Health related quality of life in Middle Eastern children with beta-thalassemia.
BACKGROUND
Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL) in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries.


METHODS
We studied 60 thalassemia children from Middle Eastern countries with a median age of 10 years (range 5 to 17 years). HRQoL was assessed with the Pediatric Quality of Life Inventory (PedsQL) 4.0. The Questionnaire was completed at baseline by all patients and their parents. The agreement between child-self and parent-proxy HRQoL reports and the relationship between HRQoL profiles and socio-demographic and clinical factors were investigated.


RESULTS
The scores of parents were generally lower than those of their children for Emotional Functioning (mean 75 vs 85; p = 0.002), Psychosocial Health Summary (mean 70.3 vs 79.1; p = 0.015) and the Total Summary Score (mean 74.3 vs 77.7 p = 0.047). HRQoL was not associated with ferritin levels, hepatomegaly or frequency of transfusions or iron chelation therapy. Multivariate analysis showed that a delayed start of iron chelation had a negative impact on total PedsQL scores of both children (p = 0.046) and their parents (p = 0.007).


CONCLUSIONS
The PedsQL 4.0 is a useful tool for the measurement of HRQoL in pediatric thalassemia patients. This study shows that delayed start of iron chelation has a negative impact on children's HRQoL.
Centro Trapianti di Midollo Osseo, P,O, "R, Binaghi", Via Is Guadazzonis, 3, 09126, Cagliari, Italy. gcaocci@alice.it.
Giovanni
G
Caocci
Fabio
F
Efficace
Francesca
F
Ciotti
Maria Grazia
MG
Roncarolo
Adriana
A
Vacca
Eugenia
E
Piras
Roberto
R
Littera
Raji Suleiman Dawood
RS
Markous
Gary Stephen
GS
Collins
Fabio
F
Ciceri
Franco
F
Mandelli
Sarah
S
Marktel
Giorgio
G
La Nasa