Background: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking.A mobile app for self-management of PERT was developed in the context of the HORIZON 2020 project MyCyFAPP. It contains an algorithm to calculate individual PERT-doses for optimal fat digestion, based on in vitro and in vivo studies carried out in the same project.In addition, the app includes a symptoms diary, educational material, and it is linked to a web tool allowing health care professionals to evaluate patient's data and provide feedback. Methods: A 6-month open label prospective multicenter interventional clinical trial was performed to assess effects of using the app on gastro-intestinal related quality of life (GI QOL), measured by the CF-PedsQL-GI (shortened, CF specific version of the Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Module). Results: One hundred and seventy-one patients with CF and PI between 2 and 18 years were recruited at 6 European CF centers. Self-reported CF-PedsQL-GI improved significantly from month 0 (M0)

Cystic fibrosis increases the risk of developing anxiety and depression in female patients and in mothers. Geographical differences were observed, with higher anxiety and depression in southern Italy for parents, but not for patients. Anxiety and depression levels also depend on clinical status. Pediatr Pulmonol. 2016;51:1311-1319. © 2016 Wiley Periodicals, Inc.

In order to overcome the cross-cultural semantic barriers related to the literal translation of the McGill Pain Questionnaire (MPQ) in non-English speaking areas, an Italian Pain Questionnaire (IPQ) has been developed, based on the 3 factorial structures proposed by Melzack and Torgerson: sensory, affective and evaluative. A group of 30 normal subjects (15 doctors and 15 university students) was used to define 5 anchor words of the intensity verbal scale by means of a visual analogue scale, and a 5-point Present Pain Intensity (PPI) verbal scale was derived. For the semantic key, a first group of 80 subjects (30 university students and patients, respectively, and 20 doctors) was asked to sort out appropriate pain descriptors from 203 pain-related words with the help of clinical literature and Italian dictionaries. Subsequently, a second group of 80 subjects (of identical structural composition) was asked to allocate the 56 words previously chosen on the basis of word frequencies (at least 45%) to the most appropriate category in the Italianized MPQ. Testees were then asked to assign an intensity value to each word, using a VAS scale. The final pain vocabulary was formed from those words, which reflected a statistically significant intensity change (P less than 0.05) within each group. The IPQ comprises 42 pain descriptors, distributed into 3 major classes (sensory, affective and evaluative) and 16 subclasses. It represents the most parsimonious, meaningful and idiomatic set of Italian pain descriptors, providing quantitative information that can be treated statistically, yet preserving a close structural parallel with the MPQ.

Managing CF can be emotionally and physically challenging for patients and their relatives. The disease and its treatment influence the ability to tackle normal tasks of daily living and unexpected life events. The context within which psychologists work varies according to different cultural backgrounds and their professional and theoretical memberships. The benchmarks presented here focus on four crucial issues: (i) identifying a common base of tools and theoretical reflections through suggested readings, (ii) interdisciplinary work within a CF team and its importance for both persons with CF and other healthcare professionals, (iii) the benefits of an eclectic approach utilising cognitive-behavioural theories for specific psychological problems and, (iv) effective and evaluated transition programmes from paediatric to adult healthcare services.