title: Impact of Burden of Thalassemia Major on Health-related Quality of Life in Omani Children.
abstract: To improve treatment outcome in young patients with homozygous β-thalassemia, evaluation of factors associated with health-related quality of life (HRQoL) is essential to develop clinical, counseling, social, and school support programs. This study was performed to evaluate HRQoL in Omani children with thalassemia who have not been previously evaluated. All patients with thalassemia (5 to 18 y) on regular hypertransfusion from August 2013 to February 2014 were included. Demographic-clinical information was obtained from electronic patient records. The 23-item Pediatric Quality of Life Inventory (PedsQL) 4.0 with 4 multidimensional scales and 3 summary scores was used to assess HRQoL by both patients and caregivers. Statistical analysis was performed using SPSS version 19. Delayed age at diagnosis and delayed initiation of chelation were independently associated with a negative impact on total PedsQL scores; children with higher pretransfusion hemoglobin and lower serum ferritin levels scored better overall. Patients self-rated their HRQoL scores lower as compared with caregivers. Although physical health was more affected than psychosocial health function from both patients' (72.26±22.34 vs. 74.55±18.25) and caregivers' (69.91±19.86 vs. 78.83±18.45) perspective, school functioning domain (68.05±21.30 and 69.71±18.96) scored the lowest. Low ratings for physical health and school functioning highlight the importance of adequate transfusion together with the need for a multidisciplinary teamwork by health care providers, social workers, counselors, family members, and school authorities to provide psychosocial support to prevent mental issues and improve academic performance in these young patients.
authors.affiliation: *Department of Child Health, Sultan Qaboos University Hospital †Department of Child Health, College of Medicine, Sultan Qaboos University, Muscat, Oman.
authors.forename: Surekha T
authors.initials: ST
authors.lastname: Mevada
authors.forename: Muna
authors.initials: M
authors.lastname: Al Saadoon
authors.forename: Mathew
authors.initials: M
authors.lastname: Zachariah
authors.forename: Abdul H
authors.initials: AH
authors.lastname: Al Rawas
authors.forename: Yasser
authors.initials: Y
authors.lastname: Wali
mesh_terms: D000293:Adolescent; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D057210:Delayed Diagnosis; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D016743:Mental Competency; D009850:Oman; D010809:Physical Fitness; D011788:Quality of Life; D017086:beta-Thalassemia / Q000175:diagnosis / Q000628:therapy