Paediatrica Indonesiana

VOLUME 51 May ‡ NUMBER 3

Original Article

Quality of life assessment of children with thalassemia

Masyitah Sri Wahyuni, Muhammad Ali, Nelly Rosdiana, Bidasari Lubis

Abstract
Background Thalassemia is a chronic disease that is becoming a
major health problem in the world, including the Mediterranean,
as well as Malaysia, Thailand and Indonesia. This condition clearly
DIIHFWVWKHSDWLHQW·VTXDOLW\RIOLIHEHFDXVHRIWKHFRQGLWLRQLWVHOIDQG
the effects of treatment. Assessment is needed to determine actions
to be taken to improve the quality of life in thalassemic children.
Objective To assess the differences in quality of life of thalassemic
children compared to their normal siblings.
T
halassemia is an inherited hemoglobin disorder
that is becoming a major health problem in
the world, especially in Mediterranean region,
the Middle East, the Indian subcontinent,
6RXWKHDVW$VLD0DOD\VLD7KDLODQGDQG,QGRQHVLD1-3
,W KDV EHHQ HVWLPDWHG WKDW DSSUR[LPDWHO\  RI WKH
world population are carriers of such disorders and
WKDW  ²  EDELHV ZLWK VHYHUH IRUPV RI
Methods:HSHUIRUPHGDFURVVVHFWLRQDOVWXG\IURP0D\
XQWLO-XQHLQ+$GDP0DOLN+RVSLWDO0HGDQDQGWKHKRPH
RIDPHPEHURIWKH1RUWK6XPDWUD&KDSWHURIWKH$VVRFLDWLRQRI
3DUHQWVRI7KDODVVHPLD6XIIHUHUV3HUKLPSXQDQ2UDQJWXD3HQGHULWD
WKHVHGLVHDVHVDUHERUQHDFK\HDU:+24 In
1RUWK6XPDWUDWKHFDUULHUSRSXODWLRQUHDFKHG
FRQVLVWLQJ RI  DOSKD WKDODVVHPLD DQG 
7DODVHPLD,QGRQHVLD3237,7KDODVVHPLFFKLOGUHQDJHG beta thalassemia.5 Children with thalassemia appear
years and their age and gender-adjusted siblings were divided into healthy at birth, but develop anemia that becomes
WZRJURXSVFDVHJURXSDQGFRQWUROJURXS3DUHQWVDQGFKLOGUHQ progressively worse due to the partial or total absence
ZHUHDVNHGWRILOOWKH3HGV4/3HGLDWULF4XDOLW\RI/LIH,QYHQWRU\
YHUVLRQTXHVWLRQQDLUHWRDVVHVVWKHLUTXDOLW\RIOLIH
Results 7KHUHZHUHFKLOGUHQLQHDFKJURXS7KHDVVHVVPHQWVRI
four quality of life domains in the thalassemic group vs the control
JURXSVKRZHGWKHIROORZLQJSK\VLFDOIXQFWLRQYV
of hemoglobin. Because of this condition, they need
regular blood transfusions for the rest of their lives to
manage chronic anemia and hemoglobin levels.1,5,6
Regular blood transfusions lead to iron accumulation in
&,WR3 HPRWLRQDOIXQFWLRQYV organs and may disrupt organ function and/or damage
&,WR3 VRFLDOIXQFWLRQYV
&,WR3 DQGVFKRROIXQFWLRQYV
organs. Iron overload causes most of the mortality
&,WR3 7RWDOVFRUHVZHUH and morbidity associated with thalassemia. Long term
YV&,WR3 6FKRROIXQFWLRQZDV transfusions should be accompanied by therapy with
the most affected parameter studied, with thalassemic children iron chelating agents.
scoring lower than the control group.
Conclusion There were significant decreases in the quality of
life parameters in the thalassemic group compared to the control
group. Thalassemic children have poorer quality of life compared
to their normal siblings, with school function being the most
affected domain. [Paediatr Indones. 2011;51:163-9]. )URP WKH 'HSDUWPHQW RI &KLOG +HDOWK 8QLYHUVLW\ RI 1RUWK 6XPDWUD
Medical school /Adam Malik Hospital, Medan, Indonesia.

