Abstract
Pediatric physical therapy (physiotherapy) is an essential component in the management of many cardio respiratory presentations in infants and children of all ages. The input will vary considerably depending on assessment findings and underlying pathology; however, the general principles remain the same across the spectrum. This chapter covers the key areas of physical therapy for specific conditions such as cystic fibrosis and bronchiectasis regarding airway clearance techniques, exercise, inhalation therapies, ventilator support, and secondary complications. It also identifies the role of cough augmentation and noninvasive ventilation in the management of children with neuromuscular conditions and spinal cord injuries. The role of the physical therapist in the critical care environment is discussed, with strategies modified for the intubated child to assist in secretion clearance and promote early rehabilitation. The importance of postoperative input through mobilization for children that require intervention and adjuncts to support therapy is outlined. Conditions that cause airway or structural abnormalities and may respond effectively to physiotherapy management are discussed. The interventions, as always, are tailored to the individual and monitored for safety and efficacy. Conditions and clinical presentations usually not amenable to physiotherapy are also reviewed. The main focus of the chapter is to highlight the essential elements of pediatric physical therapy for respiratory disease, reviewing the growing evidence base behind the many interventions available in the therapist’s “toolbox.” Physiotherapy is not only a science but also an art. It requires the therapist to constantly review, modify, and adapt to a potentially changing presentation as well as the ability to communicate with the child and family for engagement and best outcomes.
Keywords
physiotherapy, respiratory therapy, airway clearance of secretions, neuromuscular disease, bronchiectasis, cough exercise
Pediatric cardiorespiratory physical therapy (physiotherapy) management spans the spectrum of care from specialist advice to nonpharmacologic interventions for patients with a variety of respiratory conditions. Physiotherapists are an essential part of the multidisciplinary team and physiotherapy is administered from birth to the time of transition to adult services across the continuum of care settings. To provide optimal care, the therapist will clearly identify the indicators for intervention and balance these against the possible risks. Physiotherapy is not a prescribed procedure; the frequency and dosage of therapy is continually adapted and modified in response to identified outcomes and targeted goals.
Treating children can be difficult, and the physiotherapist must be responsive to individual needs and have the technical and nontechnical knowledge, skills and attributes to meet these challenges.
General Principles of Physiotherapy
Physiotherapy often focuses on treating or alleviating generic problems that are amenable to intervention rather than being disease-specific ( Fig. 17.1 ). In some instances, however, interventions are selected based upon the underlying disease process (e.g., primary ciliary dyskinesia [PCD] versus cystic fibrosis [CF]), whereby the elements of the mucociliary escalator affected by the disease process may differ (predominantly ciliary dysfunction versus altered sputum rheology). The pediatric respiratory physiotherapist/therapist will perform a wide variety of roles. The professionals and their training vary internationally. In the United Kingdom, physiotherapists are able to treat patients without a referral from a medical doctor and are therefore independent practitioners. Prior to pediatric physiotherapy, informed consent from caregivers and age-appropriate assent from the child are obtained.
The respiratory physiotherapist/therapist needs physiologic knowledge and practical skills to perform a competent respiratory assessment of the child. From this assessment, problems responsive to physiotherapy are identified and treatment strategies are recommended and implemented. Physiotherapists may also assess the reaction to inhaled pharmacologic agents (e.g., bronchodilator response and nebulized antimicrobial bronchoconstriction trials; Chapter 16 ), provide education in inhaler and nebulizer techniques, and advise on the optimal timing of inhaled medications with respect to sessions of respiratory physiotherapy. They can also assess the need for home oxygen therapy by performing exercise testing with oximetry. Physiotherapists may also help to identify potential causes of respiratory problems (e.g., gastroesophageal reflux [GER] during airway clearance). These issues can then be escalated to be reevaluated by the medical team. If it is felt that pulmonary secretions are a result of aspiration secondary to uncoordinated swallowing, a speech and language assessment is warranted (see section “Aerodigestive Disease”).
The timing of physiotherapy treatments can be important; for example, airway clearance should be timed before feeds or delayed for a sufficient time after feeds to avoid vomiting and aspiration. Likewise, physiotherapy should be timed around analgesia when clinically necessary.
Role of Physiotherapy in Pediatric Respiratory Disease
Physical therapies are essential in the removal of excess bronchopulmonary secretions and maintaining and improving exercise capacity. Physical therapy can support ventilation using high-flow nasal cannulas (HFNCs), continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV). Physical therapy should include postural education where appropriate; it can be used to prevent, correct, or improve postural problems, such as kyphosis in CF patients ( Fig. 17.2 ). Postural education can also be helpful in musculoskeletal dysfunction, in children with contractures that inhibit function, or in children with pain that limits range of motion, mobility, and ability to breathe normally. Poor posture leads to tightening of the respiratory muscles, which can lead to chest wall deformity and contribute to a decline in pulmonary function. It is therefore essential that patients with chronic lung disease have a postural assessment and treatment of any musculoskeletal disorders identified, including core muscle imbalance.
