LAUSR

Both pediatric and adult patients with a primary immunodeficiency/immune dysregulation (PID/PIDR) diagnosis report inferior quality of life (QOL) and patient-reported outcomes (PROs) as compared with their healthy peers. Recognition of the negative impact on QOL and PROs provides an opportunity for clinicians to intervene with supportive measures. However, provider perceptions of PID/PIDR patients’ quality of life, physical well-being, psychosocial health and neurocognition, and access to supportive resources have yet to be systematically evaluated. We report specialty providers’ perception of the QOL and psychosocial and physical well-being of their pediatric and adult patients with PID/PIDR through the utilization of an online survey assessing QOL and the impact of disease or its associated treatment on their physical well-being, mental health, social relationships, neurocognition, and work/school performance. Clinicians trended towards believing adult PID/PIDR patients had worse overall QOL than children with PID/PIDR. Providers additionally identified their adult patients’ QOL to be more deleteriously affected by co-morbidities than their pediatric patients. Clinicians distinguished anxiety and social relationships as the psychosocial aspects most often affected by a complex immunological diagnosis in all patients. Of physical health considerations, energy, rather than mobility or pain, was perceived to be more negatively influenced by PID/PIDR in both adult and pediatric patients. Knowledge of these clinician perceptions can affect communication of findings with patients, as well as ongoing management, and thus, it is important to understand these fully to improve healthcare delivery to, and clinical management of, these patients.