Keywords: thalassemia, normal siblings, quality of Reprint requests to0DV\LWDK6UL:DK\XQL0''HSDUWPHQWRI&KLOG

life, PedsQL +HDOWK 8QLYHUVLW\ RI 1RUWK 6XPDWUD 0HGLFDO 6FKRRO Jl. Bunga Lau
1R 0HGDQ  ,QGRQHVLD 7HO  )D[
. E-mail : chiecieyouni@yahoo.com

Paediatr Indones, Vol. 51, No. 3, May 2011 ‡163

 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia

Thalassemia has a negative impact on quality of In this study, quality of life assessment was
life due to the effects of the disease and its treatment, SHUIRUPHG XVLQJ WKH 3HGLDWULF 4XDOLW\ RI /LIH
QRWRQO\DIIHFWLQJFKLOGUHQV·SK\VLFDOIXQFWLRQEXWDOVR ,QYHQWRU\3HGV4/*HQHULF&RUH6FDOH7KLV
their social, emotional, and school function, leading questionnaire includes parallel child self-reports (age
to an impaired quality of life. The most commonly UDQJHV    DQG  \HDUV  3HGV4/
affected domains previously reported were feelings of items ask how much of a problem a particular issue
depression, anxiety, psychological problems, emotional has been for patients during a certain period. Item
burden, hopelessness, difficulty with social integration, responses are measured on a five-point rating scale,
and school problems. 6LPLODU FRQGLWLRQV DOVR UDQJLQJ IURP  QHYHU D SUREOHP WR  DOPRVW
affected their family members, such as sadness,
disappointment, hopelessness, stress, depression, and
DQ[LHW\DERXWWKHLUFKLOGUHQ·VOLYHV13 The assessment
of quality of life in thalassemic children and their family
members is important in order to determine actions to
be taken to improve quality of life. The aim of this study
was to assess differences in quality of life of thalassemic
children compared to their normal siblings.
Methods
We conducted a cross-sectional study from May -
-XQHLQ$GDP0DOLN+RVSLWDO0HGDQDQGWKH
KRPHRIRQHRI1RUWK6XPDWUD·V3237,PHPEHUV:H
LQFOXGHGFKLOGUHQDJHG\HDUVRIDOOWKDODVVHPLD
classifications and their normal siblings, with age at
RQVHWRIDQHPLD\HDUVDQGDJHDWILUVWWUDQVIXVLRQ
\HDUVDVZHOODVSUHWUDQVIXVLRQKHPRJORELQOHYHO
JG/3DUHQWVDQGFKLOGUHQJDYHLQIRUPHGZULWWHQ
consent, and filled questionnaires completely. We
excluded children with cognitive function problems,
psychotic problems and cancer.
6XEMHFWV ZHUH GLYLGHG LQWR WZR JURXSV WKH
thalassemic group and their normal siblings group. We
measured anthropometric values of each child before
the study began. Weight was measured with Camry®
VFDOHVDQGKHLJKWZDVPHDVXUHGZLWKD0LFURWRLVH0
stature meter.
3DUHQWV DQG FKLOGUHQ ZHUH DVNHG WR ILOO WKH
DOZD\V D SUREOHP 7KH  LWHPV FRQVLVW RI 
items on physical function, 5 items on emotional
function, 5 items on social function, and 5 items on
school function, yielding a total score. Each scale
KDVDVFRUHUDQJLQJIURPZLWKKLJKHUVFRUHV
indicating higher quality of life.
This study was approved by the Medical Ethics
&RPPLWWHH RI WKH 8QLYHUVLW\ RI 6XPDWUD 8WDUD
0HGLFDO6FKRRO
:HXVHG6366YHUVLRQDQG0LFURVRIW([FHO
IRUGDWDSURFHVVLQJ,QGHSHQGHQWWWHVWZDVXVHG
to compare differences in quality of life between the
WZRJURXSV'LIIHUHQFHVZHUHFRQVLGHUHGVLJQLILFDQW
DW3DQGD&,
Results
Thalassemic children and their normal siblings were
recruited to this study. There were 136 children,
ZLWKWKDODVVHPLDDQGQRUPDOVLEOLQJV2XWRI
 FKLOGUHQ  FKLOGUHQ ZHUH H[FOXGHG IURP WKLV
study because 4 children were below 5 years of age, 5
FKLOGUHQZHUHPRUHWKDQ\HDUVRIDJHFKLOGUHQ
had mental retardation, and 7 children had no
VLEOLQJV7KHFKLOGUHQZKRIXOILOOHGWKHLQFOXVLRQ
FULWHULDZHUHGHYLGHGLQWRWZRJURXSVWKDODVVHPLF
FKLOGUHQ RU FDVH JURXS DQG  QRUPDO VLEOLQJV RU
control group.
7KHDYHUDJHDJHLQERWKJURXSVZDV\HDUV
3HGV4/ YHUVLRQ  TXHVWLRQQDLUHV DIWHU UHFHLYLQJ
directions and explanations on the meaning of each
question. We collected and reviewed questionnaire
sheets after they were completely filled. The
completed questionnaires were adjusted for age and
JHQGHU'DWDIRUHDFKGRPDLQSK\VLFDOHPRWLRQDO
social, and school functions) were tabulated. Children
with significantly poor quality of life were referred to
a child psychologist.
164‡Paediatr Indones, Vol. 51, No. 3, May 2011
PRVWFRPPRQO\ER\V7KHQXWULWLRQVWDWXVRI
both groups was normal and the most common level of
HGXFDWLRQZDVSULPDU\VFKRRO0RVWFRPPRQSDUHQWV·
education level was high school with self-owned
business as their main profession. (Table 1)
Table 2 shows the hematologic profile of the
thalassemic children. This profile was consistent
with the thalassemia condition. We found the most
FRPPRQ DJH DW RQVHW RI DQHPLD ZDV ”  \HDUV
 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia

