The effects of the addition of a new airway clearance device to chest physiotherapy in children with cystic fibrosis pulmonary exacerbations

1Castellani C, Duf AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, et al. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros. 2018 Mar;17(2):153-178. doi: 10.1016/j.jcf.2018.02.006.Castellani C Duf AJA Bell SC Heijerman HGM Munck A Ratjen F et al ECFS best practice guidelines: the 2018 revision J Cyst Fibros 2018 Mar172153 178 10.1016/j.jcf.2018.02.00629506920Open DOISearch in Google Scholar

2Sanders DB, Bittner RC, Rosenfeld M, Hofman LR, Redding GJ, Goss CH. Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation. Am J Respir Crit Care Med. 2010;182(5):627-632. doi: 10.1164/rccm.200909-1421OC.Sanders DB Bittner RC Rosenfeld M Hofman LR Redding GJ Goss CH Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation Am J Respir Crit Care Med 20101825627 632 10.1164/rccm.200909-1421OC545076320463179Open DOISearch in Google Scholar

3Sanders DB, Bittner RC, Rosenfeld M, Redding GJ, Goss CH. Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis. Pediatr Pulmonol. 2011;46(4):393-400. doi: 10.1002/ppul.21374.Sanders DB Bittner RC Rosenfeld M Redding GJ Goss CH Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis Pediatr Pulmonol 2011464393 400 10.1002/ppul.2137420967845Open DOISearch in Google Scholar

4Collaco JM, Green DM, Cutting GR, Naughton KM, Mogayzel PJ Jr. Location and duration of treatment of cystic fibrosis respiratory exacerbations do not afect outcomes. Am J Respir Crit Care Med. 2010;182(9):1137-1143. doi: 10.1164/rccm.201001-0057OC.Collaco JM Green DM Cutting GR Naughton KM Mogayzel PJ Jr Location and duration of treatment of cystic fibrosis respiratory exacerbations do not afect outcomes Am J Respir Crit Care Med 201018291137 1143 10.1164/rccm.201001-0057OC300125620581166Open DOISearch in Google Scholar

5VanDevanter DR, Pasta DJ, Konstan MW. Treatment and demographic factors affecting time to next pulmonary exacerbation in cystic fibrosis. J Cyst Fibros. 2015;14(6):763-769. doi: 10.1016/j. jcf.2015.02.007.VanDevanter DR Pasta DJ Konstan MW Treatment and demographic factors affecting time to next pulmonary exacerbation in cystic fibrosis J Cyst Fibros 2015146763 769 10.1016/j.jcf.2015.02.007456103325754096Open DOISearch in Google Scholar

6Stephenson AL, Tom M, Berthiaume Y, Singer LG, Aaron SD, Whitmore GA, Stanojevic S. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Respir J. 2015;45(3):670-679. doi: 10.1183/09031936.00119714.Stephenson AL Tom M Berthiaume Y Singer LG Aaron SD Whitmore GA Stanojevic S A contemporary survival analysis of individuals with cystic fibrosis: a cohort study Eur Respir J 2015453670 679 10.1183/09031936.0011971425395034Open DOISearch in Google Scholar

7Stenbit AE, Flume PA. Pulmonary exacerbations in cystic fibrosis. Curr Opin Pulm Med. 2011;17(6):442-447. doi: 10.1097/ MCP.0b013e32834b8c04.Stenbit AE Flume PA Pulmonary exacerbations in cystic fibrosis Curr Opin Pulm Med 2011176442 447 10.1097/MCP.0b013e32834b8c0421881509Open DOISearch in Google Scholar

8Flume PA, Mogayzel PJ Jr, Robinson KA, Goss CH, Rosenblatt RL, Kuhn RJ, Marshall BC; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009;180(9):802-808. doi: 10.1164/rccm.200812-1845PP.Flume PA Mogayzel PJ Jr Robinson KA Goss CH Rosenblatt RL Kuhn RJ Marshall BC. Clinical Practice Guidelines for Pulmonary Therapies Committee Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med 20091809802 808 10.1164/rccm.200812-1845PP19729669Open DOISearch in Google Scholar

9Flume PA, Robinson KA, O’Sullivan BP, Finder JD, Vender RL, Willey-Courand DB, White TB, Marshall BC; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009;54(4):522-537.Flume PA Robinson KA O’Sullivan BP Finder JD Vender RL Willey-Courand DB White TB Marshall BC. Clinical Practice Guidelines for Pulmonary Therapies Committee Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care 2009544522 537Search in Google Scholar

