{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T02:44:16Z","timestamp":1774579456031,"version":"3.50.1"},"reference-count":109,"publisher":"Georg Thieme Verlag KG","issue":"02","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Exercise and airway clearance techniques (ACTs) have been a cornerstone of treatment for people with cystic fibrosis (pwCF) for many decades. Exercise may confer both respiratory and nonrespiratory benefits for pwCF, with greater exercise capacity associated with improved survival. A wide variety of exercise interventions for pwCF have been investigated. ACTs may assist in reducing respiratory symptoms for pwCF and are currently recommended to be performed daily, with the types of ACTs used varying globally. While recommended components of care, both exercise and ACTs are time-intensive and maintaining adherence to the recommendations over the longer term can be challenging. It has been proposed that with advances in the therapeutic options for pwCF, a rationalization of the therapeutic regimen may be possible. We summarize the current evidence for the use of exercise and ACTs by pwCF, discuss the implications of the introduction of cystic fibrosis transmembrane conductance regulator modulators on both exercise and ACTs, and highlight areas for further research.<\/jats:p>","DOI":"10.1055\/s-0042-1758729","type":"journal-article","created":{"date-parts":[[2022,12,20]],"date-time":"2022-12-20T01:12:54Z","timestamp":1671498774000},"page":"209-216","source":"Crossref","is-referenced-by-count":5,"title":["Exercise and Airway Clearance Techniques in Cystic Fibrosis"],"prefix":"10.1055","volume":"44","author":[{"given":"Nathan","family":"Ward","sequence":"additional","affiliation":[{"name":"Physiotherapy\/Department of Thoracic Medicine, Royal Adelaide Hospital, Adelaide, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathy","family":"Stiller","sequence":"additional","affiliation":[{"name":"Allied Health, Central Adelaide Local Health Network, Adelaide, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne E.","family":"Holland","sequence":"additional","affiliation":[{"name":"Department of Physiotherapy, Alfred Health, Central Clinical School, Monash University and Institute for Breathing and Sleep, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2022,12,19]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"J S Elborn","year":"2016","journal-title":"Lancet"},{"issue":"05","key":"ref2","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.jcf.2017.02.007","article-title":"Peripheral muscle abnormalities in cystic fibrosis: etiology, clinical implications and response to therapeutic interventions","volume":"16","author":"M Gruet","year":"2017","journal-title":"J Cyst Fibros"},{"issue":"04","key":"ref3","first-page":"522","article-title":"Cystic fibrosis pulmonary guidelines: airway clearance therapies","volume":"54","author":"P A Flume","year":"2009","journal-title":"Respir Care"},{"issue":"04","key":"ref4","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1111\/resp.12764","article-title":"Physiotherapy for cystic fibrosis in Australia and New Zealand: a clinical practice guideline","volume":"21","author":"B M Button","year":"2016","journal-title":"Respirology"},{"issue":"07","key":"ref5","doi-asserted-by":"crossref","first-page":"392","DOI":"10.5694\/mja10.11229","article-title":"Changes in cystic fibrosis mortality in Australia, 1979-2005","volume":"195","author":"D W Reid","year":"2011","journal-title":"Med J Aust"},{"issue":"03","key":"ref7","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1586\/ers.12.19","article-title":"Exercise as part of a cystic fibrosis therapeutic routine","volume":"6","author":"S Rand","year":"2012","journal-title":"Expert Rev Respir Med"},{"issue":"04","key":"ref8","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1016\/S0022-3476(64)80290-0","article-title":"A therapeutic regimen for patients with cystic fibrosis","volume":"65","author":"L W Matthews","year":"1964","journal-title":"J Pediatr"},{"issue":"25","key":"ref9","doi-asserted-by":"crossref","first-page":"1785","DOI":"10.1056\/NEJM199212173272504","article-title":"The prognostic value of exercise testing in patients with cystic fibrosis","volume":"327","author":"P A Nixon","year":"1992","journal-title":"N Engl J Med"},{"issue":"08","key":"ref10","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1164\/rccm.201806-1110OC","article-title":"Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis","volume":"199","author":"H Hebestreit","year":"2019","journal-title":"Am J Respir Crit Care Med"},{"issue":"06","key":"ref11","first-page":"527","article-title":"The fitter the better? 