This presentation will be looking at respiratory physiotherapy for patients with cerebral palsy.

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Obviously with cerebral palsy being such an umbrella term covering a wide range of disability, the assessment and treatment of patients with CP will vary greatly and should be individualised, considering things such as mental capacity, age, symptoms and function.

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While CP itself does not directly cause lung dysfunction, the consequences of neuromuscular impairment may lead to lung damage and reduced lung function. Proper respiratory function depends on the ability to breath properly, cough effectively, and identify respiratory infections promptly. These patients are at risk of respiratory complications due to: Immobility as a result of muscle dyscoordination and weakness, frequently complicated by cognitive impairment and shallow, irregular breathing Ineffective cough as a result of dyscoordination of bulbar muscles and weakness of the muscles of the abdomen and diaphragm (stages of a cough) Chronic aspiration as a result of dysphagia, gastroesophageal reflux, and seizures Delay in diagnosis due to inability to disclose discomfort & unsuitable assessment techniques (for example peak cough flow) Restrictive lung disease components as a result of respiratory muscle weakness and structural deformity such as scoliosis They are often premature babies- so frequently associated respiratory problems eg. bronchopulmonary dysplasia and respiratory distress syndrome Weakened immune system All of this contributing towards a greater risk of developing respiratory complications.

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What are the consequences of these respiratory complications? Retained secretions physically obstruct airways, leading to: • Infectious exacerbations and bacterial colonization, resulting from stagnation of secretions • Immobilization of cilia • Ventilation/perfusion mismatch • Atelectasis & Airway obstruction There is also chemical damage to airways as pooled secretions contain high concentrations of cytotoxic inflammatory mediators (such as cytokines and leukotrines) which can cause: • Increased mucus production • Intensified inflammatory response • Oedema • Bronchospasm • Destruction of cilia • Progressive parenchymal damage • Irreversible fibrosis (Braverman 2001)

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The main indication for treatment will come from your assessment. This may include objective measures such as PCF, InspVC, MIPS/MEPS etc, as subjective assessment of cough is generally considered to be not accurate enough, however the parameters of poor or normal or effective are less clearly defined for children than adults (eg. PCF>160lpm to be effective in adults). There may also be problems with inability to comply with Ax such an ineffective seal or comprehension or coordination difficulties. The Mental Capacity Act should also be considered with regard to whether the patient has the capacity to make decisions about their Ax & Rx. The RCH CP chest infection guidelines state that: Chest physiotherapy is helpful if children have large airway secretions and a poor cough, or focal consolidation. And this should have been identified from your assessment. The primary focus of respiratory management for individuals with CP must be to prevent mucus stasis that will lead to atelectasis and infection. Aggressive attention to airway clearance is central to breaking the lethal cycle of recurrent infection and progressive pulmonary deterioration. (Braverman 2001)

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A very general table of some interventions that may be appropriate for a patient with CP, some of these may not fit easily into one category so these are not definitive and it is far from an exhaustive list. It does show that we’ve come a long way from the ketchup bottle method, so tipping it up, shaking it and squeezing it. (Otherwise known as postural drainage, percussion, vibs and cough assistance: Falk 1984) The British Thoracic Society guidelines do not cover patients with CP, and the evidence is extremely lacking in terms of respiratory treatments, so intervention must be clinically reasoned by the assessment findings and individual requirements of the patient. Intervention is aimed at optimising quality of life for the child foremost. The views of the parents must also be taken into account. (Seddon and Khan 2003) When selecting a technique, the severity of airway clearance impairment, patient comfort, and ease of performance of the available techniques should be considered. In order to achieve optimal outcomes, it is critical to identify at an individual level which form of treatment is most effective. (Braverman 2001)

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Reduced physical activity is a risk factor for pulmonary complications, and the impaired neuromotor control in these patients can inhibit the amount of mobility achievable. But depending on their level of function, there should be an appropriate way of getting some movement. Even if their only mobility is being passively rolled or hoist transferred, sometimes that can be enough to get some secretions moving. With the more mobile, younger patients, you can use games, make believe, blowing bubbles, musical instruments are great for some expiratory muscle strengthening… Anything can be made into play, so that they don’t even realise that it’s therapy. (Instruments: Charususin 2010) A study by Ersöz et al (2006) recommended that pulmonary rehabilitation -involving breathing exercises for proper expansion, regularity of respiration and strengthening of expiratory and postural muscles- be started as early as possible to prevent worsening chest mobility and hence respiratory function. Whilst most hydrotherapy evidence in CP is relating to its effects on gross motor function and spasticity, In a study by Hutzler et al (1998) into exercise and swimming for children with CP, the intervention group showed a considerable increase in VC at a mean rate of 65% above base-line values, while the control group improvement was only 23%. This was a greater increase than shown in a previous study by Rothman (1978), which showed a 31% increase in VC using breathing exercises alone.

