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Table S1. Binary Logistic Regression analysis: Coddington Life Event Scale (CLES) versus Total Severity Score and Alexithymia Questionnaire for Children (AQC). (DOCX 20 kb)

A diagnosis of chronic illness of a child has a pervasive impact on the life of the family. Parents play a crucial role in their child’s illness experience in that they build the context for the meaning-making and the strategies to face the illness (Freda et al. 2016). In this mixed-method study we intend to explore: 1) the relation between parents’ elaboration of the diagnosis, parents-children dialogues about the illness and the management strategies in daily life;2) the influences of the the specificity of the diseases on these processes. To date, we administered the Reaction to Diagnosis Interview (RDI)(Marvin & Pianta,1996), and another ad-hoc structured interview on symptoms interpretations, parent-children dialogues and on the management strategies(Savarese et al, in press) to 40 parents of children affected form 3 different chronic diseases: Hereditary Angioedema(group1), Rheumatoid Arthritis(group2) and Diabetes(group3). Results from the analysis of the interviews show that in group 1, 82% are “resolved”; in group 2, 43% are resolved, and in group 3, 50% are resolved. In all the groups, dialogues in families with unresolved classifications are characterized from the lack or the confusion of the explanations about the the disease. These classifications will be discussed with reference to the critical areas of each disease. Moreover, the unresolved classifications are linked to strategies of avoidance that convey in heavy limitations in the physical as well as the emotional expression of the children. On the other hand, resolved classifications are linked to strategies for the promotion of resilient skills in children. In the light of the intention to develop a model of intervention for the support of the health-care relation, these results show the crucial role of parents’ elaboration of their child’s diagnosis for the construction of resilient strategies for the management of the diseases.

Background Hereditary 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). Results Mean 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. Conclusions Alexithymia 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 material The online version of this article (10.1186/s13023-018-0871-x) contains supplementary material, which is available to authorized users.