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Morrison","year":"2020","edition":"4th ed."},{"key":"ref19","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1097\/CPT.0000000000000016","article-title":"Exercise and habitual physical activity for people with cystic fibrosis: expert consensus, evidence-based guide for advising patients","volume":"26","author":"A Swisher","year":"2015","journal-title":"Cardiopulm Phys Ther J"},{"issue":"01","key":"ref20","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1249\/MSS.0b013e3182a38da8","article-title":"Effects of a low-volume aerobic-type interval exercise on VO2max and cardiac mass","volume":"46","author":"T Matsuo","year":"2014","journal-title":"Med Sci Sports Exerc"},{"issue":"03","key":"ref21","doi-asserted-by":"crossref","first-page":"151","DOI":"10.7600\/jpfsm.10.151","article-title":"Rationale and optimising of outcomes in high-intensity interval training for health and disease","volume":"10","author":"S Ito","year":"2021","journal-title":"J Phys Fit Sports 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Cox","year":"2013","journal-title":"Cochrane Database Syst Rev"},{"key":"ref32","first-page":"6641853","article-title":"Telerehabilitation using fitness application in patients with severe cystic fibrosis awaiting lung transplant: a pilot study","volume":"2021","author":"A M Layton","year":"2021","journal-title":"Int J Telemed Appl"},{"issue":"06","key":"ref33","doi-asserted-by":"crossref","first-page":"3297","DOI":"10.3390\/ijerph19063297","article-title":"Feasibility and efficacy of telehealth-based resistance exercise training in adolescents with cystic fibrosis and glucose intolerance","volume":"19","author":"C J Holmes","year":"2022","journal-title":"Int J Environ Res Public Health"},{"issue":"01","key":"ref34","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1186\/s13102-021-00323-y","article-title":"Feasibility and implementation of a personalized, web-based exercise intervention for people with cystic fibrosis for 1 year","volume":"13","author":"B Hillen","year":"2021","journal-title":"BMC Sports Sci Med Rehabil"},{"key":"ref35","first-page":"76","article-title":"Web-based physical activity promotion in CF: A clinical trial","volume":"27","author":"N Cox","year":"2022","journal-title":"Respirology"},{"issue":"01","key":"ref36","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1186\/s12890-021-01482-x","article-title":"Factors influencing physical activity in adults with cystic fibrosis","volume":"21","author":"N Hurley","year":"2021","journal-title":"BMC Pulm Med"},{"issue":"02","key":"ref37","doi-asserted-by":"crossref","first-page":"e83","DOI":"10.1016\/j.jcf.2021.08.004","article-title":"Rethinking physical exercise training in the modern era of cystic fibrosis: a step towards optimising short-term efficacy and long-term engagement","volume":"21","author":"M Gruet","year":"2022","journal-title":"J Cyst 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Int"},{"issue":"06","key":"ref47","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1007\/s00038-013-0504-z","article-title":"Population-based reference values for the 1-min sit-to-stand test","volume":"58","author":"A Strassmann","year":"2013","journal-title":"Int J Public Health"},{"issue":"05","key":"ref48","doi-asserted-by":"crossref","first-page":"e049143","DOI":"10.1136\/bmjopen-2021-049143","article-title":"Reference values and validation of the 1-minute sit-to-stand test in healthy 5-16-year-old youth: a cross-sectional study","volume":"11","author":"S R Haile","year":"2021","journal-title":"BMJ Open"},{"issue":"02","key":"ref49","doi-asserted-by":"crossref","first-page":"e0246781","DOI":"10.1371\/journal.pone.0246781","article-title":"Measurement properties of the one-minute sit-to-stand test in children and adolescents with cystic fibrosis: a multicenter randomized cross-over