Skip to main content
 

Main menu

  • Home
  • Content
    • Current Issue
    • Editor's Commentary
    • Coming Next Month
    • Archives
    • Top 10 Papers in 2020
  • Authors
    • Author Guidelines
    • Submit a Manuscript
  • Reviewers
    • Reviewer Information
    • Create Reviewer Account
    • Appreciation of Reviewers
  • CRCE
    • Through the Journal
    • JournalCasts
    • AARC University
    • PowerPoint Template
  • Open Forum
    • Call for Abstracts 2021
    • 2020 Abstracts
    • Previous Open Forums
  • Podcast
    • English
    • Español
    • Portugûes
    • 国语

User menu

  • Subscribe
  • My alerts
  • Log in

Search

  • Advanced search
American Association for Respiratory Care
  • Subscribe
  • My alerts
  • Log in
American Association for Respiratory Care

Advanced Search

  • Home
  • Content
    • Current Issue
    • Editor's Commentary
    • Coming Next Month
    • Archives
    • Top 10 Papers in 2020
  • Authors
    • Author Guidelines
    • Submit a Manuscript
  • Reviewers
    • Reviewer Information
    • Create Reviewer Account
    • Appreciation of Reviewers
  • CRCE
    • Through the Journal
    • JournalCasts
    • AARC University
    • PowerPoint Template
  • Open Forum
    • Call for Abstracts 2021
    • 2020 Abstracts
    • Previous Open Forums
  • Podcast
    • English
    • Español
    • Portugûes
    • 国语
  • Follow aarc on Twitter
  • Visit aarc on Facebook
LetterCorrespondence

CPAP in Cystic Fibrosis: Is It Time to Surrender Yet?

Simone Gambazza and Sergio Zuffo
Respiratory Care September 2013, 58 (9) e116-e117; DOI: https://doi.org/10.4187/respcare.02655
Simone Gambazza
Cystic Fibrosis Centre Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Cà Granda Ospedale Maggiore Policlinico Milan, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sergio Zuffo
Rehabilitation Unit Azienda Ospedaliero Universitaria Meyer Florence, Italy
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • References
  • Info & Metrics
  • PDF
Loading

To the editor:

Measuring the efficacy of one airway clearance technique over another is still challenging and sometimes frustrating in an evidence-based era. We want our patients clear and intuitively we assume that what they expectorate is what we have really cleared. As Sirens caught Ulysses along his journey, respiratory therapists and physiotherapists are often tempted to believe that sputum is the best available end point of any treatment. Despite this, patients use sputum output to measure the relative effectiveness of different airway clearance regimens1 and patients' preferences continue to be a factor when introducing an airway clearance technique.2

The paper by Aquino and colleagues in the November issue of Respiratory Care3 evaluated if CPAP increases the effects of hypertonic saline on mucus clearance, sputum physical properties and expectoration in patients with cystic fibrosis (CF). Their crossover study randomized 15 patients to CPAP alone or CPAP followed by hypertonic saline aerosol. Direct cough was standardized as a series of 3 coughs on command and used as control intervention. Relying on outcome measures such as sputum volume, clearability, and wettability, Aquino et al concluded that CPAP alone did not improve sputum clearance or augment the effect of hypertonic saline.

As respiratory therapists and physiotherapists involved in the care of patients with CF, we want to highlight 2 crucial points and report our experience, for a better interpretation of the results.

Hypertonic Saline

There is an extensive literature on the efficacy of inhaled hypertonic saline in CF. We already know that inhaled hypertonic saline improves mucociliary clearance, lung function, and quality of life in adults with CF.4 Indeed, airway clearance techniques and hypertonic saline are often used in treatment sessions to enhance the rheology of mucus and thus promote mucus clearance by accelerating expiratory flow. To date the effect of the timing of inhaled hypertonic saline (before, during, and after airway clearance techniques) is still debated, even though the 3 studies that demonstrated the clinical efficacy of hypertonic saline have delivered it before airway clearance techniques.4–7 Therefore, this should be the only regimen recommendable.8

CPAP

CPAP is used under several conditions, and its application in CF seems to decrease dyspnea and improve inspiratory muscle function,9 increasing tidal volume and reducing breathing frequency. The rationale for CPAP as an airway clearance technique10 might rely on its splinting effects on airways, especially during expiration. The increased airway pressure during expiration is thought to prevent premature collapse and thus reduce gas trapping in the lungs.11 Theoretically, this might help mucus transport, and not sputum, making its dislocation dependent on the movement of air along the patented airways. Several studies have indicated this mechanical advantage during positive expiratory pressure (PEP) breathing, and this positive effect seems to be sustained even 45 min following treatment.10,12 However, short-term PEP breathing improves only central expiratory air flow, as measured by FEV113: indeed, mucus transport due to expiratory air flow is more efficient in the central than in the peripheral airways, which can be completely obstructed by mucus in CF.14

We know that CPAP reopens collapsed parts of the lungs as well, and obstructed areas are thought to reopen as a result of increased collateral ventilation.15 As suggested by Falk et al,16 this might occur in patients with CF, mucus possibly being mobilized from peripheral small airways. Thus, CPAP could avoid early airways collapse, respiratory muscle fatigue, and oxygen desaturation,17 especially in advanced lung disease. Nevertheless, in our opinion, pressure alone cannot be considered a proper airway clearance technique, as presented by Aquino et al, without patient participation (eg, huff or forced expiration technique). As far as we achieve patent airways, patients need to sustain expiratory flow actively to mobilize secretions. This might happen if positive pressure is applied during the whole respiratory cycle, together with active techniques,18 and not only during the inspiratory phase alone, as previously described.10,17

However, due to the lack of data about its use in CF as an airway clearance technique, and considering the reported end points, we can assume that CPAP alone has the same effects as PEP on airway clearance during expiration in this sample. Outcomes such as those chosen by Aquino et al may not be suitable, since positive short-term effects of an airway clearance technique such CPAP may not be reflected by sputum weight19,20; rather, by radio-aerosol deposition,19 lung function methods for testing distribution for ventilation,10 volume of trapped gas assessed via the ratio of residual volume to total lung capacity,17 and patient preference.2

Our Practice

Twenty CF patients regularly followed at the CF center in Florence, Italy, have changed from PEP mask to CPAP as part of their airway clearance techniques, combining at the same time positive effects of continuous positive pressure with the active cycle of breathing technique (unpublished data). They represent the 44% of CF patients with severe flow obstruction (FEV1 < 40% of predicted) who attend the CF center. In the year before starting CPAP, 40% were chronically colonized with Pseudomonas aeruginosa, 15% with methicillin-resistant Staphylococcus aureus, and 15% with Burkholderia cepacia. Fifty percent were on oxygen therapy and 10% were on overnight noninvasive ventilation (NIV). When they were trained on CPAP, their mean age was 24.7 years, and on average they did 4 cycles of intravenous antibiotics per year prior to CPAP, although 52.9% had already done more than 4 cycles per year. The first reason patients initiated CPAP was oxyhemoglobin desaturation (50%), followed by fatigue (35%) and dyspnea (15%) during PEP mask. At the start of CPAP, being on oxygen therapy and doing more than 4 intravenous antibiotics cycles per year were significantly associated (P = .002). Moreover, when we considered time-to-exitus and time-to-lung transplantation as end points, CPAP was initiated significantly in advance, compared to NIV (P = .01). Up to now, none have returned to PEP mask therapy, but, instead, have adopted CPAP as a new and helpful airway clearance technique in everyday life.

According to our practice, patients' needs are the key factor in choosing one airway clearance technique over another, and this change occurred in our cohort when respiratory conditions became worse, which may indicate that CPAP could constitute a “bridge to bridge” in CF.

To our knowledge, very little is known about the time constant for a lung unit and lung mechanics in CF, especially if we have to tailor airway clearance interventions to the heterogeneous lung disease expressions in CF and then find appropriate outcomes. Only in the last decade have we learned about the positive effects of NIV,21 and there is still so much to discover about the respiratory care of CF patients. To improve efficacy and adherence, we need further research on the effects of CPAP and NIV on mucus transport in CF. While the results from Aquino and colleagues are of questionable clinical importance, due to methodological bias and inaccurate surrogate end points, they suggest a possible advantage. Respiratory physiotherapy is on the verge of a potentially exciting era in CF, and we require a change in approach in step with the increasing complexity of the disease and the new technology available. Positive pressure, delivered via NIV or CPAP, might be a valid tool, especially in advanced lung disease,18,21,22 and it would require more attention and smarter outcomes.

  • Copyright © 2013 by Daedalus Enterprises

References

  1. 1.↵
    1. Bradley JM,
    2. Moran FM,
    3. Elborn JS
    . Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis: an overview of five Cochrane systematic reviews. Respir Med 2006;100(2):191-201.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Morrison L,
    2. Agnew J
    . Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev 2009;(1):CD006842.
  3. 3.↵
    1. Aquino EA,
    2. Shimura F,
    3. Santos AS,
    4. Goto DM,
    5. Coelho CC,
    6. Bicalho de Fuccio M,
    7. et al
    . CPAP has no effect on clearance, sputum properties, or expectorated volume in cystic fibrosis. Respir Care 2012;57(11):1914-1919.
    OpenUrlAbstract/FREE Full Text
  4. 4.↵
    1. Dentice RL,
    2. Elkins MR,
    3. Bye PT
    . Adults with cystic fibrosis prefer hypertonic saline before or during airway clearance techniques: a randomised crossover trial. J Physiother 2012;58(1):33-40.
    OpenUrlPubMed
  5. 5.
    1. Donaldson SH,
    2. Bennett WD,
    3. Zeman KL,
    4. Knowles MR,
    5. Tarran R,
    6. Boucher RC
    . Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006;354(3):241-250.
    OpenUrlCrossRefPubMed
  6. 6.
    1. Elkins MR,
    2. Robinson M,
    3. Rose BR,
    4. Harbour C,
    5. Moriarty CP,
    6. Marks GB,
    7. et al
    . A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006;354(3):229-240.
    OpenUrlCrossRefPubMed
  7. 7.↵
    1. Eng PA,
    2. Morton J,
    3. Douglass JA,
    4. Riedler J,
    5. Wilson J,
    6. Robertson CF
    . Short-term efficacy of ultrasonically nebulized hypertonic saline in cystic fibrosis. Pediatr Pulmonol 1996;21(2):77-83.
    OpenUrlCrossRefPubMed
  8. 8.↵
    1. Elkins M,
    2. Dentice R
    . Timing of hypertonic saline inhalation for cystic fibrosis. Cochrane Database Syst Rev 2012;(2):CD008816.
  9. 9.↵
    1. Regnis JA,
    2. Piper AJ,
    3. Henke KG,
    4. Parker S,
    5. Bye PT,
    6. Sullivan CE
    . Benefits of nocturnal nasal CPAP in patients with cystic fibrosis. Chest 1994;106(6):1717-1724.
    OpenUrlCrossRefPubMed
  10. 10.↵
    1. Placidi G,
    2. Cornacchia M,
    3. Polese G,
    4. Zanolla L,
    5. Assael BM,
    6. Braggion C
    . Chest physiotherapy with positive airway pressure: a pilot study of short-term effects on sputum clearance in patients with cystic fibrosis and severe airway obstruction. Respir Care 2006;51(10):1145-1153.
    OpenUrlAbstract/FREE Full Text
  11. 11.↵
    1. Groth S,
    2. Stafanger G,
    3. Dirksen H,
    4. Andersen JB,
    5. Falk M,
    6. Kelstrup M
    . Positive expiratory pressure (PEP-mask) physiotherapy improves ventilation and reduces volume of trapped gas in cystic fibrosis. Bull Eur Physiopathol Respir 1985;21(4):339-343.
    OpenUrlPubMed
  12. 12.↵
    1. Oberwaldner B,
    2. Evans J,
    3. Zach M
    . Forced expirations against a variable resistance: a new chest physiotherapy method in cystic fibrosis. Pediatr Pulmonol 1986;2(6):358-367.
    OpenUrlCrossRefPubMed
  13. 13.↵
    1. Darbee JC,
    2. Ohtake PJ,
    3. Grant BJ,
    4. Cerny FJ
    . Physiologic evidence for the efficacy of positive expiratory pressure as an airway clearance technique in patients with cystic fibrosis. Phys Ther 2004;84(6):524-537.
    OpenUrlAbstract/FREE Full Text
  14. 14.↵
    1. van der Schans CP
    . Bronchial mucus transport. Respir Care 2007;52(9):1150-1156.
    OpenUrlAbstract/FREE Full Text
  15. 15.↵
    1. van der Schans CP,
    2. van der Mark TW,
    3. de Vries G,
    4. Piers DA,
    5. Beekhuis H,
    6. Dankert-Roelse JE,
    7. et al
    . Effect of positive expiratory pressure breathing in patients with cystic fibrosis. Thorax 1991;46(4):252-256.
    OpenUrlAbstract/FREE Full Text
  16. 16.↵
    1. Falk M,
    2. Kelstrup M,
    3. Andersen JB,
    4. Kinoshita T,
    5. Falk P,
    6. Støvring S,
    7. Gøthgen I
    . Improving the ketchup bottle method with positive expiratory pressure, PEP. A controlled study in patients with cystic fibrosis Eur J Respir Dis 1984;65(6):57-66.
    OpenUrl
  17. 17.↵
    1. Fauroux B,
    2. Boulé M,
    3. Lofaso F,
    4. Zérah F,
    5. Clément A,
    6. Harf A,
    7. Isabey D
    . Chest physiotherapy in cystic fibrosis: improved tolerance with nasal pressure support ventilation. Pediatrics 1999;103(3):E32.
    OpenUrlCrossRefPubMed
  18. 18.↵
    1. Holland AE,
    2. Ntoumenopoulos G,
    3. Naughton MT,
    4. Wilson JW
    . Noninvasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 2003;58(10):880-884.
    OpenUrlAbstract/FREE Full Text
  19. 19.↵
    1. Rubin BK
    . Designing clinical trials to evaluate mucus clearance therapy. Respir Care 2007;52(10):1348-1358.
    OpenUrlAbstract/FREE Full Text
  20. 20.↵
    1. Marques A,
    2. Bruton A,
    3. Barney A
    . Clinically useful outcomes measures for physiotherapy airway clearance techniques: a review. Phys Ther Rev 2006;11(4):299-307.
    OpenUrl
  21. 21.↵
    1. Flight WG,
    2. Shaw J,
    3. Johnson S,
    4. Webb AK,
    5. Jones AM,
    6. Bentley AM,
    7. Bright-Thomas RJ
    . Long-term non-invasive ventilation in cystic fibrosis: experience over two decades. J Cyst Fibros 2012;11(3):187-192.
    OpenUrlCrossRefPubMed
PreviousNext
Back to top

In this issue

Respiratory Care: 58 (9)
Respiratory Care
Vol. 58, Issue 9
1 Sep 2013
  • Table of Contents
  • Table of Contents (PDF)
  • Cover (PDF)
  • Index by author
  • Monthly Podcast
Print
Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Email Article

Thank you for your interest in spreading the word on American Association for Respiratory Care.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
CPAP in Cystic Fibrosis: Is It Time to Surrender Yet?
(Your Name) has sent you a message from American Association for Respiratory Care
(Your Name) thought you would like to see the American Association for Respiratory Care web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
CPAP in Cystic Fibrosis: Is It Time to Surrender Yet?
Simone Gambazza, Sergio Zuffo
Respiratory Care Sep 2013, 58 (9) e116-e117; DOI: 10.4187/respcare.02655

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
CPAP in Cystic Fibrosis: Is It Time to Surrender Yet?
Simone Gambazza, Sergio Zuffo
Respiratory Care Sep 2013, 58 (9) e116-e117; DOI: 10.4187/respcare.02655
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • References
  • Info & Metrics
  • References
  • PDF

Related Articles

Cited By...

Info For

  • Subscribers
  • Institutions
  • Advertisers

About Us

  • About Us
  • Editorial Board
  • Reprints/Permissions

AARC

  • Membership
  • Meetings
  • Clinical Practice Guidelines

More

  • Contact Us
  • RSS
American Association for Respiratory Care

Print ISSN: 0020-1324        Online ISSN: 1943-3654

© Daedalus Enterprises, Inc.

Powered by HighWire