Edited by: Francesca Santamaria, University of Naples Federico II, Italy
Reviewed by: Daniel Jerome Weiner, University of Pittsburgh School of Medicine, USA; Antonio Martinez-Gimeno, Complejo Hospitalario de Toledo, Spain
Specialty section: This article was submitted to Pediatric Pulmonology, a section of the journal Frontiers in Pediatrics
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Common symptoms of chronic suppurative lung disease or bronchiectasis in children and adolescents are chronic cough with sputum production, retention of excess secretions in dilated airways, and a history of recurrent infections. Clinical management includes the prescription of airway-clearance techniques (ACTs) to facilitate mucociliary clearance, optimize sputum expectoration, relieve symptoms, and improve well-being. A wide range of ACTs are available for selection, and these strategies may be applied in isolation or in combination. The choice of technique will depend in part on the age of the child, their clinical state, and factors which may influence treatment adherence. While the evidence base for ACTs in children and adolescent with these conditions is not robust, the current available evidence in addition to clinical expertise provides guidance for technique prescription and clinical effect. An overview of the most commonly applied ACTs, including their physiological rationale and discussion of factors influencing prescription in children and adolescents is outlined in this review.
Chronic suppurative lung disease (CSLD) is a clinical syndrome in children of respiratory symptoms and signs. Typical symptoms and signs include chronic, productive cough with purulent sputum greater than three episodes per year for longer than 4 weeks. This may be accompanied by exertional dyspnea and recurrent infections (
Airway-clearance techniques are widely prescribed in bronchiectasis (
For children and adolescents with CSLD or bronchiectasis, the principles of ACTs follow what has been described for the adult population and for children with CF. This is partially due to the challenges of ACT research, with the need for long-term studies with large patient numbers. Given the paucity of research in the bronchiectasis pediatric and adolescent population, clinical practice has evolved from existing evidence and clinical expertise. However, specific factors which may influence a technique’s effectiveness should be considered when prescribing ACTs for this age group.
Parents of children with bronchiectasis report higher levels of depression (
This review will provide suggestions for age-appropriate ACTs for children and adolescents with bronchiectasis or CSLD and will outline the most common clinical techniques, the available evidence, and practical considerations for prescription.
The choice of ACT should be tailored toward the child’s age. Their level of cooperation, maturity, and psychological adjustment to their condition is all important factors, as are the interactive skills of the physiotherapist and parents (
Technique | Age range | Advantages | Disadvantages |
---|---|---|---|
Modified GAD or GAD | All age ranges | Suitable for infants and small children who are not yet old enough to cooperate with more active techniques | Discomfort, time consuming, symptoms of gastro-esophageal reflux or breathlessness, specific contraindications or precautions |
Option for those unable to use or too fatigued to use independent techniques | |||
Percussion and vibrations | All age ranges | Suitable for infants and small children who are not yet old enough to cooperate with more active techniques | Passive, require assistant, discomfort, inconvenient, socially limiting |
Option for those unable to use or too fatigued to use independent techniques | |||
Assisted autogenic drainage | Infants | Minimal equipment required | Requires assistance, difficult technique to master and for carers to learn |
Bouncing on a fitball | Infant to toddler | Enjoyable for child | Equipment required |
Blowing games | Toddler to child | Enjoyable for child | |
Huffing | Toddler to adolescent | Enjoyable for child | |
ACBT (includes huffing) | Toddler to adolescent | Independent, flexible, requires no equipment, can be combined with other techniques | |
Bottle PEP | Toddler to adolescent | Independent technique, enjoyable for child, can be a bridge to other forms of PEP therapy, minimal cost | Need to follow instructions to avoid swallowing water |
Autogenic drainage | Toddler to adolescent | Independent technique, nil equipment required | Effect and feedback required to master the technique including sensitivity to auditory and vibratory cues of secretions |
PEP mask | Toddler to adolescent | Independent technique, can be combined with other ACTs, beneficial for those with unstable or compliant airways | Infant PEP requires assistance. Requires individual awareness of breath size |
For younger children who are afraid of a mask, this may not be the technique of choice | |||
Cost | |||
Mouthpiece PEP | Toddler to adolescent | Independent technique, can be combined with other ACTs, beneficial for those with unstable or compliant airways | No clear evidence on use of Mouthpiece PEP—either with or without nose clip |
Can be used in conjunction with hypertonic saline nebulizer (see below) | |||
Easy for younger children to use | |||
PariPEP™ with nebulizer | Toddler to adolescent | Independent technique, can be combined with other ACTs, beneficial for those with unstable or compliant airways | Cost |
Flutter® | Child to adolescent | Independent technique | Effective use dependent on angle, therefore, may be more suited to an older child (8 years). Cost |
Acapella® | Toddler to adolescent | Independent technique, can be combined with other ACTs, beneficial for those with unstable or compliant airways | Cost |
Not position dependent | |||
Aerobika™ | Toddler to adolescent | Independent technique, can be used in conjunction with nebulizer | Cost |
Physical exercise | Toddler to adolescent | Enjoyable for child | |
HFCWO | Toddler to adolescent | Independent technique | Heavy device, not easily portable |
