A structured patient empowerment programme for primary immunodeficiency significantly improves general and health-related quality of life

• Published in: Central-European journal of immunology Vol. 46; no. 2; pp. 244 - 249
• Main Authors: Fasshauer, Maria, Schuermann, Gesine, Gebert, Norbert, von Bernuth, Horst, Goldacker, Sigune, Krueger, Renate, Manzey, Petra, Notheis, Gundula, Ritterbusch, Henrike, Schauer, Uwe, Schulze, Ilka, Umlauf, Volker, Widmann, Steffi, Baumann, Ulrich
• Format: Journal Article
• Language: English
• Published: Poland Termedia Publishing House 01.01.2021
• Subjects: Clinical Immunology; education programme; Empowerment; igg replacement therapy; Immunodeficiency; Immunoglobulin G; patient empowerment programme; Patients; Primary immunodeficiencies; primary immunodeficiency; Quality of life; Rare diseases; treatment satisfaction; patient empowerment programme; primary immunodeficiency; IgG replacement therapy; treatment satisfaction; quality of life; education programme
• ISSN: 1426-3912; 1644-4124
• DOI: 10.5114/CEJI.2021.107088

Primary immunodeficiencies (PIDs) are a heterogeneous group of rare diseases characterized by increased susceptibility to infections and a reduced quality of life (QoL). The influence of a patient empowerment programme for PID (PID-PEP) on general and health-related QoL was assessed in the present study. PID-PEP is provided by a multidisciplinary team for patients with PID and immunoglobulin G (IgG) replacement therapy during a weekend course to improve patient self-management regarding chronic disease and long-term therapy. Twenty-six adult patients with PID undergoing PID-PEP were recruited. Short Form-36 (SF-36) and the Life Quality Index (LQI) were assessed as generic and disease-specific QoL instruments before as well as 6 months after the programme. Median visual analogue scale (VAS) values of present health status significantly increased from 68 at baseline to 76 after PID-PEP (p = 0.002). Furthermore, the SF-36 mental component summary (MCS) significantly improved from 36 to 43 following the programme (p = 0.042). Of the eight SF-36 dimensions, vitality (VT) significantly improved (p = 0.025). Median LQI index significantly increased from 77 at baseline to 86 after PID-PEP (p = 0.008). Furthermore, the LQI domains treatment interference (I) and therapy-related problems (II) significantly improved. Our PID-PEP significantly improved general and health-related QoL. It needs to be evaluated in future studies whether the beneficial effects of PID-PEP are sustained over longer periods of time and whether repeated PID-PEP sessions further improve QoL outcome.