Assessing and Managing Lung Disease and Sleep Disordered Breathing in Children with Cerebral Palsy Paediatric Respiratory Review

Paediatric Respiratory Reviews 10 (2009) 18–24

Contents lists available at ScienceDirect

Paediatric Respiratory Reviews

CME Article Assessing and managing lung disease and sleep disordered breathing in children with cerebral palsy

Dominic A. Fitzgerald 1,3,*, Jennifer Follett 2, Peter P. Van Asperen 1,3

1 Department of Respiratory Medicine, University of Sydney, Sydney, New South Wales, Australia 2 Department of Physiotherapy, The Children’s Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia 3 The Children’s Hospital at Westmead Clinical School, Discipline of Paediatrics & Child Health, University of Sydney, Sydney, New South Wales, Australia

EDUCATIONAL AIMS

To appreciate the insidious evolution of suppurative lung disease in children with cerebral palsy (CP). To be familiar with the management of excessive oral secretions in children with CP. To understand the range of sleep problems that are more commonly seen in children with CP. To gain an understanding of the use of non-invasive respiratory support for the management of airway clearance and sleep disordered breathing in children with CP.

ARTICLE INFO SUMMARY

Keywords: The major morbidity and mortality associated with cerebral palsy (CP) relates to respiratory compromise. Cerebral palsy This manifests through repeated pulmonary aspiration, airway colonization with pathogenic bacteria, Pulmonary aspiration the evolution of bronchiectasis and sleep disordered breathing. An accurate assessment involving a Suppurative lung disease multidisciplinary approach and relatively simple interventions for these conditions can lead to Physiotherapy signiﬁcant improvements in the quality of life of children with CP as well as their parents and carers. This Airway clearance techniques Obstructive sleep apnoea review highlights the more common problems and potential therapies with regard to suppurative lung Sleep disordered breathing disease and sleep disordered breathing in children with CP. ß 2008 Elsevier Ltd All rights reserved.

INTRODUCTION LUNG DISEASE IN CHILDREN WITH CEREBRAL PALSY

Cerebral palsy (CP) is a condition commonly seen in paediatric Lung disease is an important cause of morbidity and mortality practice. It is deﬁned as a non-progressive disorder of motor in children with CP. Reddihough et al2 published an audit of child function and occurs in approximately 1 in 500 live births. European deaths from the CP Register in Victoria, Australia. The majority of ﬁgures suggest it is associated with comorbidities of severe the 155 children who died between 1970 and 1995 had severe developmental delay/mental retardation in 31%, seizures in 21%, spastic quadriplegia, intellectual disability and epilepsy. Infection severe developmental delay and non-ambulant status in 20% and was felt to be the cause of death in almost half of the children with blindness in 11%.1 Despite the fact that respiratory and sleep pneumonia (including aspiration) in 61 (39.4%) and sepsis in a problems are common in children with CP, there is comparatively further 7 (4.5%). In two publications, Baikie et al3,4 studied 63 little in the literature to guide management of the everyday children with non-ambulant spastic quadriplegic CP to document problems of cough, pulmonary aspiration and sleep disruption. the prevalence of and the best method to detect pulmonary aspiration (Fig. 1). Respiratory symptoms were common in these patients with daily cough or wheeze in 58%, cough with feeding at least 1 day per week in 84%, ‘asthma’ episodes in the last 6 months * Corresponding author. Department of Respiratory Medicine, The Children’s in 34% and snoring in 44% with documented apnoeas in 10%.3 Focal Hospital at Westmead, Locked Bag 4001, Westmead, NSW, Australia, 2145. Tel.: +61 2 9845 3397; Fax: +61 2 9845 3396.. respiratory signs were present in 24% with crackles in 19% and E-mail address: [email protected] (D.A. Fitzgerald). wheeze in 17%, while cough during a milk feed occurred in 44%.3

Figure 2. CT scan demonstrating early bronchiectasis in a 6-year-old child.

