Treatment of patients living with Primary Antibody Deficiencies (PAD) could impose demands on daily life to plan and self-care management, as a result of the need to comply with complex therapeutic schedules, while balancing family or job commitment. This can result in a ¿burden of treatment¿. Several studies indicated that PAD patients manifest poor health-related quality of life which worsen over the time, due to associated clinical conditions. PAD patients require replacement therapy for life: on this basis, the acceptability of the schedules and settings for immunoglobulins (Ig) administration is an important factor in improving quality of life. Recently, a study on 327 Italian CVID patients showed that Ig replacement therapy schedules did not impact the health-related quality of life (HRQoL) if the choice of treatment was individualized after a long educational period focused on verifying the Ig therapy schedule ongoing at enrolment time. Starting from this observation, in this proposal we aim to collect longitudinal data on HRQoL of patients affected by PAD on different schedule and route of Ig replacement therapy. We will measure the impact of the switch from intravenously immunoglobulin preparations (IVIG) and/or subcutaneous Ig (SCIG) to treatment with facilitated Ig by human recombinant hyaluronidase (fSCIG) on HRQoL in a population of PAD patients by a prospective, longitudinal, observational study. For the evaluation of HRQoL, a recently validated specific tool for Primary antibody Deficiencies ¿the CVID_QoL questionnaires¿ will be used. This instrument allowed to highlight the impact of the immunodeficiency as an overall problem on IgG replacement therapy recipients¿ well-being. Our study sought to generate information to help health care professionals to understand factors that may impact on patient¿s everyday life and contribute to maximizing patient empowerment, satisfaction with care, and burden of treatment while minimizing the impact of illness.
