Exploring disease perception in Behçet's syndrome: combining a quantitative and a qualitative study based on a narrative medicine approach.

BACKGROUND: Behçet Syndrome (BS) has a significant psychological and social impact on patients, caregivers and families. The present study aims at exploring disease perception in BS patients, using both a co-designed survey and the narrative medicine (NM) approach. METHODS: An ad-hoc questionnaire was co-designed by clinicians expert in BS, BS patients and caregivers and BS adult patients were invited to answer the online questionnaires. Cluster analysis was used to analyse data from the survey and to identify groups of patients with diverse disease perception. To further explore real-life perspectives, the stories of illness of a smaller group of adult BS patients were anonymously collected online and analysed by means of text, sentiment and qualitative analysis. RESULTS: Two hundred and seven patients answered the survey and forty-three stories were collected. The cluster analysis highlighted that accepting or not the disease has a strong impact on the daily life, on how BS patients perceive themselves and in terms of hope for the future. The stories revealed that patients often address common issues, such as the long and complex journey faced from the disease onset until the BS diagnosis, which was strongly connected to the concept of time and perceived as an exhausting period of their lives. CONCLUSION: To our knowledge, this is the first study that addressed disease perception also applying the NM principles in BS. The current perception that BS patients have of their disease should encourage the BS scientific and patient community in joining forces in order to improve the journey of BS patients.

Sleep quality in Behçet's disease: a systematic literature review.

Behçet's Disease (BD) can be correlated with sleep impairment and fatigue, resulting in low quality of life (QoL); however, a comprehensive evaluation of this issue is still missing. We performed a systematic literature review (SLR) of existing evidence in literature regarding sleep quality in BD. Fifteen papers were included in the SLR. Two domains were mainly considered: global sleep characteristics (i) and the identification of specific sleep disorders (ii) in BD patients. From our analysis, it was found that patients affected by BD scored significantly higher Pittsburgh Sleep Quality Index (PSQI) compared to controls. Four papers out of 15 (27%) studied the relationship between sleep disturbance in BD and disease activity and with regards to disease activity measures, BD-Current Activity Form was adopted in all papers, followed by Behçet's Disease Severity (BDS) score, genital ulcer severity score and oral ulcer severity score. Poor sleep quality showed a positive correlation with active disease in 3 out of 4 studies. Six papers reported significant differences between BD patients with and without sleep disturbances regarding specific disease manifestations. Notably, arthritis and genital ulcers were found to be more severe when the PSQI score increased. Our work demonstrated lower quality of sleep in BD patients when compared to the general population, both as altered sleep parameters and higher incidence of specific sleep disorders. A global clinical patient evaluation should thereby include sleep assessment through the creation and adoption of disease-specific and accessible tests.

Empowering rare disease patients through patient education: the new BehçeTalk programme.

BACKGROUND: Educating patients and caregivers on their disease can improve their knowledge and promote the active involvement in the therapeutic decision-making process. Naturally, patient education programmes are critically important in rare systemic autoimmune diseases, where relevant knowledge and expertise still remain scattered. Behçet's disease (BD) represents a challenging rare condition, characterized by a variable spectrum of disease profile and a relapsing course. RESULTS: Recently, BehçeTalk, an educational programme tailored for BD patients, families and caregivers with, was launched. BehçeTalk, entirely co-designed with BD patients, is offering educational on-line webinars on different aspects of the disease, as well support groups for patients and caregivers coordinated by a psychologist with specific expertise in BD. CONCLUSIONS: The therapeutical management of BD is often challenging and frequently includes off-label treatments. Considering the specificities of BD, providing a specific education on the disease to patients will lead to empower them in being part of the decision-making processes, in the self-management and in improving their quality of life.

A systematic literature review of existing tools used to assess medication adherence in connective tissue diseases: the state of the art for the future development of co-designed measurement tools.

Lack of medication adherence is frequent in chronic connective tissue diseases and is associated with poorer health outcomes, low quality of life and economic loss. This research is based on a systematic literature search and aims to identify the surveys and tools used for the assessment of medication adherence in patients with connective tissue diseases (CTDs) and in particular the tools co-designed with patients. A systematic literature review was performed in PubMed and Embase databases searching for studies concerning the application of surveys or tools designed for medication adherence assessment. A specific analysis was also performed to identify which of these existing tools were developed in co-design with patients affected by CTDs. 1958 references were identified, and 31 studies were finally included. Systemic lupus erythematosus was the most investigated disease, followed by the Behçet's disease. The tools used to assess adherence in CTDs were, in most cases, valid and useful. However, the results showed a certain degree of heterogeneity among the studies and the medication adherence assessment and measurement tools adopted, which were mostly based on selfreported questionnaire. No co-designed tools with patients were found. Low- and non-adherence were explored in some CTDs with valid and useful tools, while other CTDs still need to be assessed. Therefore, more efforts should be made to better understand the specific reasons for the low- and non-adherence in CTDs patients.

Usefulness of Helicobacter pylori stool antigen test to monitor response to eradication treatment in children.

BACKGROUND: The monitoring of the results of eradication treatment is a crucial step for patients with Helicobacter pylori gastritis. A non-invasive test for H. pylori antigens in stools (HpSA) was recently validated for children. AIM: To evaluate the accuracy of HpSA in monitoring eradication treatment in children. METHODS: In 60 children, H. pylori gastritis was diagnosed by endoscopy and the 13C-urea breath test. The children were treated and returned for a follow-up (13)C-urea breath test 6 weeks after the end of treatment. Children were considered cured when the (13)C-urea breath test was negative. Stool were collected at baseline, and at 2 and 6 weeks. Stool antigens were measured by HpSA. RESULTS: According to (13)C-urea breath test, 6 weeks after the end of treatment 49 children were cured and 11 were still H. pylori-positive. The sensitivity and specificity of HpSA on stools collected 2 weeks after therapy were 100%. At 6 weeks specificity was 93.9 and sensitivity 100%. Results by visual reading were concordant with the plate-reader in all but two cases at baseline. CONCLUSIONS: HpSA is accurate for monitoring treatment in children as early as 2 weeks after therapy, when information is most useful and unachievable with other tests. Results by visual reading are accurate, and this can make the test cheaper and more practical.

Precocious puberty in immigrant children: indications for treatment.

Immigration from developing countries to Europe has greatly increased during the last few years. Very little information is available on children who immigrated with their biological family, thus this paper deals only with children immigrating by adoption. In recent years some studies have shown an increased incidence of precocious puberty among adopted children, but information on this issue is scanty. Several hypotheses have been put forward to explain precocious puberty in adopted children, including nutritional, ethnic and/or emotional factors. Untreated precocious puberty is associated with short stature and has social and psychological consequences in addition to those related to adoption. Therefore, indications for therapy need to be carefully planned. In adopted children age estimation is a possible source of error in timing of puberty as date of birth is often missing or inaccurate. Thus each child must be carefully evaluated.