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Health-related quality of life of children with primary immunodeficiency disease: a comparison study
Kathy Zebracki1, Tonya M Palermo, Robert Hostoffer
1Department of Psychology, Case Western Reserve University, Cleveland, Ohio, USA.
Insights
Children with primary immunodeficiency (PI) disease receiving intravenous immunoglobulin infusions report similar health-related quality of life (HRQOL) to those with juvenile idiopathic arthritis (JIA) but lower HRQOL than healthy children.
Area of Science:
- Pediatric immunology
- Quality of Life Research
- Chronic disease management
Background:
- Primary immunodeficiency (PI) disease management has improved, yet children face ongoing physical, social, and psychological challenges.
- Intravenous immunoglobulin (IVIg) infusion is a common treatment for PI disease symptoms.
- Limited empirical data exists on the health-related quality of life (HRQOL) for children with PI disease undergoing IVIg treatment.
Purpose of the Study:
- To compare parental reports of HRQOL in children with PI disease receiving IVIg with those of children with juvenile idiopathic arthritis (JIA) and healthy children.
- To identify specific HRQOL domains affected in children with PI disease.
Main Methods:
- A cross-sectional study involving 108 children aged 4-18 years.
- Participants included children with PI disease (n=36), children with JIA (n=36), and healthy controls (n=36).
- HRQOL was assessed using the Child Health Questionnaire-Parent Report version.
Main Results:
- Children with PI disease showed similar HRQOL to children with JIA in several domains.
- Parents of children with PI disease reported greater limitations in personal time, poorer general health, reduced physical functioning, and fewer family activities compared to parents of children with JIA.
- Children with PI disease scored lower on most HRQOL domains compared to healthy children.
Conclusions:
- Children with PI disease experience a comparable HRQOL to those with JIA, but a diminished HRQOL relative to healthy peers.
- Findings highlight potential areas for targeted medical and psychosocial interventions to improve HRQOL in children with PI disease.
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.