Figure 1. Chest radiograph of a preterm infant with severe developmental delay, subglottic stenosis and tracheostomy, chronic neonatal lung disease and recurrent pulmonary aspiration at 6 months corrected age. contribute to impaired mucociliary clearance in children with CP, including recurrent pulmonary aspiration, poor nutrition, kyphoscoliosis, motor weakness, upper and lower airway obstruction, These studies serve to highlight the importance of recognizing recurrent infection and bronchiectasis. In turn, impaired muco- potential contributors to lung disease development in children ciliary clearance may lead to the development of airway with CP and ensuring they are addressed in the management of obstruction and atelectasis, recurrent infection, bronchiectasis, these children. ventilation/perfusion inequality, reduced lung compliance and eventually, respiratory failure in these children. Kyphoscoliosis is Potential contributors to lung disease development also common in children with severe CP and occurred in 39% of children in Baikie’s cohort.3 This in turn may contribute to GOR and A number of interrelated factors can potentially contribute to pulmonary aspiration, impaired mucocilary clearance, structural the development of lung disease in children with CP (Table 1). lower airway obstruction and the development of respiratory Lower motor ability, a history of ‘asthma’ or cough and wheeze and failure. In a recent evaluation of 35 patients with severe CP, the the presence of gastro-oesophageal reflux (GOR) have been degree of spinal deformity, neck rotation deformity and severe demonstrated to predict radiological aspiration, and lower asymmetrical deformity posture, and the severity of motor adaptive ability, abnormal voice quality after feeds, lower scoliosis dysfunction were all found to predict respiratory dysfunction.5 angle and history of ‘asthma’, regurgitation or ‘aspiration’ to As highlighted previously, snoring is common in children with predict GOR in children with severe CP.3,4 Baikie et al4 also CP3 and upper airway obstruction can also occur at the supraglottic investigated the use of various tests for aspiration in their cohort of level with redundant aryepiglottic folds causing a clinical picture 63 children and found that the salivagram was most frequently of laryngomalacia. In addition, ‘asthma’ is also commonly positive (56%; CI 43–68%) with barium videofluoroscopy the next diagnosed in these children,3 but is more likely to be due to some most frequently positive test (39% CI 26–53%), while radionuclide of the other contributors to lung disease development, including scan was rarely positive (6%, CI 2–16%). However, there was poor pulmonary aspiration, bronchiectasis, impaired mucociliary clear- agreement between the tests with the best agreement being ance, kyphoscoliosis and structural upper and lower airway between the salivagram and barium videofluoroscopy. The authors obstruction. It is therefore important to be aware of the potential concluded that the salivagram was probably the most useful test at for over-treatment with asthma medications in children with CP. predicting who is at risk of aspiration in this population. An audit of 294 children with CP presenting to the dysphagia clinic at the MANAGEMENT OF LUNG DISEASE IN CHILDREN WITH CEREBRAL Children’s Hospital at Westmead demonstrated evidence of PALSY pulmonary aspiration in 72 (41%) of the 174 videofluoroscopy studies performed, while 42 (67%) of the 62 CT scans performed The management of lung disease in children with CP is demonstrated bronchiectasis (Fig. 2), again highlighting pulmon- summarized in Table 2. While thickened feeds and antireflux ary aspiration as an important cause of lung disease in children therapy should be trialled initially, many children, particularly with CP (unpublished data). A number of factors are likely to those with severe CP, will require gastrostomy feeds combined with fundoplication if there is significant GOR. However, continu- ing aspiration of saliva may be an ongoing problem. Antic- Table 1 holinergic therapy in the form of both benztropine6 and Potential contributors to lung disease development glycopyrrolate7,8 has been documented in open label studies as Pulmonary aspiration being effective in patients with CP and is generally favoured as Impaired mucociliary clearance first-line therapy.9,10 Glycopyrrolate has the theoretical advantage Recurrent infection leading to bronchiectasis of having fewer side effects.10 Botulinum toxin injections into the Kyphoscoliosis Upper airway obstruction salivary glands has been used as alternative treatment for the Lower airway obstruction – ‘asthma’ management of sialorrhea9 but trials have been limited.10,11 Surgery remains an option for patients with persistent problems 20 D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 18–24

