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

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.

[Inflammatory bowel diseases-the biopsychosocial reality in childhood and adolescence]. / Chronisch entzündliche Darmerkrankungen ­ die biopsychosoziale Realität im Kindes- und Jugendalter.

The prevalence of inflammatory bowel diseases (IBD) in children is rising. These complex diseases typically take an unpredictable course with disease flares. This review serves to illustrate the psychosocial consequences and risks of inflammatory bowel diseases in children and adolescents against the background of the essential medical aspects.Pediatric gastroenterologists should perform diagnostic procedures and medical care. Central elements of diagnostic workup include upper and lower endoscopy with multiple biopsies and imaging procedures. Therapeutic options comprise immunomodulating and immunosuppressive agents, nutrition therapy and surgical interventions. With its various symptoms, complications, diagnostic procedures, and therapies, including side effect concerns, the disease is challenging for young patients and their families. Quality of life, social life, and education may be impaired. There is also an increased risk for mental-health problems. An interdisciplinary approach with the involvement of various healthcare professions such as medical doctors, psychologists, social workers, dieticians, and nurses is necessary to satisfy the needs of children and adolescents with these complex medical conditions and their families.