Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis

Andrea Gramegna; Stefano Aliberti; Adelina Amorim; Francesco Blasi; Stephen Bourke; Pierre-Régis Burgel; Filia Diamantea; Isabelle Durieu; Libor Fila; Rosa María Girón Moreno; Barbara Messore; Eva Pokojová; Giovanni Taccetti; Stijn Verhulst; Andrea Vukić Dugac; Sabine Wege; Alistair Duff; Kevin W Southern; Carlo Castellani

doi:10.1016/j.jcf.2023.11.001

Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis

J Cyst Fibros. 2023 Nov 8:S1569-1993(23)01672-7. doi: 10.1016/j.jcf.2023.11.001. Online ahead of print.

Authors

Andrea Gramegna¹, Stefano Aliberti², Adelina Amorim³, Francesco Blasi¹, Stephen Bourke⁴, Pierre-Régis Burgel⁵, Filia Diamantea⁶, Isabelle Durieu⁷, Libor Fila⁸, Rosa María Girón Moreno⁹, Barbara Messore¹⁰, Eva Pokojová¹¹, Giovanni Taccetti¹², Stijn Verhulst¹³, Andrea Vukić Dugac¹⁴, Sabine Wege¹⁵, Alistair Duff¹⁶, Kevin W Southern¹⁷, Carlo Castellani¹⁸

Affiliations

¹ Department of Pathophysiology and Transplantation, University of Milan, Milan 20122, Italy; Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan 20122, Italy.
² Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele, Milan 20072, Italy; IRCCS Humanitas Research Hospital, Respiratory Unit, Via Manzoni 56, 20089 Rozzano, Milan, Italy. Electronic address: stefano.aliberti@hunimed.eu.
³ Adult Reference CF Center, Pulmonology Department, Faculty of Medicine, University of Porto, Portugal.
⁴ Adult Cystic Fibrosis Center, Royal Victoria Infirmary, Newcastle upon Tyne, United Kingdom.
⁵ Department of Respiratory Medicine and French National Cystic Fibrosis Reference Center, Cochin Hospital, APHP, Paris France; Université Paris Cité and Inserm U1016, Institut Cochin, Paris, France; ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany.
⁶ Adult CF Unit, Sismanoglio General Hospital, Athens, Greece.
⁷ ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany; Cystic Fibrosis Adult Refence Center, Department of Internal Medicine, Groupe Hospitalier Sud, Hospices Civils de Lyon, 69495 Pierre Benite Cedex; INSERM U1290 RESHAPE Research in Health Care Performance, Lyon 1 Claude Bernard University, Lyon, France.
⁸ Cystic Fibrosis Centre, Department of Pneumology, University Hospital in Motol, Prague, Czech Republic.
⁹ Adult Reference CF Center, Pulmonology Department. Instituto de investigacion La Princesa. Calle Diego de Leon 62, Madrid 28006 Spain.
¹⁰ Adult Cystic Fibrosis Center, Pulmonology Dept, AOU San Luigi Gonzaga, Orbassano, Turin, Italy.
¹¹ ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany; Department of Respiratory Diseases, University Hospital Brno, Jihlavska Str. 20, 62500 Brno, Czech Republic; Faculty of Medicine, Masaryk University, Brno, Czech Republic.
¹² Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy.
¹³ Lab of Experimental Medicine and Pediatrics, University of Antwerp and department of Pediatrics, Antwerp University Hospital, Antwerp, Belgium.
¹⁴ Cystic Fibrosis Centre for Children and Adults, University Hospital Centre Zagreb, Croatia; School of Medicine, University of Zagreb, Croatia.
¹⁵ Department of Pulmonology and Respiratory Medicine, Thoraxklinik at the University Hospital of Heidelberg, Röntgenstr. 1, 69126 Heidelberg, Germany.
¹⁶ Leeds Children's Hospital, Leeds, United Kingdom.
¹⁷ Women and Children's Health, University of Liverpool, Liverpool, United Kingdom.
¹⁸ Cystic Fibrosis Center, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

PMID: 37949745
DOI: 10.1016/j.jcf.2023.11.001

Abstract

Background: Although cystic fibrosis (CF) standards of care have been produced and regularly updated, they are not specifically targeting at the adult population. The ECFS Standards of Care Project established an international task force of experts to identify quality standards for adults with CF and assess their adherence.

Methods: This study was composed of two phases. In the first one, a task force of international experts derived from published guidelines and graded ten quality standards for adult CF care using a modified Delphi methodology. In the second phase, an international audit was conducted among adult CF centers to retrospectively validate the quality statements and monitor adherence.

Results: The task force identified 10 quality standards specific to the care of adults with CF, mainly based on the 2018 ECFS standards of care. 14 adult CF centers participated in the audit, which showed that most quality standards for the management of CF in adults are met across Europe. Heterogeneity in adherence to standards was found across centers according to geographical setting and centers' characteristics.

Conclusions: The identification of quality standards is a valuable resource for the standardization and monitoring of care delivery across centers taking care of adults with CF.

Keywords: Adulthood; Cystic fibrosis; Cystic fibrosis centers; Quality standards; Standards of care.