Quality of life and mental health of children with primary antibody deficiency and their caregivers

Primary antibody deficiency (PAD) is a chronic disorder primarily associated with recurrent infections. The treatment is lifelong immunoglobulin substitution. This thesis has two parts; a literature review and a clinical study, which evaluated the health-related quality of life (HRQOL) and mental health of children with PAD and their caregivers and the role of weekly subcutaneous immunoglobulin (SCIG) treatment as a stressor. From a literature search 19 papers on HRQOL and mental health of children with PAD were identified. Fifteen children with PAD (median age 12, range 9-17) and their caregivers participated in the clinical study. Data was assessed using standardized questionnaires (PedsQL, SDQ, GHQ-30, QOLS and IES-15). The literature review and cross-sectional study found children with PAD to have reduced quality of life when compared to normative values for healthy children. This was particularly evident for school functioning. Mothers, and to a lesser degree, fathers underestimated the child HRQOL. Compared to children with other chronic diseases, our clinical study found that children with PAD have reduced HRQOL. In the published literature, results are inconsistent. In our clinical study most children and caregivers tolerated the SCIG treatment well, but in a few it gave rise to severe stress responses. To improve HRQOL of children with PAD, a close collaboration with school may be valuable. The findings based on father-report gives an impression of fathers as more peripheral in paediatric PAD; closer inclusion of fathers is recommended. Clinicians should be sensitive to stress responses to treatment and alternative treatment options should be considered