7KHPRVWFRPPRQDJHDWILUVWGLDJQRVLVRI Table 3 shows there were significant differences

WKDODVVHPLDZDV!\HDUVZLWKPRVWKDYLQJ of quality of life between thalassemic children and
EHWDWKDODVVHPLDPDMRU7KHPRVWFRPPRQ their normal siblings. Assessments of the four quality
DJHDWILUVWWUDQVIXVLRQZDV\HDUVZLWK of life domains in the thalassemic vs control groups
D SUHWUDQVIXVLRQ KHPRJORELQ OHYHO RI   JG/ showed the following: physical function 53.1 vs 71.5

2XUGDWDDOVRVKRZHGWKDWRIWKDODVVHPLF
VXEMHFWVKDGSDUHQWVZKRZHUHERWKFDUULHUV,Q Table 2*GOCVQNQIKERTQſNGQHVJCNCUUGOKEEJKNFTGP
RIVXEMHFWVRQO\RQHSDUHQWZDVDFDUULHUDQGLQ Characteristic n (%)
of subjects it was unknown which parents were Age at onset of anemia
FDUULHUV 5RXWLQH WUDQVIXVLRQV ZHUH JLYHQ WR  0 2 (3.4)
ŭ[GCTU 57 (96.6)
of subjects every month, most commonly with 3 bags #IGCVſTUVFKCIPQUKUQHVJCNCUUGOKC
5RXWLQHLURQFKHODWLQJWKHUDS\ZDVJLYHQWR ŭ[GCTU 28 (47.5)
RIWKHWKDODVVHPLFFKLOGUHQ > 2 years 31 (52.5)
Parents as carrier
Unknown 35 (59.3)
1 carrier 6 (10.2)
Table 1. Baseline characteristics of subjects 2 carriers 18 (30.5)
Type of thalassemia
Thalassemia Normal Beta major 57 (96.6)
Characteristic patients siblings Beta minor 2 (3.4)
(n=59) (n=59) #IGCVſTUVVTCPUHWUKQP
Mean age, years (SD) 10.56 (3.14) 10.56 (3.14) Never transfused 2 (3.4)
Sex, n (%) ŭ[GCTU 24 (40.7)
Boy 40 (67.8) 40 (67.8) 3 years 33 (55.9)
Girl 19 (32.2) 19 (32.2) Number of transfusions per month
Mean weight, kg (SD) 27.88 (9.10) 29.32 (9.96) 0 2 (3.4)
Mean height, cm (SD) 131.37 (15.36) 133.75 (16.32) ŭDCIU 21 (35.6)
Nutritional status, n (%) 3 bags* 29 (49.2)
Moderate malnutrition 1 (1.7) 0 4 bags* 5 (8.5)
Mild malnutrition 8 (13.6) 9 (15.3) ŮDCIU 2 (3.4)
Normal 50 (84.7) 49 (83.0) Routine transfusion
Overweight 0 1 (1.7) No 5 (8.5)
Child’s level of education, n (%) Yes 54 (91.5)
Did not attend school 6 (10.2) 0 Mean pre-transfusion hemoglobin level
Kindergarten 11 (18.6) 11 (18.6) Not examined 2 (3.4)
Primary school 29 (49.2) 32 (54.2) ŭIF. 5 (8.5)
Elementary school 8 (13.6) 11 (18.6) 5 g/dL 10 (16.9)
High school 5 (8.5) 5 (8.5) 6 g/dL 42 (71.2)
Parents’ level of education, n (%) Ferritin serum level
Primary school 12 (20.3) 16 (27.1) Not examined 2 (3.4)
Elementary school 4 (6.8) 6 (10.2) < 1000 μg/L 11 (18.6)
High school 35 (59.3) 30 (50.8) > 1000 μg/L 6 (10.2)
Diploma 2 (3.4) 2 (3.4) > 2000 μg/L 40 (67.8)
Bachelor’s degree 6 (10.2) 5 (8.5) Iron chelation therapy
Parents occupation, n (%) No 13 (22)
Employees 12 (20.3) 12 (20.3) Yes (78)
Own business 47 (79.7) 47 (79.7) * Each bag contains 175 mL packed red blood cells