Table 2 Chest physiotherapy Management of lung disease in children with cerebral palsy Chest physiotherapy is now referred to by the more generic Pulmonary aspiration term of ‘airways clearance techniques’ (ACTs).12 There is a dearth Thickened feeds and antireflux treatment of evidence as to the most effective ACT for children with CP. The Gastrostomy and fundoplication Control of saliva degree of disability will influence the most appropriate ACT so, as Anticholinergics with all treatment, it should be carefully individualized and Botox injection reviewed regularly as the child grows. Due to the limited evidence Surgery for chest physiotherapy in CP children and adolescents, the Impaired mucociliary clearance evidence discussed is mostly from research done in children and Inhalations À normal/hypertonic saline or bronchodilators adolescents with cystic fibrosis who also can have difficulty with Physical therapies to improve clearance excessive secretions and impaired mucociliary transport. Infection Until recently the ‘gold standard’ ACT for all respiratory problems Antibiotics requiring physiotherapy was considered to be postural drainage Treatment vs prophylaxis (PD) with percussion and vibrations. These PD positions were Guided by sputum results 13 Prolonged courses required reported in Thorax in 1950. There are 12 positions that place the Immunization À influenza, pneumococcus patient so that gravity can assist in draining the mucus from the lungs. There is now increasing evidence that challenges the safety of Scoliosis À benefit vs risk of surgery Upper airway obstruction À surgery vs CPAP the traditional head down PD positions. GOR is well recognized as a common complication in children with CP, affecting their nutrition Lower airway obstruction and growth and perhaps their gross motor development.14 A Trial of asthma treatment (cease if no response) CPAP/BiPAP number of studies in cystic fibrosis have demonstrated an increased incidence of GOR when infants and children are tipped head down.15–17 One long term study also showed that infants with cystic fibrosis in whom no head down tilt was performed with their PD had despite trials of medical therapy.9,10 Children with impaired better lung function and less chest X-ray change after 5 years than mucociliary clearance will benefit from physical therapies to assist those who where tipped head down.18 Other documented secretion removal (see below) and this may be combined with a complications of head down tip include increased dyspnoea19 and nebulized inhalation. oxyhaemoglobin desaturation.20 There should be a low threshold for antibiotic use for Severely disabled children with CP can also have marked intercurrent respiratory infections in children with CP, particularly skeletal deformity and corresponding muscle dysfunction and in those with evidence of suppurative lung disease. Ideally, weakness. Effectively, they rely heavily on the use of the antibiotic choice should be based on sputum culture results and diaphragm for respiration which, in the head down tipped prolonged courses (3–4 weeks) are usually required in these position, is at a mechanical disadvantage. Consequently, most patients. Immunization to protect against influenza and pneumo- chest physiotherapy treatments for CP children are now performed coccus should also be administered. in modified PD positions.15 These positions are: While kyphoscoliosis may be a significant contributing factor to lung disease development in these children, the potential benefits of Supine 308 head up; surgery need to be carefully weighed up against the potential risks, Prone horizontal; particularly in children with established lung disease. As highlighted Left and right horizontal side lying; previously, ‘asthma’ is commonly diagnosed in these children but is Upright sitting for apical segment of upper lobe (this is done usually related to other causes so any use of asthma medication leaning against the therapist’s/carer’s shoulder or sitting should be seen as a trial and not continued or escalated unless there upright). is a convincing response. Finally, children with significant structural lower airway obstruction or those in respiratory failure may benefit Currently the limited available evidence18 and our experience from respiratory support in the form of non-invasive ventilation. has shown no adverse effects when modified PD is used. Percussion and vibrations are used as an adjunct to modified PD, not Physiotherapy interventions increasing the amount of secretions produced21,22 but decreasing the amount of time needed to remove secretions.21 Percussion Impaired mucociliary clearance may lead to the development of produces an energy wave that is transmitted through the chest recurrent chest infections, bronchiectasis and eventually, respira- wall to the airways and aims to mobilize sputum. It is a rhythmical tory failure. Therefore, models of care are necessary to improve technique performed using a cupped hand with flexion and mucociliary clearance and hence optimize the respiratory status of extension of the wrist with a frequency of approximately 3–6 Hz.23 children with CP. Vibrations are the shaking of the chest wall during expiration. Physiotherapy intervention is part of a 24-h care plan, designed Vibrations increase the expiratory flow rate more than percussion to complement but not overwhelm what already can be a or oscillation so should increase mucous transport.24 Once considerable burden of care. If the child can cough effectively secretions are loosened, then a cough is necessary to clear and adequately clear their secretions, they may only need to be secretions from the central airway to be expectorated or moved positioned well and repositioned throughout the day. Good to the back of the throat where they can be swallowed. positioning can be in an upright standing frame, a well supported well fitting seating system in a wheelchair or in bed with the head Practicalities of conventional therapies raised slightly. Slumped sitting should be avoided, especially at The ACTs can produce a cough spontaneously and if the mealtimes, as it promotes GOR and increases respiratory effort. secretions are not overwhelming then they can be swallowed However, if the child cannot cough effectively or clear their without adverse effect. If, however, they are excessive or the child secretions adequately, or develops an acute lower respiratory tract is having difficulty clearing the secretions, then oropharyngeal (O/ infection they may need more active chest physiotherapy. P) and nasopharyngeal (N/P) suction may be necessary. D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 18–24 21