10De Boeck K, Wilschanski M, Castellani C, Taylor C, Cuppens H, Dodge J, Sinaasappel M; Diagnostic Working Group. Cystic fibrosis: terminology and diagnostic algorithms. Thorax. 2006;61(7):627-635. doi: 10.1136/thx.2005.043539.De Boeck K Wilschanski M Castellani C Taylor C Cuppens H Dodge J Sinaasappel M Diagnostic Working Group Cystic fibrosis: terminology and diagnostic algorithms. Thorax 2006617627 635 10.1136/thx.2005.043539210467616384879Open DOISearch in Google Scholar

11Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, et al.; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008;153(2):S4-S14. doi: 10.1016/j.jpeds.2008.05.005.Farrell PM Rosenstein BJ White TB Accurso FJ Castellani C Cutting GR et al Cystic Fibrosis Foundation Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr 20081532S4 S14 10.1016/j.jpeds.2008.05.005281095818639722Open DOISearch in Google Scholar

12Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, Rosenstein BJ, Smith AL, Wohl ME. Efect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med. 1994;331(10):637-642. doi: 10.1056/NEJM199409083311003.Fuchs HJ Borowitz DS Christiansen DH Morris EM Nash ML Ramsey BW Rosenstein BJ Smith AL Wohl ME Efect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis The Pulmozyme Study Group. N Engl J Med 199433110637 642 10.1056/NEJM1994090833110037503821Open DOISearch in Google Scholar

13Horsley A. Lung clearance index in the assessment of airways disease. Respir Med. 2009;103(6):793-799. doi: 10.1016/j. rmed.2009.01.025.Horsley A Lung clearance index in the assessment of airways disease Respir Med 20091036793 799 10.1016/j.rmed.2009.01.02519246184Open DOISearch in Google Scholar

14Beydon N, Davis SD, Lombardi E, Allen JL, Arets HG, Aurora P, et al.; American Thoracic Society/European Respiratory Society Working Group on Infant and Young Children Pulmonary Function Testing. An oficial American Thoracic Society/European Respiratory Society statement: pulmonary function testing in preschool children. Am J Respir Crit Care Med. 2007;175(12):13041345. doi: 10.1164/rccm.200605-642ST.Beydon N Davis SD Lombardi E Allen JL Arets HG Aurora P et al American Thoracic Society/European Respiratory Society Working Group on Infant and Young Children Pulmonary Function Testing An oficial American Thoracic Society/European Respiratory Society statement: pulmonary function testing in preschool children. Am J Respir Crit Care Med 20071751213041345 10.1164/rccm.200605-642ST17545458Open DOISearch in Google Scholar

15Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, et al.; ATS/ERS Task Force. Standardisation of spirometry. Eur Respir J. 2005;26(2):319-338. doi: 10.1183/09031936.05.00034805.Miller MR Hankinson J Brusasco V Burgos F Casaburi R Coates A et al ATS/ERS Task Force Standardisation of spirometry. Eur Respir J 2005262319 338 10.1183/09031936.05.0003480516055882Open DOISearch in Google Scholar

16Robinson PD, Latzin P, Verbanck S, Hall GL, Horsley A, Gappa M, et al. Consensus statement for inert gas washout measurement using multiple- and single-breath tests. Eur Respir J. 2013;41(3):507-522. doi: 10.1183/09031936.00069712.Robinson PD Latzin P Verbanck S Hall GL Horsley A Gappa M et al Consensus statement for inert gas washout measurement using multiple- and single-breath tests Eur Respir J 2013413507 522 10.1183/09031936.0006971223397305Open DOISearch in Google Scholar

17Horsley A, Siddiqui S. Putting lung function and physiology into perspective: cystic fibrosis in adults. Respirology. 2015;20(1):3345. doi: 10.1111/resp.12382.Horsley A Siddiqui S Putting lung function and physiology into perspective: cystic fibrosis in adults Respirology 20152013345 10.1111/resp.1238225219816Open DOISearch in Google Scholar

18Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63(2):129-134. doi: 10.1136/thx.2007.077784.Gustafsson PM De Jong PA Tiddens HA Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis Thorax 2008632129 134 10.1136/thx.2007.07778417675316Open DOISearch in Google Scholar

