ECFS best practice guidelines: the 2018 revision

Abstract

Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.

Change in lung clearance index with microbiological status in children with cystic fibrosis

“…Consecutive CF patients admitted to outpatient clinic between February 2017 and February 2018 were analyzed for participation in the study. Subjects who met all of the inclusion criteria (male or female aged 5‐18, a confirmed diagnosis of CF based on current diagnostic criteria, ability to perform lung function tests: MBW and spirometry) and none of the exclusion criteria (lack of cooperation, severe clinical condition making it impossible to perform MBW, and spirometry, eg, dyspnea, hemoptysis, severe complications of CF) were included in the trial. Information about genotype, age of diagnosis, respiratory infection based on previous 12 months culture results was obtained from the clinical center.…”

Bronchiectasis: Treatment decisions for pulmonary exacerbations and their prevention

“…Bronchiectasis guidelines recommend long‐term antibiotics for those with ≥3 exacerbations per year, with or without chronic infection with P. aeruginosa, but the more recent guideline suggests use for ≥2 exacerbations in the community or one hospitalization as indications for long‐term antibiotics. None of the guidelines have recommended rotational antibiotic use, although this practice is standard care in CF with inhaled antibiotics …”

Aerosolized agents for airway clearance in cystic fibrosis

“…Administration of agents, such as hypertonic saline and dry powder mannitol, creates an osmotic gradient which draws water into the airway and rehydrates the ASL, increasing mucociliary clearance . Osmotic agents have a different and possibly complementary mechanism of action to dornase alfa …”

“…The European Cystic Fibrosis Society best practice guidelines consider dornase alfa as the only mucolytic agent with proven efficacy in CF and recommend long‐term maintenance therapy with dornase alfa to maintain treatment effect . The guidelines indicate the potential use of hypertonic saline and mannitol in patients with CF—hypertonic saline is used in many patients with moderate‐to‐severe lung disease and is recommended by the CF Foundation guidelines, while the recently introduced mannitol has been shown to improve lung function and its powder formulation can potentially reduce treatment time . Since both agents can result in bronchospasm, the guidelines recommend pretreatment with a bronchodilator and tolerability testing before their long‐term use …”