Health-related quality of life among a group of Egyptian children with inborn errors of immunity: a cross-sectional study

Abstract

Background: Inborn Errors of Immunity (IEI) significantly impact the patients’ life, limiting their physical and social activities. Health-related quality of life (HRQOL) can also be adversely influenced by the delay in diagnosis and treatment of infections.Methods: We sought to assess HRQOL of IEI patients at a major tertiary care hospital in Egypt by using the Arabic version of pediatric quality of life inventory generic core scale (PedQl 4.0) questionnaire answered by the patient or his/her parents.Results: We enrolled 50 IEI patients, 34 of them (68%) were males and 16 (32%) were females. Their mean ± SD age was 86.8 ± 36.9 months. The lowest HRQOL score was for school functioning with a mean score of 33.23 (SD=14.06). Social functioning score was significantly lower in older children (p=0.034). The rate and duration of hospitalization negatively affected social functioning. Pulmonary involvement in the form of bronchiectasis and interstitial lung disease caused a significant decline in quality of life. The procedures of receiving intravenous immunoglobulin (IVIG) therapy had no effect on the QOL.Conclusion: IEI patients suffer from a decreased quality of life in terms of school and physical functioning. Healthcare providers managing IEI pediatric patients should pay attention to their quality of life to improve their optimal academic achievement and emotional wellbeing.