Significant Behavioral Problems in Immunodeficient Children

Children with pediatric immunodeficiency disorders (PIDDs) have an increased risk of behavioral and emotional difficulties according to a study published this month in Annals of Allergy, Asthma & Immunology, the scientific journal of the American College of Allergy, Asthma and Immunology (ACAAI).

Carrie Piazza-Waggoner, Ph.D., post-doctoral fellow at Cincinnati Children's Hospital Medical Center, Cincinnati, and colleagues reported that children with immunodeficiency disorders are highly susceptible to infections and have higher rates of lung disease and asthma, cancer, and gastrointestinal complications than the general population.

Their investigation evaluated 20 children with PIDDs and 20 children with asthma to compare the chronic disease burden. Responses of the children and their caregivers to validated psychosocial questionnaire forms were compared to normal data.

"We found that children with PIDDs in general had increased rates of psychological difficulties, with the exception of conduct problems, when compared with a normative sample," wrote the authors. They identified the problem areas including depression, anxiety, somatization, social withdrawal and difficulties with social skills.

Since children with greater disease severity had poorer psychosocial outcomes, authors suggest that clinicians "should recognize that disease severity seems to be a good indicator for more closely monitoring psychosocial issues in patients with PIDDs."

When surveyed about themselves, 20 percent of parents of youth with PIDDS reported significant elevations of psychological distress. Authors noted that this may account for some of the discrepancy between the caregiver and child reports on the child's behavior, since distressed caregivers may view their child's behavior more negatively.

Older children with PIDDs reported having lower social skills, lower leadership skills and greater general anxiety compared with their younger counterparts.

"This study highlights the need for further research in psychosocial functions of children with PIDDs in an effort to develop interventions to promote their overall adjustment," the authors concluded.

The first national survey by the Immune Deficiency Foundation estimated a total of 50,000 diagnosed cases of primary immunodeficiency diseases (PIDs), of which approximately 40 percent are children and adolescents younger than 18 years.

Editorial author Chitra Dinakar, M.D., associate professor, Department of Pediatrics at the University of Missouri-Kansas City, wrote that the most common diagnoses in the Foundation's survey responses were common variable immunodeficiency (CVID, 34 percent), IgG subclass deficiency (24 percent), IgA subclass deficiency (17 percent) and X-linked agammaglobulinemia (8 percent).

The diagnosis of PID was frequently delayed according to the Foundation's survey findings, resulting in a heavy burden of disease. Patients often experienced two or more hospitalizations and repeated infections before diagnosis, noted Dr. Dinakar.