19Graeber SY, Dopfer C, Naehrlich L, Gyulumyan L, Scheuermann H, Hirtz S, et al. Efects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis. Am J Respir Crit Care Med. 2018;197(11):1433-1442. doi: 10.1164/ rccm.201710-1983OC.Graeber SY Dopfer C Naehrlich L Gyulumyan L Scheuermann H Hirtz S et al Efects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis Am J Respir Crit Care Med 2018197111433 1442 10.1164/rccm.201710-1983OC29327948Open DOISearch in Google Scholar

20Eckrich J, Zissler UM, Serve F, Leutz P, Smaczny C, Schmitt-Grohé S et al. Airway inflammation in mild cystic fibrosis. J Cyst Fibros. 2017;16(1):107-115. doi: 10.1016/j.jcf.2016.05.016.Eckrich J Zissler UM Serve F Leutz P Smaczny C Schmitt-Grohé S et al Airway inflammation in mild cystic fibrosis J Cyst Fibros 2017161107 115 10.1016/j.jcf.2016.05.01627296563Open DOISearch in Google Scholar

21Loeve M, Rosenow T, Gorbunova V, Hop WC, Tiddens HA, de Bruijne M. Reversibility of trapped air on chest computed tomography in cystic fibrosis patients. Eur J Radiol. 2015;84(6):11841190. doi: 10.1016/j.ejrad.2015.02.011.Loeve M Rosenow T Gorbunova V Hop WC Tiddens HA de Bruijne M. Reversibility of trapped air on chest computed tomography in cystic fibrosis patients Eur J Radiol 201584611841190 10.1016/j.ejrad.2015.02.01125840703Open DOISearch in Google Scholar

22van der Giessen LJ, de Jongste JC, Gosselink R, Hop WC, Tiddens HA. RhDNase before airway clearance therapy improves airway patency in children with CF. Pediatr Pulmonol. 2007;42(7):624-630. doi: 10.1002/ppul.20636.van der Giessen LJ de Jongste JC Gosselink R Hop WC Tiddens HA RhDNase before airway clearance therapy improves airway patency in children with CF Pediatr Pulmonol 2007427624 630 10.1002/ppul.2063617534979Open DOISearch in Google Scholar

23Rosenfeld M, VanDevanter DR, Ren CL, Elkin EP, Pasta DJ, Konstan MW, Morgan WJ; Investigators of Coordinators of the Epidemiologic Study of Cystic Fibrosis. Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. Pediatr Pulmonol. 2015;50(9):856-862. doi: 10.1002/ ppul.23227.Rosenfeld M VanDevanter DR Ren CL Elkin EP Pasta DJ Konstan MW Morgan WJ. Investigators of Coordinators of the Epidemiologic Study of Cystic Fibrosis Decline in lung function does not predict future decline in lung function in cystic fibrosis patients. Pediatr Pulmonol 2015509856 862 10.1002/ppul.2322726086901Open DOISearch in Google Scholar

24Bakker EM, Borsboom GJ, van der Wiel-Kooij EC, Caudri D, Rosenfeld M, Tiddens HA. Small airway involvement in cystic fibrosis lung disease: routine spirometry as an early and sensitive marker. Pediatr Pulmonol. 2013;48(11):1081-1088. doi: 10.1002/ppul.22777.Bakker EM Borsboom GJ van der Wiel-Kooij EC Caudri D Rosenfeld M Tiddens HA. Small airway involvement in cystic fibrosis lung disease: routine spirometry as an early and sensitive marker Pediatr Pulmonol 201348111081 1088 10.1002/ppul.2277723401260Open DOISearch in Google Scholar

25Verbanck S, Paiva M, Schuermans D, Hanon S, Vincken W, Van Muylem A. Relationships between the lung clearance index and conductive and acinar ventilation heterogeneity. J Appl Physiol (1985). 2012;112(5):782-790. doi: 10.1152/japplphysiol.01221.2011.Verbanck S Paiva M Schuermans D Hanon S Vincken W Van Muylem A Relationships between the lung clearance index and conductive and acinar ventilation heterogeneity J Appl Physiol (1985) 20121125782 790 10.1152/japplphysiol.01221.201122162528Open DOISearch in Google Scholar

