Social outcome in children treated by haematopoietic cell transplant for congenital immunodeficiency

Abstract

Previous studies have reported increased rates of social difficulties in children treated by haematopoietic cell transplant (HCT). This study assessed social functioning in children with congenital immunodeficiency treated by HCT and investigated two potential underlying mechanisms that may explain social difficulties: executive function skills and physical appearance. In total, 31 children (8-16 years of age) were assessed on measures of social functioning and peer relationships, executive function and physical appearance. Results were compared with a control group of 31 healthy children, matched for age, gender, ethnicity and cognitive ability. Parent, teacher and self-report data were collected. HCT survivors were described by parents and teachers, but not by themselves, as experiencing more difficulties with social functioning than the control group. Executive function was not associated with social functioning. However, an objective measure of physical appearance was significantly associated with social functioning. Results suggest that children treated by HCT for congenital immunodeficiency do experience significant difficulties in social functioning, not solely accounted for by below average intelligence. These difficulties are associated with physical appearance, but not with executive functional skills. This has clinical implications for identifying and treating children at increased risk of difficulties with social functioning.

Quality of Life in Children with Primary Antibody Deficiency

“…This pattern of psychological difficulties is in contrast to studies of children in the general population, where the most common types of difficulty children experience are conduct or behavioural problems. However, it is consistent with studies in children with other types of primary immunodeficiencies [9, 10], which have also found higher rates of emotional and or peer relationship difficulties in affected children. At present there are no intervention studies specifically with children with these types of medical conditions.…”

A systematic review and meta-analysis of changes in cognitive functioning in adults undergoing hematopoietic cell transplantation

“…Study heterogeneity has resulted in limited power to examine potentially important predictors of cognitive change, such as severity of graft-versus-host disease and history of intensive chemotherapy prior to HCT, although more recent, higher-quality studies are starting to examine these questions (36). Similarly, recent studies are starting to examine impaired cognition as one aspect of a constellation of negative psychosocial effects of HCT (37, 38). Cooperative group studies have been suggested to address design limitations and increase statistical power (33).…”

How I treat severe combined immunodeficiency

“…Among the latter, we monitor for hearing deficit, which may be part of the original disorder, as in ADA SCID, 65 or a consequence of the HSCT procedure; dental defects as previously described 66 ; endocrine and fertility deficits in those receiving conditioning chemotherapy; and, importantly, neurobehavioral disorders, which we have shown to be potential problems in this group of patients. 64,67 Most families will want genetic and family planning counseling. Related to these, we will help facilitate preimplantation, prenatal, and neonatal diagnostic testing, and cord blood storage for potential future-directed cord blood transplants.…”