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Open Access Research article

Health related quality of life in Middle Eastern children with beta-thalassemia

Giovanni Caocci17*, Fabio Efficace2, Francesca Ciotti3, Maria Grazia Roncarolo3, Adriana Vacca1, Eugenia Piras1, Roberto Littera4, Raji Suleiman Dawood Markous5, Gary Stephen Collins6, Fabio Ciceri3, Franco Mandelli2, Sarah Marktel3 and Giorgio La Nasa17

Author affiliations

1 Centro Trapianti di Midollo Osseo, P.O. “R. Binaghi”, Via Is Guadazzonis, 3, 09126, Cagliari, Italy

2 Health Outcomes Research Unit, Italian Group for Adult Hematologic Diseases (GIMEMA) Data Center, Rome, Italy

3 Pediatric Immuno-Hematology and BMT Unit, IRSS San Raffaele Hospital, Milan, Italy

4 Regional Organ Transplantation Center, R. Binaghi Hospital, Cagliari, Italy

5 Thalassemia Center, Hevi Pediatric Hospital, Duhok, Iraq

6 Centre for Statistics in Medicine, University of Oxford, Oxford, UK

7 Department of Hematology, University of Cagliari, Cagliari, Italy

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Citation and License

BMC Blood Disorders 2012, 12:6  doi:10.1186/1471-2326-12-6

Published: 22 June 2012

Abstract

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.

Keywords:
Quality of life; Thalassemia; PEDsQL 4.0