Zebracki K, Palermo TM, Hostoffer R, Duff K, Drotar D. Ann Allergy Asthma Immunol. 2004;93:557–561
Purpose of the Study.
To compare parental perceptions of health-related quality of life (HRQOL) in children with primary immunodeficiency (PI) with children with juvenile idiopathic arthritis (JIA) and healthy children.
Thirty-six children in each of 3 groups (108 total): those with PI, those with JIA, and those who were healthy. Patients were matched for age, ethnicity, and parental marital status. The age ranged from 4 to 18 years, and 94% were white. All patients with PI received regular infusions of intravenous immunoglobulin. Of the patients with JIA, 77% had either oligoarthritis or polyarthritis. The JIA group had a significantly higher proportion of females.
Parents were interviewed and completed the Child Health Questionnaire-Parental Form 50. Treating physicians completed forms documenting any complications of the underlying disease.
In comparison to healthy children, those with PI had significantly lower scores on physical functioning, school and social activities, limitations on parental time and family activities, and parental emotional distress. They were equivalent to the healthy group with respect to overall psychosocial health, daily pain and discomfort, social limitations, self-esteem, mental health, general behavior, and family cohesion. In comparison to the JIA group, children with PI were similar. However, they scored lower than the JIA group with respect to perception of general health and limitations on parental time and family activities. The children with JIA had more bodily pain and discomfort than the children with PI.
Children with PI have significant impairment in several measures of HRQOL in comparison to healthy children. These limitations are similar to, and in some cases more severe than, those occurring in another group of chronically ill children, those with JIA.
This study begins to fill the gap in our understanding of the impact of PI on the quality of life of children and families. Despite some limitations in size and scope, it is clear that HRQOL in children and families with PI is impaired (even when the disease is treated with intravenous immunoglobulin) to a degree that is on a par with other serious chronic disorders that are generally better recognized. Overall, PI is underdiagnosed, to what extent is unknown. This study suggests that not only is there room for improvement in HRQOL aspects of disease management or patient care in those who already have a diagnosis of PI but also implies that there are gains in HRQOL to be made with improved diagnosis of PI.