New international guidelines for cystic fibrosis

The European CF Society Exercise Working Group (ECFS) has developed recommendations and instructions for healthcare professionals carrying out exercise tests on individuals with cystic fibrosis (CF).

Dr Zoe Saynor from the University of Portsmouth in England led the project for this inherited condition caused by a faulty gene that affects the movement of salt and water across cell surfaces. It is a multisystem chronic condition, and the mutation in the gene results in the accumulation of sticky mucus in the lungs and digestive system, leading to a range of challenging symptoms.

While there is no cure, a wide range of treatments including physical activity and exercise are recommended to manage CF. International guidelines recommend regular exercise testing of people with the condition. These tests include establishing aerobic fitness, measuring performance and assessing muscle strength — they are also able to assist in evaluating health trends, response to treatment and health outlook.

The document, published in European Respiratory Reviews, was a collaboration between more than 60 experts, from countries including Australia, the UK, France, America, Switzerland, Germany and others.

Dr Saynor from the School of Sport, Health & Exercise Science at the University of Portsmouth said, “The big focus of the project was to change and improve clinical practice across the world, so we’ve been working together collectively over a number of years.

“We wanted to involve colleagues from areas with different medical care systems, and from both low- and middle-income countries, to ensure our recommendations had as much stakeholder involvement as possible.

“Our goal is that all people with CF of an appropriate age have access to regular exercise testing to better understand their health and be given individualised exercise advice.”

The document also highlights previously used tests that are no longer recommended for individuals with CF, as well as areas that require further research. It represents the work of a multidisciplinary panel of physiotherapists, exercise scientists and clinicians, all members of the European CF Society Exercise Working Group.

The Deputy Coordinator of the ECFS Exercise Working Group, Dr Don Urquhart from the University of Edinburgh, said, “There is still a lot we don’t yet know about people living with CF, but exercise is becoming increasingly important in the management of the condition. We hope that these guidelines will help improve confidence and understanding by having everyone undertake tests in a standardised manner.”

CF Physio President Jenny Hauser added: “Global standardisation across many aspects of physiotherapy management in CF is a strong focus for clinicians in the rapidly developing and changing landscape of this chronic condition.

“Moving towards a consensus internationally on the choices of validated exercise tests, standardised protocols and objective measures in exercise testing will assist in developing robust multicentre research opportunities.

“The guidelines will improve equity in practice across CF centres and will help clinicians in appropriately supporting their patients to develop individualised exercise programs for improved health outcomes.”

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