Dataset Information

Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL).

ABSTRACT:

Background

Common variable immunodeficiency (CVID) and other primary antibody deficiencies (PAD) are a heterogeneous group of >?300 congenital disorders affecting the immune system. Until recently, efforts to measure health-related quality of life (QoL) in PAD patients have utilised generic QoL tools and disease-specific tools for other conditions. Still, the full impact of the disease is probably not understood. We evaluated the performance of the CVID_QoL, a novel disease-specific QoL instrument for adults with CVID, on Norwegian PAD patients and compared the results to those of the generic WHOQOL-BREF.

Methods

Respondents were recruited through the Norwegian Centre for Rare Disorders' patient database. Included patients fulfilled the following criteria (all three): 1.) Age ?18 years, 2.) a PAD diagnosis, 3.) currently on immunoglobulin therapy. The CVID_QoL is a 32-item questionnaire. Global CVID_QoL scores were compared between Norwegian PAD patients and Italian CVID patients.

Results

In total, 83 PAD patients filled out the CVID_QoL, 63% had CVID, 76% were females. 32 patients filled out the WHOQOL-BREF. Feasibility was high (<1% missing). Internal consistency for the emotional- (Cronbach's ?-value = 0.91) and relational functioning (? = 0.77) subscales was high, but questionable for the gastrointestinal and skin symptoms subscale (? = 0.66). Convergent validity varied from weak to strong (range 0.3-0.8). Floor and ceiling effects were present.

Conclusions

Although many disease-specific characteristics are probably shared with CVID and other PAD, the CVID_QoL captures some, but not all, dimensions of PAD patients' QoL. More evaluations of the CVID_QoL's performance in different contexts are needed.

SUBMITTER: Andersen JB

PROVIDER: S-EPMC6391500 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature

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Publications

Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL).

Andersen Jintana B JB Midttun Knut K Feragen Kristin J B KJB

Journal of patient-reported outcomes 20190226 1

<h4>Background</h4>Common variable immunodeficiency (CVID) and other primary antibody deficiencies (PAD) are a heterogeneous group of > 300 congenital disorders affecting the immune system. Until recently, efforts to measure health-related quality of life (QoL) in PAD patients have utilised generic QoL tools and disease-specific tools for other conditions. Still, the full impact of the disease is probably not understood. We evaluated the performance of the CVID_QoL, a novel disease-specific QoL ...[more]

PMID: 30806830

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Project description:BACKGROUND:Domains other than those commonly measured (physical, psychological, social, and sometimes existential/spiritual) are important to the quality of life of people with life-threatening illness. The McGill Quality of Life Questionnaire (MQOL) - Revised measures the four common domains. The aim of this study was to create a psychometrically sound instrument, MQOL - Expanded, to comprehensively measure quality of life by adding to MQOL-Revised the domains of cognition, healthcare, environment, (feeling like a) burden, and possibly, finance. METHODS:Confirmatory factor analyses were conducted on three datasets to ascertain whether seven new items belonged with existing MQOL-Revised domains, whether good model fit was obtained with their addition as five separate domains to MQOL-Revised, and whether a second-order factor representing overall quality of life was present. People with life-threatening illnesses (mainly cancer) or aged > 80 were recruited from 15 healthcare sites in seven Canadian provinces. Settings included: palliative home care and inpatient units; acute care units; oncology outpatient clinics. RESULTS:Good model fit was obtained when adding each of the five domains separately to MQOL-Revised and for the nine correlated domains. Fit was acceptable for a second-order factor model. The financial domain was removed because of low importance. The resulting MQOL-Expanded is a 21-item instrument with eight domains (fit of eight correlated domains: Comparative Fit Index = .96; Root Mean Square Error of Approximation = .033). CONCLUSIONS:MQOL-Expanded builds on MQOL-Revised to more comprehensively measure the quality of life of people with life-threatening illness. Our analyses provide validity evidence for the MQOL-Expanded domain and summary scores; the need for further validation research is discussed. Use of MQOL-Expanded will enable a more holistic understanding of the quality of life of people with a life-threatening illness and the impact of treatments and interventions upon it. It will allow for a better understanding of less commonly assessed but important life domains (cognition, healthcare, environment, feeling like a burden) and their relationship to the more commonly assessed domains (physical, psychological, social, existential/spiritual).

Dataset Information

Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL).

Background

Methods

Results

Conclusions

Publications

Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL).

Similar Datasets

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