Specifically in neuromuscular disease (NMD), physical therapy is essential to maintain ambulation or facilitate standing, where possible, to improve lung function. Optimizing the maturing musculoskeletal and neuromuscular systems of a child with CF may play an important role in the long-term outcome of the child’s mental and physical state. It is essential to ask children with chronic lung disease about urinary and fecal incontinence in a private and empathetic setting; reluctance to cough may stem from fear of incontinence. Physical therapies should be directed toward managing this problem.
Respiratory Physiotherapy in Specific Conditions
Cystic Fibrosis and Noncystic Fibrosis Bronchiectasis (Including Primary Ciliary Dyskinesia)
Airway Clearance
Treatment options for airway clearance depend on the child’s age and ability to participate in treatment. There is a wide variety of airway clearance techniques (ACTs; Figs. 17.3–17.5 ). There is no single best technique, so the therapist should not assume that the findings all apply to non-CF bronchiectasis.
The technique should be tailored to the individual, and choice is dependent on efficacy, simplicity of use, and cost. A good starting point is with the technique that is simplest to use and that impinges least on the patient’s life. The term airway clearance describes a number of different treatment modalities that aim to enhance the clearance of bronchopulmonary secretions. Through clinical reasoning, the therapist decides the aim of treatment and how to address the specific issue (or issues). Lannefors and colleagues clearly identified the following four stages of airway clearance; they are the cornerstones to decision making.
- 1.
To get air behind mucus so as to open up the airways
- 2.
To loosen/unstick the secretions from the small airways ( )
- 3.
To mobilize the secretions through the smaller airways to the larger airways
- 4.
To clear the secretions from the central airways
The age and adherence of the individual and caregivers as well as disease severity will affect the modalities introduced and in what combination. In the infant, manual techniques ( Figs. 17.6–17.8 ), positioning, infant positive expiratory pressure (PEP ; Fig. 17.9 ), and assisted autogenic drainage (AAD) are used. PEP and AAD focus on enhancing changes in air flow and ensuring the move from “passive” techniques to a more dynamic approach. The use of movement is encouraged from an early age, as it is not only more effective but also more realistic in the younger age group ( ).
As the child grows older and can become an active participant in therapy, the emphasis will change. The therapist can incorporate techniques that augment volume and introduce the concept of a change in expiratory flow; in many cases, this is a forced expiration. Forced expiration or “huffing” is integral to many techniques and utilizes the theory of the equal pressure point to move mucus to the larger airways. It is also a valuable assessment tool for children, as chest palpation during a “huff” can often be abnormal, with crackles being palpable, even when there are no abnormalities on auscultation. In young children, forced expiration will start as blowing games and then become a more formal component of ACT.
With the child’s increasing ability to participate, the active cycle of breathing techniques (ACBT ; Fig. 17.10 ) can be taught and may be used in postural drainage (PD) positions (see Figs. 17.3–17.5 ). Physiotherapy may consist of modified PD targeting the area of lung affected or rotating through different areas to ensure that the lung fields are clear. Other techniques such as autogenic drainage (AD; Fig. 17.11 ) also can be considered. In addition, many adjuncts are available, with PEP ( Figs. 17.12 and 17.13 ) or oscillatory PEP ( Figs. 17.14 and 17.15 ) commonly used to facilitate clearance and to help move the child toward independence if appropriate. PEP has been shown to reduce exacerbations significantly more than other ACTs. However, different physiotherapy techniques and devices may be more or less effective at varying times (e.g., stable state and during an exacerbation) and in different individuals. CF registry data from 2011 found that oscillating PEP and “huffing” were the most commonly used techniques in the United Kingdom and that PD and high-frequency chest wall oscillation (HFCWO; Fig. 17.16 ) were the least common. Several of these adjuncts can be used in combination with inhaled medications (e.g., hypertonic saline; Fig. 17.17 ) or in conjunction with exercise.
HFCWO (see Figs. 17.3–17.5 ) is widely used across North America. Although evidence indicates that it may be less effective than other therapies, including one study showing significantly greater pulmonary exacerbations in patients using HFCWO compared with PEP, it can still be considered for specific individuals or used in combination with other ACTs. One other device that may be of benefit is intrapulmonary percussive ventilation (IPV; Fig. 17.18 and 17.19 ). Previous studies have investigated sputum mobilization in CF patients by comparing the use of IPV to other modes of airway clearance (e.g., PD and percussion, HFCWO, and oscillatory PEP). These studies have shown IPV to be as effective as the other methods of airway clearance in sputum mobilization.
Exercise has many benefits for people with CF and bronchiectasis: improving cardiovascular fitness, bone mineral density, and quality of life ( Fig. 17.20 ). In CF it has been shown to slow the rate of pulmonary function decline and may increase survival independent of FEV 1 . Exercise has also been shown to have an additive effect on sputum production, and it improves oxygen saturation in adolescents and adults with CF when used before airway clearance. In fact, Dwyer and coworkers have shown that exercising on a treadmill increases expiratory air flow and moves sputum from peripheral lung regions, but this must be combined with “huffing” to be an effective method of airway clearance.