26Vermeulen F, Proesmans M, Boon M, Havermans T, De Boeck K. Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis. Thorax. 2014;69(1):39-45. doi: 10.1136/thoraxjnl-2013-203807.Vermeulen F Proesmans M Boon M Havermans T De Boeck K. Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis Thorax 201469139 45 10.1136/thoraxjnl-2013-20380724021874Open DOISearch in Google Scholar

27Rayment JH, Stanojevic S, Davis SD, Retsch-Bogart G, Ratjen F. Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis. Thorax. 2018;73(5):451-458. doi: 10.1136/thoraxjnl-2017-210979.Rayment JH Stanojevic S Davis SD Retsch-Bogart G Ratjen F Lung clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis Thorax 2018735451 458 10.1136/thoraxjnl-2017-21097929449440Open DOISearch in Google Scholar

28Stanojevic S, Davis SD, Retsch-Bogart G, Webster H, Davis M, Johnson RC, et al. Progression of Lung Disease in Preschool Patients with Cystic Fibrosis. Am J Respir Crit Care Med. 2017;195(9):1216-1225. doi: 10.1164/rccm.201610-2158OC.Stanojevic S Davis SD Retsch-Bogart G Webster H Davis M Johnson RC et al Progression of Lung Disease in Preschool Patients with Cystic Fibrosis Am J Respir Crit Care Med 201719591216 1225 10.1164/rccm.201610-2158OC543901827943680Open DOISearch in Google Scholar

29Welsh L, Nesci C, Tran H, Tomai M, Ranganathan S. Lung clearance index during hospital admission in school-age children with cystic fibrosis. J Cyst Fibros. 2014;13(6):687-691. doi: 10.1016/j.jcf.2014.05.012.Welsh L Nesci C Tran H Tomai M Ranganathan S Lung clearance index during hospital admission in school-age children with cystic fibrosis J Cyst Fibros 2014136687 691 10.1016/j.jcf.2014.05.01224917113Open DOISearch in Google Scholar

30Horsley AR, Davies JC, Gray RD, Macleod KA, Donovan J, Aziz ZA, et al. Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax. 2013;68(6):532-539. doi: 10.1136/ thoraxjnl-2012-202538.Horsley AR Davies JC Gray RD Macleod KA Donovan J Aziz ZA et al Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation Thorax 2013686532 539 10.1136/thoraxjnl-2012-20253823396354Open DOISearch in Google Scholar

31Robinson PD, Cooper P, Van Asperen P, Fitzgerald D, Selvadurai H. Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis. Pediatr Pulmonol. 2009;44(8):733-742. doi: 10.1002/ppul.20956.Robinson PD Cooper P Van Asperen P Fitzgerald D Selvadurai H. Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis Pediatr Pulmonol 2009448733 742 10.1002/ppul.2095619598271Open DOISearch in Google Scholar

32Sonneveld N, Stanojevic S, Amin R, Aurora P, Davies J, Elborn JS, Horsley A, Latzin P, O’Neill K, Robinson P, Scrase E, Selvadurai H, Subbarao P, Welsh L, Yammine S, Ratjen F. Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations. Eur Respir J. 2015;46(4):1055-1064. doi: 10.1183/09031936.00211914.Sonneveld N Stanojevic S Amin R Aurora P Davies J Elborn JS Horsley A Latzin P O’Neill K Robinson P Scrase E Selvadurai H Subbarao P Welsh L Yammine S Ratjen F Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations Eur Respir J 20154641055 1064 10.1183/09031936.0021191426160868Open DOISearch in Google Scholar

33Yammine S, Bigler A, Casaulta C, Singer F, Latzin P. Reasons for heterogeneous change in LCI in children with cystic fibrosis after antibiotic treatment. Thorax. 2014;69(2):183. doi: 10.1136/ thoraxjnl-2013-204283.Yammine S Bigler A Casaulta C Singer F Latzin P Reasons for heterogeneous change in LCI in children with cystic fibrosis after antibiotic treatment Thorax 2014692183 10.1136/thoraxjnl-2013-20428323988751Open DOISearch in Google Scholar

34Rowan SA, Bradley JM, Bradbury I, Lawson J, Lynch T, Gustafsson P, et al. Lung clearance index is a repeatable and sensitive indicator of radiological changes in bronchiectasis. Am J Respir Crit Care Med. 2014;189(5):586-592. doi: 10.1164/ rccm.201310-1747OC.Rowan SA Bradley JM Bradbury I Lawson J Lynch T Gustafsson P et al Lung clearance index is a repeatable and sensitive indicator of radiological changes in bronchiectasis Am J Respir Crit Care Med 20141895586 592 10.1164/rccm.201310-1747OC24428575Open DOISearch in Google Scholar

