Improving quality of life in patients with rare autoimmune liver diseases by structured peer-delivered support (Q.RARE.LI): study protocol for a transnational effectiveness-implementation hybrid trial.

BACKGROUND: Psychosocial support is a crucial component of adequate rare disease care, but to date psychosocial support needs of this patient population are insufficiently met. Within Q.RARE.LI, we strive to evaluate the effectiveness of a structured, transdiagnostic, and location-independent psychosocial support intervention in routine care of patients with rare autoimmune liver diseases in five countries and prepare its implementation. METHODS: Within an effectiveness-implementation hybrid trial, we aim to a) investigate the effectiveness of the intervention in routine care in five diverse healthcare systems and b) assess implementation outcomes, examine and prepare the implementation context, and develop country-specific implementation strategies. To assess effectiveness, we will include N = 240 patients with rare autoimmune liver diseases. Within a two-armed randomized controlled trial (allocation ratio 1:1), we will compare structured and peer-delivered psychosocial support in addition to care-as-usual (CAU) with CAU alone. Outcomes will be assessed via electronic database entry prior to intervention, directly after, and at a three-month follow-up. Our primary effectiveness outcome will be mental health-related quality of life at post-assessment. Secondary outcomes include depression and anxiety severity, perceived social support, helplessness, and disease acceptance. Implementation outcomes will be assessed within a mixed-methods process evaluation. In a quantitative cross-sectional survey, we will examine perceived acceptability and feasibility in patients, peer-counselors, and healthcare providers involved in delivery of the intervention. In qualitative focus groups, we will analyze the implementation context and determine barriers and facilitators for implementation with different stakeholders (patients and/or representatives, peer-counselors, healthcare providers, health insurers). Based on these results, we will derive country-specific implementation strategies and develop a concrete implementation plan for each country. DISCUSSION: The intervention is expected to help patients adjust to their disease and improve their mental quality of life. The transdiagnostic and location-independent program has the potential to reach patients for psychosocial support who are usually hard to reach. By preparing the implementation in five countries, the project can help to make low-threshold psychosocial support available to many patients with rare diseases and improve comprehensive healthcare for an often neglected group. TRIAL REGISTRATION: ISRCTN15030282.

Association of adherence to therapy and complementary and alternative medicine use with demographic factors and disease phenotype in patients with inflammatory bowel disease.

BACKGROUND AND AIMS: Previous studies have suggested an increasing use of complementary and alternative medicine (CAM) in patients with inflammatory bowel disease (IBD). Furthermore, a significant number of IBD patients fail to comply with treatment. The aim of our study was to evaluate the prevalence of non-adherence and the use of CAM in Hungarian patients with IBD. METHODS: A total of 655 consecutive IBD patients (CD: 344, age: 38.2 [SD 12.9]years; UC: 311, age: 44.9 [15.3]years) were interviewed during the specialist visit by self-administered questionnaire including demographic and disease-related data as well as items analyzing the extent of non-adherence and CAM use. Patients taking more than 80% of each prescribed medication were classified as adherent. RESULTS: The overall rate of self-reported non-adherence (CD: 20.9%, UC: 20.6%) and CAM (CD: 31.7%, UC: 30.9%) use did not differ between Crohn's disease (CD) and ulcerative colitis (UC). The most common causes of non-adherence were: forgetfulness (47.8%), too many/unnecessary pills (39.7%), being afraid of side effects (27.9%) and too frequent dosing. Most common forms of CAM were herbal tea (47.3%), homeopathy (14.6%), special diet (12.2%), and acupuncture (5.8%). In CD, disease duration, date of last follow-up visit, educational level and previous surgeries were predicting factors for non-adherence. Alternative medicine use was associated in both diseases with younger age, higher educational level, and immunosuppressant use. In addition, CAM use in UC was more common in females and in patients with supportive psychiatric/psychological therapy. CONCLUSIONS: Non-adherence and CAM use is common in patients with IBD. Special attention should be paid to explore the identified predictive factors during follow-up visits to improve adherence to therapy and improving patient-doctor relationship.

[Treatment adherence and use of complementary and alternative medicine in patients with inflammatory bowel disease]. / A terápiás adherencia, valamint a komplementer és alternatív gyógymódok használata gyulladásos bélbetegek kezelésében.

