Standards for the care of people with cystic fibrosis; establishing and maintaining health.

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.

Exploring Strategies for Developing Enabling Environments for People with Chronic Heart Disease: An Ethnographic Study Protocol.

The impact of chronic diseases on people's daily lives and the exponential number of people affected is a major public health issue. The consequences on individuals and their families is significant, particularly in terms of quality of life. In the literature, this phenomenon is well described in terms of care policy and cost. Although there is a link between a supportive environment and empowerment, there is little literature describing a supportive environment and the daily lives of people living with cardiovascular disease. The objectives of this study are to identify the strategies people use to develop an enabling environment. It will be a qualitative ethnographic study that will address both human behavior and the notion of culture in a broad sense. In the context of this study, an orientation towards critical ethnography will be considered for its particular interest in vulnerable people and in the power relations that may exist in the socio-cultural system. Data will be collected directly in people's homes through observations and interviews with 10 people with cardiovascular disease. For each person, the data collection will take place over three days and will represent approximately 210 h of observation. This protocol was registered in the Research Register on 30 June 2021 and its number is 6933. This study will explore strategies for developing an enabling environment for people living with heart disease and eventually provide recommendations for nursing practices in terms of support.

Defining an enabling environment for those with chronic disease: an integrative review.

BACKGROUND: Health policies are currently being implemented to cope with the 37% of those affected by chronic disease and 63% of deaths worldwide. Among the proposals, there is accelerating support for greater autonomy for patients, which incorporates several concepts, including empowerment. To achieve this, develop an environment to increase an individual's capacity for action seems to be a fundamental step. The aim of this study is to characterize an enabling environment for patients in the context of chronic disease management. METHODS: An integrative review design was applied. Medline, CINAHL, and Web of Science databases were searched to identify relevant literature published between 2009 and 2019. Overall, the review process was guided by the PRISMA 2020 checklist. The Mixed Methods Appraisal Tool for quality evaluation was used. RESULTS: A total of 40 articles were analyzed, divided into 18 quantitative studies, 11 qualitative studies, two mixed studies, seven expert opinions, one theory and one conference report. The following characteristics defining an enabling environment were taken from the literature relating to those with a chronic condition: Needs assessment-adaptation of responses, supporting "take care", involvement in support, knowledge improvement, engagement with professionals, use of information and communication technologies, and organization of care. Beyond that, the interactions highlighted between these seven categories characterize an enabling environment. CONCLUSION: This review specifies the essential elements of an enabling environment for patients with chronic conditions. It encompasses the partnership between the healthcare professional, such as the advanced practice nurse, and the individual for whom interventions and care strategies must be devised.

Using registry data to improve quality of care.

Patient registries provide clinicians, patients and families with the ability to track important health outcomes at a population, cystic fibrosis (CF) center, and patient level. International quality improvement (QI) work driven by registries has been effective at improving the health and the care delivered to the individual patient. In this review, we examine the role CF registries have played in the QI process over the years and discuss the inherent strengths and limitations that exist when using registry data for this purpose.

European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis.

Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful treatments and patient outcomes to be recognized and improvement programmes to be implemented in specialist CF centres. Over the past decades, the greater volumes of data becoming available through Centre databases and patient registries led to the possibility of making comparisons between different therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons.