectively,
with a mean score M±SD (65.5±17.9 compared to 62.1±16.0).
The findings of current study were in agreement with Torcharus et al.
(Torcharus and Pankaew 2011)
(Kaheni, Yaghobian et al. 2013)
Trachtenberg et al
(Trachtenberg, Mednick et al. 2012)
(Ali, Tarawah et
who reported that is no statistical significant differences were found
in their studies regarding QOL based on type of iron chelation therapy, and
these findings are inconsistent with other studies Surapolchai et al 2010
(Surapolchai, Satayasai et al. 2010)
and Tavorncharoensap et al 2010
(Thavorncharoensap, Torcharus et al. 2010)
who found there was statistical
significant differences in quality of life scores due to iron chelation therapy.
On the other hand, Dhirar et al
(Dhirar, Khandekar et al. 2016)
, Goulas et al
(Goulas, Kourakli-Symeonidis et al. 2012)
and Cheuk et al.
(Cheuk, Mok et
, Osborne et al.
(Osborne, Lourenço et al. 2007)
reported that patients
on Desferasiox (oral chelation) had significantly better self-related QoL than
patients on other iron chelation regimen. Other studies also reported that the
use of Desferal was a strong impediment of QoL, and effective oral chelation
therapy might improve QoL
(Ratip, Skuse et al. 1995, Caro, Ward et al. 2002,
Kuo, Tsai et al. 2006, Thavorncharoensap, Torcharus et al. 2010, Ansari,
Baghersalimi et al. 2014, Senol, Tiftik et al. 2016)
In this study we assessed the effects of knowledge, attitude and
practice of thalassemia patients and their caregivers, on the patient's quality of
life. Our findings highlighted that there was lack and scarcity of patients’
knowledge and skills regarding the disease. There has been a remarkable
improvement in both knowledge and performance after patients received the
teaching guide. It is recommended to pay more attention in different aspects
of life of these patients helping to improve their quality of life in all
dimensions. Furthermore, it is necessary to improve patients’ and their
families’ awareness regarding this disease. We also found that thalassemia and
its complications have a negative impact on physical function, education, time
off school, difference from friends/sibling, social interaction, playing and
engaging in group activities and stigmatization. In addition, patients with
thalassemia and their families often require support in many aspects,
especially psychological support to prevent mental disorders.
Moreover, quality of life of patients in the four domains; physical,
social, psychological and environmental health regarding to the patients’ age
was above average, also it was above average in physical, psychological and
environmental domains regarding the patients' gender while it was less than
average in social domain in females. The findings show there were differences
between the four domains of quality of life regarding two types of iron
chelation treatment (subcutaneous and oral), subcutaneous treatment was
higher in all domains but the overall quality of life was higher also in
subcutaneous but not statistically significant.
Thalassemia patients have many limitations in their daily life. Proper
strategies are required for an effective prevention and treatment of this disease.
Medical staff should focus on disease specific educational programs for
patients with thalassemia about the entity and the process of the disease, its
managements, self-care needs, importance of regular iron chelation therapy
and its role in preventing or delaying complications. By improving these
methods and giving much more social and psychological support, we could
improve their quality of life.
The authors are grateful to acknowledge with extreme thanks the
patients and their parents who participated and cooperated us in this study.
Also we would like to thank Ministry of Health, the National Thalassemic
center in Damascus and University of Gezira who supported us during this
84. Thavorncharoensap, M., et al. (2010). "Factors affecting health-related
quality of life in Thai children with thalassemia." BMC Hematology
85. Torcharus, K. and T. Pankaew (2011). "Health-related quality of life
in Thai thalassemic children treated with iron chelation." Southeast
Asian Journal of Tropical Medicine and Public Health 42(4): 951.
86. Touma, H., et al. (2013). Clinical Effectiveness of Iron Chelation
Therapies in Syrian Patients with β Thalassemia Major: Suboptimal
Clinical Outcomes and High Prevalence of Iron Overload, Am Soc
87. Trachtenberg, F. L., et al. (2012). "Beliefs about chelation among
thalassemia patients." Health and quality of life outcomes 10(1): 148.
88. Tsiantis, J., et al. (1990). "A multi-level intervention approach for care
of HIV-positive haemophiliac and thalassaemic patients and their
families." AIDS care 2(3): 253-266.
89. Tuysuz, G. and F. Tayfun (2017). "Health-related Quality of Life and
its Predictors Among Transfusion-dependent Thalassemia Patients."