Table 3. Differences in quality of life between thalassemic children and their normal siblings
Case group Control group
Quality of life (n=59) (n=59) 95% CI P
mean (SD) mean (SD)
Physical function 53.1 (9.49) 71.5 (7.23) -21.41 to -15.26 0.0001
Emotional function 50.9 (13.96) 62.9 (11.75) -16.82 to -7.41 0.0001
Social function 62.5 (10.92) 72.8 (6.25) -13.50 to -7.01 0.0001
School function 36.2 (10.06) 56.0 (6.75) -22.95 to -16.71 0.0001
Total score 50.9 (7.55) 66.1 (4.35) -18.20 to -13.12 0.0001

Paediatr Indones, Vol. 51, No. 3, May 2011 ‡165

 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia

Figure 1. Differences in quality of life between thalassemic children and their normal siblings

 &,  WR  3  HPRWLRQDO is a broad-ranging concept, affected in a complex way
IXQFWLRQ  YV   &,  WR  E\WKHSHUVRQ·VKHDOWKSV\FKRORJLFDOVWDWHSHUVRQDO
3  VRFLDO IXQFWLRQ  YV   &, beliefs, social relationships and their relationship to
WR3 DQGVFKRROIXQFWLRQ salient features of their environment.
YV&,WR3 7KH 7KHKHPDWRORJ\SURILOHVKRZHGRI
WRWDO VFRUHV ZHUH  YV   &,  WR thalassemic patients to have beta thalassemia major
3 6FKRROIXQFWLRQZDVPRVWDIIHFWHG ZLWKPHDQDJHDWRQVHWRIDQHPLD\HDUVROGSUH
parameter (Figure 1). WUDQVIXVLRQKHPRJORELQOHYHOJG/DQGDJHDWILUVW
Discussion
We compared the quality of life of thalassemic
children to their normal siblings, as representative
family members to assess the effects of the disease
and its treatment. The assessment of quality of life
in children, especially in those with chronic diseases
such as thalassemia is important. The negative
impact of the disease and its treatment can affect the
quality of life of patients and their family members,
hence the need to assess both groups.The WHO
GHILQHVTXDOLW\RIOLIHDVDQLQGLYLGXDO·VSHUFHSWLRQRI
their position in life in the context of the culture and
value systems in which they live and in relation to
their goals, expectations, standards and concerns. It
166‡Paediatr Indones, Vol. 51, No. 3, May 2011
WUDQVIXVLRQ\HDUVROG0RUHWKDQKDOI
of the subjects were diagnosed with thalassemia at
WKHDJHRI!\HDUVDOWKRXJKWKH\KDGEHHQDQHPLF
EHIRUHWKHDJHRI\HDUV
Thalassemia is a chronic disease with serious
clinical symptoms. A study in Thailand found several
conditions that correlate to the severity of disease
LQ WKDODVVHPLD SDWLHQWV DJH DW RQVHW RI DQHPLD 
 \HDUV ROG DJH DW ILUVW WUDQVIXVLRQ   \HDUV ROG
SUHWUDQVIXVLRQKHPRJORELQOHYHOJG/DQGEHWD
thalassemia major.6
:H IRXQG WKDW RI DOO WKDODVVHPLF FKLOGUHQ·V
SDUHQWVKDGERWKSDUHQWVDVFDUULHUV
KDGRQHSDUHQWDVDFDUULHUDQGKDGXQNQRZQ
parental status. The above results are consistent with
thalassemic offspring often having carrier parents.
:H IRXQG  RI WKH WKDODVVHPLF FKLOGUHQ WR EH
 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia
thalassemia major, a condition typically inherited from
two carrier parents. There was also a small number of
other thalassemic children.
Thalassemia major can often be prevented
by avoiding marriage of two carrier parents or by
performing prenatal diagnoses in high risk mothers.5,15
0DUULDJHRIWZRFDUULHUSDUHQWVUHVXOWVLQDFKDQFH
RI SURGXFLQJ WKDODVVHPLF RIIVSULQJ  FKDQFH RI
producing a carrier who can pass on the disease if
KHVKHPDUULHVDQRWKHUFDUULHUDQGDFKDQFHRI
normal offspring (not thalassemic or carrier).5,16
3URJUHVVLYH LURQ RYHUORDG LV D FRQVHTXHQFH RI
ineffective erythropoiesis, increased gastrointestinal
absorption of iron and above all, multiple blood
transfusions.  (YHU\  PO RI UHG EORRG FHOOV
WUDQVIXVHGFRQWDLQVDSSUR[LPDWHO\PJRILURQWKDW
will circulate in the body, but the body cannot excrete
more than 1 mg of iron per day.The accumulation
of iron results in progressive dysfunction of the heart,
liver and endocrine glands.
