Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease Activity

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

doi:10.1007/s10875-021-01013-7

Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease Activity

J Clin Immunol. 2021 Aug;41(6):1198-1207. doi: 10.1007/s10875-021-01013-7. Epub 2021 Mar 17.

Authors

Linde N Nijhof¹, Marco van Brussel², Esther M Pots³, Raphaële R L van Litsenburg⁴, Elise M van de Putte³, Joris M van Montfrans⁵, Sanne L Nijhof³

Affiliations

¹ Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Surface mail: HP KE.04.133.1, Post box 85090, 3508 AB, Utrecht, The Netherlands. l.n.nijhof@umcutrecht.nl.
² Department of medical physiology, Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
³ Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Surface mail: HP KE.04.133.1, Post box 85090, 3508 AB, Utrecht, The Netherlands.
⁴ Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
⁵ Department of Pediatric Immunology and Infectious Diseases, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.

Abstract

Purpose: Fatigue is a distressing symptom commonly reported among pediatric patients with primary immunodeficiency (PID). However, the relationship between fatigue and disease activity is currently unknown.

Methods: In this cross-sectional study, we examined the prevalence of severe fatigue, the effect of fatigue on health-related quality of life (HRQoL), and the effects of disease activity and comorbidity on fatigue severity among pediatric patients 2-18 years of age with PID. Fatigue and HRQoL were assessed using the pediatric quality of life inventory multidimensional fatigue scale (PedsQL MFS) and generic core scales (PedsQL GCS), respectively. Linear regression analyses and an analysis of covariance were used to compare the fatigue scores with the scores obtained from a healthy control group. Data were adjusted for age and sex.

Results: Of the 91 eligible patients, 79 were assessed (87% participation rate), with a mean age of 10.4 ± 4.4 years. Pediatric patients with PID reported significantly higher fatigue levels compared to healthy peers, with an 18.9% prevalence of severe fatigue. Moreover, higher fatigue levels were inversely associated with HRQoL in all domains and directly associated with school absences. We found that severe fatigue was comparable between common variable immunodeficiency (CVID), combined immunodeficiency (CID), and selective immunoglobulin A deficiency (SIgAD) patients, but was not reported in the X-linked agammaglobulinemia (XLA) patients studied. Finally, fatigue severity was not significantly associated with disease activity or comorbidity.

Conclusions: Nearly 20% of pediatric patients with PID reported experiencing severe fatigue, and fatigue was reported among a wide range of PID subcategories. In addition, severe fatigue negatively affected the patient's quality of life and daily functioning, but was not associated with disease activity or comorbidity. Thus, targeting severe fatigue might be a promising strategy for improving the overall well-being and quality of life of pediatric patients with PID.

Keywords: Pediatric; disease activity; fatigue; health-related quality of life; immunodeficiency.

MeSH terms

Child
Common Variable Immunodeficiency / etiology
Comorbidity
Cross-Sectional Studies
Fatigue / etiology*
Female
Health Status
Humans
Male
Primary Immunodeficiency Diseases / complications*
Quality of Life
Severity of Illness Index