Travelling with cystic fibrosis: Recommendations for patients and care team members

There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation.

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“…[192]. The use of knowledge questionnaires and other tools like clinical cases and scenarios facilitates the achievement of these objectives.…”

Section: Other Topicsmentioning

confidence: 99%

Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis

Hirche¹,

Knoop

Hebestreit

et al. 2014

Pulmonary Medicine

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“…This identification process mainly takes part during the control process of all answered questions in the English archive as described in the Background section. One concrete result of this process is the recently published European paper on "Travelling with cystic fibrosis" [14]. Other projects will follow leading to more unequivocal counseling and treatment strategies for patients with CF all over Europe.…”

Section: Discussionmentioning

confidence: 99%

Quality assessment of expert answers to lay questions about cystic fibrosis from various language zones in Europe: the ECORN-CF project

d'Alquen

Boeck

Bradley³

et al. 2012

BMC Med Res Methodol

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BackgroundThe European Centres of Reference Network for Cystic Fibrosis (ECORN-CF) established an Internet forum which provides the opportunity for CF patients and other interested people to ask experts questions about CF in their mother language. The objectives of this study were to: 1) develop a detailed quality assessment tool to analyze quality of expert answers, 2) evaluate the intra- and inter-rater agreement of this tool, and 3) explore changes in the quality of expert answers over the time frame of the project.MethodsThe quality assessment tool was developed by an expert panel. Five experts within the ECORN-CF project used the quality assessment tool to analyze the quality of 108 expert answers published on ECORN-CF from six language zones. 25 expert answers were scored at two time points, one year apart. Quality of answers was also assessed at an early and later period of the project. Individual rater scores and group mean scores were analyzed for each expert answer.ResultsA scoring system and training manual were developed analyzing two quality categories of answers: content and formal quality. For content quality, the grades based on group mean scores for all raters showed substantial agreement between two time points, however this was not the case for the grades based on individual rater scores. For formal quality the grades based on group mean scores showed only slight agreement between two time points and there was also poor agreement between time points for the individual grades. The inter-rater agreement for content quality was fair (mean kappa value 0.232 ± 0.036, p < 0.001) while only slight agreement was observed for the grades of the formal quality (mean kappa value 0.105 ± 0.024, p < 0.001). The quality of expert answers was rated high (four language zones) or satisfactory (two language zones) and did not change over time.ConclusionsThe quality assessment tool described in this study was feasible and reliable when content quality was assessed by a group of raters. Within ECORN-CF, the tool will help ensure that CF patients all over Europe have equal possibility of access to high quality expert advice on their illness.

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“…There are no European recommendations on issues specifically related to travel [74]. The main predictors of medical safety during air travel are the duration of flight and patients' condition [74].…”

Section: Travelmentioning

confidence: 99%

Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

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Travelling with cystic fibrosis: Recommendations for patients and care team members

Cited by 17 publications

References 58 publications

Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis

Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis

Quality assessment of expert answers to lay questions about cystic fibrosis from various language zones in Europe: the ECORN-CF project

Adult cystic fibrosis care in the 21st century

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