Most primary immunodeficiencies (PID) are characterized by recurrent infections. Evaluating quality of life (QoL) is important for patient care.
The main objective was to evaluate the quality of life of children with PID under intravenous replacement infusions of polyvalent immunoglobulins (IVIG). The second objective was to describe the adverse effects associated with IVIG and the social and financial impact of this therapy on families.
This was a cross-sectional study of forty cases of PID. All immunocompromised patients receiving intravenous replacement immunoglobulins in the Pediatric Department Immuno-Heamatology and Stem Cell Transplant of the National Bone Marrow Transplantation Center in Tunis were enrolled.
The mean age of the patients was 14 years ± 147.36 months (11 months to 47 years). The common variable immune deficiency CVIDs was the most common DIPs in our series (45%). Fourteen (35%) patients have IVIG-related reactions or side effects such as headaches (17%) and fever (10%). This therapy was the cause of school absenteeism in 81.8% of patients and professional absenteeism in 52% of parents. These patients had a reduced quality of life compared to the standards of the healthy subject, mainly for physical health. However, the overall score is not too far from norms, which is probably due to the protective action of IVIG. The emotional and social health of our patients did not differ from healthy controls. The best quality of life score was observed with infants, while the lowest was observed with patients over 18 years of age. Boys had significantly better quality of life than girls. There was no significant correlation between age and type of illness and quality of life. However, high level of residual immunoglobulin G was significantly correlated to better quality of life.
Despite regular immunoglobulin substitutions, the quality of life of these antibody-deficient patients was reduced by standards established for a healthy subject. This quality of life was directly related to the residual IgG level. Adverse events related to IVIG were common but not severe. Moreover, IVIG substitution resulted in additional costs for the family and considerable absenteeism rate. Subcutaneous infusions of immunoglobulins are an alternative that could alleviate these financial and social problems.
