airway-clearance therapy guidelines and implementation

Airway-Clearance Therapy Guidelines and Implementation

Mary K Lester RRT and Patrick A Flume MD

IntroductionWhat Is Airway Clearance?Cystic Fibrosis Pulmonary GuidelinesImplementing the Airway-Clearance Therapy GuidelinesImplementing Airway-Clearance Therapies: A Timeline for the Newly

Diagnosed PatientImplementing ACT: The Patient Who Transfers to Your CenterImplementing Airway-Clearance Therapy: The Hospitalized PatientA Role for ExerciseSummary

The clearance of secretions from the lungs of patients with cystic fibrosis (CF) is an important compo-nent in the fight to preserve their lung function. There is excessive inflammation in the airways of thesepatients, which is thought to be exaggerated by ineffective mucociliary clearance and bacterial infection.In 2005 the Cystic Fibrosis Foundation formed the Pulmonary Therapies Committee to review all of themedical literature on the various airway-clearance therapies used in treating CF lung disease. Therecommendations were: an airway-clearance therapy should be performed by all patients with CF, noform of airway-clearance therapy stood out as being superior to another, and that patients may expressa preference of one therapy over another. They also concluded that aerobic exercise is beneficial topatients with CF, as it is to everyone, and that exercise should be a component to the overall healthroutine of patients with CF. The challenge for respiratory and physical therapists together with thepatient/family is to develop a plan of attack through the use of various airway-clearance therapies. Therespiratory and physical therapists are integral in helping patients and families develop airway-clearance routines that aid in the removal of the secretions that cause airway obstruction. There is a widerange of airway-clearance therapies that therapists can choose from when they are teaching the patientsand family members the strategies of secretion removal. The questions are: What therapy is best forwhat age or stage of lung disease? What therapies will the patient do? And which therapies will becovered by medical insurance? These are all fundamental questions that must be answered whenguiding families in finding therapies that are effective and appropriate for each CF patient’s uniquesituation. Key words: cystic fibrosis, airway-clearance therapies, secretion removal, airway-clearance teach-ing, airway-clearance guidelines. [Respir Care 2009;54(6):733–750. © 2009 Daedalus Enterprises]

Mary K Lester RRT is affiliated with the Departments of RespiratoryTherapy, Medicine, and Pediatrics; Patrick A Flume MD is affiliated withthe Departments of Medicine and Pediatrics, Medical University of SouthCarolina, Charleston, South Carolina.

The authors have disclosed no conflicts of interest.

Ms Lester presented a version of this paper at the 43rd RESPIRATORY

CARE Journal Conference, “Respiratory Care and Cystic Fibrosis,” heldSeptember 26-28, 2008, in Scottsdale, Arizona.

Correspondence: Mary K Lester RRT, Department of Respiratory Ther-apy, Medical University of South Carolina, 169 Ashley Avenue, PO Box250906, Charleston SC 29425. E-mail: [email protected].

RESPIRATORY CARE • JUNE 2009 VOL 54 NO 6 733

Introduction

Airway-clearance therapies (ACTs) have long been con-sidered an important part of care of the patient with cysticfibrosis (CF). This is based upon our understanding of thepathophysiology of CF lung disease. There is ineffectivemucociliary clearance1 and, from very early in life, there isobstruction of the small airways by mucus.2 Mucociliaryclearance is an important defense mechanism of the air-ways, and patients are vulnerable to chronic infection ofthe airways. There is an exaggerated inflammatory re-sponse, with the airways containing bacteria, inflamma-tory cells, and cellular breakdown products (eg, neutro-phil-derived deoxyribonucleic acid and filamentous actin(F-actin).3-6 All of this material increases the tenacity andstickiness of the airway secretions, and the airways be-come further obstructed.7,8 Since the mucociliary clear-ance system isn’t working, it makes sense to use othermethods to help clear the airway secretions, if only torelieve the obstruction. In addition, these secretions con-tain bacteria and inflammatory mediators that continue toinjure the airways as well as recruit new inflammatorycells. Therefore, clearance of airway secretions does morethan merely relieve obstruction; it also reduces the amountof infection and inflammation present in the airways.

What Is Airway Clearance?

