1. Gadgets and Gizmos: Reviewing pulmonary therapies for optimizing airway clearance
Stacey Denver DNP, RN, FNP-BC
Pediatric Pulmonary Division
University of Texas Health Science Center
Director of Operations for Pediatric Ambulatory
University Health Systems

2. Disclosures
I have no financial or commercial disclosures

3. Objectives
Review the physiologic airway differences between children and adults
Review disease processes that benefit from chest physiotherapy
Options of chest physiotherapy reviewed in context of the hospital and home settings

4. Basic Respiratory Mechanics
Muscles of Respiration
Factors which affect airflow
Mucous production
Ciliary movement
Equal Pressure Point
Collateral Ventilation

5. Physiologic Differences Between Children and Adults
Airway mucous
Greater density of submucosal glands
In a normal adult, the gland constitutes 12% of the airway wall, in children the area is about 17%.
More acidic mucins

6. Physiologic Differences Between Children and Adults
Respiratory Mechanics
Greater airway wall compliance
This contributes to less airway support and increase collapsibility of the airway.
Lower functional residual capacity
Smaller airway diameter
Fewer collateral airway channels
The interalveolar pores of Kohn and bronchiolar-alveolar channels of Lambert

7. Pathophysiology of a Cough
The initial phase of cough is characterized by the inhalation of gas.
After inhaling a volume of air, the glottis is closed and the intrathoracic pressures begin to build.
Once the glottis is opened, the expiratory phase of cough ensues.
and the high intrathoracic pressures developed during the compressive phase of cough promote high expiratory flow rates.

8. What is Chest physiotherapy (CPT) or Airway clearance therapy (ACT)?
Postural drainage
Percussion
Chest-wall vibration
Coughing
Four components
Chest physiotherapy or airway clearance is a set of manuveurs to assist in getting secretions out of the airways to improve airflow in the lungs.
Postural drainage uses gravity to move secretions from smaller to larger airways where they then can be coughed out
Percussion loosens secretions from the bronchial walls through clapping or the use of high frequencies. The palm cup or electric percussor are examples of these
Chest wall vibration adds vibration to help loosen the bronchial secretions . Similar to percussion but with vibration, the device or hands do not loose contact with the patient.
Coughing: Active process to expel secretions from the airway. Remember the cough is the strongest component to airway maintenance.
Walsh, Hood & Merritt, 2011

9. Who benefits from airway clearance therapies and how do we decide??
Why we order ACT for a child may be related to a number of reasons.
We may think “it can’t hurt and maybe this will help”
Or “this is how you should treat pneumonia or prevent pneumonia”
But we need to be more thoughtful than this

10. A more thoughtful approach
What is the underlying condition of the patient?
Neuromuscular disease
Cystic Fibrosis
Bronchiectasis
Ciliary dyskinesia
What is the current diagnosis?
What is the current quality of their cough?
Condition of their lungs?
We will review underlying diseases that have demonstrated usefulness of CPT and others that have not shortly
Current diagnosis? What are we treating? What do we ‘hope’ to achieve with CPT?
Quality of their cough? How much is this patient able to expectorate their secretions?
Literature reviews are unfortunate in that there are a lack of sufficiently powered studies assessing the application of CPT to the majority of diagnosis so recommendations must be made on what we do know

11. Remember…
Primary objective of ACT or CPT is to reduce the obstructing secretions and possibly remove ineffective materials such as mediators of inflammation or oxidative agents from the bronchioles.
We need to ask ourselves ‘Could I do harm?’
Documented cases of rib fractures and neurologic injury from inappropriate use of therapies
Gastroesophageal reflux has been shown to worsen with the use of CPT
Be mindful that this is not a benign set of therapies. First ‘do no harm’
Schechter, 2007

12. Diseases benefiting from ACT
Cystic Fibrosis
Cystic Fibrosis is caused by an abnormality of the gene that encodes for the CF transmembrame conductance regulator. This leads to an imbalance in salt and water on the airway surfaces. The abnormal airway surface promote chronic inflammation and infection that progresses to lung damage in the form of bronchiectasis.
We know that the mucociliary clearance is impaired in patients with CF and so airway clearance devices have been used to help remove the sticky secretions from their airways to minimize scarring and inflammation.
Despite the lack of well powered studies due to multiple complexities, the Cystic Fibrosis Foundation Pulmonary Therapies Committee recommended in 2007 that airway clearance should be utilized in patients with CF with no type of airway clearance demonstrating to be superior to another.
Schechter, 2007

13. Diseases likely benefiting from ACT
Neuromuscular diseases ie: muscular dystrophy
Bronchiectasis of unknown origin
Cerebral Palsy
Atelectasis (with mechanical ventilation)
Neuromuscular diseases: I include patients with muscular dystrophy, metabolic muscle diseases such as carnitine deficiencies and other myopathies who are characterized by weakness of muscles either centrally, peripherally or both. This leads to a decrease in thoracic muscular support with normal lung recoil. As they age, scoliosis and other spinal deformities develop which further compromise the bronchial mechanics.
Bronchiectasis of unknown origin: Similar to CF but without the actual diagnosis (mutations and sweat test) for CF
Cerebral Palsy: Because of their combination of aspiration/scoliosis and impaired cough, these patients often develop pulmonary disease and pneumonias.
Atelectasis: Because of sedation and pain medications, patients on ventilators have a decrease in cough and movement. Atelectasis or collapsing segments of the lung results.
These diseases are placed in the ‘Likely to benefit from ACT’ because of their lack of well powered studies to direct guidelines and standard practices.
Schechter, 2007