In children the suction catheter is only passed as far as the Upper airway obstruction: glossoptosis, poor pharyngeal muscle pharynx to stimulate a cough; then the secretions are produced tone, adenoid and tonsillar enlargement; and suctioned out. Suction is a traumatic experience and there are Severe visual impairment: altered timing and maintenance of necessary precautions that need to be followed. Hands should be sleep; washed and gloved. There is a risk of vomiting so the child should Brainstem dysfunction: cardiac and respiratory control may be be positioned on their side and routine suction following chest altered as well as level of alertness; physiotherapy should be performed when the child has an empty Postural limitations and discomfort: muscle spasms resulting stomach or at least 1 h after eating. Swaddling of the child may be from dislocated hips, scoliosis and joint contractures together necessary to prevent unnecessary struggling and to decrease with an inability to alter posture in response to discomfort; trauma to the mouth or nose. The suction pressure (vacuum Epilepsy treatment with anticonvulsants may have excessive pressure) should be sufficient to draw the secretions up the somnolence as a side effect which may disrupt normal catheter but no greater than 10–20 kPa (75–150 mmHg). Suction sleepÀwake patterns; pressure should only be applied on extraction of the catheter. The Pulmonary aspiration: micro-aspiration and cough will disrupt catheter should not be too large, generally an 8 or 10 French sleep architecture. gauge.25 Suction can cause adverse effects which include mucosal trauma, hypoxia, tachycardia or bradycardia and bronchospasm so Upper airway obstruction close monitoring of the child is required.26 An external tracheal stimulation may also be used to stimulate This is an underappreciated issue in sleep in many children with a cough. This is done by applying manual pressure to the trachea in CP and may not be considered until the question of obstructive sleep the sternal notch below the cricoid cartilage. This should be done apnoea (OSA) is raised because of a lengthy history of snoring. A with care and used judiciously as there are currently no guidelines, recent retrospective small case series described eight children with consensus or research on this technique. severe spastic quadriplegic CP presenting over an 8-month period to a large paediatric teaching hospital with upper airway obstruction. Alternative therapies Seven required admission to the intensive care unit for a median stay Due to the time consuming and physically demanding nature of of 8 days.31 Seven underwent awake nasendoscopy which demon- the modified PD with percussion and vibrations, many more ACTs stratedenlargedadenoidsintwopatients.However,intheremaining have been developed that allow independence of the child and less six patients, nostructuralabnormalitywas foundatendoscopy ofthe physical involvement of the carer. A group of therapists in Sweden supraglottic regions, but collapse of the pharyngeal/supraglottic have performed research on the use of positive expiratory pressure structures was noted.31 Only two of the eight subjects had been (PEP) on a small group of severely disabled CP children. They found referredforsleepstudies.Twoofthepatientshadtracheostomiesand that the transcutaneous oxygen tension improved after PEP in the one had an adenotonsillectomy and continued on nocturnal CPAP. short term but there was no change in the respiratory rate. The The eighth patient, with a 7-month history of upper airway children accepted the treatment without any problems. The long obstruction, was shown to have a diffuse malignant brainstem term effect or amount of secretions produced were not men- glioma, was managed palliatively and died 6 months later. tioned.27 It has also been suggested that the low flow Acapella The interrelationship between awake upper airway obstruction (oscillating PEP) may be an alternate ACT, however there is no and OSA is not clear-cut. It is possible to have neither, one without research in this area. The high frequency chest wall oscillation vest the other or indeed both. For those children with stridor and has been effective in patients with cystic fibrosis in the US, helping obstructive symptoms when awake, but not in sleep, the clear their secretions,28 but due to its high cost and limited symptomatology has been attributed to laryngeal dystonia, and availability it has not been widely used worldwide. reported in a small cohort of children with dystonic CP32 or severe In conclusion, not all children with CP need daily chest laryngomalacia.31 The laryngomalacia seen in older children with physiotherapy. If they have difficulty clearing excessive secretions CP reflects the reduced tone of the supraglottic structures, which of the lower respiratory tract then chest physiotherapy is on forceful inspiration collapse into the airway, reducing the indicated, but the need should be reassessed regularly. There calibre of the airway, compromising airflow and resulting in are limited guidelines to influence clinical practice.29 The fact must stridor. The concurrent presence of a pseudobulbar palsy may be recognized that ACTs impose an extra demand on carers who serve to predispose to this tendency.31,32 already have a significant workload in caring for a disabled child. This suggests that the clinician should consider both waking and sleeping upper airway obstruction in children with CP, be mindful of changes in symptomatology related to the upper airway SLEEP BREATHING PROBLEMS and consider the morbidity that may follow from an under- appreciation of upper airway obstruction and its management. Children