trial","volume":"16","author":"Y Combret","year":"2021","journal-title":"PLoS 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Pulmonol"},{"issue":"08","key":"ref53","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.4187\/respcare.01016","article-title":"Desaturation during the 3-minute step test predicts impaired 12-month outcomes in adult patients with cystic fibrosis","volume":"56","author":"A E Holland","year":"2011","journal-title":"Respir Care"},{"issue":"02","key":"ref54","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1002\/ppul.10213","article-title":"Three-minute step test to assess exercise capacity in children with cystic fibrosis with mild lung disease","volume":"35","author":"I Narang","year":"2003","journal-title":"Pediatr Pulmonol"},{"issue":"12","key":"ref55","doi-asserted-by":"crossref","first-page":"3777","DOI":"10.1002\/ppul.25667","article-title":"Development of the A-STEP: a new incremental maximal exercise capacity step test in cystic fibrosis","volume":"56","author":"L M Wilson","year":"2021","journal-title":"Pediatr Pulmonol"},{"issue":"20","key":"ref56","doi-asserted-by":"crossref","first-page":"1941","DOI":"10.1056\/NEJMra1813799","article-title":"Muco-obstructive lung diseases","volume":"380","author":"R C Boucher","year":"2019","journal-title":"N Engl J Med"},{"issue":"05","key":"ref57","doi-asserted-by":"crossref","first-page":"595","DOI":"10.4187\/aarc0427","article-title":"Cystic fibrosis: pathogenesis and future treatment strategies","volume":"54","author":"F A Ratjen","year":"2009","journal-title":"Respir Care"},{"key":"ref58","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1016\/S0140-6736(09)60327-5","article-title":"Cystic fibrosis","volume":"373","author":"B P O'Sullivan","year":"2009","journal-title":"Lancet"},{"issue":"143","key":"ref59","doi-asserted-by":"crossref","first-page":"160086","DOI":"10.1183\/16000617.0086-2016","article-title":"Personalising airway clearance in chronic lung disease","volume":"26","author":"M McIlwaine","year":"2017","journal-title":"Eur Respir Rev"},{"issue":"05","key":"ref60","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1152\/jappl.1972.32.5.681","article-title":"Interdependence of lung units in intact dog lungs","volume":"32","author":"H Menkes","year":"1972","journal-title":"J Appl Physiol"},{"issue":"01","key":"ref61","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1152\/jappl.1979.46.1.8","article-title":"Lung interdependence and lung-chest wall interaction of sublobar and lobar units in pigs","volume":"46","author":"A Zidulka","year":"1979","journal-title":"J Appl Physiol"},{"issue":"05","key":"ref62","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1152\/jappl.1966.21.5.1443","article-title":"Respiratory bronchioles as the pathway for collateral ventilation","volume":"21","author":"H B Martin","year":"1966","journal-title":"J Appl Physiol"},{"issue":"02","key":"ref63","doi-asserted-by":"crossref","first-page":"368","DOI":"10.1152\/physrev.1971.51.2.368","article-title":"Airway obstruction and collateral ventilation","volume":"51","author":"P T Macklem","year":"1971","journal-title":"Physiol Rev"},{"issue":"06","key":"ref64","first-page":"423","article-title":"Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis","volume":"65","author":"M Falk","year":"1984","journal-title":"Eur J Respir Dis"},{"issue":"03","key":"ref65","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1152\/jappl.1987.62.3.959","article-title":"Mucus clearance by two-phase gas-liquid flow mechanism: asymmetric periodic flow model","volume":"62","author":"C S Kim","year":"1987","journal-title":"J Appl Physiol"},{"issue":"06","key":"ref66","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1183\/09031936.06.00083605","article-title":"Physiological effects of vibration in subjects with cystic fibrosis","volume":"27","author":"B McCarren","year":"2006","journal-title":"Eur Respir J"},{"issue":"S 02","key":"ref67","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1016\/S1569-1993(14)60091-6","article-title":"Analysis of expiratory flow rates used in autogenic drainage. Are they sufficiently high to mobilize secretions?","volume":"13","author":"M McIllwaine","year":"2014","journal-title":"J Cyst