Inhalation Therapy
Lesson 1: CFTR Modulator Therapy
Middleton, P. G., M. A. Mall, P. Drevinek, L. C. Lands, E. F. McKone, D. Polineni, B. W. Ramsey, J. L. Taylor-Cousar, E. Tullis, F. Vermeulen, G. Marigowda, C. M. McKee, S. M. Moskowitz, 182 N. Nair, J. Savage, C. Simard, S. Tian, D. Waltz, F. Xuan, S. M. Rowe and R. Jain (2019). “Elexacaftor-Tezacaftor Ivacaftor for cystic fibrosis with a single Phe508del allele.” New England Journal of Medicine 1(19): 1809-1819.
Mayer-Hamblett, N., F. Ratjen, R. Russell, S. H. Donaldson, K. A. Riekert, G. S. Sawicki, K. Odem-Davis, J. K. Young, D. Rosenbluth, J. L. Taylor-Cousar, C. H. Goss, G. Retsch Bogart, J. P. Clancy, A. Genatossio, B. P. O’Sullivan, A. Berlinski, S. L. Millard, G. Omlor, C. A. Wyatt, K. Moffett, D. P. Nichols and A. H. Gifford (2023). Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials. The Lancet Respiratory Medicine 11(4): 329-340.
Stanford G, Morrison L, Brown C. Nebuliser systems for drug delivery in cystic fibrosis. Cochrane Database of Systemic Reviews 2023, Issue 11. Art. No.: CD007639. DOI: 10.1002/14651858.CD007639.pub3.
Wark, P., V. M. McDonald and S. Smith (2023). “Nebulised hypertonic saline for cystic fibrosis.” Cochrane Database of Systematic Reviews (6): CD001506.
LESSON 2: Principles of inhalation therapy and inhalation technique
Agent P, Parrott H. Inhaled therapy in cystic fibrosis: agents, devices and regimens. Breathe (Sheff). 2015 Jun;11(2):110-8.
Boe J, Dennis JH, O’Driscoll BR, Bauer TT, Carone M, Dautzenberg B, Diot P, Heslop K, Lannefors L. European Respiratory Society Guidelines on the use of nebulizers. Eur Respir J. 2001; 18: 228-42.
Bronchiectasis toolbox: www.brochiectasis.com.auendorsed and supported by Lung Foundation Australia and the Thoracic Society of Australia and New Zealand.
Heijermann H, Westerman E, Conway S, et al. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2009; 8: 295–315
IPG-CF Blue Booklet on Physiotherapy Techniques https://www.ecfs.eu/ipg_cf/booklet
Le J, Ashley E D, Neuhauser M M, Brown J, Gentry C, Klepser M E,Wood G C. Consensus summary of aerosolized antimicrobial agents: application of guideline criteria insights from the society of infectious diseases pharmacists. Pharmacotherapy 2010; 30 (6): 562-584
Physiotherapy for Cystic Fibrosis in Australia: A Consensus Statement. http://www.thoracic.org.au/documents/papers/physiotherapyforcf. pdf. Accessed 20th March 2015
LESSON 3: Different modes of delivery
Agent P, Parrott H. 2015. Inhaled therapy in cystic fibrosis: agents, devices and regimens. Breathe (Sheff) June 11 (2): 110-8
Boe J, Dennis JH, O’Driscoll BR, et al. European Respiratory Society Guidelines on the use of nebulizers. Eur Respir J. 2001;18:228-42.
Button B et al. 2016. Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology. May ;21;(4):656-67. doi: 10.1111/resp.12764. Epub 2016 Apr 18.
Cipolla D, Gonda I, Shire SJ. Characterization of aerosols of human recombinant deoxyribonuclease I (rhDNase) generated by jet nebulizers. Pharmaceutical Research. 1994;11:491-8.
Daniel T, Mills N, Whitaker P. Nebuliser systems for drug delivery in cystic fibrosis. Cochrane Database of Systematic Reviews 2013
Lannefors L. Inhalation therapy: practical considerations for nebulisation therapy. Physical Therapy Reviews. 2006;11:21-7.
Raynor EM, Butler A, Guill M, et al. Nasally inhaled dornase alfa in the postoperative management of chronic sinusitis due to cystic fibrosis. Archives of otolaryngology–head & neck surgery. 2000;126:581-3.
Daniel T, Mills N, Whitaker P. Nebuliser systems for drug delivery in cystic fibrosis. Cochrane Database of Systematic Reviews 2013
LESSON 4: Mechanism of action
Daniels T, Mills N, Whitaker P. Nebuliser systems for drug delivery in cystic fibrosis. Cochrane Database of Systematic Reviews 2013, Issue 4. Art. No.: CD007639. DOI: 10.1002/14651858.CD007639.pub2.
Dentice R, Elkins M. Timing of dornase alfa inhalation for cystic fibrosis. Cochrane Database of Systematic Reviews 2018, Issue 11. Art. No.: CD007923. DOI: 10.1002/14651858.CD007923.pub5.
Dentice, R.L., Elkins, M.R., Middleton, P.G., Bishop, J.R., Wark, P.A., Dorahy, D.J., Harmer, C.J., Hu, H. and Bye, P.T (2016). A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis.Thorax, 71(2), pp.141-147.
Dwyer TJ, Elkins MR, Dentice R, Forbes S, Cooper P, Jaffe A, Bishop J, Middleton PG, Wark P, Bye PTP; SALTI-CF Study Group. Saline at lower tonicity in cystic fibrosis (SALTI-CF) trial comparing 0.9% versus 3% versus 6% nebulised saline. Eur Respir J. 2023 Jul 7;62(1):2100960. doi: 10.1183/13993003.00960-2021. PMID: 37343977.
Elkins M, Dentice R. Timing of hypertonic saline inhalation for cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 12. Art. No.: CD008816. DOI: 10.1002/14651858.CD008816.pub3.
Nevitt SJ, Thornton J, Murray CS, Dwyer T. Inhaled mannitol for cystic fibrosis. Cochrane Database of Systematic Reviews 2018, Issue 2. Art. No.: CD008649. DOI: 10.1002/14651858.CD008649.pub3.
O’Neill K, Moran F, Tunney MM, Elborn JS, Bradbury I, Downey DG, Rendall J, Bradley JM(2017) Timing of hypertonic saline and airway clearance techniques in adults with cystic fibrosis during pulmonary exacerbation: pilot data from a randomised crossover study [with consumer summary]BMJ Open Respiratory Research Jan 12;4(1):e000168
Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database of Systematic Reviews 2018, Issue 9. Art. No.: CD001506. DOI: 10.1002/14651858.CD001506.pub4.
Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database of Systematic Reviews 2018, Issue 9. Art. No.: CD001127. DOI: 10.1002/14651858.CD001127.pub4.
Lesson 5: Timing of Inhaled Therapy
Dentice, R. and M. Elkins (2021). “Timing of dornase alfa inhalation for cystic fibrosis.” Cochrane Database of Systematic Reviews (1): CD007923.
Lesson 6: Inhalation Therapy Technique
Dentice RL, Elkins MR, Dwyer GM, Bye PTP. Alternate side-lying during inhalation therapy does not prolong nebulisation time in adults with Cystic Fibrosis: randomised crossover trial. BMC Pulmonary Medicine. 2018;18(1):3.
Dentice RL, Elkins MR, Verschuer J, Eberl S, Dwyer G, Bye PTP. Side-lying during nebulisation significantly improves apical deposition in healthy adults and those with mild cystic fibrosis lung disease: randomised crossover trial. BMC Pulmonary Medicine. 2019;19(1):128.
Laube, BL, Geller, DE, Lin, TC, Dalby, RN, Diener-West, M, and Zeitlin, PL, Positive expiratory pressure changes aerosol distribution in patients with cystic fibrosis. Respiratory Care, 2005. 50(11): p. 1438-1444.
O’Connell, OJ, O’Farrell, C, Harrison, MJ, Eustace, JA, Henry, MT, and Plant, BJ, Nebulized hypertonic saline via positive expiratory pressure versus via jet nebulizer in patients with severe cystic fibrosis. Respiratory Care, 2011. 56(6): p. 771-775.
Standards of Care and Good Clinical Practice for the Physiotherapy Management of Cystic Fibrosis (2020) Fourth edition.
LESSON 7: Cleaning and Maintenance
Bell, J., L. Alexander, J. Carson, A. Crossan, J. McCaughan, H. Mills, D. O’Neill, J. E. Moore and B. C. Millar (2020). “Nebuliser hygiene in cystic fibrosis: evidence-based recommendations.” Breathe 16(2): 190328.
Brzezinski, Lorena Xavier Costa, Riedi, Carlos Antônio, Kussek, Paulo, Souza, Helena Homem de Melo de, & Rosário, Nelson. (2011). Nebulizers in cystic fibrosis: a source of bacterial contamination in cystic fibrosis patients?. Jornal Brasileiro de Pneumologia, 37(3), 341-347
Tabatabaii, S. A., G. Khanbabaee, S. Sadr, N. Farahbakhsh, M. K. Aghdam, S. Lotfollahzadeh, A. Hosseini, N. Dara, M. Nanbakhsh and F. A. Gorji (2020). “Microbial contamination of home nebulizers in children with cystic fibrosis and clinical implication on the number of pulmonary exacerbations.” BMC Pulmonary Medicine 20(1): 33.
Physiotherapy for Cystic Fibrosis in Australia: A Consensus Statement. http://www.thoracic.org.au/documents/papers/physiotherapyforcf. pdf. Accessed 20th March 2015
Reychler G, Aarab K, Van Ossel C, Gigi J, Simon A, Leal T, et al. In vitro evaluation of efficacy of 5 methods of disinfection on mouthpieces and facemasks contaminated by strains of cystic fibrosis patients. J Cyst Fibros. 2005;4(3):183-7.
http://www.pulmomed.com.au/pari-lc-sprint-family-nebulisers/
https://www.pari.com/fileadmin/user_upload/PARI.com_DE/Doc/de/Docs/178D1051-EN-eFlow-rapid-Flyer.pdf
https://www.trudellmed.com/sites/trudellmed.com/files/pdf/consumer/aexl_manual.pdf
