Quality of life in children with primary antibody deficiency

Abstract

Primary antibody deficiency disorders (PADs) can have an excellent outlook if diagnosed early and treated appropriately, but require lifelong treatment with immunoglobulin replacement. Some carry risks of inflammatory complications even with optimal treatment. Quality of life (QoL) and the psychological impact of PADs has been relatively little studied, particularly in children. The purpose of this study was to evaluate QoL and psychological impact in a large group of children affected by a range of PADs, as well as a group with transient hypogammaglobulinemia of infancy (THI). Both parental and, where appropriate, child ratings, were collected using standardised questionnaires (PedsQL and SDQ). Higher rates of psychological difficulties, particularly emotional and peer-relationship difficulties were found in children with PAD when compared with healthy controls. Quality of life was poorer than in healthy controls, and also worse than in children affected by diabetes mellitus. Variations in QoL and the degree of psychological difficulties were found between specific diagnostic groups, with children affected by THI being amongst those with the lowest scores for QoL. Further studies are needed to corroborate and extend these findings, but this study confirms previous findings that primary antibody deficiency has a significant impact on quality of life and psychological well-being, and additionally suggests that the impact varies according to severity of the underlying condition. For those with significant difficulties psychological intervention at an early stage may be beneficial.

Fig. 1

Mean total parent and child SDQ scores for psychological difficulties (including conduct, emotional, peer relationship and hyperactivity scores). Higher scores indicate more difficulties, with a maximum possible score of 40. Error bars indicate 95 % confidence intervals; and statistically significant differences are indicated by an asterisk *

Fig. 2

Mean parent rated SDQ individual subscale scores. Higher scores indicate higher levels of psychological difficulties with a maximum possible score of 10. Error bars indicate 95 % confidence intervals; statistically significant differences are indicated by an asterisk *

Fig. 3

Mean child rated individual SDQ subscale scores. Higher scores indicate higher levels of psychological difficulty with a maximum possible score of 10.. Error bars are only shown for the emotional subscale; the intervals for other subscales are too large due to the small number of children who were old enough to complete this measure. Statistically significant differences are indicated by an asterisk *

Fig. 4

Mean parent rated overall quality of life and subscale scores for children with PAD, compared both with healthy children and with a group of children with diabetes mellitus included in the UK standardization study of the PedsQL [17]. Higher scores indicate better QoL and there is a maximum score of 100. Error bars indicate 95 % confidence intervals; statistically significant differences are indicated by an asterisk *

Fig. 5

Mean child rated overall quality of life and subscale scores compared both with healthy children and with a group of children with diabetes mellitus included in the UK standardization study of the PedsQL [17]. Higher scores indicate better QoL and there is a maximum score of 100.. Error bars indicate 95 % confidence intervals; statistically significant differences are indicated by an asterisk *

Fig. 6

Mean parent rated overall quality of life by diagnostic group Higher scores indicate better QoL and there is a maximum score of 100. For analysis of results by diagnosis the children with ‘CVID’ and ‘possible CVID’ have been amalgamated as ‘Combined’

Fig. 7

Mean child rated overall quality of life by diagnostic group Higher scores indicate better QoL and there is a maximum score of 100. For analysis of results by diagnosis the children with ‘CVID’ and ‘possible CVID’ have been amalgamated as ‘Combined’

Fig. 8

Mean clinician severity ratings for each of the diagnostic groups. The maximum score is 5, indicating most severe. For analysis of results by diagnosis the children with ‘CVID’ and ‘possible CVID’ have been amalgamated as ‘Combined’