Cost |
Gravity-assisted drainage involves placing the patient in specific (including semi-recumbent) positions which enables gravity to drain excess secretions from bronchopulmonary segments (
This technique is often combined with manual techniques, such as chest percussion or vibrations, with the technique application adjusted for the child’s age (
Despite the lack of focused research of these techniques in children and adolescents with bronchiectasis, GAD or more commonly ModGAD improves secretion clearance and, combined with manual techniques, is equally effective as other ACTs (
The ACBT consists of thoracic expansion exercises and the forced expiratory technique (FET) (
To encourage glottis opening, a peak-flow mouthpiece or similar piece of tubing may be used, providing audible feedback to children learning the technique (Figure
Only studies of adults with bronchiectasis undertaking ACBT (in recumbent positions) have been conducted, and when compared to other techniques, similar improvements in quality of life and lung function have been consistently noted (
Positive expiratory pressure therapy uses a one-way valve that allows unrestricted inspiration and a resistance to expiration. The theoretical rationale for PEP is that in the presence of small airway obstruction, PEP therapy promotes airflow past the obstruction or through the collateral channels. This allows an increased volume of air to accumulate behind secretions while the pressure gradient across the sputum plug forces secretions toward the larger airways (
A commonly applied PEP therapy system consists of a close-fitting mask and a one-way valve to which expiratory resistors are attached. Alternatively, a mouthpiece with holes of varying diameters (PariPEP™) to apply expiratory resistance or a TheraPEP® is used. A manometer determines the correct pressure generated during initial therapy instruction. For low pressure PEP therapy, the resistor giving a pressure level of 10–25 cmH2O during the middle of expiration is an ideal selection (
Most studies of PEP therapy in children and adolescents have been conducted in CF. Short-term PEP therapy is associated with similar sputum expectoration, lung function, and well-being as other ACTs, although PEP is a preferred technique (
Oscillating PEP therapy offers the combination of PEP with high-frequency oscillations which elicit shear forces within the airways during exhalation to facilitate secretion clearance. The oscillations are believed to induce vibrations within the airway wall to displace secretions and the repeated accelerations of expiratory airflow favor movement of secretions from the peripheral to the central airways (
The Flutter® is a small pipe-shaped hand-held device with a mouthpiece, a perforated cover which encases a stainless steel ball resting in a circular cone. Inhalation occurs through the nose or around the mouthpiece. During expiration, at a slightly faster rate than normal, the high-density ball rolls up and down the cone, creating interruptions in expiratory flow and generating a PEP within the range of 18–35 cmH2O (
The Acapella® uses a counterweighted plug and magnet to create airflow oscillations (
The RC-Cornet® consists of a mouthpiece, curved tube, a valve hose, and a sound damper, with expiration through the tube creating an increasing pressure within the hose until it opens, allowing air to flow through the device and creating a PEP and vibrations within the airways. Similar to an Acapella, the Aerobika™ includes a mouthpiece and one-way valve. Nebulizer therapy can also be incorporated into the circuit as well to deliver mucolytic therapy.
The majority of research of OscPEP in bronchiectasis has focused on the Flutter®, Acapella®, and RC-Cornet® in adults. When applied in the short term (single treatment session) or long term (4 weeks or 3 months), OscPEP improved sputum expectoration and HRQOL compared to no treatment (
Bottle or Bubble PEP is an alternative method to administer low pressure OscPEP therapy, particularly for children less than 4 years, who no longer tolerate infant PEP but are not yet able to progress to other forms of ACT. The resistance in this set up is a water column, with the expiratory pressure remaining constant once the tubing diameter is >5 mm (
Autogenic drainage is a technique which maximizes airflow to promote ventilation and secretion clearance. It employs the principles of breathing at different lung volumes to loosen and mobilize secretions (
In those with bronchiectasis, a single session of AD cleared more secretions compared to no treatment (
An alternative adaptation is assisted AD, which may be applied for infants and young children. Assisted AD is achieved by applying gentle manual pressure to the child’s chest wall by the physiotherapist/parent/caregivers’ hands to increase expiratory flow and achieve different lung volume breathing (
High-frequency chest wall oscillation applies external chest wall oscillations
Physical exercise, particularly endurance activity, is highly recommended for all ages in the management of bronchiectasis and CSLD (
Other options which may facilitate ACTs include mucoactive agents, delivered by nebulized inhalation therapy. Hypertonic saline is classed as an expectorant and may be inhaled before or during an ACT (
A key component of managing CSLD or bronchiectasis in children and adolescents involves ACTs and exercise and although the evidence base is lacking, clinical practice reflects their regular prescription and use. Technique choice will vary according to age and specific factors influencing patient adherence. Working closely with the child or adolescent and their family in a therapeutic alliance and providing ongoing education, engagement, and encouragement to assist with adherence to therapy is critical in maximizing the effectiveness of airway-clearance therapy in these populations.
AL drafted the manuscript, BB and E-LT provided critical input to the manuscript, and all the authors approved the final version.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.