Journal of pediatric hematology/oncology 39(5): 332-336.
90. Vichinsky, E., et al. (2008). "Standards of care guidelines for
thalassemia." Children’s Hospital & Research Center Oakland, USA.
91. Wong, L. P., et al. (2011). "Public perceptions and attitudes toward
thalassaemia: Influencing factors in a multi-racial population." BMC
Public Health 11(1): 193.
92. Wonke, B. (2001). Clinical management of β-thalassemia major.
Seminars in hematology, Elsevier.
93. Yang, H., et al. (2001). "Illness knowledge, social support and self care
behavior in adolescents with beta-thalassemia major." Hu li yan jiu=
Nursing research 9(2): 114-124.
94. Zahed, L. and J. BOU‐DAMES (1997). "ACCEPTANCE OF FIRST‐
TRIMESTER PRENATAL DIAGNOSIS FOR THE
HAEMOGLOBINOPATHIES IN LEBANON." Prenatal diagnosis
95. Zahed, L., et al. (2002). "Attitudes towards prenatal diagnosis and
termination of pregnancy among health professionals in Lebanon."
Prenatal diagnosis 22(10): 880-886.
96. Zani, B., et al. (1995). "Psychosocial aspects of chronic illness in
adolescents with thalassaemia major." Journal of adolescence 18(4):
1. Abetz , L. , et al. ( 2006 ). "The impact of iron overload and its treatment on quality of life: results from a literature review . " Health and Quality of Life Outcomes 4 ( 1 ): 73 .
2. Al-Zir , K. N. ( 2009 ). "Prevention of hemoglobinopathies in Syria." Hemoglobin 33 ( sup1 ): S25 - S27 .
3. Al‐Wahadni , A. M. , et al. ( 2002 ). "Dental diseases in subjects with β‐ thalassemia major." Community dentistry and oral epidemiology 30(6 ): 418 - 422 .
4. Al Hajeri , A . and S. Al Arrayed ( 2012 ). "Public awareness of beta thalassemia in Bahrain." Bahrain Medical Bulletin 34 ( 1 ): 26 - 29 .
5. Ali , S. S. , et al. ( 2015 ). "Comprehensive patient care improves quality of life in transfusion dependent patients with β-thalassemia." Saudi medical journal 36 (5) : 575 .
6. Alkuraya , F. S. and R. A . Kilani ( 2001 ). "Attitude of Saudi families affected with hemoglobinopathies towards prenatal screening and abortion and the influence of religious ruling (Fatwa)." Prenatal diagnosis 21(6 ): 448 - 451 .
7. Amani , F. , et al. ( 2015 ). "Quality of life among Ardabil patients with beta-thalassemia major." 8 . Angastiniotis , M. ( 2002 ). "The adolescent thalassemic . The complicant rebel." Minerva pediatrica 54 (6) : 511 - 515 .
9. Angastiniotis , M. and M. Hadjiminas ( 1981 ). "Prevention of thalassaemia in Cyprus." The Lancet 317 ( 8216 ): 369 - 371 .
10. Ansari , S. , et al. ( 2014 ). "Quality of life in patients with thalassemia major." Iranian journal of pediatric hematology and oncology 4(2 ): 57 .
11. Atkin , K. and W. Ahmad ( 2000 ). "Pumping iron: compliance with chelation therapy among young people who have thalassaemia major." Sociology of Health & Illness 22 ( 4 ): 500 - 524 .
12. Aydinok , Y. , et al. ( 2005 ). "Psychosocial implications of thalassemia major." Pediatrics International 47 ( 1 ): 84 - 89 .
13. Ayoub , M. D. , et al. ( 2013 ). "Quality of life among children with betathalassemia major treated in Western Saudi Arabia." Saudi Med J 34 ( 12 ): 1281 - 1286 .
14. Basu , M. ( 2015 ). "A study on knowledge, attitude and practice about thalassemia among general population in outpatient department at a Tertiary Care Hospital of Kolkata." J Preven Medic Holistic Health 1 ( 1 ): 6 - 13 .
15. Beratis , S. ( 1993 ). "Psychosocial status in pre-adolescent children with β-thalassaemia." Journal of psychosomatic research 37 (3) : 271 - 279 .
16. Borgna-Pignatti , C. and M. R. Gamberini ( 2011 ). "Complications of thalassemia major and their treatment." Expert review of hematology 4(3 ): 353 - 366 .
