Gerald Ullrich

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Different opinions of physicians on the importance of measures to prevent acquisition of Pseudomonas aeruginosa from the environment

BACKGROUND Since chronic infection with mucoid Pseudomonas aeruginosa (PA) is associated with deteriorating lung function, many parents of young children with cystic fibrosis (CF) fear the first PA positive throat swab as a milestone in the progression of the disease. To reduce the risk of PA acquisition from the environment, they perform preventive measures at home or outdoors. METHODS In an attempt to evaluate the attitude of CF physicians towards these measures and the respective consulting practice, we mailed a questionnaire to all 65 certified paediatric CF centres in Germany. RESULTS Physicians from 54 (83%) CF clinics replied. They expressed widely different ideas about the impact of the environment for the acquisition of P. aeruginosa, and recommended a large spectrum of preventive measures. Some physicians proposed only few precautions, which focussed on the prevention of cross-infection between patients, whereas others suggested prevention of any contact with moist or wet places, e.g. use different toothbrushes for mornings and evenings, or do without air-conditioning in the car. CONCLUSIONS CF physicians have different opinions on the risk of PA acquisition from the environment. Doctors who recommend strict precautions could engender a parental fear of a ubiquous threat from invisible bacteria. The resulting extended safety measures might impair the familys quality of life.

Patient experience in cystic fibrosis care: Development of a disease-specific questionnaire.

Objectives The aim of this study was to develop valid and reliable disease-specific questionnaires for adult patients with cystic fibrosis and for parents of minors with cystic fibrosis for assessing patient experience with cystic fibrosis care. Methods The pilot versions of the questionnaires were developed based on a literature review, interviews with health professionals and focus groups. A postal survey with two reminders was conducted in 56 German cystic fibrosis centres recruiting 2874 participants. Psychometric evaluation was done via exploratory factor analysis and reliability and regression analysis. The questionnaires’ ability to differentiate between subgroups and between cystic fibrosis centres was evaluated. Results Response rates were 74% for both adult patients and parents. Ten factors were extracted for both the adult and the parents’ models (Cronbach’s alpha between 0.6 and 0.9), explaining 50% and 48% of the variance, respectively. The factors organisation & access and the doctor–patient/parent-interaction had the highest relevance for a good overall care experience. The questionnaires were able to distinguish between different cystic fibrosis centres. Discussion The questionnaires are well suited for use in internal and external quality management of cystic fibrosis care due to their good psychometric properties, the ability to differentiate between centres and its practicability.

Lung transplant recipients' views on the integration of their new organs

Aim. Although donorship issues and the integration of the new organs are the most distinguishing aspects of lung transplantation (LTx) compared to other kinds of ‘high-tech’ medicine, there is a paucity of papers on that matter. Therefore, we aimed to evaluate these aspects in young adult LTx recipients with at least 1-year survival. Methods. Semi-structured interviews; content analysis of specific parts of the interviews; frequency distributions of resulting categories. Sample. Forty-five adults aged 18–42 years (mean: 32 ± 5.5 years). Post-transplant survival ranged from 1 to 11 years. Results. The majority of recipients (60%) fulfilled criteria of denial towards the foreign organ and/or the donor. However, they were rather sensitive towards the ethical and psychological impact of the upcoming option of living donorship in LTx. Also, the majority of recipients (79%) understood that there might as well be good reason not to opt for a LTx. Conclusions. LTx recipients appeared at first sight ‘pragmatic’ towards the gift of life, but they remained sensitive to its ethical and psychological challenges.

Parenting stress in mothers with cystic fibrosis.

Abstract Aim: To assess the parenting experience of mothers with cystic fibrosis (CF) and to compare with normative data. Methods: Cross-sectional study with a validated generic parental stress questionnaire (PSQ). This PSQ differentiates four components of parental stress: main factor “parental stress”, compounding factor “role restrictions”, protective factors “support from spouse”, and “social support”. Cut-off scores categorise results as “normal”, “borderline” or “concerning”. Sample: Seventy-three women were informed by their local CF centre. Of these, 36 enrolled and had a first-born child aged 1–12 years (consistent with reference values of the PSQ). Of these, 31 (86%) returned the PSQ. Mean age of mothers was 32.6 years ± 6.9 years, mean age of first-born child was 5.2 years ± 3.4 years. Most of the mothers had one biological child, five women had two children and one had three children. Results: Parental stress scores were normally distributed, the same applies for contributing factors and for the two protective factors. Favourable scores were twice as frequent as concerning scores. Mothers of younger children scored slightly better than mothers of school-aged children. Conclusion: In line with the only comparable study, mothers with CF seem to be a remarkably resilient group who mostly cope well with parental stress even in the face of a progressive, chronic disease requiring time-consuming treatment. Implications for Rehabilitation Today, motherhood is increasingly becoming an option in fertile women with cystic fibrosis. The additional burden of parenting seems to be rewarded by fulfilling essential personal goals. CF clinics should routinely address a possible wish for a child and to discuss it, openly.

The ideal and the real world of mental health care delivery

According to a European Consensus, mental health care should be an integral component of CF centre care [1]. Abbott et al. surveyed CF health care professionals in Europe (n = 627) and in the US (n = 618) whether their patients receive regular mental health screening, and how members in their team address mental health issues. This web-based survey [2] revealed that the majority of respondents (73%) had no personal experience with mental health screening. Those who did used well-established instruments. In addition, about one third of respondents from Europe were unable or unsure if they could refer to mental health care clinicians in their hospital. Even though screening itself may not yet be called health care, the current data tell us about the inadequate state of mental care delivery in CF centres. The data raise doubts if mental health problems of CF patients are properly dealt with, whether noticed by screening or because symptoms and problems have occurred during routine care. While the European standards expect psychologists to be part of the CF-team, Abbott et al. reported that 39% of CF professionals in Europe not even had “up-to-date lists of mental health resources and referrals to provide to patients and families”. This is in contrast to an earlier survey, where 80% of respondents indicated “good” access to healthcare professionals, except for clinical pharmacists [3]. Similarly, data from Germany add information obtained directly from adult patients (n = 1220) and parents of children with CF (n = 891). In a nationwide German survey [4] on patient experience and satisfaction we assessed whether or not patients saw allied health professionals (AHPs) in the preceding year. The rate of AHP utilisation was lower than expected, particularly with respect to psychosocial staff: only 48% of parents and 26% of adult patients reported at least one contact with psychosocial staff in the preceding year. Remarkably,

390 Motivational interviewing as a bridge to shared decision making: Further results from a team-centred programme

Professional practice of participants • All (n=120) German CF centers were informed about the course, – 30% of the centers are small (< 20 CF patients) [8] – 65% are accredited as CF centers (including among others a full range multiprofessional staff) • 34 centers registered – 28 of whom are accredited – 18 of whom registered as a „whole team“ • So far 9 courses have been accomplished, counting 170 participants from 17 centres • The maximum number of returned evaluation sheets is 144 (drop of response rate mostly due to organizational failings) 0 25 50 75 100 day 3 day 2 day 1 physic nurse