35Caskey S, Gough A, Rowan S, Gillespie S, Clarke J, Riley M, et al. Structural and Functional Lung Impairment in Adult Survivors of Bronchopulmonary Dysplasia. Ann Am Thorac Soc. 2016;13(8):1262-1270. doi: 10.1513/AnnalsATS.201509-578OC.Caskey S Gough A Rowan S Gillespie S Clarke J Riley M et al Structural and Functional Lung Impairment in Adult Survivors of Bronchopulmonary Dysplasia Ann Am Thorac Soc 20161381262 1270 10.1513/AnnalsATS.201509-578OC27222921Open DOISearch in Google Scholar

36Gustafsson PM. Peripheral airway involvement in CF and asthma compared by inert gas washout. Pediatr Pulmonol. 2007;42(2):168-176. doi: 10.1002/ppul.20554.Gustafsson PM Peripheral airway involvement in CF and asthma compared by inert gas washout Pediatr Pulmonol 2007422168 176 10.1002/ppul.2055417186546Open DOISearch in Google Scholar

37Sonnappa S, Bastardo CM, Wade A, Bush A, Stocks J, Aurora P. Repeatability and bronchodilator reversibility of lung function in young children. Eur Respir J. 2013;42(1):116-124. doi: 10.1183/09031936.00076012.Sonnappa S Bastardo CM Wade A Bush A Stocks J Aurora P Repeatability and bronchodilator reversibility of lung function in young children Eur Respir J 2013421116 124 10.1183/09031936.0007601223222876Open DOISearch in Google Scholar

38Fuchs SI, Toussaint S, Edlhaimb B, Ballmann M, Gappa M. Short-term effect of physiotherapy on variability of the lung clearance index in children with cystic fibrosis. Pediatr Pulmonol. 2010;45(3):301-306. doi: 10.1002/ppul.21180.Fuchs SI Toussaint S Edlhaimb B Ballmann M Gappa M Short-term effect of physiotherapy on variability of the lung clearance index in children with cystic fibrosis Pediatr Pulmonol 2010453301 306 10.1002/ppul.2118020146388Open DOISearch in Google Scholar

39Pfleger A, Steinbacher M, Schwantzer G, Weinhandl E, Wagner M, Eber E. Short-term effects of physiotherapy on ventilation inhomogeneity in cystic fibrosis patients with a wide range of lung disease severity. J Cyst Fibros. 2015;14(5):627-631. doi: 10.1016/j.jcf.2014.12.017.Pfleger A Steinbacher M Schwantzer G Weinhandl E Wagner M Eber E Short-term effects of physiotherapy on ventilation inhomogeneity in cystic fibrosis patients with a wide range of lung disease severity J Cyst Fibros 2015145627 631 10.1016/j.jcf.2014.12.01725612899Open DOISearch in Google Scholar

40Marques A, Cruz J, Jácome C, Oliveira A. Outcome measures for respiratory physiotherapy in cystic fibrosis - challenges and advances. online book chapter]. s. 37-71. doi: 10.5772/60674. IN: Cystic fibrosis in the light of new research. Ed. by Wat D. London, UK : IntechOpen Limited, 2015. doi: 10.5772/59523. ISBN 978-953-51-2152-7, eBook ISBN 978-953-51-7248-2. Available from: https://www.intechopen.com/books/cystic-fibrosis-in-the-light-of-new-research/outcome-measures-for-respiratory-physiotherapy-in-cystic-fibrosis-challenges-and-advances (accessed 5 Jun 2019).Marques A Cruz J Jácome C Oliveira A Outcome measures for respiratory physiotherapy in cystic fibrosis - challenges and advances online book chapter] s 37–71 10.5772/60674 IN: Cystic fibrosis in the light of new research. Ed. by Wat D. London, UK : IntechOpen Limited, 2015. 10.5772/59523. ISBN 978-953-51-2152-7, eBook ISBN 978-953-51-7248-2. Available from https://www.intechopen.com/books/cystic-fibrosis-in-the-light-of-new-research/outcome-measures-for-respiratory-physiotherapy-in-cystic-fibrosis-challenges-and-advances (accessed 5 Jun 2019)Open DOISearch in Google Scholar