Research article

BMC Blood Disorders

, 12:6

First online:

Open Access This content is freely available online to anyone, anywhere at any time.

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

  • Giovanni CaocciAffiliated withCentro Trapianti di Midollo OsseoDepartment of Hematology, University of Cagliari Email author 
  • , Fabio EfficaceAffiliated withHealth Outcomes Research Unit, Italian Group for Adult Hematologic Diseases (GIMEMA) Data Center
  • , Francesca CiottiAffiliated withPediatric Immuno-Hematology and BMT Unit, IRSS San Raffaele Hospital
  • , Maria Grazia RoncaroloAffiliated withPediatric Immuno-Hematology and BMT Unit, IRSS San Raffaele Hospital
  • , Adriana VaccaAffiliated withCentro Trapianti di Midollo Osseo
  • , Eugenia PirasAffiliated withCentro Trapianti di Midollo Osseo
  • , Roberto LitteraAffiliated withRegional Organ Transplantation Center, R. Binaghi Hospital
  • , Raji Suleiman Dawood MarkousAffiliated withThalassemia Center, Hevi Pediatric Hospital
  • , Gary Stephen CollinsAffiliated withCentre for Statistics in Medicine, University of Oxford
    • , Fabio CiceriAffiliated withPediatric Immuno-Hematology and BMT Unit, IRSS San Raffaele Hospital
    • , Franco MandelliAffiliated withHealth Outcomes Research Unit, Italian Group for Adult Hematologic Diseases (GIMEMA) Data Center
    • , Sarah MarktelAffiliated withPediatric Immuno-Hematology and BMT Unit, IRSS San Raffaele Hospital
    • , Giorgio La NasaAffiliated withCentro Trapianti di Midollo OsseoDepartment of Hematology, University of Cagliari

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