Does the route of immunoglobin replacement therapy impact quality of life and satisfaction in patients with primary immunodeficiency? Insights from the French cohort “Visages”

B Bienvenu,P Cherin,M Pasquet,P Clerson,G Cozon,J C Delain,R Jaussaud,C Hoarau,E Hachulla,J C Crave

doi:10.1186/s13023-016-0452-9

Abstract

BackgroundIgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home. The objective of the VISAGE study was to investigate if route and/or place for IgRT impact patients’ satisfaction regarding IgRT and quality of life (QoL) in real-life conditions.MethodsThe study enrolled PID patients at least 15 years old receiving IgRT for at least 3 months. Satisfaction and QoL were evaluated at enrollment and over a 12-month follow-up period by Life Quality Index (LQI) which measures 3 dimensions of satisfaction: treatment interference, therapy related problems and therapy settings (factors I, II and III) and SF-36 v2 questionnaire.ResultsThe study included 116 PID patients (mean age 42 ± 18 years, 44 % males, 58 % with scholar or professional occupation) receiving IgRT for a mean of 8.5 ± 8.4 years. At enrollment they were receiving either home-based SCIg (51 %), hospital-based IVIg (40 %) or home-based IVIg (9 %). Patients exhibited a high degree of satisfaction regarding IgRT whatever the route and place for administration. LQI factor I was higher for home-based SCIg (86 ± 2) than for hospital-based IVIg (81 ± 3) and home-based IVIg (73 ± 5; p = 0.02 versus home-based SCIg); no difference was found for LQI factor II; LQI factor III was higher for home-based SCIg (92 ± 2) than for hospital-based IVIg (87 ± 5) and hospital-based IVIg (82 ± 3; p = 0.005 versus home-based SCIg). By contrast, every dimension of QoL was impaired. Over the follow-up period, 10 patients switched from hospital-based IVIg to home-based SCIg and improved LQI factor I (p = 0.004) and factor III (p = 0.02), while no change was noticed in LQI factors II and QoL. Meanwhile, no change in satisfaction or QoL was found in patients with stable route of IgRT. When asked on their preferred place of treatment all but one patient with home-based treatment would choose to be treated at home and 29 % of patients treated at hospital would prefer home-based IgRT.ConclusionPID patients expressed a high degree of satisfaction regarding IgRT, contrasting with impaired QoL. In real-life conditions awareness of patient’s expectations regarding the route or place of IgRT may be associated with further improvement of satisfaction.

Highlights

IgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home
Most of them like agammaglobulinemia, X-linked agammaglobulinemia, common variable immune deficiency (CVID), severe combined immunodeficiency (SCID), and immunoglobulin subclass deficiency associated with recurrent infections, are characterized by a low level of circulating immunoglobulins (Ig)
Patients were suffering from agammaglobulinemia (n = 1), X-linked agammaglobulinemia (n = 5), hypogammaglobulinemia (n = 9), CVID (n = 76), severe combined immunodeficiency (n = 4), IgG subclass deficiency (n = 12) or of other types of PID (n = 9). 81 patients were suffering from at least one concomitant disease

Summary

Introduction

IgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home. Local reactions are more frequent with SCIg whereas general systemic reactions are more often observed with IVIg [14, 18, 22, 25,26,27], a point that could be explained by supraphysiological Ig peaks [17] Both routes have comparable efficacy in the prevention of serious bacterial infections [28]. Some patients prefer to receive injections at hospital because of the ease of organization and the quality of care and follow-up; while others prefer homebased treatment due to the lower impact on daily activities These dimensions are encompassed in the general concept of satisfaction regarding the treatment [31]

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Discussion

Conclusion