Health-Related Quality of Life and Patients’ Empowerment in the Health Care of Primary Immune Deficiencies

Excerpt

The health care of Primary Immune Deficiencies (PIDs) has always been challenging for clinicians due to the wide range of clinical phenotypes, difficulties in diagnosis, and complexity of treatments. Over the last decades, considerable progress has been made in awareness, prompt diagnosis, and therapy leading to a profound change in attitude and approach to primary antibody deficiencies (PADs). In particular, the advent of preparations of polyvalent immunoglobulin (IG) in the 1980s has dramatically improved morbidity and mortality. However, while IG replacement therapy has proven useful to control infections, other PADs-associated conditions related to chronic inflammation and cancer have not appeared to be improved by IG treatment. Disability in PADs is currently due to autoimmune complications, malignancies, recurrent gastrointestinal infections, and chronic lung involvement, with a strong impact on patients’ daily functioning [1]. The focus on the patients’ experience of illness requires a rigorous scientific approach to determine factors affecting the burden of disease to maximize patient’s well-being and to minimize the impact of disease. …