Health-related quality of life in primary immunodeficiencies: Impact of delayed diagnosis and treatment burden

Anderson, J.; Cowan, Juthaporn; Condino‐Neto, Antônio; Levy, Donald S.; Prusty, Subhransu

doi:10.1016/j.clim.2022.108931

Cited by 32 publications

(15 citation statements)

References 81 publications

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“…Even with the availability of treatment, patients with immunodeficiencies can still experience a significant disease burden that can impact their physical function, emotional well-being, work productivity, disability, social interactions, and family life [5]. They have greater risk of lower school/work productivity, reduced life satisfaction, and experience anxiety or depressive symptoms in response to living with their chronic condition compared with healthy individuals [6,7]. Regular long-term treatment regimens, while reducing disease burden, can introduce treatment-related burden in terms of interference with daily life, increased risk of adverse events (AEs), and acting as a constant reminder of the disease [8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

Mallick

Solomon²,

Bassett³

et al. 2022

Allergy Asthma Clin Immunol

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Background Understanding the impact of different immunoglobulin (Ig) infusion methods (intravenous [IVIg] and subcutaneous [SCIg]) upon treatment experience can potentially facilitate optimization of patient outcomes. Here, the perspective of patients with primary and secondary immunodeficiency diseases (PID and SID, respectively) receiving IVIg and SCIg was evaluated, in terms of treatment satisfaction, accounting for treatment history, using Association des Patients Immunodéficients du Québec (APIQ) survey data. Methods The online APIQ survey (shared October 2020–March 2021) of patients with immunodeficiencies in Canada contained 101 questions on: Ig use, history, and detailed infusion characteristics; as well as structured patient-reported outcomes such as treatment satisfaction (via TSQM-9), symptom state (via PASS), general health perception (via GHP), and physical and mental function (via PROMIS). Adult respondents (≥ 18 years old) currently using Ig were compared by their current Ig infusion method (IVIg or SCIg cohort) overall, and in a sub-analysis, the IVIg cohort was compared with the SCIg cohort after stratification by respondents who started SCIg when naïve to Ig (‘SCIg naïve’) or with previous IVIg experience (‘SCIg switch’). Results In total, 54 respondents currently used IVIg and 242 used SCIg. The average duration per infusion of a weekly SCIg infusion was significantly shorter compared with the average duration of a 3–4 weekly IVIg infusion (p < 0.001). The SCIg cohort was associated with significantly higher scores for the TSQM-9 effectiveness domain compared with the IVIg cohort. The scores for TSQM-9 convenience and global satisfaction domains were similar in the two cohorts. The SCIg cohort was also associated with a significantly higher proportion of respondents who were in an acceptable symptom state and a lower proportion who reported very poor or poor perception of health compared with the IVIg cohort. Further, the SCIg naïve subgroup was associated with significantly higher TSQM-9 effectiveness and convenience domain scores compared with the IVIg cohort, while there was no significant difference between the SCIg switch subgroup and the IVIg cohort in terms of convenience. Conclusions A better understanding of how different IgRT administration methods impact treatment experience and satisfaction may assist with informed treatment decision making and ultimately further improvements in patient outcomes.

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Section: Introductionmentioning

confidence: 99%

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

Mallick

Solomon²,

Bassett³

et al. 2022

Allergy Asthma Clin Immunol

View full text Add to dashboard Cite

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“…34 Other studies have shown that delayed diagnosis was correlated with some health-related quality of life (HRQL) dimensions in pediatric and adult patients, suggesting that early diagnosis and treatment may improve some of these HRQL parameters. 35,36 Because our study relied on patient report rather than collection of data from clinical charts, we were unable to confirm any possible relationship between delay in diagnosis and HRQL. Given the long diagnostic delay patients often experience, they may have been subjected to treatment for other conditions, which can confound immunodiagnostic studies when they do see immunologists.…”

Section: Discussionmentioning

confidence: 97%

Neurologic Manifestations of Common Variable Immunodeficiency

Almeida

Smith

Delic

et al. 2023

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesCommon variable immunodeficiency is a systemic disease and not solely a disease of humoral immunity. Neurologic symptoms associated with common variable immunodeficiency are underrecognized and warrant further study. This work aimed to characterize the neurologic symptoms reported by people living with common variable immunodeficiency.MethodsWe conducted a single academic medical center study of neurologic symptoms reported by adults previously diagnosed with common variable immunodeficiency. We used a survey of common neurologic symptoms to determine the prevalence of these symptoms in a population with common variable immunodeficiency and further assessed these patient-reported symptoms with validated questionnaires and compared symptom burden with other neurologic conditions.ResultsA volunteer sample of adults (aged 18 years or older) previously diagnosed with common variable immunodeficiency at the University of Utah Clinical Immunology/Immune Deficiency Clinic who were able to read and comprehend English and willing and able to answer survey-based questions were recruited. Of 148 eligible participants identified, 80 responded and 78 completed the surveys. The mean age of respondents was 51.3 years (range 20–78 years); 73.1% female and 94.8% White. Patients with common variable immunodeficiency reported many common neurologic symptoms (mean 14.6, SD 5.9, range 1–25), with sleep issues, fatigue, and headache reported by more than 85%. Validated questionnaires addressing specific neurologic symptoms supported these results. T-scores on Neuro QoL questionnaires for sleep (mean 56.4, SD 10.4) and fatigue (mean 54.1, SD 11) were higher, indicating more dysfunction, than in the reference clinical population (p< 0.005). The Neuro QoL questionnaire for cognitive function showed a lower T-score (mean 44.8, SD 11.1) than that in the reference general population (p< 0.005), indicating worse function in this domain.DiscussionAmong survey respondents, there is a marked burden of neurologic symptoms. Given the impact of neurologic symptoms on health-related quality-of-life measures, clinicians should screen patients with common variable immunodeficiency for the presence of these symptoms and offer referral to neurologists and/or symptomatic treatment when indicated. Frequently prescribed neurologic medications may also affect the immune system, and neurologists should consider screening patients for immune deficiency before prescribing them.

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“…They are more prone to have recurrent pulmonary infections, infective diarrhoea, granulomatous disease, enteropathy, and development of autoimmunity later in life. Thus, early detection by primary care doctors may help to prevent the complications of PID [8].…”

Section: Discussionmentioning

confidence: 99%

Primary immunodeficiency disease in children: A significant but rare cause of failure to thrive

2023

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This case illustrates the rare cause of failure to thrive (FTT) that initially presented with recurrent ear discharge. A five-year-old boy with a history of recurrent ear infections for the past year was treated for acute symptoms during each visit. He later was diagnosed with acute mastoiditis secondary to otitis media by a private otorhinolaryngologist and was referred to a tertiary hospital for admission and parenteral antibiotic commencement. The anthropometric evaluation noted he fell under the group of FTT and had an incidental finding of lower tract respiratory infection that turned out to be tuberculosis infection. Multiple complications occurred during his admission, including candidemia, disseminated tuberculosis, and deep-seated collections. Hence, he was worked up for primary immunodeficiency and was given extensive supportive treatment.

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Health-related quality of life in primary immunodeficiencies: Impact of delayed diagnosis and treatment burden

Cited by 32 publications

References 81 publications

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

Neurologic Manifestations of Common Variable Immunodeficiency

Primary immunodeficiency disease in children: A significant but rare cause of failure to thrive

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