Psychological processes in the experience of hereditary angioedema in adult patients: an observational study

Abstract

Background Hereditary angioedema associated to C1 inhibitor deficiency (C1-INH-HAE) is a pathological condition characterized by episodes of subcutaneous swelling and it is frequently associated with discomfort and social impairment of the patients, due to the anxiety experienced for an unpreventable manifestation of an attack during daily life. In children increased level of stress and alexithymia have been associated to C1-INH-HAE, and the latter correlated also with the severity of the disease. We hypothesized that the involvement of psychological issues may impact on the severity of C1-INH-HAE in adult patients as well, interfering with their ability to engage with the management of the disease. Methods 28 adult patients with C1-INH-HAE were evaluated for clinical (C1-INH-HAE Severity Score) and psychological factors (alexithymia, emotion regulation, stress, patient health engagement, general severity index) by means of validated questionnaires. Results Mean age (standard deviation [SD]) was 45 (11) years and time from diagnosis was 20 (12) years. The mean C1-INH-HAE severity score was 6.4. Alexithymia was absent in 22 (78%) patients. Moderate and high stress levels were present in 17 (61%) and 4 (14%) patients, respectively. Moderate-high discomfort was experienced by 9 (36%) patients and a discomfort beyond the clinical attention threshold was shown by 3 (12%) patients. Stress correlated with patient health engagement and with psychological discomfort. Conclusions In C1-INH-HAE, patients health engagement and moderate-high psychological discomfort are linked with stress but not with the severity of the disease or alexithymia. A better patient health engagement may be a target for psychological intervention in clinics to ameliorate the stress perceived by C1-INH-HAE patients.

Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany

“…Although reduced emotional regulation enforces the inability to manage one's health, a better knowledge of disease has a positive impact on a patient's health engagement, possibly reducing clinically relevant stress. As psychological stress is described as most common trigger factor for HAE attacks, which was also seen in our study group, the authors suggest that psychological and experiential processes of HAE patients might offer a possible therapeutic target for clinical psychologists [41,43,44].…”

“…It remains unclear, whether the duration of long-term treatment is essential to improve or maintain mental health, as our patients were taking their prophylactic medication for <1 year. It should be also discussed, whether the lifelong persisting burden of disease, unpredictable breakthrough attacks in spite of prophylactic treatment or even common neurobiological processes are critical features, altogether leading to a substantial prevalence of mental diseases in HAE patients [9,13,[39][40][41].…”

Immunogenicity and Safety of Anti-SARS-CoV-2 mRNA Vaccines in a Cohort of Patients with Hereditary Angioedema

“…HAE is a potentially life-threatening disorder associated with a significant emotional burden [ 33 , 34 ]. Indeed, this rare clinically heterogeneous condition is often accompanied by uncertainty due to the variability in the clinical phenotype, even in patients with the same genetic mutation, and the lack of reliable biomarkers able to predict the severity of the disease [ 35 , 36 ].…”

Hereditary angioedema with C1 inhibitor deficiency: traps in the diagnosis, treatment, and understanding

“…15 Savarese et al also linked emotional disorders and lack of knowledge to poor adherence to treatment. 16 According to Graffigna et al, when a patient is given an important diagnosis, he or she may not be as actively engaged in their treatment because of the emotional instability inherent to the process. 17 The psychological processes associated with HAE are extremely important because they can act as triggers of crises and interfere in disease management by affecting patients' quality of life.…”