Health-related quality of life of children with primary immunodeficiency disease: a comparison study

Background: Many symptoms of primary immunodeficiency (PI) disease can be successfully managed with intravenous immunoglobulin infusion. Although survival rates and prognosis have greatly improved, children with PI disease are still at risk for physical, social, and psychological problems owing to their chronic health condition. However, to our knowledge, there are no empirical data concerning health-related quality of life (HRQOL) in children with PI disease receiving intravenous immunoglobulin infusion. Objective: To compare parental reports of HRQOL of children with PI disease receiving intravenous immunoglobulin infusion with children with juvenile idiopathic arthritis (JIA) and a healthy sample. Methods: Demographic, illness, and HRQOL data were collected from parents of 4- to 18-year-old children with PI disease (n = 36), children with JIA (n = 36), and healthy children (n = 36). The HRQOL was evaluated using the Child Health Questionnaire-Parent Report version. Results: Compared with children with JIA, children with PI disease were similar in many aspects of their HRQOL. However, parents of children with PI disease reported greater limitations in their personal time, poorer general health of their children, greater limitations in their children's physical functioning and family activities, and less bodily pain than children with JIA. In contrast, children with PI disease scored lower on most HRQOL domains compared with healthy children. Conclusion: Children with PI disease experience similar HRQOL to children with JIA and poorer HRQOL than healthy children, indicating potential areas to be addressed by future medical and psychosocial interventions.