Saida Ridao-Manonellas1, Anna Fábregas-Bofill1, Gloria Núñez-Rueda1, Míriam González-Amores1, Marina García-Prat1, Laura López-Seguer1, Jacques G Rivière1, Andrea Martín-Nalda1, Natalia Mendoza-Palomar1, Susana Melendo-Pérez1, Pere Soler-Palacín2,3. 1. Paediatric Infectious Diseases and Immunodeficiencies Unit (UPIIP), Hospital Infantil Vall d'Hebron (HIVH), Jeffrey Modell Foundation Excellence Centre, ERN RITA Centre, Vall d'Hebron Research Institute (VHIR), Passeig Vall d'Hebron 119-129, 08035, Barcelona, Catalonia, Spain. 2. Paediatric Infectious Diseases and Immunodeficiencies Unit (UPIIP), Hospital Infantil Vall d'Hebron (HIVH), Jeffrey Modell Foundation Excellence Centre, ERN RITA Centre, Vall d'Hebron Research Institute (VHIR), Passeig Vall d'Hebron 119-129, 08035, Barcelona, Catalonia, Spain. psoler@vhebron.net. 3. Universitat Autònoma de Barcelona, Barcelona, Catalonia, Spain. psoler@vhebron.net.
Abstract
PURPOSE: Patients with primary immunodeficiency disease (PID) have an increased risk of experiencing physical activity limitations, social difficulties, and psychological problems due to their chronic condition. Evaluation of their health-related quality of life (HRQOL) and fatigue is crucial in these patients to help understand their complex disease and provide adequate medical care. METHODS: In this study, we evaluated HRQOL and fatigue in pediatric and young adult patients with PID attending our center. Participants completed the Pediatric Quality of Life Inventory (PedsQL), version 4.0, and the PedsQL multidimensional fatigue module, standard version. RESULTS: Fifty-three PID patients were recruited (age range: 2-23 years). The mean HRQOL score obtained was 66.61 (SD: 18.73) out of 100, and the emotional and work/school dimensions were the ones most highly affected. There were no significant differences in reported quality of life between patients and their caregivers. The mean patient-reported fatigue value was 68.81 (SD: 17.80) out of 100, and the rest-related dimension was the one most highly affected. In the caregivers' assessment, general fatigue was the most highly affected dimension. CONCLUSIONS: The results of this study show that quality of life is poor and fatigue measures are considerably increased in our young adult and pediatric patients with PIDs. These findings can indicate areas requiring more intensive interventions, and they will serve as a basis for comparison of future results.
PURPOSE:Patients with primary immunodeficiency disease (PID) have an increased risk of experiencing physical activity limitations, social difficulties, and psychological problems due to their chronic condition. Evaluation of their health-related quality of life (HRQOL) and fatigue is crucial in these patients to help understand their complex disease and provide adequate medical care. METHODS: In this study, we evaluated HRQOL and fatigue in pediatric and young adult patients with PID attending our center. Participants completed the Pediatric Quality of Life Inventory (PedsQL), version 4.0, and the PedsQL multidimensional fatigue module, standard version. RESULTS: Fifty-three PIDpatients were recruited (age range: 2-23 years). The mean HRQOL score obtained was 66.61 (SD: 18.73) out of 100, and the emotional and work/school dimensions were the ones most highly affected. There were no significant differences in reported quality of life between patients and their caregivers. The mean patient-reported fatigue value was 68.81 (SD: 17.80) out of 100, and the rest-related dimension was the one most highly affected. In the caregivers' assessment, general fatigue was the most highly affected dimension. CONCLUSIONS: The results of this study show that quality of life is poor and fatigue measures are considerably increased in our young adult and pediatric patients with PIDs. These findings can indicate areas requiring more intensive interventions, and they will serve as a basis for comparison of future results.
Entities:
Keywords:
Primary immunodeficiencies; adolescence; children; fatigue; health-related quality of life
Authors: Matthew F Smout; Gian Mauro Manzoni; Sofia Tamini; Nicoletta Marazzi; Alessandra De Col; Giada Pietrabissa; Gianluca Castelnuovo; Enrico Molinari; Alessandro Sartorio Journal: Health Qual Life Outcomes Date: 2022-01-10 Impact factor: 3.186
Authors: Linde N Nijhof; Marco van Brussel; Esther M Pots; Raphaële R L van Litsenburg; Elise M van de Putte; Joris M van Montfrans; Sanne L Nijhof Journal: J Clin Immunol Date: 2021-03-17 Impact factor: 8.317