New guideline highlights individualized physical activity, exercise plans for cystic fibrosis

A new statement outlines recommendations for health care professionals to determine physical activity and exercise plans for patients with cystic fibrosis.

The Exeter Activity Unlimited Statement is a 24-statement evidence-driven document. The statement was published in Chronic Respiratory Disease.

“The promotion of physical activity for people with complex medical conditions is not usual, although evidence shows it to be effective in disease management,” Craig A. Williams, MD, professor of pediatric physiology and health at the Children’s Health and Exercise Research Centre, Sport and Health Science at the University of Exeter and the Royal Devon and Exeter NHS Foundation Trust Hospital, Exeter, U.K., said in a related press release. “This consensus integrates physiological, psychological and social benefits of being physically active to support clinical teams and help people with cystic fibrosis lead a life unlimited.”

Expert consensus

An international, multidisciplinary group of 39 stakeholders that included researchers, health care professionals and patients from 11 countries participated in a virtual conference June 30 and July 1, 2021, to develop evidence-based and informed expert consensus on physical activity and exercise for individuals with cystic fibrosis.

Twenty-four statements in the final document surpassed the consensus threshold of 80% or more agreement for people with cystic fibrosis, including:

• Physical activity and exercise affect survival and improve overall patient well-being; engagement can potentially positively influence cost associated with hospitalizations, exacerbations and antibiotic use.
• Physical activity and exercise improve cardiovascular health; resistance training improves limb muscle strength; exercise of adequate frequency and progressive intensity improves fitness; health-related quality of life is an important outcome for inclusion in such interventions.
• Accelerometers can provide accurate information on physical activity; there are many safe, effective and informative options for exercise testing; lab-based tests provide insights to the determinants of exercise capacity, but field-based tests provide useful information; standardized methods should be utilized to enable within- and between-person comparisons;
• Individualized and comprehensive training programs are recommended; lifelong adherence to habitual physical activity and exercise throughout life is important and challenging; the provision of exercise is important and requires additional staffing and expertise to offer annual exercise testing and prescription; and remote exercise training is feasible and accessible;
• Exercise with huff or cough improves mucus clearance; clinical efficacy of cystic fibrosis transmembrane conductance regulator therapy on fitness and physical activity behaviors warrants further study; physical activity and exercise are strongly recommended; both energy intake and hydration should be adjusted to meet the elevated caloric and fluid needs of this patient population.
• Physical activity and exercise prescription should be individualized; clinical trials should include outcomes associated with physical activity and exercise to understand the effect of lifestyle parameters; future trials should include outcomes like CV health, mental health, endocrine, inflammatory function and patient-reported outcomes; and examine if exercise can replace airway clearance techniques; the interactive effects of cystic fibrosis transmembrane conductance regulator therapies, physical activity and exercise; and how to improve adherence.

“Research has shown that there is a lack of confidence and understanding in how to utilize exercise for management of cystic fibrosis, therefore, we hope that these resources can provide an easy-to-use method to plug that knowledge gap,” Owen Tomlinson, PhD, co-author and lecturer at the University of Exeter, said in the release.

Implications

According to the authors, this statement, along with critical future directions in cystic fibrosis, provides information to aid initiation, implementation and promotion of physical activity recommendations and exercise programs across hospitals, outpatient clinics, home, schools and workplaces.

“This research is really important in highlighting what we do and do not currently know about the relationship between cystic fibrosis and exercise,” Daniel Beever, MPH, researcher at the School of Health and Related Research at the University of Sheffield, U.K., said in the release. “Hopefully it can lead to more effective, focused and personalized approaches to exercise that, alongside other developments in care and treatment, further help people with cystic fibrosis to lead longer, healthier lives.”

Reference:

• Experts unite to provide guidelines on exercise and physical activity in cystic fibrosis. Published Sept. 7, 2022. Accessed Sept. 7, 2022.