17. Burckhardt , C. S. and K. L. Anderson ( 2003 ). "The Quality of Life Scale (QOLS): reliability, validity, and utilization." Health and quality of life outcomes 1(1 ): 60 .
18. Bush , S. , et al. ( 1998 ). "Future orientation and life expectations of adolescents and young adults with thalassemia major." Annals of the New York Academy of Sciences 850 ( 1 ): 361 - 369 .
19. Cao , A. and Y. W. Kan ( 2013 ). "The prevention of thalassemia." Cold Spring Harbor perspectives in medicine 3(2): a011775.
20. Caocci , G. , et al. ( 2012 ). "Health related quality of life in Middle Eastern children with beta-thalassemia." BMC blood disorders 12(1): 6 .
21. Capellini , M. , et al. ( 2008 ). "Guidelines for the clinical management of thalassemia." Thalassaemia International Federation (TIF ) April 2000 .
22. Caro , J. J. , et al. ( 2002 ). "Impact of thalassemia major on patients and their families . " Acta haematologica 107 ( 3 ): 150 - 157 .
23. Cheuk , D. , et al. ( 2008 ). "Quality of life in patients with transfusiondependent thalassemia after hematopoietic SCT." Bone marrow transplantation 42(5 ): 319 .
24. Cianciulli , P. ( 2008 ). "Treatment of iron overload in thalassemia." Pediatric endocrinology reviews: PER 6 : 208 - 213 .
25. Cohen , A. R. ( 2006 ). "New advances in iron chelation therapy . " ASH Education Program Book 2006 ( 1 ): 42 - 47 .
26. Dahlui , M. , et al. ( 2009 ). "Quality of life in transfusion-dependent thalassaemia patients on desferrioxamine treatment." Singapore medical journal 50 (8) : 794 .
27. Dehkordi , A. H. and M. S. Heydarnejad ( 2008 ). Enhancement of parents' awareness about ß-thalassemia major disorder through two educational programs , Professional Medical Publications.
28. Dhirar , N. , et al. ( 2016 ). "Thalassemia Major: how do we improve quality of life?" SpringerPlus 5(1 ): 1895 .
29. El Dakhakhny , A. M. , et al. ( 2011 ). "Quality of Life of School Age Thalassemic Children at Zagazig City Amal M El Dakhakhny , Mervat A Hesham, Samah E Mohamed, Fawzia N Mohammad . " Journal of American Science 7 ( 1 ).
30. Elalfy , M. S. , et al. ( 2014 ). "Quality of life of Egyptian b-thalassemia major children and adolescents." The Egyptian Journal of Haematology 39 ( 4 ): 222 .
31. Gharaibeh , H. , et al. ( 2009 ). "The psychological burden of patients with beta thalassemia major in Syria." Pediatrics international 51(5 ): 630 - 636 .
32. Gharaibeh , H. and M. Gharaibeh ( 2012 ). "Factors influencing healthrelated quality of life of thalassaemic Jordanian children." Child: care, health and development 38(2 ): 211 - 218 .
33. Gharaibeh , H. and F. Mater ( 2009 ). "Young Syrian adults' knowledge, perceptions and attitudes to premarital testing." International nursing review 56(4 ): 450 - 455 .
34. Goulas , V. , et al. ( 2012 ). "Comparative effects of three iron chelation therapies on the quality of life of greek patients with homozygous transfusion-dependent Beta-thalassemia." ISRN hematology 2012 .
35. Haghpanah , S. , et al. ( 2013 ). "Quality of life among Iranian patients with beta-thalassemia major using the SF- 36 questionnaire." Sao Paulo Medical Journal 131 ( 3 ): 166 - 172 .
36. Hanl , A. M. , et al. ( 1992 ). "Thalassemia in the outpatient department of the Yangon Children's Hospital in Myanmar: cost analysis of the day-care-room services for thalassemia . " Medicine 3 ( 2 ): 3 , 183 .
37. Hatami , G. and N. Motamed ( 2014 ). "Life Satisfaction in children and adolescents with beta thalassemia major in southwest Iran." Electronic physician 6(1 ): 759 .
38. Heydarnejad , M. and A. Hasanpour-Dehkordi ( 2008 ). "Effect of booklet and combined method on parents' awareness of children with β-thalassemia major disorder . " Journal of the Pakistan Medical Association 58 ( 9 ).
39. Hoch , C. , et al. ( 2000 ). "Psychosocial support of patients with homozygous beta-thalassaemia." Klinische Padiatrie 212 ( 4 ): 216 - 219 .