UNLABELLED: Previous studies have suggested an increasing use of complementary and alternative medicine (CAM) in patients with inflammatory bowel disease (IBD). Furthermore, a significant number of IBD patients fail to comply with treatment. The aim of our study was to evaluate the prevalence of non-adherence the use of CAM in Hungarian patients with IBD. METHODS: A total of 655 consecutive IBD patients (Crohn's disease [CD]: 344, age: 38.2 + or - 12.9 years; ulcerative colitis [UC]: 311, age: 44.9 + or - 15.3 years) were interviewed during the visit at specialists by self-administered questionnaire including demographic and disease-related data, as well as items analyzing the extent of non-adherence and CAM use. Patients taking more then 80% of each prescribed medicine were classified as adherent. RESULTS: The overall rate of self reported non-adherence (CD: 20.9%, UC: 20.6%) and CAM (CD: 31.7%, UC: 30.9%) use was not different between CD and UC. The most common causes of non-adherence were: forgetfulness (47.8%), too many/unnecessary pills (39.7%), being afraid of side effects (27.9%) and too frequent dosing. Most common forms of CAM were herbal tee (47.3%), homeopathy (14.6%), special diet (12.2%), and acupuncture (5.8%). In CD, disease duration, date of last follow-up visit, educational level and previous surgeries were predicting factors for non-adherence. Alternative medicine use was associated in both diseases with younger age, higher educational level and immunosuppressant use. In addition, CAM use in UC was more common in females and in patients with supportive psychiatric/psychological therapy. CONCLUSIONS: Non-adherence and CAM use is common in patients with IBD. Special attention should be paid to explore the identified predictive factors during follow-up visits to improve adherence to therapy and improving patient-doctor relationship.

Early azathioprine/biological therapy is associated with decreased risk for first surgery and delays time to surgery but not reoperation in both smokers and nonsmokers with Crohn's disease, while smoking decreases the risk of colectomy in ulcerative colitis.

BACKGROUND/AIMS: Smoking may alter the natural course of Crohn's disease (CD). Smokers are more likely to develop complications, relapses and have a greater risk for surgery. In contrast, in ulcerative colitis (UC), smoking might improve the disease course. Our aim was to assess the combined effect of disease phenotype, smoking, and immunomodulator [azathioprine (AZA), AZA/biological] treatment on the risk of intestinal resection/reoperation in CD and colectomy in UC. PATIENTS/METHODS: Six hundred and eighty-one inflammatory bowel disease patients were analyzed (CD: 340, male/female: 155/185, duration: 9.4+/-7.5 years; UC: 341, male/female: 174/164, duration: 11.5+/-9.7 years). Patients were interviewed on their smoking habits at the time of diagnosis and during the regular follow-up visits. Medical records were retrospectively analyzed. RESULTS: Smoking was present in 45.5% in CD and 15.8% in UC. CD patients who underwent at least one bowel resection comprised 46.5%. In an univariate analysis, disease location, behavior, AZA, or AZA/biological use before surgery [odds ratio (OR): 0.26 and 0.22, P<0.001] and smoking (OR: 1.61, P = 0.03) were associated with risk for first surgery. Smoking, AZA, or AZA/biological (P<0.001) use before first surgery and disease behavior were independently associated with risk for surgery in a proportional Cox-regression analysis. Perianal disease (OR: 3.2, P = 0.001) and frequent relapses (OR: 4.8, P<0.001) but not smoking, AZA, or AZA/biological use after first surgery were predictive for reoperation. In UC, the rate of colectomy was 5.6%. Disease location (P = 0.001) and smoking status (P = 0.02) were independently associated with risk for colectomy in a proportional Cox-regression analysis. CONCLUSION: Our data suggest that early AZA/biological therapy reduces the risk for first operation but not reoperation in CD, in both smokers and nonsmokers. In contrast, smoking was associated with a decreased need for colectomy in UC.

[Bacterial infections in liver cirrhosis]. / Bakteriális fertozések májcirrhosisban.

Bacterial infections are well described complications of cirrhosis that greatly increase mortality rates. Two factors play important roles in the development of bacterial infections in these patients: the severity of liver disease and gastrointestinal haemorrhage. The most common infections are spontaneous bacterial peritonitis, urinary tract infections, pneumonia and sepsis. Gram-negative and gram-positive bacteria are equal causative organisms. For primary prophylaxis, short-term antibiotic treatment (oral norfloxacin or ciprofloxacin) is indicated in cirrhotic patients (with or without ascites) admitted with gastrointestinal haemorrhage (variceal or non-variceal). Administration of norfloxacin is advisable for hospitalized patients with low ascitic protein even without gastrointestinal haemorrhage. The first choice in empirical treatment of spontaneous bacterial peritonitis is the iv. III. generation cephalosporin; which can be switched for a targeted antibiotic regime based on the result of the culture. The duration of therapy is 5-8 days. Amoxicillin/clavulanic acid and fluoroquinolones--patients not on prior quinolone prophylaxis--were shown to be as effective and safe as cefotaxime. In patients with evidence of improvement, iv. antibiotics can be switched safely to oral antibiotics after 2 days. In case of renal dysfunction, iv albumin should also be administered. Long-term antibiotic prophylaxis is recommended in patients who have recovered from an episode of spontaneous bacterial peritonitis (secondary prevention). For "selective intestinal decontamination", poorly absorbed oral norfloxacin is the preferred schedule. Oral ciprofloxacin or levofloxacin (added gram positive spectrum) all the more are reasonable alternatives. Trimethoprim/sulfamethoxazole is only for patients who are intolerant to quinolones. Prophylaxis is indefinite until disappearance of ascites, transplant or death. Long-term prophylaxis is currently not recommended for patients without previous spontaneous bacterial peritonitis episode, not even when refractory ascites or low ascites protein content is present.