Iron chelating agents are needed to control
iron levels in patients who receive regular blood
$OPRVWDOORIWKHWKDODVVHPLFVXEMHFWV transfusions.'HIHUR[DPLQHKDVXQWLOQRZEHHQ
received monthly transfusions, with various blood considered the treatment of choice for patients with
volumes transfused depending on hemoglobin level chronic iron overload due to blood transfusion.
upon visiting the hospital. The most common amount The time to start the iron chelating agents is
RIEORRGWUDQVIXVHGZDVEDJVRIVXEMHFWVDQG GHWHUPLQHGE\WKHSDWLHQW·VVHUXPIHUULWLQDQGLURQ
the most common hemoglobin level upon hospital visit levels, as well as the total iron binding capacity after
ZDVJG/
In the management of thalassemia, regular red
blood cell transfusions can reduce the complications of
anemia and ineffective erythropoiesis, permit normal
growth and development throughout childhood,
and extend survival in thalassemic children.1,15,17
The decision to initiate regular transfusions is
generally based on the observation of a hemoglobin
concentration less than 6 g/dL.1,15
regular transfusions. Chelating therapy is typically
VWDUWHGLIWKHVHUXPIHUULWLQOHYHOH[FHHGV—J/
RUDIWHUUHFHLYLQJWUDQVIXVLRQV
We assessed the quality of life of thalassemic
FKLOGUHQDQGWKHLUQRUPDOVLEOLQJVZLWKWKH3HGV4/
YHUVLRQTXHVWLRQDLUH,QWKLVVWXG\ZHXVHGWKH
(QJOLVK,QGRQHVLDQYHUVLRQRI3HGV4/JHQHULFFRUH
module in a sample of healthy children and children
with thalassemia.
:HIRXQGPRVWVXEMHFWVKDGDQRUPDO
nutritional status, which is not in keeping with the
theory that children with thalassemia have growth
problem. This was caused by the anthropometry data
showed abnormal condition (short stature) which in
turn resulting in normal nutritional status.
Children with thalassemia are known to have
abnormal growth. The etiology of these co-morbidities
is typically ascribed to the toxic effect of regular blood
transfusions related to iron overload. Malnutrition
is primarily caused by inadequate nutrient intake, as
indicated by the capacity to gain weight appropriately
when provided with nutritional support in the absence
of intestinal malabsorption. for certain symptoms or side effects of the disease.
,QVWXGLHVRIFKLOGUHQ·VTXDOLW\RIOLIHWKHUHDUH
generally two types of tools that can be used, generic
measures and disease-specific measures. Each has its
own advantages and disadvantages. Generic measure
tools can be used in healthy or sick children with
various diseases. Hence, they can be used to compare
the quality of life of children with chronic illness to
healthy children or children with other types of diseases.
The disadvantage of these tools is that they cannot be
used to assess specific symptoms of certain illnesses
or possible side effects. On the other hand, specific
measure tools cannot be used to compare sick children
to healthy ones, but they are more sensitive and specific
0RVWVXEMHFWVLQRXUVWXG\KDGUHFHLYHG
iron chelating therapy regularly after blood transfusions,
with serum ferritin examinations every 3 months. We
IRXQGWKDWRIVXEMHFWVKDGVHUXPIHUULWLQ!
—J/DQGKDGVHUXPIHUULWLQ!—J/$IHZ
subjects had not received chelating therapy because
WKHLUVHUXPIHUULWLQZDVOHVVWKDQ—J/RUWKH\KDG
not been checked yet.
The choice of the tools depends on the subjects,
specific conditions or the illness.14 The reliability and
validity of the tool also determines the validity of the
assessment. For practical purposes, the ideal tool must
be brief, but retain good reliability and validity, provide
useful information, able to be filled by children of
various ages and their parents, and be available both
in generic and specific measures.
Paediatr Indones, Vol. 51, No. 3, May 2011 ‡167
 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia
3HGV4/LVDWRROUHFRPPHQGHGIRUTXDOLW\RIOLIH
assessment because it fulfills several criteria for such a
valid tool. It has high validity and reliability (has been
proven in several studies in children with malignancies,
diabetes and heart disease), is available in generic and
VSHFLILFIRUPVIRUFHUWDLQGLVHDVHVHJ3HGV4/PRGXOH
for cancer, asthma, diabetes, rheumatic heart disease,
cerebral palsy, epilepsy and thalassemia), can be filled
by children (self-report) or parents/guardians (proxy
report), has been translated to several languages to
facilitate usage, and is available for various age groups:
supported other studies in Malaysia and Thailand in
that it also showed that children with thalassemia have
a poorer quality of life. However, we were unable to
evaluate if there was any improvement to quality of
life after intervention, such as education for parents
and consultations with child psychologists. Further
study is needed in this area.
In conclusion, we found that thalassemic
children had a lower quality of life compared to their
clinically normal siblings, with school function the
most negatively affected.
 \HDUV SUR[\ UHSRUW  \HDUV VHOI DQG SUR[\
UHSRUW\HDUVVHOIDQGSUR[\UHSRUWDVZHOODV
\HDUVVHOIDQGSUR[\UHSRUW Acknowledgements
7KHRULJLQDODXWKRURI3HGV4/LV'U-DPHV:9DUQL
He has developed and made several modifications since :HZRXOGOLNHWRWKDQN'U-DPHV:9DUQLWKHRULJLQDODXWKRURI
7KLVWRROFRQVLVWVRIVHYHUDOYHUVLRQVYHUVLRQ 3HGV4/IRUDOORZLQJXVWRXVHWKLVLQVWUXPHQWLQRXUVWXG\:H
XQWLOZLWKTXHVWLRQVLQGRPDLQVRIDVVHVVPHQW
SK\VLFDOHPRWLRQDOVRFLDODQGVFKRROGRPDLQV7KH
YHUVLRQLVDJHQHULFPHDVXUHVWRRO,Q3HGV4/WKHTXDOLW\
of life of children with chronic illness may be compared
to healthy/normal children as a control.
The results of our study are comparable to a
previous study where the quality of life of thalassemic
children was significantly lower than the control
group. Our study also showed the results of each
domain in the quality of life assessment: physical,
VRFLDOHPRWLRQDODQGVFKRROGRPDLQ6LPLODUWRWKH
previous study, school domain had the lowest mean
would also like to acknowledge the thalassemic children, their
siblings, parents and those who assisted in this study.
References
 3HUPRQR % 8JUDVHQD ,'* +HPRJORELQ DEQRUPDO ,Q
3HUPRQR +% 6XWDU\R 8JUDVHQD ,'* :LQGLDVWXWL (
Abdulsalam M. Buku ajar hematologi-onkologi anak. Jakarta:
,NDWDQ'RNWHU$QDN,QGRQHVLDS
 ,VPDLO $ &DPSEHOO 0- ,EUDKLP +0 -RQHV */ +HDOWK
related quality of life in Malaysian children with thalassaemia.
VFRUH6'LQWKHFDVHJURXS
6WXGLHVRQTXDOLW\RIOLIHRIWKDODVVHPLFFKLOGUHQ
have been limited, but needed. A study in Malaysia
LQDQGWKHQDQRWKHULQ7KDLODQGLQXVHG
WKH3HGV4/YHUVLRQTXHVWLRQQDLUHWRDVVHVVWKH
quality of life of thalassemic children compared to
normal children as a control. These study reported
that the quality of life of thalassemic children was
significantly lower comparared to control group.
Effects of thalassemia on quality of life included
physical, social, emotional and school issues. A study
in Malaysia reported that school function was the
parameter most affected. Thalassemic children are
frequently absent from school, since they routinely
go to the hospital for blood transfusions and iron
chelating therapy.
7KLV LV WKH ILUVW VWXG\ IURP 1RUWK 6XPDWUD
Indonesia used to assess quality of life in children with
chronic illness, in particular, thalassemia. Our findings
168‡Paediatr Indones, Vol. 51, No. 3, May 2011
%LRPHG
 (UPD\D <6 +LOPDQWR ' 5HQLDUWL / +XEXQJDQ NDGDU
KHPRJORELQVHEHOXPWUDQVIXVLGDQ]DWSHQJLNDWEHVLGHQJDQ
kecepatan pertumbuhan penderita thalassemia mayor.
0DMDODK.HGRNWHUDQ,QGRQHVLD
 :HDWKHUDOO'-&OHJJ-%,QKHULWHGKDHPRJORELQGLVRUGHUV
an increasing global health problem. Bull WHO.

