Quality of life in thalassemia: A comparison of SF‐36 results from the thalassemia longitudinal cohort to reported literature and the US norms

Abstract

Thalassemia is a chronic, inherited blood disorder, which, in its most severe form, causes life-threatening anemia. Advances in treatment have led to increased life expectancy however the need for chronic blood transfusions and chelation therapy remains a significant burden for patients. Our study compared health related quality of life (HRQOL) from the Thalassemia Clinical Research Network's (TCRNs) Thalassemia Longitudinal Cohort (TLC) study to US norms and assessed association with clinical variables. There were 264 patients over age 14 who completed the Medical Outcomes Study 36-Item Short Form Health Survey version 2 (SF36v2) baseline assessment. When compared to US norms, TLC patients had statistically significant (p<0.05) worse HRQOL on 5 of the 8 subscales (physical functioning, role-physical, general health, social functioning and role-emotional) and on both summary scales (physical component summary and mental component summary). Women, older patients, and those with more disease complications and side effects from chelation reported lower HRQOL. In general, adolescents and adults with thalassemia report worse HRQOL than the US population, despite contemporary therapy. The SF-36 should become a standard instrument for assessing HRQOL in thalassemia to determine predictors of low HRQOL which may be better addressed by a multidisciplinary team.

Quality of life among Ardabil patients with beta-thalassemia major

“…Sobotaet al Investigated quality of life in a longitudinal thalassemia cohort aged over 14 years, compared with the norms in the United States, along with the influence of clinical factors. 21 Differing from our results, they showed worse quality of life among older patients. The results have also demonstrated that sex is influential in GH domain and the girls' status seemed to be better.…”

Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire

“…We did not find any significant relationship between age and the QoL scores. Sobota et al 23 investigated QoL in a longitudinal thalassemia cohort aged over 14 years, compared with the norms in the United States, along with the influence of clinical factors. Differing from our results, they showed worse QoL among older patients.…”

Deferiprone (GPO‐L‐ONE®) monotherapy reduces iron overload in transfusion‐dependent thalassemias: 1‐year results from a multicenter prospective, single arm, open label, dose escalating phase III pediatric study (GPO‐L‐ONE; A001) from Thailand

“…The majority of Thai patients also have elevated liver iron concentration (LIC) at a high risk level (>15 mg iron/g dry weight (dw) [12]), values associated with increased risk of cardiac complications in the longterm [13,14]. Although management for thalassemia patients has improved significantly in past recent years resulting in increased life expectancy, however the need for life-long blood transfusions and iron chelation remains a significant burden for patients and this results in a poor health-related quality of life (HRQOL) evaluation [15].…”