40. Ishaq , F. , et al. ( 2012 ). "Awareness Among Parents of ββ-Thalassemia Major Patients, Regarding Prenatal Diagnosis and Premarital Screening . " Journal of the College of Physicians and Surgeons Pakistan 22 ( 4 ): 218 - 221 .
41. Jafari , H. , et al. ( 2008 ). "Quality of life in thalassemia major: reliability and validity of the Persian version of the SF-36 questionnaire." Journal of postgraduate medicine 54 (4) : 273 .
42. Jantan , A. W. , et al. ( 2011 ). "Thalassaemia: a study on the perception of patients and family members." Medical Journal of Malaysia 66 ( 4 ): 326 - 334 .
43. Kaheni , S. , et al. ( 2013 ). "Quality of life in children with β-thalassemia major at center for special diseases." Iranian journal of pediatric hematology and oncology 3(3 ): 108 .
44. Khurana , A. , et al. ( 2006 ). "Psychosocial burden in thalassemia." The Indian Journal of Pediatrics 73 ( 10 ): 877 - 880 .
45. Kourorian , Z. , et al. ( 2014 ). The effect of knowledge, attitude and practice on the function of thalassemic patients .
46. Kuo , H. -T. , et al. ( 2006 ). "Pilot study on the “quality of life” as reflected by psychosocial adjustment of children with thalassemia major undergoing iron-chelating treatment in western Taiwan." Hemoglobin 30 ( 2 ): 291 - 299 .
47. Kyriacou , K. , et al. ( 2000 ). "Molecular characterization of β- thalassemia in Syria." Hemoglobin 24 ( 1 ): 1 - 13 .
48. Liem , R. I. , et al. ( 2011 ). "THE IMPACT OF THALASSEMIA ON SOUTHEAST ASIAN AND ASIAN INDIAN FAMILIES IN THE UNITED STATES : AQualitative STUDY." childhood 11 : 12 .
49. Mann‐Jiles , V. and D. L. Morris ( 2009 ). "Quality of life of adult patients with sickle cell disease . " Journal of the American Association of Nurse Practitioners 21 ( 6 ): 340 - 349 .
50. Massaglia , P. , et al. ( 1986 ). "Psychological aspects of Thalassemia." La Pediatria medica e chirurgica: Medical and surgical pediatrics 8(1 ): 27 - 31 .
51. Mehdizadeh , M. , et al. ( 2008 ). "Orodental complications in patients with major beta-thalassemia." Dent Res J 5 ( 1 ): 17 - 20 .
52. Milat , F. , et al. ( 2012 ). "A case of hypophosphatemic osteomalacia secondary to deferasirox therapy . " Journal of Bone and Mineral Research 27 ( 1 ): 219 - 222 .
53. Miri‐Moghaddam , E. , et al. ( 2011 ). "Molecular basis and prenatal diagnosis of β‐thalassemia among Balouch population in Iran." Prenatal diagnosis 31(8 ): 788 - 791 .
54. Mrayyan , M. T. , et al. "Jordanian Families' Attitudes toward Thalassaemia and Genetic Counseling."
55. Muncie Jr, H. L. and J. Campbell ( 2009 ). "Alpha and beta thalassemia." American family physician 80(4 ): 339 - 344 .
56. Murad , H. , et al. ( 2014 ). "Prenatal Molecular Diagnosis of β- Thalassemia and Sickle Cell Anemia in the Syrian Population . " Hemoglobin 38 ( 6 ): 390 - 393 .
57. Nagaraj , T. , et al. ( 2011 ). "[Beta] Thalassemia major: A case report." Journal of International Oral Health 3 ( 5 ): 67 .
58. Olivieri , N. F. , et al. ( 1994 ). "Survival in medically treated patients with homozygous β-thalassemia." New England Journal of Medicine 331 ( 9 ): 574 - 578 .
59. Osborne , R. H. , et al. ( 2007 ). "Quality of life related to oral versus subcutaneous iron chelation: a time trade-off study." Value in health 10(6 ): 451 - 456 .
60. Othman , H. and M. Saadat ( 2009 ). "Prevalence of consanguineous marriages in Syria." Journal of biosocial science 41 ( 5 ): 685 - 692 .
61. Pakbaz , Z. , et al. ( 2005 ). "A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia . " Annals of the New York Academy of Sciences 1054 ( 1 ): 486 - 491 .
