Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.

This is the third 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 recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.

Improving end-of-life care for adults with cystic fibrosis: an improvement project.

BACKGROUND: Cystic fibrosis (CF) is a life-limiting disease that results in premature death mainly due to respiratory failure. Literature suggests that for many people with CF end-of-life wishes are discussed too late or not at all, with most dying in hospital. The aim of this project was to improve end-of-life care for adults with CF. DESIGN: Three improvement cycles were carried out over a 2-year period in one of the largest adult CF centres in Europe. The first cycle involved implementing regular multidisciplinary team (MDT) debriefs after a patient death with increased education. The second cycle involved codesigning a CF-specific advance care plan (ACP) with patients, families, bereaved relatives and experts across the UK, then implementing this into our service. The final cycle was designing a CF-specific end of life, online course for clinicians. Success was measured by: use of ACP and whether patients had died in their preferred location, patient feedback via a survey and satisfaction with the online course using a postcourse report. RESULTS: The number of patients given the opportunity to discuss their end of life wishes increased from 10% to 85%. The number of patients who died in their preferred location increased from 7% to 85% over the 2-year project time. Patient feedback has been overwhelmingly positive. The key barrier has been changing MDT culture, overcoming this required the engagement of the whole team. The online course has been successful with 258 participants to date from 26 countries. CONCLUSION: Education, staff support and a CF-specific ACP document empowered healthcare professionals to initiate difficult conversations to improve end-of-life care.

Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis.

The tidal and raised volume rapid thoracoabdominal compression techniques are increasingly used to detect diminished airway function in infancy. The aim of this study was to assess the relative ability of parameters measured by these techniques to identify diminished airway function in infants newly diagnosed with cystic fibrosis (CF) with and without clinical evidence of prior lower respiratory illness. A cross-sectional, prospective study design was used in which maximal flow at functional residual capacity (VmaxFRC) from the tidal technique and FVC, FEV0.5, FEF75, and FEF25-75 from the raised volume technique were measured in 47 infants with CF and 187 healthy infants of similar body size, sex distribution, ethnic group, and exposure to maternal smoking. Multiple linear regression was used to assess group differences and to calculate SD scores for each parameter for the infants with CF. Airway function was also compared with clinical assessments of respiratory status made by pediatric pulmonologists. FEV0.5 was significantly diminished in 13 infants with CF, of whom 4 had been identified by clinicians as having normal respiratory status. Only one infant with CF had a VmaxFRC below the estimated normal range. Airway function is diminished in infants with CF irrespective of prior lower respiratory illness and in those whose respiratory status is considered normal by pediatric pulmonologists. In infants with CF, the raised volume technique identified diminished airway function more frequently than the tidal technique.