14. Diseases with minimal benefit from ACT
Bronchopulmonary dysplasia
Bronchiolitis
Asthma (acute)
Preventative post op measures on ventilated child
Respiratory failure without atelectasis
We now reach a group of diagnosis in which oftentimes CPT is ordered thinking ‘it may be helpful’ but yet studies have not demonstrated efficacy
Bronchiolitis often times seen as RSV: Acute inflammation with increased mucus production. A 2007 Cochrane review demonstrated no improvement in oxygenation or illness severity with CPT and a muticenter randomized trial with over 450 infants showed no significant impact on time to recovery with the addition of CPT
Asthma: Here we see bronchoreactivity and inflammation: Again, studies have not demonstrated that CPT assists in clearing secretions.
Schechter, 2007

15. Airway clearance ‘toolbox’
So many options!
What to consider?
Age and size of child
Cooperation/maturity of child
Tolerability of device
Mental Status of child
Turn our attention to what options there are for the patient that would benefit from CPT.
Remember our primary objective is to reduce the obstructing secretions and to remove ineffective materials from the airways.
I will focus on the inpatient application of these devices and Stacey will end our presentation with outpatient options.

16. Manual CPT
Utilizes percussion or clapping on the chest wall with hand or cup Done over 1 area for at least 1 minute Must be aware of head down position which may induce GERD
Gold standard of CPT is manual therapy-
Done before all the devices were invented
Concept:
Utilizing percussion, vibration and postural drainage in a range of different positions.
Recommended to be done before meals or 1 hour after eating to minimize nausea and emesis.
On infants, the head down positioning is not utilized due to reflux concerns
Downside of this is that eventually the person doing the therapy will tire out and develop carpal tunnel

18. The High Frequency Chest Wall Oscillation
3 different companies- depends on which one your hospital contracts with
Most offer disposable vests and wrap options for the hospital
Cleanable cloth option for home use
The inflatable vest that attaches to hoses and to the generator.
The vest inflates and deflates at different herzes or frequencies. The pressure is attempting to manuvuer secretions from the lower airways to the upper airways for the patient to then cough it out
Different sizes of vest jackets. Measurement determined by the chest circumference at the nipple line.
Able to nebulized medications in during the vest treatment- typically Albuterol with or without hypertonic saline
Remember Inhaled antibiotics and inhaled steroids should not be nebulized in during a treatment as the patient will end up coughing out medicine you most likely want to stay in the lungs.
In Courage Vest system

19. The VEST 2 settings to program Hertz (8-18)- age dependent
Pressure (6-10)
Jacket or wrap option
Standard time ~ 30 min per session
Can nebulize medications
during treatment

20. Vest settings per age

21. Tricks for vest therapy
Before inflationing hold bottom of vest to prevent ride up
Make sure vest jacket fits properly. It should ride at top of hip and snug but not tight
Use washcloth and twist into donut shape and place over ports or G-tubes for protection
No food one hour before or one hour after treatment to prevent nausea/vomiting

22. Intrapulmonary Percussive Ventilation (IPV)
High frequency puffs open atelectatic alveoli delivering air behind the mucus plugs
Enhanced delivery of aerosolized medications
Provides CPAP support to the patients airways during treatments
IPV delivers high flow mini bursts of air into the lungs at rates of per minute at pressures of 5-35 ccmH2O. Mist of 1ml per minute is delivered during percussion
Runs off of a 50 PSI gas source
If the patient is awake:
1. Sit upright
2. Place the mouthpiece in and have them keep a tight seal while maintaining tight cheeks
Remember 1ml = 1 minute of treatment so if you plan on just using Albuterol or 3 ml you are telling the RT to do 3 min of therapy.
In our practice, we add additional ml of Normal saline to increase the treatment time to at least min.
3. Start with frequency or Hz of 300 and work down to Hz of 100 as tolerated
4. Patient will inhale and exhale through the percussions
May need to rest or take a break during the treatment.
If the patient is on a ventilator, the IPV will connect to the vent circuit

23. Tricks for IPV Can be used inline with vent or with mouthpiece or mask
Always use no less than 12 ml of liquid in system for effective treatment
Pucker up lips to make check muscles taunt and prevent check puffs
Never put inhaled antibiotics in the system
Adjust pressures to achieve an adequate pulse power to mobilize secretions

24. Positive expiratory pressure devices (PEP therapy)
TheraPEP®
AccuPEP®
EzPAP ®

25. PEP therapy
Uses a simple flow resistor device which the patient breathes out of 5-20 times
By exhaling through the device, a positive pressure (back pressure) is created in the airways helping small airways and aveoli open up
Vibrations create airflow that help to loosen secretions from the bronchial walls
You can nebulize medications through PEP therapy- minimum of 12 ml (similar concept to IPV)

26. Gadgets and Gizmos for Home
Currently, IPV is not approved for home use.
Vest therapy is approved for certain diagnoses, such as Cystic Fibrosis. However, no diagnosis triggers an automatic approval.

