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Health-Related Quality of Life and Multidimensional Fatigue Scale in Children with Primary Immunodeficiencies

Journal of Clinical Immunology volume 40, pages 602–609 (2020)Cite this article

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.

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Acknowledgments

We are deeply grateful to the affected individuals and their families who participated in this study. The authors thank Celine Cavallo for English language support and acknowledge Santiago Pérez-Hoyos for carrying out the statistical analysis. The program I Have A PID, But I Am Not Alone is conducted with the support of the Barcelona PID Foundation, the Catalan Association of Primary Immune Deficiencies (ACADIP), and the social work of La Caixa Foundation.

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Affiliations

  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

    Saida Ridao-Manonellas, Anna Fábregas-Bofill, Gloria Núñez-Rueda, Míriam González-Amores, Marina García-Prat, Laura López-Seguer, Jacques G. Rivière, Andrea Martín-Nalda, Natalia Mendoza-Palomar, Susana Melendo-Pérez & Pere Soler-Palacín

  2. Universitat Autònoma de Barcelona, Barcelona, Catalonia, Spain

    Pere Soler-Palacín

Authors
  1. Saida Ridao-Manonellas
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  2. Anna Fábregas-Bofill
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  3. Gloria Núñez-Rueda
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  4. Míriam González-Amores
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  5. Marina García-Prat
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  6. Laura López-Seguer
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  7. Jacques G. Rivière
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  8. Andrea Martín-Nalda
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  9. Natalia Mendoza-Palomar
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  10. Susana Melendo-Pérez
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  11. Pere Soler-Palacín
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Corresponding author

Correspondence to Pere Soler-Palacín.

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Electronic supplementary material

S-Table 1 Comparison of PedsQL quality of life scores between PID studies, mean and CI

S-Table 2 Comparison of PedsQL quality of life scores between healthy individuals and present study, mean and CI

S-Table 3 Comparison of PedsQL quality of life scores between chronic conditions and present study, mean and CI

S-Table 4 Comparison of fatigue scores between PID, healthy individuals, CF and present study, mean and CI

S-Table 5 Detailed data

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Ridao-Manonellas, S., Fábregas-Bofill, A., Núñez-Rueda, G. et al. Health-Related Quality of Life and Multidimensional Fatigue Scale in Children with Primary Immunodeficiencies. J Clin Immunol 40, 602–609 (2020). https://doi.org/10.1007/s10875-020-00775-w

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  • DOI: https://doi.org/10.1007/s10875-020-00775-w

Keywords

  • Primary immunodeficiencies
  • health-related quality of life
  • fatigue
  • children
  • adolescence