Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study

Abstract

BackgroundHereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is characterized by recurrent edema of unpredictable frequency and severity. Stress, anxiety, and low mood are among the triggering factors most frequently reported. Impaired regulation and processing of emotions, also known as alexithymia, may influence outcomes. The aim of this study was to confirm the presence of alexithymia and stress in children with C1-INH-HAE, to determine whether they are also present in children affected by other chronic diseases, and to investigate their relationship with C1-INH-HAE severity. Data from children with C1-INH-HAE (n = 28) from four reference centers in Italy were compared with data from children with type 1 diabetes (T1D; n = 23) and rheumatoid arthritis (RA; n = 25). Alexithymia was assessed using the Alexithymia Questionnaire for Children scale; perceived stress was assessed using the Coddington Life Event Scale for Children (CLES-C).ResultsMean age (standard deviation [SD]) in the C1-INH-HAE, T1D, and RA groups was 11.8 (3.3), 11.7 (2.9), and 11.1 (2.6) years, respectively. Mean C1-INH-HAE severity score was 5.9 (2.1), indicating moderate disease. Alexithymia scores were similar among disease groups and suggestive of difficulties in identifying and describing emotions; CLES-C scores tended to be worse in C1-INH-HAE children. C1-INH-HAE severity was found to correlate significantly and positively with alexithymia (p = 0.046), but not with perceived stress. Alexithymia correlated positively with perceived stress.ConclusionsAlexithymia is common in children with chronic diseases. In C1-INH-HAE, it may result in increased perceived stress and act as a trigger of edema attacks. Comprehensive management of C1-INH-HAE children should consider psychological factors.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0871-x) contains supplementary material, which is available to authorized users.

Increased fibrinolysis-induced bradykinin formation in hereditary angioedema confirmed using stored plasma and biotechnological inhibitors

“…This suggests that the instability of the kallikrein-kinin system in HAE-1/-2 resides in the upstream fibrinolytic system, as previously discussed [1]. Known triggers of HAE attacks include plausible situations where fibrinolysis may be activated: trauma, infection, medical and dental procedures, menstruation, and, singularly, mental stress [16, 17]. Indeed, mental stress determines tPA release and fibrin turnover in healthy human subjects [18].…”

Going Beyond the Visible in Type 2 Diabetes Mellitus: Defense Mechanisms and Their Associations With Depression and Health-Related Quality of Life

“…Due to this, self-care could be considered as a suggestive index of patients' adaptation to diabetes, which may involve psychological adjustment to disease and its related emotional distress (Lapolla et al, 2012;Schmitt et al, 2014;Del Piccolo et al, 2015;Craparo et al, 2016;Settineri et al, 2019a,b;Knowles et al, 2020). Emotional distress could exist independently from such chronic disease, though considering the higher risk of mortality and morbidity due to diabetes, a psychological elaboration processing would be useful for the psychic integration of the chronic illness experience within patients' daily life (Whittemore et al, 2010;Castelnuovo et al, 2015;Van Houtum et al, 2015;Stanton and Hoyt, 2017;Di Giuseppe et al, 2018Savarese et al, 2018;Catalano et al, 2019a). Demographical and physical characteristics, such as body mass index (BMI) and smoking and alcohol consumption, could predict depression in Type 2 Diabetes Mellitus (T2DM) (Bouwman et al, 2010;Rosa et al, 2019).…”

Caregivers’ Sensemaking of Children’s Hereditary Angioedema: A Semiotic Narrative Analysis of the Sense of Grip on the Disease

“…Moreover, some parents report difficulties in leaving the child as well as in letting the child/adolescent go far from them. The sense of unpredictability is made worse by the poor awareness of the disease and its medical protocols and prophylaxis within medical and social settings (Freda et al, 2016a; Savarese et al, 2017, 2018).…”

“…The “psychological hypothesis” has a long tradition in the history of HAE since the first case identified in the medical literature was attributed to a nervous and psychosomatic cause (Ossler, 1888; Bannister, 1894; McDougall, 1989). Our previous pilot studies on HAE highlighted that clinicians should be aware that the common-sense hypothesis concerning physical and psychological triggers may lead to the development of unjustified and excessive fears of engaging in physical and social activities, as well as to an attitude of avoidance and denial toward negative emotions and the events that may potentially trigger them (Freda et al, 2016a; Savarese et al, 2018). It is therefore necessary to find effective ways to collect anamnestic information and to share suggestions and knowledge on these aspects of HAE within the health-care system.…”