Children with hereditary angioedema frequently have difficulties recognizing their own emotions (alexithymia), which could be a key source of psychological stress that triggers acute attacks of swelling, an Italian study found.
Guidelines recommend that management of these patients should include psychological intervention to ease stress and reduce the risk of disease flares.
The study, “Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study,” was published in the journal Orphanet Journal of Rare Diseases.
Hereditary angioedema, caused by a deficiency in the C1-inhibitor protein, is marked by recurrent episodes of swelling, which can be painful, life-threatening, and affect children as young as 4.
The condition poses a burden to patients and their families, and can impair quality of life and increase anxiety in affected children.
What triggers angioedema flares is still poorly understood. The unpredictability, frequency, and severity of the episodes present a significant obstacle to disease management.
Based on prior research suggesting that hereditary angioedema led to alexithymia in children, researchers evaluated the relationship between emotional and psychological factors and disease manifestations in these patients.
The study compared the levels of alexithymia, stress, and disease severity among children with hereditary angioedema and two control groups — children with type 1 diabetes and those with rheumatoid arthritis.
Patients were recruited from four reference centers in Italy. They included 28 children with hereditary angioedema, 23 with type 1 diabetes, and 25 with rheumatoid arthritis.
Clinical data were collected from medical charts and patient diaries. Alexithymia, perceived stress, and anxiety were assessed using the Alexithymia Questionnaire for Children (AQC) scale for those 15 or younger; the Toronto Alexithymia Scale 20 (TAS-20) in children over 15; plus the Coddington Life Event Scale for Children (CLES-C) and Physiological Hyperarousal in Children (PH-C).
Children were, on average, 11 or 12 years old. Frequency of symptoms was similar among the three groups, and overall disease severity was considered moderate in the group with angioedema.
On average, children with the disease had experienced two to three attacks over the past month. Most were affected by swelling in the skin (89.3%) and abdomen (82.1%).
According to AQC self-reports, all the groups had signs of difficulty in identifying and describing feelings when compared to reference values. They were more prone to externally oriented (concrete) thinking, and were moderately confused about their physical perceptions.
But children with hereditary angioedema more frequently (72%) reported critical levels of such difficulties when compared with those with diabetes (68%) or rheumatoid arthritis (57%).
They also indicated on the CLES-C survey to have experienced more stressful events over the past 12 months. Although these differences in alexithymia and perceived stress were noted among the three groups, they did not reach statistical significance.
But there was a significant and positive correlation between disease severity and impairment in recognizing emotions in children with angioedema. Increasing difficulties with identifying feelings were associated with poorer disease outcomes.
Also, children who experienced more severe alexithymia were more likely to experience critical levels of perceived stress.
Thus, in children with hereditary angioedema, “the presence of marked alexithymia might be predictive of even worse outcomes,” researchers said.
“Our findings suggest that impaired emotional competence may also lead to an increase in perceived stress, which is a possible trigger of angioedema attacks.”
As recommended by current guidelines, management of these patients should include psychological intervention, if needed, and encourage cooperation between physicians and psychologists.
“Such interventions may help reduce stress levels and, ultimately, control factors that trigger attacks and contribute to the unpredictability of disease manifestations,” investigators said.