Quality of life in children and adults with primary immunodeficiencies
Published online: February 20, 2019
Primary Immunodeficiency Diseases (PID) are a heterogeneous group of inherited disorders. Around 6 million people may be living with a PID worldwide. Patients with PID are generally more prone to develop recurrent infections and/or autoimmune diseases. PID significantly influences patient quality of life, limiting their working ability, physical and social activities. This group of conditions has been linked with a few negative effects on physical and social activities and is associated with increased anxiety and fatigue. Despite an increasing number of papers on health-related quality of life in PID patients, little attention has been paid to the systematic evaluation of available data. Improved knowledge can highlight unmet needs, stimulating further research in the field and improve our understanding of patient needs.
To analyze the current knowledge on the health-related quality of life in PID patients Peshko, Kulbachinskaya (Titova) and co-authors performed a comprehensive review of all available evidence focusing on 2 aspects: (1) assessment of the PID effect on patient quality of life and (2) identification of strengths and weaknesses in existing approaches to the quality of life assessment. As a basis of this review, a comprehensive literature search was performed and all human studies, published in English and available in medical databases, were analyzed. The results of this systematic review are now published in The Journal of Allergy and Clinical Immunology: In Practice.
Authors found that The Child Health Questionnaire - Parent Form 50 (CHQ-PF50) in children and the Short Form Survey (SF-36) in adults, were the most frequently used instruments for the health-related quality of life assessment across studies. Surprisingly there are no disease-specific instruments available for quality of life assessment in children. Health-related quality of life (HRQL) is significantly lower in PID adults and children compared to both healthy individuals and patients with other chronic conditions, such as diabetes mellitus and juvenile idiopathic arthritis. Evidence from a small number of studies suggests an agreement between parental- and child-reported health-related quality of life, but parents report child school-related health-related quality of life as more impaired than children. However, we note that the research reviewed here is cross-sectional and has not assessed changes in child–parent agreement over time.
There is an urgent need to develop PID-specific instruments and rigorously evaluate existing measures. This would allow for a more sensitive measurement of treatment efficiency, as well as the impact of PID on patient health and well-being. Furthermore, recognizing the role of HRQL in PID outcomes may constitute an important step in selecting and supporting those patients for whom therapy is appropriate and who will benefit most from a particular treatment. It can also help us to tailor both clinical and psycho-social interventions to the characteristics and needs of patients and to provide targeted support for patients who would benefit from treatments, but only with specific care.
The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.