Main Article Content
cystic fibrosis, lung clearance index, nocturnal cardio-respiratory polygraphy, non-invasive ventilation, normocapnic; quality of life.
Background and aim: In patients with cystic fibrosis (CF) non-invasive ventilation (NIV) improves lung mechanics and gas exchange, and decreases the work of breathing. Domiciliary NIV is mainly used in hypercapnic patients with severe disease, because it counteracts the progression of lung functional impairment and it is often used as a useful “bridge” to lung transplantation. However, to date, there are no standardized criteria to indicate the effect of a precocious starting of NIV in patients with functional ventilation inhomogeneity without hypercapnia. In this pilot study we assessed whether an early NIV treatment might influence functional and clinical outcomes in CF patients.
Methods: Six normocapnic CF patients were treated for one year with NIV. At baseline and after 1 year of NIV treatment, arterial gas analysis, spirometry, MBW to derive LCI, nocturnal cardio-respiratory polygraphy (PG), and Pittsburgh Sleep Quality Index (PSQI) were perfomed in all enrolled patients.
Results: After one year, despite spirometric and LCI values remain statistically not modified, the number of infectious exacerbations was reduced by 50%.
Conclusions: These results suggest that nocturnal NIV improves clinical conditions of stable CF patients. Finally, we suggest that this procedure can be useful to counteract the progression of lung disease even in normocapnic patients
2. Fauroux B, Le Roux E, Ravilly S, Bellis G, Clément A. Long-term noninvasive ventilation in patients with cystic fibrosis. Respiration 2008;76(2):168-74.
3. Archangelidi O, Carr SB, Simmonds NJ, Bilton D, Banya W, Cullinan P; CF-EpiNet. Non-invasive ventilation and clinical outcomes in cystic fibrosis: Findings from the UK CF registry. J Cyst Fibros 2019;18(5):665-670
4. Poncin W, Lebecue P. Lung Clearance index in Cystic Fibrosis. Rev Mal Respir 2019; S0761-8425(18)31015-5
5. Horsley A, Wild JM. Ventilation heterogeneity and the benefits and challenges of multiple breath washout testing in patients with cystic fibrosis. Paediatric Respiratory Reviews 2015;16 Suppl 1:15-18.
6. Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, Howenstine M, McColley SA, Rock M, Rosenfeld M, Sermet-Gaudelus I, Southern KW, Marshall BC, Sosnay PR. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr 2017;181S:S4-S15.e1
7. Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, Rosenstein BJ, Smith AL, Wohl ME; The Pulmozyme Study Group. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med. 1994;331:637–642
8. Robinson PD, Latzin P, Verbanck S, Hall GL, Horsley A, Gappa M, Thamrin C, Arets HG, Aurora P, Fuchs SI, King GG, Lum S, Macleod K, Paiva M, Pillow JJ, Ranganathan S, Ratjen F, Singer F, Sonnappa S, Stocks J, Subbarao P, Thompson BR, Gustafsson PM. Consensus statement for inert gas washout measurement using multiple- and single- breath tests. Eur Respir J 2013;41(3):507-22
9. Kapur VK, Auckley DH, Chowdhuri S, Kuhlmann DC, Mehra R, Ramar K, Harrod CG. Clinical Practice Guideline for Diagnostic Testing for Adult Obstructive Sleep Apnea: An American Academy of Sleep Medicine Clinical Practice Guideline. J Clin Sleep Med 2017;(3):479-504
10. Buysse DJ, Reynolds CF 3rd, Monk TH, Berman SR, Kupfer DJ. The Pittsburgh Sleep Quality Index: a new instrument for psychiatric practice and research. Psychiatry Res 1989;28(2):193-213.
11. Graham BL, Steenbruggen I, Miller MR, Barjaktarevic IZ, Cooper BG, Hall GL, Hallstrand TS, Kaminsky DA, McCarthy K, McCormack MC, Oropez CE, Rosenfeld M, Stanojevic S, Swanney MP, Thompson BR. Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement. Am J Respir Crit Care Med 2019;200(8):e70-e88
12. Foong RE, Harper AJ, Skoric B. The clinical utility of lung clearance index in early cystic fibrosis lung disease is not impacted by the number of multiple-breath washout trials. ERJ Open Res 2018;4(1):00094-2017
13. Pleil JD. QQ-plots for assessing distributions of biomarker measurements and generating defensible summary statistics. J Breath Res 2016;10(3):035001
14. Rayment JH, Stanojevic S, Davis SD, Retsch-Bogart G, Ratjen F. clearance index to monitor treatment response in pulmonary exacerbations in preschool children with cystic fibrosis. Thorax 2018;73(5):451-458.