ACTs are physical or mechanical means of facilitatingthe removal of tracheobronchial phlegm through the ex-ternal and/or internal manipulation of air flow, and theevacuation of phlegm via coughing. One of the earliestforms of therapy, which continues to be a primary method,is chest physiotherapy, or percussion and postural drain-age. In 1959, British physiotherapist Jocelyn Reed firstreported that “clapping and pressure vibrations, during longexpirations, are the most effective forms of mechanicalstimulus to elimination of secretions in the treatment oflung abscess, collapsed lobes, and bronchiectasis.”9 ACTshave evolved over the years, using imaginative strategiessuch as high-frequency chest-wall compression using aninflatable vest connected to an air compressor, hand-heldexpiratory vibratory devices, and, more recently, acousticwaves to vibrate the mucus from the airway walls. Therespiratory therapist has an extensive menu of airway-clearance modalities from which to choose (Table 1), eachwith advantages and disadvantages. The intent of this pa-per is not to discuss the techniques for delivering the nu-merous airway-clearance modalities; however, we haveincluded the instructions that we give to our patients andfamilies on these therapies (Appendix). The intent is tohelp guide the therapist in choosing an ACT that is appro-priate for a CF patient at different stages of life. Thechallenge for the therapist is to match the therapy with the

patient so that there is the most effective clearance ofairway phlegm.

Cystic Fibrosis Pulmonary Guidelines

The Cystic Fibrosis Foundation established the Pulmo-nary Therapies Committee to develop guidelines for med-ications and therapies to maintain the lung health of patientswith CF. Thus far, there are published recommendationsfor medications used chronically10 and for ACTs.11 A sys-tematic review of the published literature was used to de-velop these recommendations. An important realization inthe development of the ACT guidelines was the lack oflarge, long-term studies of airway clearance. It is not thatthere isn’t any evidence to support ACT for patients withCF; there are, in fact, a large number of studies, but mosthave small numbers of patients, are performed over shortperiods of time (some only a single treatment), and manyhave no comparator. A prior systematic review concludedthat ACT increases mucus transport for the short-term, butthe authors could not draw conclusions about the long-term effects or benefits.12 Another review noted that, de-spite the “dearth of high-level evidence to support anysecretion clearance technique,” the lack of evidence doesnot mean lack of benefit.13 The Pulmonary Therapies Com-mittee felt that the existing evidence supporting ACT com-pared to no therapies was fair in quality overall. Theyexpressed concern that because ACT is so fundamental toCF treatment, a large controlled trial designed to explicitlyprove the benefits of ACT could not be completed. Theydetermined that the overall benefit of ACT was moderate,based upon the various outcomes that had been studiedwith a variety of therapies, including increased sputumproduction, increased lung function in the short-term, re-duced rate of decline of lung function, and increased ex-ercise tolerance. In the end, they recommended that someform of ACT be performed as a routine in all patients.11

There were other important findings in the systematicreview that led to specific recommendations by the com-mittee. First, there weren’t any data to suggest that onetype of ACT was better than any other. Second, patientswill express preferences, and it is believed that patients aremore likely to use therapies they believe to be beneficial.Finally, aerobic exercise seemed to offer benefits to aregimen of airway clearance in addition to the other well-known benefits of exercise.14

Implementing theAirway-Clearance Therapy Guidelines

Now that we have recommendations regarding ACT, wemust address how to implement them in our CF clinics.We must find a way to communicate these recommenda-tions to our patients and their families, and help them find

AIRWAY-CLEARANCE THERAPY GUIDELINES AND IMPLEMENTATION

734 RESPIRATORY CARE • JUNE 2009 VOL 54 NO 6

a method of ACT that works best for them. Given the thirdrecommendation that patient preference matters, it seemsrational to introduce the patients to all of the availabletherapies. Then they can make an informed decision as towhich of the therapies they are most likely to use. How-ever, there are important factors in the choice of therapies.For example, an infant is not capable of performing mostof the available therapies and is dependent upon others (ie,parents) to perform the treatment, typically percussion andpostural drainage. On the other hand, a person who livesalone will not have a partner to assist with percussion andpostural drainage, and so will probably benefit more froma type of ACT that can be performed independently (eg,high-frequency chest compression). Therefore, there are anumber of factors to consider regarding recommendationsof specific ACT, including independence, effectiveness,ease of use, flexibility, treatment durations, comfort, con-venience, and interruption to daily living. It has been ourexperience that patients will benefit from more than onetype of ACT, and so we introduce them to all of theoptions at an appropriate time of their life (Fig. 1). Beforediscussing how to introduce various therapies to patients,it is useful to express our other basic tenets of ACT:

1. The physician prescribes the therapy but does notnecessarily know what is best. The CF team typically in-cludes an expert in ACT, most often a respiratory therapistor a physical therapist. The therapist’s role is to work withthe patient and family to determine the most appropriateACT and educate them as to its proper performance.