5. Ganie RA. Thalassemia: permasalahan dan penanganannya.
3UHVHQWHGDW3URIHVVRUVKLS,QDJXUDWLRQ8QLYHUVLW\RI1RUWK
6XPDWUD0HGDQ1RYHPEHU
6. Thavorncharoensap M, Torcharus K, Nuchprayoon I,
Riewpaiboon A, Indaratna K, Ubol B. Factors affecting
health-related quality of life in Thai children with
WKDODVVHPLD%LRPHG
 3LJQDWWL &% 5XJRORWWR 6 6WHIDQR 3 =KDR + &DSSHOOLQL
0' 9HFFKLR *& HW DO 6XUYLYDO DQG FRPSOLFDWLRQV LQ
patients with thalassemia major treated with transfusion and
GHIHUR[DPLQH+DHPDWRO
 Masyitah Sri Wahyuni et al: Quality of life assessment of children with thalassemia
 5XQG ' 5DFKPLOHZLW] ( E thalassemia. N Eng J Med.

 0DUHQJR5RZH$-7KHWKDODVVHPLDVDQGUHODWHGGLVRUGHUV
3URF
 7HOIHU3&RQVWDQWLQLGRX*$QGUHRX3&KULVWRX60RGHOO
%$QJDVWLQLRWLV04XDOLW\RIOLIHLQWKDODVVHPLD$QQ1<
$FDG6FL
 0D]]RQH/%DWWDJOLD/$QGUHR]]L)5RPHR0$0D]]RQH'
Emotional impact in E thalassaemia major children following
cognitive behavioural family therapy and quality of life of
FDUHJLYLQJPRWKHUV%LRPHG
 6KDOLJUDP ' *LULPDML 6& &KDWXWYHGL 6. 3V\FKRORJLFDO
problems and quality of life in children with thalassemia.
,QGLDQ-3HGLDWU
 $OL03XWUD67*DWRW'6DVWURDVPRUR6/HIWYHQWULFXODU
functions and mass of the adolescents and young adults
ZLWKWKDODVVHPLDPDMRUDQHFKRFDUGLRJUDSK\VWXG\3DHGLDWU
,QGRQHV
 )LFD6$OEX$9ODGDUHDQX)%DUEX&%XQJKH]51LWX
L, et al. Endocrine disorders in E thalassemia major: cross-
VHFWLRQDOGDWD$FWD(QGRFULQRO
 $EHW] / %DODGL -) -RQHV 3 5RIDLO ' 7KH LPSDFW RI LURQ
overload and its treatment on quality of life: results from a
OLWHUDWXUHUHYLHZ%LRPHG
 9LQFHQW.$+LJJLQVRQ,-$VVHVVLQJTXDOLW\RIOLIHLQFKLOGUHQ
,Q&DUU$-+LJJLQVRQ,-5RELQVRQ3*HGLWRUV%0-ERRNV
/RQGRQ%ULWLVK/LEUDU\&DWDORJXLQJS
 6LWDUHVPL013HQLODLDQNXDOLWDVKLGXS\DQJEHUKXEXQJDQ
 6KDUJKL$.DUEDNKVK01DEDHL%0H\VDPLH$)DUURNKL$
'HSUHVVLRQLQPRWKHUVRIFKLOGUHQZLWKWKDODVVHPLDRUEORRG
PDOLJQDQFLHVDVWXG\IURP,UDQ%LRPHG
 7HVWD 0$ 6LPRQVRQ '& $VVHVVPHQW RI TXDOLW\ RI OLIH
RXWFRPHV1(QJO-0HG
 2OLYLHUL 1) :HDWKHUDOO '- 7KDODVVHPLDV ,Q $UFHFL 5-
+DQQ,06PLWK23HGLWRUV3HGLDWULFKHPDWRORJ\$XVWUDOLD
%ODFNZHOO3XEOLVKLQJS
