Health-Related Quality of Life and Patients’ Empowerment in the Health Care of Primary Immune Deficiencies

Quinti, Isabella; Pulvirenti, Federica

doi:10.1007/s10875-017-0428-0

Cited by 11 publications

(10 citation statements)

References 11 publications

(11 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…That said, these attributes and levels were de ned with direct patient input and in consultation with clinical experts. Finally, while the characteristics of survey respondents were largely consistent with samples from previous studies conducted in this population [20,22,23], our sampling framework does not guarantee that our preference estimates are representative of the broader PAD patient population.…”

Section: Discussionmentioning

confidence: 76%

Primary Immune Deficiency: Patients’ Preferences for Replacement Immunoglobulin Therapy

González

Ballow

Fairchild³

et al. 2021

Preprint

View full text Add to dashboard Cite

Purpose: Immunoglobulin (Ig) replacement therapy is an important life-saving treatment modality for patients with primary antibody immune deficiency disorders (PAD). IVIG and SCIg are suitable alternatives to treat patients with PAD but vary in key ways. Existing evidence on patient preferences for Ig treatments given the complexities associated with IVIG and SCIg treatment is limited and fails to account for variations in preferences across patients. For this reason, we sought to evaluate PAD patient preferences for features of IVIG and SCIg across different patient characteristics.Methods: 119 PAD patients completed a discrete-choice experiment (DCE) survey. The DCE asked respondents to make choices between carefully constructed treatment alternatives described in terms of generic treatment features. Choices from the DCE were analyzed to determine the relative influence of attribute changes on treatment preferences. We used subgroup analysis to evaluate systematic variations in preferences by patients’ age, gender, time since diagnosis, and treatment experience. Results: Patients were primarily concerned about the duration of treatment side effects, but preferences were heterogeneous. This was particularly true around administration features. Time since diagnosis was associated with an increase in patients’ concerns with the number of needles required per infusion. Also, patients appear to prefer the kind of therapy they are currently using which could be the result of properly aligned patient preferences or evidence of patient adaptive behavior. Conclusions: Heterogeneity in preferences for Ig replacement treatments suggests that a formal shared decision making process could have an important role in improving patient care.

show abstract

Section: Discussionmentioning

confidence: 76%

Primary Immune Deficiency: Patients’ Preferences for Replacement Immunoglobulin Therapy

González

Ballow

Fairchild³

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…Moreover, no differences in efficacy, tolerability, and HRQoL scores were found between patients receiving intravenous immunoglobulins at different concentrations. This lack of impact of the IgRT route on HRQoL in adult CVID further emphasizes the need of empowering patients in the management of their life-long disease and to focus on their psychological status [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

“…This should improve outcomes in the treatment of CVID with Ig, given its life-long administration, as several factors may impact on replacement therapies. These include the route and setting of administration, as assessed in our study [ 16 , 17 ]. The groups under different IgRT were homogeneous in terms of frequency of CVID-associated conditions and, as already shown [ 29 ], no difference was observed on the IgRT efficacy in terms of severe and mild infectious episodes and hospital admissions among different Ig formulations.…”

Section: Discussionmentioning

confidence: 99%

“…This can result in a “burden of treatment” [ 11 , 12 ]. Hence, the importance of defining a tailored immunoglobulin treatment plan for each patient’s situation has been shown [ 13 – 15 ], with clear implications on patient well-being [ 16 , 17 ]. Our own work indicates that patients with CVID manifest substantial restrictions and poor HRQoL, which worsen over the time, mainly due to CVID-associated clinical conditions [ 8 – 10 ].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Health-Related Quality of Life in Patients with CVID Under Different Schedules of Immunoglobulin Administration: Prospective Multicenter Study

et al. 2019

Self Cite

View full text Add to dashboard Cite

Objective We assessed the health-related quality of life (HRQoL) in CVID adults receiving different schedules of immunoglobulin replacement therapy (IgRT) by intravenous (IVIG), subcutaneous (SCIG), and facilitated (fSCIG) preparations. For these patients, IgRT schedule was chosen after a period focused on identifying the most suitable individual option. Methods Three hundred twenty-seven participants were enrolled in a prospective, observational, 18-month study. Participants received IgRT for at least 2 years. The first 6 months were devoted to the educational process during which the choices related to IgRT were regularly re-assessed, and the shift to alternative regimen was permitted. During the following 12 months, clinical data were prospectively collected, and only patients who did not further modify their IgRT schedule were included in the analysis of HRQoL measured by CVID_QoL, a specific instrument, and by GHQ-12, a tool to assess minor psychiatric nonpsychotic disorders. Results Three hundred four patients were included in the analysis. CVID_QoL global score and its dimensions (emotional functioning, relational functioning, gastrointestinal symptoms) were similar in IVIG, SCIG, and fSCIG recipients. Patients receiving IgRT by different routes of administration reported similar capacity to make long-term plans, discomfort due to therapy, and concern to run out of medications. Multivariate analysis revealed the GHQ-12 status, but not the IgRT mode of administration, as the major factor impacting on treatment-related QoL items, and a significant impact of age on discomfort related to IgRT. Conclusions IgRT schedules do not impact the HRQoL in CVID if the treatment is established after an extensive educational period focused on individualizing the best therapeutic regimen. Electronic supplementary material The online version of this article (10.1007/s10875-019-0592-5) contains supplementary material, which is available to authorized users.

show abstract

“…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]. Although immunoglobulin (Ig) replacement therapy has greatly improved the morbidity and mortality of PAD patients over the past decades [2, 3], it seems less effective on other PAD-associated challenges such as autoimmunity, malignancy, recurring gastrointestinal disorders, and chronic lung complications [4]. The true incidence of PAD is unknown [3], and primary immunodeficiency disorders as a group might not be as rare as previously thought [5].…”

Section: Introductionmentioning

confidence: 99%

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)

Andersen¹,

Midttun²,

Feragen³

2019

J Patient Rep Outcomes

View full text Add to dashboard Cite

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 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. 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. Electronic supplementary material The online version of this article (10.1186/s41687-019-0101-x) contains supplementary material, which is available to authorized users.

show abstract

Health-Related Quality of Life and Patients’ Empowerment in the Health Care of Primary Immune Deficiencies

Cited by 11 publications

References 11 publications

Primary Immune Deficiency: Patients’ Preferences for Replacement Immunoglobulin Therapy

Primary Immune Deficiency: Patients’ Preferences for Replacement Immunoglobulin Therapy

Health-Related Quality of Life in Patients with CVID Under Different Schedules of Immunoglobulin Administration: Prospective Multicenter Study

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)

Contact Info

Product

Resources

About