Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment

Abstract

PurposeCommon variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment.MethodsA 75-question survey developed by the IDF and a 12-item Short Form Health Survey (SF-12) to assess QOL were mailed to adults with CVID. Mean SF-12 scores were compared between patients with CVID and the general US adult population normative sample.ResultsOverall, 945 patients with CVID completed the surveys. More than half of the patients (54.9%) received intravenous Ig and 44.9% received subcutaneous Ig treatment. Patients with CVID had significantly lower SF-12 scores compared with the general US population regardless of sex or age (p < 0.05). Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home.ConclusionThese data provide insight into what factors are most associated with physical and mental health, which can serve to improve QOL in patients in this population. Improvements in QOL can result from early detection of disease, limiting digestive system disease, attention to fatigue, and implementation of an individual treatment plan for the patient.Electronic supplementary materialThe online version of this article (doi:10.1007/s10875-017-0404-8) contains supplementary material, which is available to authorized users.

Fatigue and the wear-off effect in adult patients with common variable immunodeficiency

“…As reported previously [7], 4000 surveys were mailed and 1608 were received. A second mailing to non-respondents was conducted on 27 January 2014.…”

“…Infections are a hallmark of immune abnormality, and their incidence has been correlated inversely with QoL [7]. Thus, we evaluated the association between fatigue and infection frequency.…”

Perceived health of patients with common variable immunodeficiency – a cluster analysis

“…Previous studies have shown that there are subgroups of patients with CVID with respect to their genotype, B cell or T cell profiles and clinical phenotypes [10][11][12][13][14][15][16]. In contrast to previous studies on HRQoL [2,21,22], we were able to analyze it according to the patterns of physical and mental health scores. As has been shown for other chronic diseases, patients are not usually a homogeneous group [17][18][19], but differ according to medical situation, social and psychological support and resilience.…”

“…Perceived health as a subjective measure of general health of individuals is a significant predictor of a number of outcomes, such as recovery from illness, greater satisfaction with care, physicians' ratings of health status, new morbidity and health-care utilization [1,2]. It is also an important predictor of mortality [3].…”

“…Most CVID patients receive immunoglobulin (Ig)G substitution, which has a significant impact on health-related quality of life [8,9]. While several studies targeting CVID patients have assessed perceived health and health-related quality of life (HRQoL) [1,2,20], little is known about subgroups of CVID patients differing by physical and mental health status. However, very few studies analyzing the heterogeneity of CVID go beyond biomedical perspectives and focus on the psychosocial aspects of the disease including perceived health.…”