2. Airway clearance is an active process. Most patientswill be performing their therapies on themselves in theout-patient setting. Even in the hospital setting, the thera-pist or parent who is performing ACT on a patient workswith the patient to assist clearance of the airway secre-tions. The patient should play an active role in making thetherapy effective by coughing throughout and performingdeep breath-holds and forced expiratory maneuvers at ap-propriate times. No single method of airway clearance isbetter than another. This is similar to the CF PulmonaryTherapies Committee’s second recommendation. Althoughno therapy has been shown better than the others, this doesnot mean that one form of therapy won’t prove superior tothe others for the individual patient. What is also apparentis that one form of ACT may prove useful at one time, butanother will be more effective at another time. Similarly,patients may be able to use one form of therapy when theyare home (eg, percussion and postural drainage) but mayneed a different therapy when they are away from homeliving alone (eg, college).

3. Airway clearance is boring. There is no way aroundthis reality. ACT is tedious, monotonous, and takes time toperform effectively. This is another reason for patients tohave access to more than one form of therapy.

4. Be creative. This has more than one meaning. First,there are a number of fun activities and toys for childrenthat can serve as a method of airway clearance. Take awalk down an aisle of party favors and look at all of thetoys that behave like positive-expiratory-pressure (PEP)

Table 1. Methods of Airway Clearance

Therapy Age Range Advantages Disadvantages

Percussion and postural drainage All ages No cost/no equipmentTarget specific areas of lung

Requires caregiverTiresomeDoes not promote independence

Blowing games 18 mo to 5 y Low costPrepares child for future spirometry

Patient must have appropriate cognitiveability

Appropriate coughing � 18 mo Teaches to cover mouthPromotes good hand-washing

None

High-frequency chest compression � 2 y Promotes independencePortableAdminister nebulized medications at

same time

Expensive

Huff cough � 3–4 y Gentle coughing technique NoneActive cycle breathing technique � 4 y Independent, inconspicuous

Can be performed anywhere, anytimePatient must have appropriate cognitive

abilityAutogenic drainage � 8 y Independent, inconspicuous

Can be performed anywhere, anytimePatient must have appropriate cognitive

abilityHandheld devices, PEP, oscillating PEP � 4 y Low cost

Promotes independenceSome devices allow nebulized

medications

Patient must have appropriate cognitiveability

Intrapulmonary percussive ventilation � 5 y Can nebulize medications while performing Expensive

PEP � positive expiratory pressure



devices. Second, we ask our patients to perform lengthytherapies several times daily. We need to work with themto help them figure out a way to get these therapies intotheir routine. Listen to the patients to learn how they spendtheir day, either at work or at school, and discuss ways toincorporate ACT into that schedule. We must try to adaptthe therapies to the patient’s needs and let the therapies beless interruptive to their daily living.

Implementing Airway-Clearance Therapies:A Timeline for the Newly Diagnosed Patient

The infant (birth to 18 months old) is completely de-pendent upon the caregiver (eg, parent) for performance ofACT. Most of the other therapies are inappropriate for theinfant. Therefore, the parents of the newly diagnosed in-fant with CF should be introduced to percussion and pos-tural drainage. The therapist will meet with the parentsinitially to teach them the technique, and will meet withthem at subsequent clinic visits to evaluate their techniqueand re-educate as needed.

Toddlers (18 months to 4 years) are often ready to tryhigh-frequency chest compression. There are currently 3high-frequency chest-compression devices available on themarket: The Vest (Hill-Rom, St Paul, Minnesota), InCour-age (Respirtech, St Paul, Minnesota), and SmartVest (Elec-tromed, New Prague, Minnesota). These devices are ex-pensive, and so we confirm with the patient’s insurancecarrier that such durable medical equipment will be cov-ered. We introduce each of the devices to the patient andfamily at a clinic visit or in the hospital; the parent orcaregiver will then be able to make an informed decisionon which machine to choose, if it is clear the patient willuse the device. Also, at this young age the patient shouldbe taught blowing games (eg, pinwheel, bubbles) to pre-

pare them for the prolonged exhalation maneuvers theywill need to master prior to their first spirometric test. Huffcough instruction can also start during this time.

School-age children (5 years to preteen) can begin basicactive cycle breathing techniques. Patients can be taughtbreath-holds and prolonged exhalation maneuvers. The huffcough or forced expiratory technique can be mastered atthese ages, and portable devices such as PEP or oscillatingPEP (eg, Acapella or Flutter) should be used. Patients needto have access to portable devices for use while away fromhome, such as at camp, on sleep-overs, or while beingdriven to school. Patients this age should be encouraged toparticipate in sports. Exercise needs to be a part of CFpatient’s daily routine. Not only does exercise improvecardiovascular health and self esteem, it can also act as amethod of removing secretions.15 Some patients report thatwhen they run, bike, or swim, these movements cause avibration and loosening of the mucus in the chest. Therehave not been enough controlled trials to demonstrate thatexercise is an effective form of airway clearance, but thePulmonary Guidelines Committee found that exerciseshould be considered an adjunct to a patient’s airway-clearance routine.

Preteen to early teen CF patients express a need to be“not different” from their peers. It is common for parentsto complain that their child “will not do anything” whenasked about airway clearance. This is a very challengingtime in life because teenagers want to be independent andto make their own decisions, and often they choose to skipperformance of ACT. But this is the most vulnerable timefor patients, and one in which we see a greater rate ofdecline in their lung function.16 Patients at this age canimprove on the techniques of active cycle breathing tech-nique and huff cough, and learn autogenic drainage. Whendone properly, these techniques are effective and they can

Fig. 1. Not all airway-clearance modalities are appropriate for all ages. We have a strategy of introducing airway-clearance techniques toall patients at our center. We introduce the various airway-clearance techniques based upon the patient’s age and ability to perform thetherapy. This timeline approximates the appropriate age the therapy can be introduced. AD � autogenic drainage. CCPT � conventionalchest physiotherapy. HFCC � high-frequency chest compression. ACBT � active cycle breathing technique. IPV � intrapulmonarypercussive ventilation.



be done inconspicuously. We must negotiate with the teensto help them establish a routine that is effective, conve-nient, and causes little interruption in their lifestyle. Theyshould learn that breathing techniques can be done at anytime and they do not require an external device or care-giver.

By the time they reach adulthood (age 18 years and up),patients with CF will have been introduced to the entireACT armamentarium, including percussion and posturaldrainage, high-frequency chest compression, PEP, oscil-lating PEP (eg, Acapella), autogenic drainage, active cyclebreathing technique, and intrapulmonary percussive ven-tilation. All adults should have more than one techniqueavailable to them, and we must work with them to estab-lish a routine that fits with a busy lifestyle that includesschool, work, and family time. As their lung disease reachesa more severe stage, they must realize that they are likelyto require increased time spent with ACT.

Implementing Airway-Clearance Therapy:The Patient Who Transfers to Your Center

Like many others, people with CF may move to a dif-ferent city, and will transfer their care to a different CFcenter. When we see a new patient for the first time, we tryto learn what therapies the patient is currently performingand assess their technique, providing tips for improvementas needed. We also perform a survey of their knowledge ofall of the other therapies. Do they have knowledge of theother therapeutic options? Have they tried them? What didthey like or not like about those therapies? We will thenmake plans for a future clinic visit to introduce them to thetherapies they have not tried, following the same timelinenoted above.

Implementing Airway-Clearance Therapy:The Hospitalized Patient

Patients with CF may experience acute worsening ofsymptoms, such as increasing cough and sputum produc-tion, often called a pulmonary exacerbation. The CF pul-monary guidelines on treatment of a pulmonary exacerba-tion suggest that ACTs may need to be changed duringsuch an event.11 In situations where the exacerbation ismild and out-patient therapy is planned, the patient can beadvised to increase the time spent with therapy, or to per-form the therapies more frequently, or even to consider analternative therapy. For those who are ill enough to war-rant admission to the hospital, there is an opportunity fortherapists to become fully engaged. At the start of thehospitalization it is most important to begin with a therapythe patient finds the most comfortable. It is a time toestablish early success in clearance of secretions and toestablish a trusting relationship between the therapist and

the patient. As the patient improves, this is now an oppor-tunity to evaluate the patient’s ability to perform airwayclearance on their own as well as introduce therapies theyhave not yet tried.

A Role for Exercise

A question asked frequently in the CF clinic is, “Can my30 min of running take the place of my ACT?” Indeed,there are many patients with CF who participate in regularexercise activities that are fairly rigorous. The PulmonaryGuidelines Committee recommended aerobic exercise asan adjunctive therapy for airway clearance. Although therewas not sufficient evidence to conclude that exercise wasas effective as other ACT, there was evidence that exercisedoes result in clearance of secretions and there is improve-ment in quality of life.15 There are additional benefits toaerobic exercise: increased endurance, increased self es-teem, and improved cardiovascular health.14 Patients withCF who have greater fitness have better survival.17 It is ourperception that the patients in our clinic who exercise reg-ularly tend to have better overall health.

Summary

The CF Pulmonary Guidelines Committee came to thesame conclusion that most clinicians had already believed:that performance of techniques of airway clearance worksfor patients with CF. We do not have evidence that anysingle method of ACT is best for all patients, but we arefortunate to have many therapies from which to choose.The opportunity for the therapist and the entire CF team isto work with the patients and families to find the therapiesthat work for the individuals, given the myriad of resourcesthey have and the hurdles they face. It is important to listento the patients as they will help us figure out what worksbest for them, and they will teach us tricks that will workfor other patients as well.

REFERENCES

1. Boucher RC. Evidence for airway surface dehydration as the initi-ating event in CF airway disease. J Intern Med 2007;261(1):5-16.

2. Robinson M, Bye PT. Mucociliary clearance in cystic fibrosis. Pe-diatr Pulmonol 2002;33(4):293-306.

3. Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolarlavage findings in cystic fibrosis patients with stable, clinically mildlung disease suggest ongoing infection and inflammation. Am J Re-spir Crit Care Med 1994;150(2):448-454. Erratum in: Am J RespirCrit Care Med 1995;151(1):260.

4. Birrer P, McElvaney NG, Rudeberg A, Sommer CW, Liechti-GallatiS, Kraemer R, et al. Protease-antiprotease imbalance in the lungs ofchildren with cystic fibrosis. Am J Respir Crit Care Med 1994;150(1):207-213.

5. Bonfield TL, Panuska JR, Konstan MW, Hilliard KA, Hilliard JB,Ghnaim H, et al. Inflammatory cytokines in cystic fibrosis lungs.



Am J Respir Crit Care Med 1995;152(6 Pt 1):2111-2118. Erratum in:Am J Respir Crit Care Med 1996;154(4 Pt 1):following 1217.

6. Bonfield TL, Konstan MW, Burfeind P, Panuska JR, Hilliard JB,Berger M. Normal bronchial epithelial cells constitutively producethe anti-inflammatory cytokine interleukin-10, which is downregu-lated in cystic fibrosis. Am J Respir Cell Mol Biol 1995;13(3):257-261.

7. Chernick WS, Barbero GJ. Composition of tracheobronchial secre-tions in cystic fibrosis of the pancreas and bronchiectasis. Pediatrics1959;24:739-745.

8. Vasconcellos CA, Allen PG, Wohl ME, Drazen JM, Janmey PA,Stossel TP. Reduction in viscosity of cystic fibrosis sputum in vitroby gelsolin. Science 1994;263(5149):969-971.

9. Doershuk CF, editor. Cystic fibrosis in the 20th century, people,events, and progress. Cleveland, Ohio: AM Publishing; 2001.

10. Flume PA, O’Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ,Willey- Courand DB, et al; Cystic Fibrosis Foundation PulmonaryTherapies Committee. Cystic fibrosis pulmonary guidelines: chronicmedications for maintenance of lung health. Am J Respir Crit CareMed 2007;176(10):957-969.

11. Flume PA, Robinson KA, O’Sullivan BP, Finder JD, Vender RL,Willey-Courand DB, et al; Clinical Practice Guidelines for Pul-

monary Therapies Committee. Cystic fibrosis pulmonary guide-lines: airway clearance therapies. Respir Care 2009;54(4):522-537.

12. van der Schans C, Prasad A, Main E. Chest physiotherapy comparedto no chest physiotherapy for cystic fibrosis. Cochrane Database SystRev 2000(2):CD001401.

13. Hess DR. The evidence for secretion clearance techniques. RespirCare 2001;46(11):1276-1293.

14. Kesaniemi YK, Danforth E Jr, Jensen MD, Kopelman PG, LefebvreP, Reeder BA. Dose-response issues concerning physical activityand health: an evidence- based symposium. Med Sci Sport Exerc2001;33(6 Suppl):S531-S538.

15. Balestri E, Ambroni M, Dall’Ara S, Miano A. Efficacy of physicalexercise for mucus clearance in patients with cystic fibrosis (ab-stract). Pediatr Pulmonol 2004;38:316.

16. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Reg-istry, 2006 Annual Data Report to the Center Directors. Bethesda,Maryland: Cystic Fibrosis Foundation; 2007.