with CP have a higher prevalence of sleep breathing problems than otherwise healthy children reported in community Altered sleep cycles based surveys.30 The disruption of normal sleep patterns may have an enormous impact upon the interactive and cognitive skills of the The establishment and maintenance of normal sleep patterns in child with CP, adversely affect their quality of life and interfere young children with CP is often challenging. In addition to the with the normal sleeping patterns of siblings and parents. Indeed, negative impact upon the child with CP, altered sleep cycles are sleep problems in children with CP are under-reported, under- frustrating for the parents and siblings as they are often disruptive recognized and under-treated. to their sleep requirements. Sleep may be affected by a seizure Disturbed sleep patterns may involve fragmented sleep, disorder and its treatment, altered perception of light with visual increased nocturnal awakenings and even inversion of the impairment as well as pain and physical discomfort. sleepÀwake cycle.30 A range of factors predispose children with CP to sleep disordered breathing. Children with CP may be Insomnia vulnerable to sleep problems for a variety of reasons which may The prevalence of difficulties in settling off to sleep in the include, but are by no means limited to: general paediatric population is generally high and is higher again 22 D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 18–24 in special needs populations, including children with CP.33,34 apparent in the supine position during sleep and therefore a full Figures quoted relate to difficulty settling and frequent night history of sleep breathing concerns should include consideration of awakenings in 40% of infants; delayed sleep onset and disruptive cough, its aetiology, GOR and oral secretion management. night awakenings in up to 50% of preschoolers and bed-time resistance in up to 27% of school-aged children.34 These events may MANAGEMENT OF SLEEP PROBLEMS IN CHILDREN WITH be disruptive for the child whilst tiring and distressing for the CEREBRAL PALSY family.34,35 Poor sleep quality will adversely impact upon behaviour, mood, attention, cognition, memory and school After considering the history of sleep breathing concerns and performance.35 In children with CP, and indeed with many other completing a physical examination of the chest and upper airway, neurocognitive abnormalities, the mechanisms of sleep disruption it is likely that the possible problems will fall into either are not well understood. obstructive breathing patterns or altered sleep cycles which have resulted in fragmented sleep. The presence of snoring on the Seizure disorders majority of nights, with or without a clear history of witnessed Sleep patterns may be adversely affected by the existence and apnoea, would favour the possibility of OSA. Conversely, the treatment of a coexisting seizure disorder.33,36 The interaction presence of predominantly lengthy periods of wakefulness and between epilepsy and sleep disorders is not well understood, but some daytime sleepiness may favour a problem with altered sleep the most frequently reported problems are those of sleep cycles. Certainly, both problems can occur concurrently but the fragmentation with reduced sleep efficiency and more frequent approach to their management differs. arousals from sleep.37 Conversely, poor sleep quality may also increase seizure frequency.38 Possible contributory factors include Management of obstructive sleep apnoea associated psychological and mood disturbance and an alteration in sleep architecture that may persist after seizures.39 For children who snore, it would be reasonable to consider a A child with frequent seizures may have post-ictal phases with lateral airways radiograph to assess the size of adenoidal tissue somnolence as the initial symptom. Alternatively, the treatment of in relation to the airway. If there was a lengthy history of a difficult seizure disorder, often with a number of anticonvulsants, witnessed apnoea, it would be appropriate to obtain a venous may also result in somnolence as a side effect of the medications.40 blood gas or serum bicarbonate as a quick screen for carbon A range of antiepileptic medications have been shown to alter dioxide retention. The definitive test is a polysomnogram to sleep architecture.41 The alteration and more typically reduction in characterize the presence and severity of sleep disordered an anticonvulsant regimen may improve sleep-wake cycling and breathing. This will also demonstrate the presence of excessive reduce somnolence. This frequently will reduce sleep related central apnoea (Æoxygen desaturation), a more common finding in hypoventilation and perhaps concurrent OSA with a resulting children with central neurological abnormalities. Where poly- improvement in gas exchange. somnography is not available, the clinician can rely on a combination of clinical judgement, the lateral airways radiograph, Altered light perception a measure of serum bicarbonate (if not a blood gas) and perhaps The presence of blindness or greatly reduced vision may impair oximetry during sleep. the benefits of natural light and its facilitation of wakefulness The therapeutic response to the presence of significant during the daytime. A reduction in the perception of natural light obstructive apnoea (an obstructive apnoea index > 5 episodes/h will disrupt the natural secretion of the sleep-inducing hormone with oxygen desaturation of > 3%) would be adenoidectomy and melatonin, released from the pineal gland. The secretion of tonsillectomy. In more severe cases, or in those children deemed to melatonin