Abstract

Augmentation therapy with human intravenous alpha1-proteinase inhibitors is the only specific treatment of pulmonary manifestations of Alpha-1 Antitrypsin Deficiency (AATD). The weekly intravenous administration of augmentation therapy in patients with AATD may impair quality of life; interfere with family, social, and leisure activities; and pose a challenge for school or work attendance. Self-administration of augmentation therapy is an alternative clinic-based administration that may increase patient satisfaction, self-care competence and independence. We review the experience with self-administration of intravenous drugs in other clinical conditions, the perceived advantages and barriers to self-administration of intravenous augmentation therapy, the clinical experience with self-administration of augmentation therapy, and the published recommendations for the implementation of self-administration of augmentation therapy in clinical practice. Self-administration is a very attractive option for many patients with AADT who desire to maintain or gain independence. Self-administration is easy to implement, providing the patients are properly selected and receive adequate training, and is associated with increased patient satisfaction.

Keywords: Alpha-1 antitrypsin deficiency; Augmentation therapy; Self-administration; Self-infusion independence; Satisfaction