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

Maria Fasshauer; Gesine Schuermann; Norbert Gebert; Horst von Bernuth; Sigune Goldacker; Renate Krueger; Petra Manzey; Gundula Notheis; Henrike Ritterbusch; Uwe Schauer; Ilka Schulze; Volker Umlauf; Steffi Widmann; Ulrich Baumann

doi:10.5114/ceji.2021.107088

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

Cent Eur J Immunol. 2021;46(2):244-249. doi: 10.5114/ceji.2021.107088. Epub 2021 Jun 20.

Authors

Affiliations

¹ Klinikum St. Georg, Klinik für Kinder- und Jugendmedizin - ImmundefektCentrum Leipzig IDCL, Leipzig, Germany.
² Medizinische Hochschule Hannover, Immundefektambulanz, Hannover, Germany.
³ Private Psychotherapeutische Praxis, Panketal, Germany.
⁴ Department of Pediatric Pulmonology, Immunology and Intensive Care Medicine, Charité Universitätsmedizin Berlin, Berlin, Germany.
⁵ Universitätsklinikum Freiburg, Medizinische Fakultät, Institut für Immundefizienz, Centrum für Chronische Immundefizienz (CCI), Freiburg, Germany.
⁶ Ludwig Maximilian Universität München, Dr. von Haunersches Kinderspital, Immundefektambulanz, München, Germany.
⁷ Universitätsklinikum Augsburg, Kinderklinik, Immunologische Sprechstunde, Augsburg, Germany.
⁸ Ruhr-Universität Bochum, St. Josef Hospital, Klinik für Kinder- und Jugendmedizin, Bochum, Germany.
⁹ KiJuMed-Gemeinschaftspraxis für Kinder- und Jugendmedizin sowie Kinder- und Jugendpsychotherapie, Bern, Switzerland.
¹⁰ Department of Pediatric Intensive Care, Inselspital University Hospital Bern, University Children's Hospital, Bern, Switzerland.

Abstract

Introduction: 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.

Material and methods: 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.

Results: 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.

Conclusions: 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.

Keywords: IgG replacement therapy; education programme; patient empowerment programme; primary immunodeficiency; quality of life; treatment satisfaction.