is inhibited by light and enhanced during darkness.42 have too great an operative risk, a period of non-invasive In healthy human controls, melatonin secretion will increase soon respiratory support with nasal CPAP during sleep will improve after dark, peak in the middle of the night and steadily decline gas exchange, reset altered chemoreceptors and provide a bridge to during the second half of the night.43 The consequences of reduced surgery to remove enlarged tonsils or adenoids. In children or absent light perception upon sleep can be dramatic. Children without enlarged tonsils or adenoids, and presumably low with CP and blindness may have a free running circadian rhythm or pharyngeal muscle tone in sleep, the use of CPAP in sleep should even an inverted sleep-wake cycle.44 overcome upper airway obstruction. Those with altered respiratory drive may benefit from a more complex form of non-invasive Pain and physical discomfort respiratory support with bi-level pressure devices which can be set The effect of physical discomfort and the impaired ability to at a guaranteed rate, generally of up to 30 breaths/min. Both adjust position in sleep may also impact adversely upon sleep devices can be established at the bedside with an approximation of quality. The children may present with increased tiredness, the pressures required to overcome snoring and minimize restless sleep, night time crying or agitation. Initial responses paradoxical respirations. Importantly, fine tuning of the pressures may be to offer the child sedation with antihistamines or and rate would take place during further polysomnography as a benzodiazepines, but such responses may overlook the underlying titration study. With growth over time and alterations in airway problem such as a dislocated hip or pressure sores on dependent dimensions, such as after surgery on adenoids or tonsils, the need areas. The physical examination should always consider these for ongoing respiratory support will need reassessment. possibilities. Interestingly, the sedative agents may also influence upper airway muscle tone, increasing snoring and problems with Management of altered sleep cycles the coordinated swallowing of oral secretions, which may further complicate the assessment. It is useful to begin with a sleep diary, filled in by the parents over a 10–14-day period which will demonstrate the sleeping Cough and pulmonary aspiration patterns, length of sleep and duration of overnight awakenings. This can serve as a useful baseline and can be repeated at This has been highlighted earlier with regard to the develop- subsequent intervals if needed to demonstrate progress. Once the ment of lung disease. Interestingly, the symptoms may be more sleep pattern is documented, the use of sleep settling routines D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 18–24 23 should be the first action. This is designed to make the 2. Reddihough DS, Baikie G, Walstab JE. Cerebral palsy in Victoria, Australia: Mortality and causes of death. J Paediatr Child Health 2001; 37: 183–186. environment, usually the child’s bedroom, more conducive to 3. Baikie G. Inhalation pneumonitis in children with severe cerebral palsy. MD 1999 sleep. A regular meal time, bathing routine and a lack of University of Melbourne, Melbourne, Victoria, Australia. stimulation from computers, television or DVDs in the pre-bed 4. Baikie G, South MJ, Reddihough DS et al. Agreement of aspiration tests using barium videofluroscopy, salivagram, and milk scan in children with cerebral palsy. Dev Med hours is often helpful. Typically, whilst helpful, these measures are Child Neurol 2005; 47: 86–93. insufficient to normalize the sleeping patterns of a child with a 5. Mori N, Kurosawa H, Yosida M et al. What factors are related to respiratory delayed sleep phase or an inverted sleep cycle. dysfunction in patients with severe cerebral palsy? Eur Respir J 2007; 30: 376s. Cognitive behavioural therapies have been shown to be 6. Camp-Bruno JA, Winsberg BG, Green-Parsons AR, Abrams JP. Efficacy of benztropine therapy for drooling. Dev Med Child Neurol 1989; 31: 309–319. 45 successful in children with insomnia. In a survey of American 7. Blasco PA, Stansbury JCK. Glycopyrrolate treatment of chronic drooling. Arch Pediatr community-based paediatricians, Owens and colleagues reported Adolesc Med 1996; 150: 932–935. that the prescription of sedatives and hypnotics is common for 8. Bachrach SJ, Walter RS, Trzcinski K. Use of glycopyrollate and other anticholinergic medications for sialorrhea in children with cerebral palsy. Clin Pediatr 1998; 37: 485– treating paediatric insomnia despite reservations regarding this 490. practice and the lack of specific clinical guidelines in this area.45 9. Blasco PA. Management of drooling: 10 years after the Consortium on Drooling, 1990. Over-the-counter sedating antihistamines are widely recom- Dev Med Child Neurol 2002; 44: 778–781. 10. Tscheng DZ. Sialorrhea - therapeutic drug options. Ann Pharmacother 2002; 36: mended followed by melatonin. 1785–1790. The use of melatonin has become common for the treatment of 11. Dogu O, Apaydin D, Sevim S, Talas DU, Aral M. Ultrasound-guided versus ‘‘blind’’ circadian rhythm disorders (especially delayed sleep phase intraparotid injections of botulinum toxin-A for the treatment of sialorrhea in patients 44 with Parkinson’s disease. Clin Neurol Neurosurg 2004; 106: 93–96. syndrome). Generally administered in doses of 3–6 mg as a 12. Prasad A. Physiotherapy in cystic fibrosis. J R Soc Med 2000; 93(Suppl. 38): 27–36. liquid or tablet 30 min before desired sleep onset at night, the 13. Society T. The nomenclature of bronchopulmonary anatomy. Thorax 1950; 5: 222– medication has become popular and has been considered 228. 