The mental state characteristics, quality of lives and family characteristics in patients with serious primary immune deficiency syndrome who receive intravenous immunglobulin treatment

Objective: Primary immune deficiency (PID) is one of the childhood chronical diseases which causes severe morbidity and mortality. In recent years survival rates have increased with the advancement of the treatment of this disorder. However, these patients and their families deal with difficulties of this chronic condition. The burden of such a chronic disorder may increase the prevalence of psychopathologies and deteriorate their quality of lives. Nevertheless, there is sparse of studies which investigate the quality of life and psychopathology in this population. In this study, we aimed to assess and compare children with PID, who receive intravenous immunoglobulin (IVIG) treatment, Juvenile Idiopathic Arthritis (JIA) and healthy controls with respect to their quality of life and mental health status. Methods: Forty-four children with PID, 32 children with JIA and 30 healthy controls, whose ages were between 4-18 years, were included the study. Schedule for Affective Disorders and Schizophrenia for School-Age Children- Present and Lifetime Version was administered in the psychiatric evaluation of participants. Children forms of Pediatric Quality of Life Questionnare, Childhood Depression Inventory, and Screen for Child Anxiety and Related Disorders were filled by participants. Parent forms of Pediatric Quality of Life Questionnaire, Children Behavior Checklist (CBCL), and The Symptom Checklist-90-Revised (SCL-90-R) were completed by mothers of participants. Results: Differences were detected among children with PID on IVIG therapy, JIA, and healthy controls with respect to the prevalence of psychopathology. This result was due to the difference of the frequency of mood disorders among these three groups. Nevertheless, there was no difference between the groups of PID and JIA with respect to the prevalence of mood disorders and other psychopathologies. The quality of life was found reduced in the PID and JIA groups relative to the healthy controls while there was no difference between the PID and JIA groups. According to SCL-90-R, mothers stated more psychiatric symptoms in both patient groups relative to controls whereas no substantial distinction was found between the PID and JIA groups. Conclusions: As a conclusion, the rate of psychopathology found similar in patients with PID and JIA and higher than the controls. In addition, quality of life scores were found similar in patient groups and lower than the healthy controls. Frequency of hospital visits and IVIG treatment sessions, the number of undergone infection, and severity level of illness were found associated with the frequency of observed mood disorders in children with PID. Similarly, mental state features of the mothers of the PID and JIA groups were similar but differed from the healthy controls. In PID group, we found an association between the prevalance of psychopathology and mood disorder of children and stated psychiatric symptoms of their mothers.