Fibros"},{"issue":"04","key":"ref68","doi-asserted-by":"crossref","first-page":"398","DOI":"10.4187\/respcare.06329","article-title":"Oscillating positive expiratory pressure therapy may be performed poorly by children with cystic fibrosis","volume":"64","author":"K J O'Sullivan","year":"2019","journal-title":"Respir Care"},{"issue":"06","key":"ref69","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1136\/thx.49.6.557","article-title":"Regional lung clearance during cough and forced expiration technique (FET): effects of flow and viscoelasticity","volume":"49","author":"A Hasani","year":"1994","journal-title":"Thorax"},{"issue":"S 01","key":"ref70","first-page":"S50","article-title":"Airway clearance and exercise for people with cystic fibrosis: balancing longevity with life","volume":"57","author":"N J Rowbotham","year":"2022","journal-title":"Pediatr Pulmonol"},{"key":"ref72","first-page":"CD009595","article-title":"Autogenic drainage for airway clearance in cystic fibrosis","volume":"12","author":"P Burnham","year":"2021","journal-title":"Cochrane Database Syst Rev"},{"issue":"01","key":"ref73","first-page":"CD002011","article-title":"Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis","author":"E Main","year":"2005","journal-title":"Cochrane Database Syst Rev"},{"issue":"11","key":"ref74","first-page":"CD003147","article-title":"Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis","volume":"2019","author":"M McIlwaine","year":"2019","journal-title":"Cochrane Database Syst Rev"},{"issue":"07","key":"ref75","first-page":"CD007862","article-title":"Active cycle of breathing technique for cystic fibrosis","volume":"7","author":"N A Mckoy","year":"2016","journal-title":"Cochrane Database Syst Rev"},{"issue":"04","key":"ref76","first-page":"CD006842","article-title":"Oscillating devices for airway clearance in people with cystic fibrosis","volume":"4","author":"L Morrison","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"issue":"12","key":"ref77","first-page":"CD001401","article-title":"Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis","author":"L Warnock","year":"2015","journal-title":"Cochrane Database Syst Rev"},{"issue":"08","key":"ref78","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1136\/thoraxjnl-2012-202915","article-title":"Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis","volume":"68","author":"M P McIlwaine","year":"2013","journal-title":"Thorax"},{"issue":"01","key":"ref82","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.jphys.2018.11.006","article-title":"Exercise is commonly used as a substitute for traditional airway clearance techniques by adults with cystic fibrosis in Australia: a survey","volume":"65","author":"N Ward","year":"2019","journal-title":"J Physiother"},{"issue":"02","key":"ref83","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1080\/17476348.2017.1280399","article-title":"Adherence to therapies in cystic fibrosis: a targeted literature review","volume":"11","author":"S Narayanan","year":"2017","journal-title":"Expert Rev Respir Med"},{"issue":"05","key":"ref84","doi-asserted-by":"crossref","first-page":"762","DOI":"10.1016\/j.jcf.2019.11.007","article-title":"Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy","volume":"19","author":"A H Gifford","year":"2020","journal-title":"J Cyst Fibros"},{"issue":"05","key":"ref85","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/j.jcf.2008.01.003","article-title":"Treatment compliance in children and adults with cystic fibrosis","volume":"7","author":"R P Arias Llorente","year":"2008","journal-title":"J Cyst Fibros"},{"issue":"06","key":"ref86","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1177\/1359105306069093","article-title":"Adherence to chest physiotherapy in adults with cystic fibrosis","volume":"11","author":"L B Myers","year":"2006","journal-title":"J Health Psychol"},{"issue":"S 01","key":"ref87","doi-asserted-by":"crossref","first-page":"i50","DOI":"10.1136\/bmjqs-2013-002352","article-title":"The impact of re-education of airway clearance techniques (REACT) on adherence and pulmonary function in patients with cystic fibrosis","volume":"23","author":"R L Zanni","year":"2014","journal-title":"BMJ Qual Saf"},{"key":"ref88","first-page":"46","article-title":"Striving for perfection, accepting the reality: a