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.Electronic supplementary materialThe online version of this article (doi:10.1007/s10875-014-0072-x) contains supplementary material, which is available to authorized users.

A cohort of French pediatric patients with primary immunodeficiencies: are patient preferences regarding replacement immunotherapy fulfilled in real-life conditions?

“…In comparison with healthy children, our patients rated lower for physical functioning (IVIg patients), bodily pain, general health, and parental impact – time, although the differences did not reach significance. Similar results have been reported previously 37–40,44. We found no difference on the behavioral subscale, as previously highlighted by Titman et al40…”

“…Similar results have been reported previously 37–40,44. We found no difference on the behavioral subscale, as previously highlighted by Titman et al40…”

“…Most studies on patients’ QoL and satisfaction with substitutive immunotherapy have mixed cohorts of children and adult patients,34,35 and few have reported results of specific analyses on pediatric patients 30–32,36–40. These cohorts however included a few children (aged 8–37 years), and most of them were receiving IVIg.…”

Psychological burden of pediatric primary immunodeficiency

“…According to limited research in this group, children with PID are at increased risk of school absenteeism, restriction of participation in social and sport activities, and of anxiety and depression symptoms . Another study found that children with PID have higher psychosocial difficulties such as emotional difficulties, peer relationship difficulties and hyperactivity . In another study in which patients with 22q.11 deletion syndrome were evaluated, social obstacles and attention problems were the most important difficulties in these patients .…”

“…10 Another study found that children with PID have higher psychosocial difficulties such as emotional difficulties, peer relationship difficulties and hyperactivity. 12,13 In another study in which patients with 22q.11 deletion syndrome were evaluated, social obstacles and attention problems were the most important difficulties in these patients. 9 All of these studies indicate that children with PID are at risk for psychosocial problems.…”