Quality of Life Evaluation in Saudi Arabian Pediatric Patients with Primary Immunodeficiency Diseases Receiving Subcutaneous IgG Infusions at Home

Abstract

Background and aims Subcutaneous immunoglobulin (SCIG) home infusion is widely used as an alternative to intravenous immunoglobulin (IVIG). The aim of this study is to determine the quality of life in primary immunodeficiency (PID) patient’s after switching to home based SCIG. Methods Prospective open-label, single center study. Quality of life (QoL) was determined using a validated Child Health Questionnaire (CHQ) (HealthActCHQ, Boston, MA, USA) in Arabic at baseline, 3 and 6 months after switching from IVIG to SCIG. Results From July 2018 to August 2021, 29 patients were recruited and 25 patient’s data were available for analysis: 15 females and 10 males. The median age was 5 years old (Range 0–14 years). The patient’s diagnoses were 11 severe combined immunodeficiency, 3 combined immunodeficiency, 2 agammaglobulinemia, 2 Omenn syndrome, 2 immunodysregulation, 2 hyper-IgE syndrome, 1 common variable immunodeficiency, 1 bare lymphocyte syndrome, and 1 chronic granulomatous disease. The median time on IVIG was 57 months (Range 3–125 months). The baseline mean serum IgG trough levels was 8.8 g/L ± 2.1. The mean serum IgG level was significantly higher on SCIG at 11.7 ± 2.3 and 11.7 ± 2.5 at 3 months and 6 months respectively. The QoL score showed a significant improvement in the child’s global health at 3 and 6 months compared to baseline (P-value 0.001 and 0.001 respectively) and a significant improvement in the child general health in the 3 and 6 months compared to the baseline (P-value 0.01 and 0.03 respectively). Conclusions This is the first study in an Arab population showing improvement in the QoL for PID patients after switching from hospital based IVIG to home based SCIG.