27. Positive Expiratory Pressure Devices
These devices are considered to allow more air to enter the peripheral airways via collateral channels, to allow pressure air to go behind the secretions, moving them toward the larger airways where they can be expelled.
TheraPEP® EzPAP® AccuPAP®

28. Positive Expiratory Pressure (PEP)
TheraPEP® application
Patient slowly inspires to vital capacity then holds his breath for 3 seconds. Then slowly exhales through the mouthpiece with a fixed resister creating an expiratory pressure resistance between cm H2O.
The breathing maneuver is repeated times, followed by a “huff” or forced exhalation.

29. PEP Vibration Systems Acapella® choice Device Flutter® Device
These systems combine positive expiratory pressure and cyclic oscillations of the airway
Acapella® choice Device Flutter® Device

30. PEP Vibration Systems Application
The patient is instructed to inhale deeply and hold his breath for 3 seconds.
Exhale slowly through the devise, which causes positive pressure. The vibrations created helps loosen secretions.
Routinely, three sets of 15 exhalations are performed over minutes.

31. PEP Vibration Systems Huff Coughing:
Take a breath that is slightly deeper than normal.
Use your stomach muscles to make a series of 3 rapid exhalations with the airway open, making a "ha, ha, ha" sound.
Follow this by controlled diaphragmatic breathing and a deep cough if you feel mucus moving.

32. Positive Expiratory Pressure (PEP)
Advantages
Independence to patients
Convenient and Easy to use
Can be used simultaneous with nebulizer
Disadvantages
Limited by age
Dependent of patient effort
Cleaning required after each use
Can be carried out without an assistant

33. Cough Assist Device How does it work?
The system supplies positive pressure (inhale) to inflate the lungs, then quickly shifts to supply negative pressure (exhale). After the exhale, the system pauses and maintains a resting positive pressure flow to the patient.
American Thoracic Society (ATS) clinical practice guidelines strongly recommend CoughAssist to prevent respiratory complications in patients with neuromuscular disease.

34. Diseases benefiting from a Cough Assist Device
Duchane’s Muscular Dystrophy
SMA
Cerebral palsy
Neuromuscular Diseases
Children with neurological devastation

35. Cough Assist Device
Prescription should include positive and negative pressures
Start at +20/-20 cm H2O and gradually increase to +40/-40 cm H2o
A series of 4-5 breaths with cough assist for 4-6 sequences

36. Factors to consider for Home Airway Clearance
Will insurance cover it?
Use the proper diagnostic
ICD 10 Codes
E84.9 Cystic Fibrosis
J47.9-Brochiectasis
G71.0- Muscular Dystrophy
G12.1-SMA
Airway clearance may be the most frequently omitted therapy outside of the hospital.
The greatest success of airway clearance of any type is adherence and compliance.
These are affected by the home environment, family dynamics, social situation and many other things that can contribute to adherence or nonadherence to therapy at home.
No diagnosis is a “slam dunk” for insurance approval of many of these devices.
Most insurances will require a prior auth and a letter of medical necessity.
There is a lack of high level scientific evidence in the literature. There are some meta analysis that demonstrate effectiveness in some populations, primarily cystic fibrosis and children with neuromuscular disease.

37. How do I get a device for my patient???
Prescription forms- usually located on the companies websites
Letters of Medical Necessity
Lots and lots and lots and lots of clinical documentation
Hospitalizations
Antibiotics use
Failure of lesser expensive options

38. Things to consider for Home Airway Clearance
Provide patient/family with a well and sick plan
Plans should include order of therapies
SABA, 3% or 7% saline with airway clearance, followed by inhaled antibiotic and/or inhaled corticosteroids.
Well plan is usually BID and sick plan is every 4-6 hours

39. Things to consider for Home Airway Clearance
Airway clearance on a full stomach may cause vomiting.
To prevent this from happening do it:
Before a meal
2 hours after meals
Bedtime

40. Considerations regarding airway clearance therapies in Infants and children
Airway clearance performed in a head-down position aggravates reflux.
The small infant may not have adequate musculoskeletal support to resist injury from CPT.
Behavioral issues
Consider CPT without a head-down tilt for infants and children with a tendency to reflux

41. Questions??

42. References
Schechter, M. (2007). Airway clearance applications in infants and children. Respiratory Care, 52(10),
Walsch, B. Hood, K. & Merritt, G. (2011). Pediatric airway maintenance and clearance in the acute care setting: how to stay out of trouble. Respiratory Care, 56(9),
Boundless. “The Mechanics of Human Breathing.” Boundless Biology. Boundless, 08 Jan Retrieved 30 Jan from
Bach JR. Mechanical insufflation/exsufflation: has it come of age? A commentary. Eur Respir J. 2003;21: