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Pediatric Cystic Fibrosis

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Andrew Han

on 14 September 2012

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Transcript of Pediatric Cystic Fibrosis

Assisted Devices in Airway Clearance Cystic Fibrosis By Annabelle Introduction NO CURE at the moment!! Inherited recessive genetic condition (CFTR)
Main characteristic: increase in amount of mucus in airways and difficulty breathing
Children
Caucasian population
1 in every 2500 babies in AUS Cystic Fibrosis Airway Obstruction and decreased cough effectiveness
Persistent cough, particularly with physical effort
Some difficulty in breathing or wheezing with physical effort
Tiredness and impaired exercise ability.
Re-occuring inflammation and infection which cause injury and structural changes to the lungs Health Problems Introduction By Kazuko Description Description PEP therapy was first introduced in Denmark following the first report of PEP for physiotherapy
M Falk et al. 1984


Before the introduction of PEP, conventional chest physiotherapy for airways clearance in CF included PD, percussion and vibrations, FET(cough) Why Was It Implemented? If resting lung volume ↑ more air behind secretions →therefore more effective cough?

PEP, usually CPAP or PEEP to treat acute respiratory failure;
- Low resting lung volumes
- Diffuse airway obstruction

How about applying this to CF?

→ 1984 Falk M et al. “Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis.”, Herlev Hospital, Denmark
Aim: To evaluate whether PEP applied with a face mask is of advantage to CF patients

Method: Each of 14 CF patients was subjected to 4 different chest physio regimens in 2 days;
- A= PD + percussion & vibrations
- B= PD + PEP mask
- C= PEP in sitting position
(A,B,C were followed by FET and cough)
- D= FET and cough in sitting position “Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis.”
1984 Falk M et al. Copenhagen, Denmark C   Subjective Ax of acceptability
11 patients preferred Rx C – easy, non-tiring, effective and possibility of self-administration

11 patients unaccepted Rx D – exhausting

Rx’s involving PD, i.e. Rx A and B were rejected by 7 and 9 patients respectively
uncomfortable, time consuming, ineffective and dependent on help from others Many subsequent papers supporting the use of the PEP

1986 Tyrrell JC et al, the first study of PEP in UK also showed that;

the use of a PEP mask with FET (cough) was as effective as conventional physiotherapy over a short period.

the mask was well accepted and allowed independent treatment by older paediatric patients. 1997 Mcllwaine PM et al. in Vancouver, Canada showed PEP mask more effective than PD and percussion.

Concluded that the PEP was more effective method of physiotherapy PEP was well accepted by the patients. Suggestion to incorporate PEP in chest physio regiments if the aim is to increase expectoration. Conclusion Results Conclusion F PEP mask became a popular method physiotherapy
Effective in secretion clearance
Well accepted by the patients
Some degree of independence to the patient (T. R. Myers. 2007) The theoretical benefit of PEP therapy is the ability to enhance and promote mucus clearance by either;
preventing airway collapse by stenting the airways OR
increasing intrathoracic pressure distal to retained secretions by collateral ventilation or by increasing FRC Goals of PEP By Joey Description By Andrew Conclusion By Amy Analysis By Bobbie Analysis - A= PD + percussion & vibrations
- B= PD + PEP mask
- C= PEP in sitting position
(A,B,C were followed by FET and cough)
- D= FET and cough in sitting position
Total sputum yields
A = 10.0g (1.9-51.1)
B = 21.6g (12.5- 53.5)
C = 17.4g (5.8- 50.7)
D = 15.0g (5.4- 44.9)

Sputum Expectorated per min clearance rate
A = 209mg/min (54-409)
B = 557mg/min (386-910)
C = 681 mg/min (472-896)
D = 607mg/min (388-1017) Results How It Has Been Implemented Why it was implemented Goals of PEP Analysis References Ventilation and airflow can occur between lung segments via anatomical channels
Healthy adults: resistance in collateral channels is greater than normal conducting airways
CF: resistance is increased within normal conducting airways due to increased secretions + airway collapse  relative resistance to airflow through collateral channels is lower
In children these channels are not developed until 2-3 years of age Theoretical assumptions No difference in measures of lung function, exercise capacity, and QoL measures between ACBT, AD, Cornet, Flutter, PEP over 1 year
Pryor et al 2010
No evidence that PEP is better than CCPT or vice versa
Marks 2007
No difference in lung function, exercise performance, or user satisfaction between Acapella device and PEP mask for CF during acute exacerbation
West, Wallen & Follett 2010 Known outcomes Require appropriate education and re-education to ensure technique is being performed correctly

Need to be cleaned correctly to avoid infection

Requires significant effort, may not be suitable for patients who tire easily

For children:
May not be able to master technique especially if young
Potentially useless on children younger than 3 years

Sinusitis, epistaxis, ear infection may contraindicate use of PEP

Devices may need replacing ‘In patients with CF, PEP is recommended over conventional chest physiotherapy because it is approximately as effective as chest physiotherapy, and is inexpensive, safe, and can be self-administered’
McCool & Rosen 2006

Treatment promotes independence and is preferred by patients, thus can increase compliance Sustainability Weaknesses Able to be used independently by the patient after around age 10
Flume et al 2009

Just as effective as other chest physiotherapy techniques e.g. percussion, PD

Can be used in conjunction with other techniques e.g. PD, ACBT

Relatively inexpensive Strengths
McIlwaine PM et al. "Long-term comparative trial of conventional postural drainage and percussion versus positive expiratry pressure physiotherapt in the treatment of cystic fibrosis"J Pediatr 1997; 506-508
Pryor, J. A., Tannenbaum, E., Scott, S. F., Burgess, J., Cramer, D., Gyi, K., & Hodson, M. E. (2010). Beyond postural drainage and percussion: Airway clearance in people with cystic fibrosis. Journal of Cystic Fibrosis, 9(3), 187-192. doi: 10.1016/j.jcf.2010.01.004
Rogers, D., & Doull, I. J. M. (2005). Physiological principles of airway clearance techniques used in the physiotherapy management of cystic fibrosis. Current Paediatrics, 15(3), 233-238. doi: 10.1016/j.cupe.2005.02.007
Tyrrell JC et al. " Face mask physiotherapy in cystic fibrosis" Achieves of Disease in Childood (1986) 61, 598-611
T.R.Myers 2007. Poaitive Expiratory pressure and Osillatory Positive Expiratory Pressure Therapies. Respiratory Care. Vol.. 52(10)
West, K., Wallen, M., & Follett, J. (2010). Acapella vs. PEP mask therapy: A randomised trial in children with cystic fibrosis during respiratory exacerbation. Physiotherapy Theory and Practice, 26(3), 143-143. doi: 10.3109/09593980903015268 References Strengths and Weaknesses Theoretical Assumptions & Outcomes Clinical Safety Financial Practicality Research, research and more research
Higher quality studies
Look into survival, exercise tolerance, cost

Latest advancements in airway clearance devices

Gene therapy Improvements
Alexandra Hough, Physiotherapy in Respiratory Care 3rd edition, Nelson Thornes Ltd 
Cystic Fibrosis Australia. Available at: http://www.cysticfibrosis.org.au/. On 23/08/2012.
Elkins MR, Jones A, van der Shans C. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. In: The Cochrane Library, Issue 2. Chichester:Wiley; 2006.
Falk M et al. “Improving the ketchup bottle method with positive expiratory pressure, PEP, in cystic fibrosis.” Eur J Respir Dis (1984) 65, 423-432
Flume, P. A., Robinson, K. A., O'Sullivan, B. P., Finder, J. D., Vender, R. L., Willey-Courand, D.-B., . . . Clinical Practice Guidelines for Pulmonary Therapies, C. (2009). Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respiratory care, 54(4), 522.
Marks, J. H. (2007). Airway clearance devices in cystic fibrosis. Paediatric Respiratory Reviews, 8(1), 17-23. doi: 10.1016/j.prrv.2007.02.003
McCool, F. D., & Rosen, M. J. (2006). Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest, 129(1 Suppl), 250S-259S. doi: 10.1378/chest.129.1_suppl.250S References How PEP is being implemented in the real world http://www.gofightingfish.com/meet-the-fishes.html
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