17. Nixon PA, Orenstein DM, Kelsey SF, Doershuk CF. The prognosticvalue of exercise testing in patients with cystic fibrosis. N EnglJ Med 1992;327(25):1785-1788.



Discussion

Geller: It’s great to hear somebodytalk about the practicalities of how toget people to do airway clearance,which is neglected by a lot of people,and it’s really fundamental. You men-tioned Rule 4, airway clearance is bor-ing, and Rule 5, think outside the box.I ask my patients, “Have you figuredout a way to make airway clearancefun?” As of yet, I have not had a sin-gle person give me an answer otherthan, “I sit in front of the computer orTV,” and that’s their exercise too: us-ing the remote control.

Some of my healthiest patients playwind instruments: trumpet, trombone,saxophone, or clarinet, but not flute:instruments that create some resis-tance. With wind instruments, they’rebasically performing high-pressurePEP, and it’s a wonderful airway-clearance technique. Usually a prac-tice session lasts about 30 minutes toan hour a day, and if they like theinstrument, then it’s fun. That’s oneway to make airway clearance fun.

Exercise is an adjunct to therapy,and when they get old enough, theycan join the marching band and playand march and get exercise at the sametime. That’s the only thing that I’vefound, besides putting a vest in thecar, that might actually be fun, at leastfor some patients. Unfortunately, whenthey graduate high school, they usu-ally drop the instrument and don’t playany more, and then lung function de-teriorates because they never learnedany other technique. But it was funwhile it lasted.

Lester: On YouTube an airway-clearance video showed a duck-call-ing contest, and the 3 finalists wereCF patients. They exhaled through avibrating duck whistle. They had funwith it and probably didn’t know thatthey were doing airway clearance. Vesttherapy can be a passive therapy, andwhen I went to Minnesota last yearfor adult benchmarking, they used the

Minnesota Protocol,1 in which they in-crease the frequency throughout thetreatment, and every 5 minutes theypop a hose and do some coughing. Itadds time to the therapy session, but itmakes the session more active, becausethey’re doing true airway clearance bystopping to cough. I’ve had some goodresponse with that treatment plan.Some patients really take on the Min-nesota Protocol, and they do their neb-ulizer treatments at the same time. Thatmakes vest therapy more active thanpassive.

REFERENCE

1. Kempainen RR, Williams CB, HazelwoodA, Rubin BK, Milla CE. Comparison ofhigh-frequency chest wall oscillation withdiffering waveforms for airway clearancein cystic fibrosis. Chest 2007;132(4):1227-1232.

Geller: After vest therapy, how im-portant is the addition of posturaldrainage? They’re usually sitting up-right during vest therapy, so how im-portant is it to add postural drainage,for someone who has ineffectivecough?

Lester: I’ve never suggested that.Sometimes at the hospital we mightadd CPT [chest physiotherapy] andpostural drainage to target specificproblem areas of the lung.

O’Malley: If you want to adverselyimpact adherence, asking them to do2 consecutive treatments would prob-ably be the way. I wouldn’t ask a pa-tient to do 30 minutes of vest therapyand then spend more time doing an-other therapy, at least not in home man-agement.

Lester: We had a 5-year-old CF pa-tient recently, and her main therapywas vest therapy. While she was anin-patient, we thought it was a goodtime to try different therapies. She wasso sick and had a lot of secretions, andthe therapist thought vest therapy wasnot being effective, so they tried chest

physiotherapy and postural drainageand got a lot of mucus out. If onetherapy is not working, try another. Ihave not had any patients who feltlike doing drainage positions after vesttherapy.

Flume: Or during.

Lester: Sure, or during.

Ratjen: The guidelines reflect ourlack of data on whether one airway-clearance therapy is better than an-other, and I’m a bit frustrated by that,because we’ve been doing airwayclearance techniques in CF for decadesnow, and the evidence we have comesfrom rather small studies. On manyquestions we’ve done large, well-powered studies that provided goodevidence, but we haven’t done thatfor airway clearance. Should we justgive up on that, or, rather than saying,“One method is probably as good asthe other, but we don’t really know,”should we try to get some more evi-dence?

Our physiotherapists are getting to-gether to compare PEP and vest ther-apy, and to determine whether it’s go-ing to be feasible and whether you canpower the study to find an outcomemeasure that really shows a difference.I still think that having stronger evi-dence than we have now would help,and maybe some of the more sensitivetechniques, such as the lung-clearanceindex, will help us sort this out andgive us better outcome measures. Thecurrent evidence is as you presentedit, but I still feel a little uncomfortableabout this, because in many areas wesay we’ll build our guidelines on thedata, but lack the data for airway clear-ance.

Lester: It’s an n-of-1 study some-times. What works for one patient maynot work for another. If vest therapyis the best way to do it, but the patientwon’t do vest therapy, then we haveto use other therapies.



Flume: The guidelines committeedid ask whether we should do a de-finitive study of airway clearance. Thefirst question was to compare airwayclearance to no airway clearance. Asmuch as we all know the importanceof airway clearance, the evidence tosupport it was only level B. The guide-lines1 state that, although we mightwant more robust data, the chance ofa study being completed successfullyis probably nil, because I don’t thinkanybody would enroll a patient in sucha controlled trial.

As for the studies of devices, therigor of the approval studies for a de-vice is far less than that for a medi-cation, and that’s why device studieshave few subjects. We would like morerigorous evaluation of new devices,but the challenge is, what would welike to see measured? As Bruce said,what might seem to be the most intu-itive outcome measure (sputum vol-ume) may not be very useful.

REFERENCE

1. Flume PA, Robinson KA, O’Sullivan BP,Finder JD, Vender RL, Wiley-CourandDB, et al. Cystic fibrosis pulmonary guide-lines: airway clearance therapies. RespirCare 2009;54(4):522-537.

Newton: Would you put the patientin the head-down position after vesttherapy?

Geller: I’m not the right person toask that question, you are. I asked thatquestion because before the advent ofvest therapy we had hands-on CPTfollowed by postural drainage. Withvest therapy there’s no postural drain-age recommended, so the question is,did that do any good in addition toCPT, or did we just do it because itmade sense that gravity might help?And maybe it did help in some peo-ple, maybe it didn’t in others, but itwas just the standard regimen: CPTand postural drainage. I’m not sure itwould have an effect. I don’t knowthat there’s any evidence.

That said, if an individual patient isnot improving with vest therapy andthey don’t have an effective cough,Pulmozyme’s not going to work—nothing will work—you have to beable to cough it out. If they don’t havean effective cough, they might jigglethe secretions around, but they won’tget them out. So the question is, wouldpostural drainage help? I don’t knowthe answer.

Volsko: We haven’t paired vest ther-apy with postural drainage, but wehave paired postural drainage with os-cillating PEP devices, such as Flutteror Acapella, and that seemed to help.

Mary, you brought up a really goodpoint about thinking outside the box.We use the Acapella with a mask invery young patients who can’t per-form all the postural-drainage posi-tions because of reflux, and that worksnicely, as it does with patients at endof life, who have very low expiratoryflow. We get a good cough and someexpectoration from those patients aswell.

Rubin: If I can address this: at theairway clearance journal conference ayear ago1,2 there was a full reviewabout airway clearance physical ther-apy, and Cees van der Schans, whodoes the Cochrane reviews, looked atthe role of postural drainage.3 It wasclear that from the studies that weredone, which were few, there’s no ad-vantage with postural drainage at all,under any circumstances, other thanthe fact that it prolonged physical ther-apy. There are some risks for increasedintracranial pressure in small babies,increased reflux, and discomfort in ahead-down position. So in our center,for example, we do not use posturaldrainage.

REFERENCES

1. Airway clearance: physiology, pharmacol-ogy, techniques, and practice. Respir Care2007;52(9):1070-1238.

2. Airway clearance: physiology, pharmacol-ogy, techniques, and practice. Respir Care2007;52(10):1239-1405.

3. van der Schans CP. Bronchial mucus trans-port. Respir Care 2007;52(9):1150-1156;discussion 1156-1158.

McFall:* Do you find that patientshave a big resistance to airway clear-ance, or is it just that they do not un-derstand the reasons for this therapy?Or is it the time required? Patientsdon’t necessarily realize that they’reneeding to do their therapy. If I don’ttake my enzymes, I notice within12 hours, but patients adapt very wellto feeling short of breath; they get usedto feeling congested, and they don’tnecessarily make the connection thatif they did their airway clearance, itwould be beneficial. How do you ad-dress that?

Lester: It’s different at differentphases in life. With parents of a newlydiagnosed and asymptomatic infant, itcan be difficult to get them to buy inand do the therapies twice a day. With5-to-8-year-olds who are starting tobecome symptomatic and have hadtheir first Pseudomonas-positive cul-ture, usually the family will buy in atthat point. Teenagers don’t want to dotheir therapies; they want to be incon-spicuous and fit in with their peers, sotherapies stop. When they becomeadults and the lung disease gets moresevere, then they’re on board, and usu-ally very adherent to therapy. At ourcenter we teach all the therapies bythe time they’re 12 years old. Life cir-cumstances get in the way a lot oftimes.