reflection on adherence to airway clearance and inhalation therapy for paediatric patients with chronic suppurative lung disease","volume":"34","author":"E Dixon","year":"2020","journal-title":"Paediatr Respir Rev"},{"issue":"01","key":"ref89","doi-asserted-by":"crossref","first-page":"e000601","DOI":"10.1136\/bmjresp-2020-000601","article-title":"What effective ways of motivation, support and technologies help people with cystic fibrosis improve and sustain adherence to treatment?","volume":"7","author":"R J Calthorpe","year":"2020","journal-title":"BMJ Open Respir Res"},{"issue":"04","key":"ref90","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1007\/s10880-018-9598-z","article-title":"Identifying factors that facilitate treatment adherence in cystic fibrosis: qualitative analyses of interviews with parents and adolescents","volume":"26","author":"C J Nicolais","year":"2019","journal-title":"J Clin Psychol Med Settings"},{"key":"ref91","doi-asserted-by":"crossref","first-page":"1201","DOI":"10.1016\/S0140-6736(81)91440-9","article-title":"Effect of swimming on forced expiration and sputum clearance in cystic fibrosis","volume":"2","author":"M S Zach","year":"1981","journal-title":"Lancet"},{"issue":"08","key":"ref92","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1136\/adc.57.8.587","article-title":"Cystic fibrosis: physical exercise versus chest physiotherapy","volume":"57","author":"M Zach","year":"1982","journal-title":"Arch Dis Child"},{"issue":"12","key":"ref93","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1136\/thx.44.12.1006","article-title":"Effect of exercise and physiotherapy in aiding sputum expectoration in adults with cystic fibrosis","volume":"44","author":"W Salh","year":"1989","journal-title":"Thorax"},{"issue":"01","key":"ref94","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/s12890-016-0360-8","article-title":"Effects of treadmill exercise versus Flutter on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial","volume":"17","author":"T J Dwyer","year":"2017","journal-title":"BMC Pulm Med"},{"issue":"04","key":"ref95","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1378\/chest.10-1158","article-title":"Effects of exercise on respiratory flow and sputum properties in patients with cystic fibrosis","volume":"139","author":"T J Dwyer","year":"2011","journal-title":"Chest"},{"issue":"03","key":"ref96","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1164\/ajrccm.164.3.2007168","article-title":"Exercise inhibits epithelial sodium channels in patients with cystic fibrosis","volume":"164","author":"A Hebestreit","year":"2001","journal-title":"Am J Respir Crit Care Med"},{"issue":"02","key":"ref97","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1183\/09031936.00197309","article-title":"Exercise reduces airway sodium ion reabsorption in cystic fibrosis but not in exercise asthma","volume":"37","author":"L Schmitt","year":"2011","journal-title":"Eur Respir 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Ward","year":"2020","journal-title":"Thorax"},{"issue":"04","key":"ref101","doi-asserted-by":"crossref","first-page":"e19","DOI":"10.1016\/j.jcf.2019.10.026","article-title":"Can exercise replace airway clearance techniques in cystic fibrosis? 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Edgeworth","year":"2017","journal-title":"Clin Sci (Lond)"},{"issue":"01","key":"ref107","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1186\/s12931-020-01406-z","article-title":"Short-term effects of Lumacaftor\/Ivacaftor (Orkambi) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis","volume":"21","author":"B S Quon","year":"2020","journal-title":"Respir Res"},{"issue":"03","key":"ref108","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.jcf.2020.12.006","article-title":"VO2max as an exercise tolerance endpoint in people with cystic fibrosis: lessons from a lumacaftor\/ivacaftor trial","volume":"20","author":"J Wilson","year":"2021","journal-title":"J Cyst Fibros"},{"issue":"07","key":"ref109","doi-asserted-by":"crossref","first-page":"1364","DOI":"10.1016\/j.jand.2020.03.014","article-title":"Effect of CFTR modulators on anthropometric parameters in individuals with cystic fibrosis: an evidence analysis 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