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

• Published in: Orphanet journal of rare diseases Vol. 11; no. 1; p. 83
• Main Authors: Bienvenu, B, Cozon, G, Hoarau, C, Pasquet, M, Cherin, P, Clerson, P, Hachulla, E, Crave, J C, Delain, J C, Jaussaud, R
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 22.06.2016; BioMed Central
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Analysis; Care and treatment; Cohort Studies; Female; Humans; Immunodeficiency; Immunoglobulins - therapeutic use; Immunologic Deficiency Syndromes - therapy; Immunotherapy; Male; Medical research; Middle Aged; Patient Satisfaction; Personal Satisfaction; Quality of Life; Rare diseases; Surveys and Questionnaires; Young Adult; Immunoglobulins; Preference; Cohort study; Immunotherapy; Satisfaction; Replacement; Primary immunodeficiency; Quality of life
• ISSN: 1750-1172; 1750-1172
• DOI: 10.1186/s13023-016-0452-9

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. 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. The 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. The 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. PID 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.