62. Pakbaz , Z. , et al. ( 2005 ). "A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia . " Annals of the New York Academy of Sciences 1054 : 486 - 491 .
63. Patricia Ault , R. ( 2009 ). "Understanding iron overload: screening, monitoring, and caring for patients with transfusion-dependent anemias." Clinical journal of oncology nursing 13 (5) : 511 .
64. Payne , K. A. , et al. ( 2008 ). "Iron chelation therapy: clinical effectiveness, economic burden and quality of life in patients with iron overload." Advances in therapy 25(8 ): 725 .
65. Pennell , D. , et al. ( 2011 ). "On improvement in ejection fraction with iron chelation in thalassemia major and the risk of future heart failure . " Journal of Cardiovascular Magnetic Resonance 13 ( 1 ): 45 .
66. Perisano , C. , et al. ( 2012 ). "Physiopathology of bone modifications inthalassemia . " Anemia 2012 .
67. Politis , C. ( 1998 ). "The psychosocial impact of chronic illness." Annals of the New York Academy of Sciences 850 ( 1 ): 349 - 354 .
68. Politis , C. , et al. ( 1990 ). "Social integration of the older thalassaemic patient." Archives of disease in Childhood 65 ( 9 ): 984 - 986 .
69. Prasomsuk , S. , et al. ( 2007 ). "Lived experiences of mothers caring for children with thalassemia major in Thailand." Journal for Specialists in Pediatric Nursing 12 ( 1 ): 13 - 23 .
70. Ratip , S. , et al. ( 1995 ). "Psychosocial and clinical burden of thalassaemia intermedia and its implications for prenatal diagnosis." Archives of disease in childhood 72(5 ): 408 - 412 .
71. Romeo , M. A. , et al. ( 1997 ). "Persistence of Mediterranean anaemia in Sicily." Journal of medical genetics 34 ( 10 ): 869 - 870 .
72. Roy , T. and S. C. Chatterjee ( 2007 ). "The experiences of adolescents with thalassemia in West Bengal , India." Qualitative Health Research 17 (1) : 85 - 93 .
73. Safizadeh , H. and Z. Farahmandinia ( 2012 ). "Quality of life in patients with thalassemia major and intermedia in kerman-iran (IR)." Mediterranean journal of hematology and infectious diseases 4(1).
74. Scalone , L. , et al. ( 2008 ). "Costs, quality of life, treatment satisfaction and compliance in patients with β-thalassemia major undergoing iron chelation therapy: the ITHACA study." Current medical research and opinion 24(7 ): 1905 - 1917 .
75. Schrier , S. L. ( 2002 ). "Pathophysiology of thalassemia." Current opinion in hematology 9(2 ): 123 - 126 .
76. Schrier , S. L. and E. Angelucci ( 2005 ). "New strategies in the treatment of the thalassemias . " Annu. Rev. Med . 56 : 157 - 171 .
77. Senol , S. P. , et al. ( 2016 ). "Quality of life, clinical effectiveness, and satisfaction in patients with beta thalassemia major and sickle cell anemia receiving deferasirox chelation therapy." Journal of basic and clinical pharmacy 7(2 ): 49 .
78. Seyam , S. and A . Assemi ( 2010 ). "Study of the knowledge in guilan university students about thalassemia . " Journal of Urmia Nursing And Midwifery Faculty 8 ( 3 ): 0 - 0 .
79. Seyedifar , M. , et al. ( 2016 ). "Health-related quality of life and health utility values in beta thalassemia major patients receiving different types of iron chelators in Iran." International journal of hematologyoncology and stem cell research 10 (4): 224 .
80. Shaligram , D. , et al. ( 2007 ). "Psychological problems and quality of life in children with thalassemia." The Indian Journal of Pediatrics 74 ( 8 ): 727 - 730 .
81. Shrivastava , K. , et al. ( 2011 ). "Knowledge, attitude and practice study of beta-thalassemia in rural bengal." Genetic clinics 4(4 ): 13 - 15 .
82. Surapolchai , P. , et al. ( 2010 ). "Biopsychosocial predictors of healthrelated quality of life in children with thalassemia in Thammasat University Hospital." J Med Assoc Thai 93 ( Suppl 7 ): 65 - 75 .
83. Telfer , P. , et al. ( 2005 ). "Quality of life in thalassemia." Annals of the New York Academy of Sciences 1054 ( 1 ): 273 - 282 .