title: Quality of life in patients with thalassemia intermedia compared to thalassemia major.
abstract: The impact of thalassemia major and thalassemia intermedia and their associated complications on quality of life (QOL) is largely unknown. Determining the degree of health impairment as perceived by the patient is essential information needed to recommend suitable therapy. The objective of this study was to evaluate QOL in transfusion-independent patients with thalassemia (non-Tx) compared with that in transfused patients (Tx) and to identify the factors that affect QOL in thalassemia. A convenient sample of 48 thalassemia patients (29 Tx and 19 non-Tx) with mean age of 14.6 years (SD = 7.5 years) were selected during a comprehensive visit to complete a Dartmouth Primary Care Cooperative Information Chart System (COOP) questionnaire. Patients rated QOL from excellent (1) to poor (5) on five dimensions of health status. Scores of 4 or 5 represent major limitations. These results were augmented by a brief medical history and chart review. Forty-one percent of Tx patients and 47% of non-Tx patients reported severe impairments in 1-6 and 1-2 domains, respectively. The most commonly reported affected domains were feelings such as anxiety, depression, and concern of overall health status or indications of recent deterioration in health. In contrast with previous beliefs, transfusion-independent thalassemia patients also suffer serious impairment in QOL. Presented data suggest that all patients with thalassemia undergo QOL assessment so that interventions focused on affected domains can be implemented.
authors.affiliation: Children's Hospital & Research Center at Oakland, 747 52nd St., Oakland, CA 94609, USA. zpakbaz@mail.cho.org
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pakbaz
authors.forename: Marsha
authors.initials: M
authors.lastname: Treadwell
authors.forename: Robert
authors.initials: R
authors.lastname: Yamashita
authors.forename: Keith
authors.initials: K
authors.lastname: Quirolo
authors.forename: Drucilla
authors.initials: D
authors.lastname: Foote
authors.forename: Laura
authors.initials: L
authors.lastname: Quill
authors.forename: Titi
authors.initials: T
authors.lastname: Singer
authors.forename: Elliott P
authors.initials: EP
authors.lastname: Vichinsky
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion / Q000523:psychology; D002140:California; D002648:Child; D004644:Emotions; D005260:Female; D006801:Humans; D008297:Male; D010146:Pain / Q000209:etiology; D010809:Physical Fitness; D011788:Quality of Life; D012919:Social Behavior; D012944:Social Support; D011795:Surveys and Questionnaires; D017086:beta-Thalassemia / Q000145:classification / Q000150:complications / Q000523:psychology* / Q000628:therapy