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There are different reasons for altering the position of a patient, to reduce breathless, to improve ventilation/perfusion matching and also for postural drainage. Hussey (1992) describes how it is important to regularly change position to prevent the mucous pooling and atelectasis that occurs in the pos basal regions when supine. Lying prone has been shown by Mackenzie et al (1989) to increase TV and MV with a 25% increase in arterial oxygen tension in babies, this stabilises the anterior chest wall, limiting inward collapse of the ribcage, but patients should be monitored carefully due to the position being implicated in sudden infant death syndrome. SL can remove abdominal loading on diaphragm and being sat upright gives better diaphragm excursion. [V/Q] Young children (below about 10) ventilate their uppermost lung preferentially, due to an incomplete airway support system, and so to optimise gas exchange, the ‘good’ lung should be places uppermost. However, physiotherapists may wish to place the ‘bad’ lung up in order to drain secretions and to open up areas of atelectasis. This may cause oxygen saturation to drop unless an increased fraction of inspired oxygen is given, as shown by Davies et al (1985). There is now increasing evidence that challenges the safety of the traditional head down PD positions, due to gastroesophageal reflux, and the diaphragm being at a mechanical disadvantage causing dyspnoea and desaturation. Consequently the modified postural drainage positions are now more commonly used for this pt group. (Finder 2010)

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[Modified postural drainage positions] •Supine 30° head up •Prone horizontal •Left and right horizontal side lying •Upright sitting for apical segment of upper lobe (this is done leaning against the therapist's/carer's shoulder or sitting upright).

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Often used in CP, as it is passive and effective at mobilising secretions. Should be combined with the modified postural drainage positions to remove secretions faster than positioning alone, but still remains time consuming for the patient. (Gallon 1992) There is great variation in how percussion and chest wall vibrations can be delivered, and should be adapted based on the individual patient circumstances, for instance for small infants, a face mask may be used to tap on. Wollmer et al (1985) suggested that a slow rate of percussion is preferred, as firm or fast percussion can disturb airflow, causing local airway closure and timing to allow an infant to take a breath between each percussion reduces this risk. Whilst an article by Balachandran et al (2005) suggests percussion should be delivered at a rate of 3 per second, Blazey et al (1998) discusses how the rate of optimal percussion has not and possibly can not be established due to the differences in patient circumstances, recommending that it be considered for each individual patient alone. A study by Shannon and Robyn Stiger et al (2010) suggested that chest wall vibrations may vary in application but must be applied at the beginning of, or slightly before, expiration to generate sufficient expiratory flows to be effective.

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Aka. The Vest. It works by an air pressure generator rapidly inflating and deflating the vest, which compresses and releases the chest wall at a frequency and pressure designated by settings on the machine, up to 25 times per second. This process dislodges mucus from the bronchial walls, moving it along toward more central airways for eventual expectoration. It is commonly used in CF, however evidence for use in cerebral palsy is limited, a randomised control trial by Yuan et al (2010) showed excellent safety, tolerability, and better compliance with high-frequency chest wall oscillation compared to standard chest physiotherapy. Another small study (Plioplys et al 2002) demonstrated more frequent removal of secretions by suction, reduced incidence of pneumonia, and reduced number of hospitalizations for pneumonia. No special positioning or breathing techniques are required, and it is technique independent, as is Intrapulmonary Percussive Ventilation, however it doesn’t require an effective seal around a mouthpiece like IPV does, so therefore can be useful for patients with CP, however it could potentially be strange or uncomfortable to a patient without the capacity to comprehend the therapy.

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There are different devices available, bubble PEP, PEP by facemask, by mouthpiece, the Cornet, Acapella, Flutter… An evidence review by Finder (2010) stated that most devices which deliver oscillating PEP (positive airway pressure) are effort dependent and as such are generally ineffective in the very weak neuromuscular patient. However a study into PEP treatment in children with multiple severe disabilities showed that PEP increases blood oxygen tension immediately after treatment in severely disabled children with airway mucus accumulation. (Lagerkvist 2005)

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These techniques often require a level of comprehension and cooperation, glossopharyngeal breathing in particular is a difficult technique and requires intact bulbar function and coordination, and is rarely used in the presence of a trachy. The bird (IPPB) may be useful for individuals who are unable to take a sufficiently deep breath for an effective cough, however may require some ability to synchronize and tolerate inspiration with the machine. (Braverman 2001)

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It may be said that without an effective cough, secretion mobilization will not result in secretion clearance. When considering manual assisted cough it is important to consider the patient’s comprehension as it can be quite a forceful manoeuvre. Cough stimulation for expectoration may be something as simple as tickling, or more clinical using a suction catheter or tracheal stimulation (which is still somewhat controversial). The mechanical in-exsufflator augments both insp & exp and may be usesul if tolerated by the patient.

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Combining (using timing) of physio treatment and medications for optimal effect And a combination of techniques to unstick the secretions, move them up the respiratory tract, augment inspiration to achieve greater lung volumes and aid expiration for effective expectoration.

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Involving the consideration of all elements of patient needs such as spasticity, pressure sores, positioning, transfers, social situation etc. Health promotion- is not only encouraging exercise and stop smoking advice but also giving advise on ensuring that they have their regular vaccinations, are taking their medications as prescribed, wearing any splints and anything else that may be of pertinence to the individual patient and their holistic care needs. (Jones et al 2007)

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