Flume: Folks with CF are not dif-ferent from the rest of us. Most of usaren’t going to get on the treadmilltoday, even though we know it hashealth benefits; it’s boring and it’s hardto get into that routine.

* Kristin McFall, Hill-Rom, St Paul, Minne-sota.



Lester: And it’d be hard to fit in to-day. Every day is different. I don’tthink it’s a lack of knowledge of theclearance techniques; it’s just, “whatam I going to do at this particulartime?” That’s why if you can incor-porate sports or wind instruments intoactivities of daily living, the patientwill get health benefits and be doingnormal activities without necessarilyrealizing how much benefit they’regetting, and they’ll buy into it more.

Marshall: I’m always struck by howcomplicated it is for pediatricians withthe developmental stages and dealingwith all those little intricacies, as op-posed to internists, for whom it’s alittle more straightforward. I’m curi-ous about differences in what airwayclearance techniques are prescribedand how exercise is approached in Eu-rope and Canada versus the UnitedStates.

Davies: Something that struck me ina couple of the talks today is that inthe United Kingdom I think we usefewer devices, we certainly use vesttherapy very much less commonly thanwhat you’re describing. In my center,and at Great Ormond Street Hospital,where I’ve also worked, we very muchmore rely on the parents and the childto administer the straightforward tech-niques, as opposed to using the de-vices that you’ve mentioned. We havethem available, and we use them indifficult cases, and we depend on ourphysiotherapy colleagues to advise uson what they think is best for the child,but in general I suspect that we use

rather fewer devices, and certainly rel-atively fewer vests. Part of that mightbe cost. At my institution there arestudies underway on whether vest ther-apy is as effective as other techniques.

Lester: Do you emphasize physicalexercise?

Davies: We emphasize physical ex-ercise, but not as a substitute for air-way clearance. We try to promote thatas a healthy lifestyle, but certainly inthe pediatric clinic we’re always say-ing, “That’s not instead of your phys-iotherapy; that’s as well as.”

Ratjen: In Canada the primary formof physiotherapy is PEP, based on theToronto study of PEP versus posturaldrainage.1 It was a small study but itlooked rather positive, and it used arelatively simple technique, so this isthe primary form of physiotherapy.The cost of vest therapy is one of themajor concerns, and there’s a lot ofinterest in looking at that.

We helped our physiotherapy col-leagues Maggie McIlwaine and Jen-nifer Agnew design a study to com-pare PEP to vest therapy in a cohort ofCF patients, with pulmonary exacer-bation as a primary outcome.2 I thinklooking at lung function is probablynot going to be very useful, and if Iuse the analogy from hypertonic sa-line, then I expect that pulmonary ex-acerbation is probably the outcome wehave to look at. It’s going to be a one-year study. The problem is that thereis no placebo arm, and ideally youwould like to do that study with a pla-

cebo arm, but I don’t think that’s fea-sible.

In various countries throughout theworld there’s a lot of belief in certaintechniques. In Belgium, Chevalier in-troduced autogenic drainage and justabout every patient is going to usethat technique, and he goes from cen-ter to center teaching those techniques.The problem I have is that there’s alot of belief in the field, but a lack ofdata. We strongly encourage exerciseand we have a long-term exercise studygoing on in Toronto. One interestingthing we see in the data from morethan 6 years is a correlation betweendaily physical activity and lung-func-tion decline in CF patients, across thewhole population [unpublished data],so we strongly believe that exerciseshould be a focus. The only problemis that we have the ideal interventionto promote that as a long-term goal,because exercise protocols are diffi-cult to follow, so you have to findways to promote regular exercise; thedata support that.

REFERENCES

1. Newbold ME, Tullis E, Corey M, Ross B,Brooks D. The Flutter device versus thePEP mask in the treatment of adults withcystic fibrosis. Physiotherapy Canada 2005;57(3):199-207.

2. McIlwaine M, Davidson G, Bjornson C,Smith C, Pasterkamp H, Kraemer L, et al;University of British Columbia; CanadianCystic Fibrosis Foundation. Long-termstudy, comparing vest therapy to positiveexpiratory pressure (PEP) therapy in thetreatment of cystic fibrosis. http://clinicaltrials.gov/ct2/show/nct00817180. AccessedApril 24, 2009.



airway-clearance therapy guidelines and implementation

Documents