15. Milross MA, Piper AJ, Norman M, Becker HF, Willson GN, Grunstein RR, Sullivan CE, Bye PT. Low-flow oxygen and bilevelventilatorysupport: effects on ventilationduringsleep in cysticfibrosis.Am J Respir Crit Care Med 2001;163(1):129-34
16. Kraemer R, Latzin P, Pramana I, Ballinari P, Gallati S, Frey U. Long-term gas exchange characteristics as markers of deterioration in patients with cystic fibrosis. Respir Res 2009;10(1):106
17. Gustafsson 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
18. Owens CM, Aurora P, Stanojevic S, et al. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax 2011;66(6):481–488
19. Hajian B, De Backer J, Sneyers C, Ferreira F, Barboza KC, Leemans G, Vos W, De Backer W. Pathophysiological mechanism of long-term noninvasive ventilation in stable hypercapnic patients with COPD using functional respiratory imaging. Int J Chron Obstruct Pulmon Dis 2017;12:2197-2205.
20. Loeve M, Gerbrands K, Hop WC, Rosenfeld M, Hartmann IC, Tiddens HA. Bronchiectasis and pulmonary exacerbations in children and young adults with cystic fibrosis. Chest 2011;140(1):178-185.
21. Leonardi S, Cuppari C, Lanzafame A, Attardo D, Tardino L, Parisi G, Giacchi V, Manti S, Arrigo T. Exhaled breath temperature in asthmatic children. J Biol Regul Homeost Agents 2015;29(2 Suppl 1):47-54
22. Manti S, Leonardi S, Parisi GF, De Vivo D, Salpietro A, Spinuzza A, Arrigo T, Salpietro C, Cuppari C. High mobility group box 1: Biomarker of inhaled corticosteroid treatment response in children with moderate-severe asthma. Allergy Asthma Proc. 2017;38(3):197-203
23. Leonardi S, Parisi GF, Capizzi A, Manti S, Cuppari C, Scuderi MG, Rotolo N, Lanzafame A, Musumeci M, Salpietro C. YKL-40 as marker of severe lung disease in cystic fibrosis patients. J Cyst Fibros. 2016;15(5):583-6
24. Spicuzza L, Parisi GF, Tardino L, Ciancio N, Nenna R, Midulla F, Leonardi S. Exhaled markers of antioxidant activity and oxidative stress in stable cystic fibrosis patients with moderate lung disease. J Breath Res. 2018 Feb 20;12(2):026010
25. Madden BP, Kariyawasam H, Siddiqi AJ, Machin A, Pryor JA, Hodson ME. Noninvasive ventilation in cystic fibrosis patients with acute or chronic respiratory failure [published correction appears in Eur Respir J. 2002 Sep;20(3):790.]. Eur Respir J 2002;19(2):310–313
26. Rodriguez Hortal MC, Hedborg A, Biguet G, Nygren-Bonnier M. Experience of using non-invasive ventilation as an adjunct to airway clearance techniques in adults with cystic fibrosis-A qualitative study. Physiother Theory Pract 2018;34(4):264-275
27. Papale M, Parisi GF, Spicuzza L, Licari A, Bongiovanni A, Mulè E, Rotolo N, Manti S, Leonardi S. Lung clearance index evaluation in detecting nocturnal hypoxemia in cystic fibrosis patients: Toward a new diagnostic tool. Respir Med 2020;164:105906
28. Abbott J, Morton AM, Hurley MA, Conway SP. Longitudinal impact of demographic and clinical variables on health-related quality of life in cystic fibrosis. BMJ Open 2015;5(5):e007418.
29. Clini E, Sturani C, Porta R, et al. Outcome of COPD patients performing nocturnal non-invasive mechanical ventilation. Respir Med 1998;92(10):1215–1222
30. Flight WG, Shaw J, Johnson S, et al. Long-term non-invasive ventilation in cystic fibrosis - experience over two decades. J Cyst Fibros 2012;11(3):187–192