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

Linde N Nijhof,Marco Van Brussel,Elise M Van De Putte,Joris M Van Montfrans,Sanne L Nijhof,Esther M Pots,Raphaële R L Van Litsenburg

doi:10.1007/s10875-021-01013-7

Abstract

PurposeFatigue is a distressing symptom commonly reported among pediatric patients with primary immunodeficiency (PID). However, the relationship between fatigue and disease activity is currently unknown.MethodsIn 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.ResultsOf 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.ConclusionsNearly 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.

Highlights

Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease [1, 2]
Major advances in pediatric medicine have resulted in many patients with PID transitioning from having a potentially fatal disease to living with a chronic condition [4]; this has led to a new set of challenges, as growing up with a chronic disease can place considerable psychological, social, emotional, and financial burden on the children themselves, as well as their relatives [5, 6]
Using retrospective data collected from the US Immunodeficiency Network (USIDNET) patient registry, Hajjar et al recently estimated that the prevalence of fatigue among adults with PID is 18%, compared to 6–7.5% in the general population [4]

Summary

Introduction

Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease [1, 2]. Using retrospective data collected from the US Immunodeficiency Network (USIDNET) patient registry, Hajjar et al recently estimated that the prevalence of fatigue among adults with PID is 18%, compared to 6–7.5% in the general population [4]. Recent research reported a similar prevalence of fatigue among children with a chronic condition (21.1%), regardless of the type of underlying condition [8]. It appears that fatigue severity can be better explained by transdiagnostic psychosocial factors—for example, coping strategies, cognitive, emotional, and motivational factors underlying neurobiological factors and universal protective factors such as resilience—rather than specific characteristics of the disease [8]

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Results

Conclusion