16. Wahidiyat WI. Genetic problems at present and their
FKDOOHQJHVLQWKHIXWXUHWKDODVVHPLDDVDPRGHO3DHGLDWU
,QGRQHV
17. Honig GR. Thalassemia syndromes. In: Behrman RE,
Kliegman RM, Arvin AN, penyunting. Nelson textbook of
pediatrics. 15th HG 3KLODGHOSKLD 6DXQGHUV (OVHYLHU 
S
 )XQJ(%;X<.ZLDWNRZVNL-/9RJLDW]L0*1HXIHOG(
Olivieri N, et al. Relationship between chronic transfusion
therapy and body composition in subjects with thalassemia.
-3HGLDWU
GHQJDQNHVHKDWDQKHDOWKUHODWHGTXDOLW\RIOLIH3UHVHQWHGDW
4th$QQXDO6FLHQWLILF0HHWLQJ,QGRQHVLDQ3HGLDWULFV6RFLHW\
)HEUXDU\0HGDQ,QGRQHVLD
 9DUQL-:%XUZLQNOH70.DW](50HHVNH.'LFNLQVRQ3
7KH3HGV4/TM in pediatric cancer reliability and validity of
the pediatric quality of life inventoryTM generic core scales,
multidimensional fatigue scale, and cancer module. Am
&DQF6RF
 9DUQL -: 6HLG 0 .XUWLQ 36 3HGV4/  UHOLDELOLW\ DQG
validity of the pediatric quality of life inventory TM version
JHQHULFFRUHVFDOHVLQKHDOWK\DQGSDWLHQWSRSXODWLRQ
0HG&DUH
 %DDUV50$WKHUWRQ&,.RRSPDQ+0%XOOLQJHU03RZHU
0',6$%.,'6JURXS7KH(XURSHDQ',6$%.,'6SURMHFW
development of seven condition-specific modules to measure
health related quality of life in children and adolescents.
%LRPHG
 (ZLQJ-(.LQJ076PLWK1)9DOLGDWLRQRIPRGLILHGIRUPV
RIWKH3HGV4/JHQHULFFRUHVFDOHVDQGFDQFHUPRGXOHVFDOHV
 )XFKV*-7LHQERRQ3/LQSLVDUQ61LPVDNXO6/HHODSDW3
7RYDQDEXWUD6HWDO1XWULWLRQDOIDFWRUVDQGWKDODVVDHPLD
PDMRU$UFK'LV&KLOG
 ,NUDP 1 +DVVDQ . <RXQDV 0 $PDQDW 6 )HUULWLQ OHYHOV
LQ SDWLHQWV RI EHWD WKDODVVDHPLD PDMRU ,QWHU - 3DWKRO

 6DLG06DVWURDVPRUR6*DWRW'6XSUL\DWQR%$QDQWD<
Comparison of pulmonary function of thalassemic and of
IRUDGROHVFHQWVDQG\RXQJDGXOWV$<$ZLWKFDQFHURUD
EORRGGLVRUGHUV4XDO/LIH5HV
 9DUQL -: 6KHUPDQ 6$ %XUZLQNOH 70 'LFNLQVRQ 3(
'R[RQ37KH3HGV4/TM family impact module: preliminary
UHOLDELOLW\DQGYDOLGLW\%LRPHG
 8SWRQ 3 (LVHU & &KHXQJ , +XWFKLQJV +$ -HQQH\ 0
Maddocks A, et al. Measurement properties of the UK-
(QJOLVKYHUVLRQRIWKHSHGLDWULFTXDOLW\RIOLIHLQYHQWRU\
KHDOWK\FKLOGUHQ3DHGLDWU,QGRQHV 3HGV4/JHQHULFFRUHVFDOH%LRPHG

Paediatr Indones, Vol. 51, No. 3, May 2011 ‡169