46 14. Campanozzi A, Capano G, Miele E et al. Impact of malnutrition on gastrointestinal efficacious. The only reservation may relate to an inconsistent disorders and gross motor abilities in children with cerebral palsy. Brain Dev 2007; 29: 30,47 decrease in seizure control. The effect is to reduce the time to 25–29. sleep onset (typically by 30–60 min), increase total sleep time 15. Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Postural drainage and (typically by 45–60 min) and decrease the frequency of night gastro-oesophageal reflux in infants with cystic fibrosis. Arch Dis Child 1997; 76: 148– 150. awakenings. The medication has been used continuously in clinical 16. Vandenplas Y, Diericx A, Blecker U, Lanciers S, Deneyer M. Esophageal pH studies for 6 months with no suggestion of tachyphylaxis. monitoring data during chest physiotherapy. J Pediatr Gastroenterol Nutr 1991; Other therapies that have been used in children include 13: 23–26. 17. Button BM, Heine RG, Catto-Smith AG, Phelan PD. Postural drainage in cystic fibrosis: alternative medicines such as herbal preparations, chloral hydrate, is there a link with gastro-oesophageal reflux? J Paediatr Child Health 1998; 34: 330– benzodiazepines, barbiturates, phenothiazines and diphenhydra- 334. mine.45 It is presumed that these medications are also being used 18. Button BM, Heine RG, Catto-Smith AG et al. Chest physiotherapy in infants with cystic fibrosis: to tip or not? A five-year study. Pediatr Pulmonol 2003; 35: 208– in children with CP and neurocognitive deficits, again with little in 213. the way of evidence to guide practice. 19. Cecins N, Jenkins S, Pengelly J, Ryan G. The active cycle of breathing techniques - to tip or not to tip? Respir Med 1999; 93: 660–665. 20. McDonnell T, McNicholas WT, FitzGerald MX. Hypoxaemia during chest physiotherapy in patients with cystic fibrosis. Ir J Med Sci 1986; 155: 345–348. 21. Gallon A. The use of percussion. Physiotherapy 1992; 78: 85–89. PRACTICE POINTS 22. Pryor JA, Parker RA, Webber BA. A comparison of mechanical and manual percussion as adjuncts to postural drainage in the treatment of cystic fibrosis in adolescents and Lung disease is common particularly in children with adults. Physiotherapy 1981; 67: 140–141. severe CP. 23. van der Schans CP, Postma DS, Koeter GH, Rubin BK. Physiotherapy and bronchial mucus transport. Eur Respir J 1999; 13: 1477–1486. The aetiology is multifactorial, including pulmonary 24. McCarren B, Alison JA, Herbert R. Vibration and its effect on the respiratory system. aspiration, decreased mucociliary clearance, suppuration, Aust J Physiotherapy 2006; 52: 39–43. kyphoscoliosis and airway obstruction. 25. Parker A, Prassad A. In: Pryor J, Webber B, eds: Physiotherapy for Respiratory and Cardiac Problems 2nd edn. Edinburgh: Churchill Livingstone. Asthma is over-diagnosed and over-treated with wheeze 26. MacMillan C. Nasopharyngeal suction study reveals knowledge deficit. Nurs Times usually due to other aetiologies. 1995; 91: 28–30. CP is also commonly associated with sleep disordered 27. Lagerkvist A, Sten G, Westerberg B, Ericsson-Sagsjo A, Bjure J. Positive expiratory pressure (PEP) treatment in children with multiple severe disabilities. Acta Paediatr breathing that is under-recognized and under-treated. 2005; 94: 538–542. Management needs to address the relatively predictable 28. Hansen LG, Warwick WJ, Hansen KL. Mucus transport mechanisms in relation to the lung and sleep breathing problems. effect of high frequency chest compression (HFCC) on mucous clearance. Pediatr Pulmonol 1994; 17: 113–118. Recurrent suppurative lung disease is often amenable to 29. AARC Clinical Practice Guideline. Postural drainage therapy. Resp Care 1991; 36: simple and inexpensive therapies that will improve the 1418–1426. quality of life of the person with CP and their family and 30. Kotagal S, Gibbons VP. Sleep disorders in severe cerebral palsy. Dev Med Child Neurol carers. 1994; 36: 304–311. 31. Wilkinson DJ, Baikie G, Berkowitz RG, Reddihough DS. Awake upper airway obstruc- A range of airway clearance techniques are available for tion in children with spastic quadriplegic cerebral palsy. J Paediatr Child Health 2006; children with CP. 42: 44–48. Non-invasive respiratory support is useful for airway 32. Worley G, Witsell DL, Hulka GF. Laryngeal dystonia causing inspiratory stridor in children with cerebral palsy. Laryngoscope 2003; 113: 2192–2195. clearance and sleep disordered breathing. 33. Quine L. Sleep problems in children with mental handicap. J Ment Defic Res 1991; 35: Life expectancy for children with CP is improving due in 269–290. part to a more aggressive approach to the use of 34. Owens J, Palmermo T, Rosen C. Overview of current management of sleep disturbances in children: II Behavioural interventions. Curr Ther Res 2002; 63(Suppl B): respiratory therapies. B38–B52. 35. Wiggs L, Stores G. Behavioural treatment for sleep problems in children with severe learning disabilities and challenging daytime behaviour. J Child Psychol Psychiatry 1999; 40: 627–635. 36. Coppola G, Iervolino G, Mastrosimone M, La Torre G, Ruiu F, Pascotto A. Melatonin REFERENCES in wake-sleep disorders in children, adolescents and young adults with mental retardation with or without epilepsy: a double-blind, cross-over, placebo-controlled 1. Johnson A. Prevalence and characteristics of children with cerebral palsy in Europe. trial. Brain Dev 2004; 26: 373–376. Dev Med Child Neurol 2002; 44: 633–640. 37. Bazil CW. Epilepsy and sleep disturbance. Epilepsy Behav 2003; 4(Suppl 2): S39–45. 24 D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 18–24

