Abstract
BackgroundCommon 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.MethodsRespondents 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.ResultsIn 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 α-value = 0.91) and relational functioning (α = 0.77) subscales was high, but questionable for the gastrointestinal and skin symptoms subscale (α = 0.66). Convergent validity varied from weak to strong (range 0.3–0.8). Floor and ceiling effects were present.ConclusionsAlthough 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.
Highlights
Primary antibody deficiencies (PAD) are the most frequent primary immunodeficiency, where common variable immunodeficiency (CVID) is the most common clinically relevant diagnosis, characterized by severe and chronic infections [1]
The attention to the patient experience of health-related quality of life (HRQoL) as a factor influencing the individual’s well-being and treatment choices are increasingly explored by clinicians and researchers [8,9,10,11]
We found that the internal consistency was excellent or acceptable for the subscales of Emotional functioning (EF) and relational functioning (RF) respectively, but was questionable for the gastrointestinal and skin symptoms (GSS) subscale
Summary
Primary antibody deficiencies (PAD) are the most frequent primary immunodeficiency, where common variable immunodeficiency (CVID) is the most common clinically relevant diagnosis, characterized by severe and chronic infections [1]. The true incidence of PAD is unknown [3], and primary immunodeficiency disorders as a group might not be as rare as previously thought [5]. The psychological and social impact of having a rare diagnosis may differ from having a more common medical disease, due to a lack of knowledge both in society and in health care [6, 7]. Common variable immunodeficiency (CVID) and other primary antibody deficiencies (PAD) are a heterogeneous group of > 300 congenital disorders affecting the immune system. Efforts to measure health-related quality of life (QoL) in PAD patients have utilised generic QoL tools and disease-specific tools for other conditions. 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
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