Pain as an emergent issue in thalassemia

Abstract

Thalassemia is a congenital blood disorder often requiring chronic blood transfusions and iron chelation therapy [1,2]. While advances in treatment have resulted in increased life expectancy [3], extended life spans have exposed previously unidentified issues, including bodily pain. The aim of this study was to examine the prevalence, severity, predictors, and effects of pain in 265 adults/adolescents and 103 children with thalassemia. Overall, 69% of adults/adolescents reported bodily pain on the SF-36v2 health survey, with 28% reporting at least moderate pain. Parents reported pain in 56% of children using the PF-28 child health questionnaire, with only 11% reporting pain fairly often. There were no significant differences in pain in children with thalassemia compared with the general population. In adults/adolescents, pain increased significantly with age (P = 0.005), more so than in the general population. This study highlights the fact that children and young adults with thalassemia experience pain comparable to the general population, whereas older adults (aged 35+) experience greater pain. Our findings show that increased pain is associated with decreased quality of life and increased anxiety and depression.

Figure 1

Bodily pain: thalassemia cohort by country versus U.S. norms. (A) Adults (Bodily pain (BP) scale of the SF-36v2 quality of life health survey compared with U.S. adult norms [16]. Higher scores indicate higher QOL (i.e., less pain). There are no normed data available for age <18.). (B) Children (Bodily pain (BP) scale of the PF-28 child health questionnaire (CHQ) compared with U.S. pediatric norms [17]. Higher scores indicate higher QOL (i.e., less pain).). As norms differ by culture, only thalassemia patients in North America are compared with U.S. norms; age trends are similar for patients in the U.K., but scores are consistently lower than in the North American group.

Figure 2

Quality of Life by pain severity (excludes patients from the U.K. because of significant country effects on these scales; however, U.K. patients show similar trends). (A) PCS and MCS summary scales of the SF-36 in adults/adolescents (Physical Component Summary and Mental Component Summary scales of the SF-36v2 health survey administered to participants aged 14+ years. Higher scores indicate higher QOL.). (B) Anxiety and Depression in adults/adolescents (Hospital Anxiety and Depression Scale (HADS) administered to participants aged 141 years. Higher scores indicate higher levels of anxiety and depression.). (C) PhS and PsS summary scales of the CHQ in children (Physical Summary and Psychosocial Summary scales of the PF-28 child health questionnaire (CHQ) for participants <14 years. Higher scores indicate higher QOL.).