Genetic impairment and emotion regulation in the variability of Heredirary Angioedema symptoms: an empirical research

Abstract

Recent studies suggest that Hereditary Angioedema(HAE) attacks may be triggered or affected by psychological stress. HAE is a rare genetic disease characterized by unpredictable and variable attacks of cutaneous and mucosal edema, carrying a major impact on daily life. We explore the connection between stress, emotion regulation and the variability of the attacks. Twelve children with HAE, aged 6 to 14, as well as their parents, were involved in a mixed-method study: 1. a qualitative thematic analysis of parent semi-structured interviews on disease variability; 2. a quantitative evaluation of the psychological profile (CBCL), perceived stress levels (CLES) and emotion regulation competences (AQC ; LEAS-C) of the young patients. In the interviews, 91% of parents believe emotional and stress factors are involved in trigger-ing HAE. 83% of the children experience an above-average levels of stress, 91% of them manifest deficits in recognizing and naming emotions. Recognition of stress as a trigger may lead parents and physicians to implicitly or explicitly suggest that stressful and intensely emotional events should be avoided, paradoxically increas-ing the vulnerability to stress.