38. Bazil CW. Sleep and epilepsy. Curr Opin Neurol 2000; 13: 171–175. 44. Jan MM. Melatonin for the treatment of handicapped children with severe sleep 39. De Haas S, Otte A, de Weerd A, van Erp G, Cohen A, van Gerven J. Exploratory disorders. Pediatr Neurol 2000; 23: 229–232. polysomnographic evaluation of pregabalin on sleep disturbance in patients with 45. Owens JA, Rosen CL, Mindell JA. Medication use in the treatment of pediatric epilepsy. J Clin Sleep Med 2007; 3: 473–478. insomnia: results of a survey of community-based pediatricians. Pediatrics 2003; 40. Kothare SV, Kalevius J. The adverse effects of anti-epileptic drugs in children. Expert 111: e628–e635. Opin Drug Saf 2007; 6: 251–265. 46. Wasdell MB, Jan JE, Bomben MM et al. A randomized, placebo-controlled trial of 41. Sammaritano M, Sherwin A. Effect of anticonvulsants on sleep. Neurology 2000; 54: controlled release melatonin in the treatment of delayed sleep phase syndrome and S16–S24. impaired sleep maintenance in children with neurodevelopmental disabilities. J Pineal 42. Fischer S, Smolnik R, Herms M, Born J, Fehm H. Melatonin acutely improves the Res 2008; 44: 57–64. neuroendocrine architecture of sleep in blind individuals. J Clin Endocrinol Metab 47. Braam W, Didden R, Smits M, Curfs L. Melatonin treatment in individuals with 2003; 88: 5315–5320. intellectual disability and chronic insomnia: a randomized placebo-controlled study. J 43. Brzezinski A. Melatonin in humans. N Engl J Med 1997; 336: 186–195. Intellect Disabil Res 2008; 52: 256–264.

CME SECTION c. Non-invasive respiratory support has a role in improving This article has been accredited for CME learning by the impaired mucociliary clearance. European Board for Accreditation in Pneumology (EBAP). You d. Cough in infants with cerebral palsy does not require can receive 1 CME credit by successfully answering these intervention. questions online. e. The use of nebulized hypertonic saline may be of benefit in children with suppurative lung disease complicating (A) Visit the journal CME site at http://www.prrjournal.com. cerebral palsy. (B) Complete the answers online, and receive your final score 3. Children with cerebral palsy have difficulties with insomnia. upon completion of the test. Aspects of their care to consider include: (C) Should you successfully complete the test, you may a. Environmental issues such as light perception. download your accreditation certificate (subject to an b. Pain from pressure areas or musculoskeletal problems administrative charge). such as a dislocated hip. c. Circadian rhythm disturbances. d. Short sleep requirements. Educational questions e. Disrupted melatonin secretion. Answer true or false. 4. Children with cerebral palsy have more frequent sleep problems than other children. Contributing factors to 1. Regarding lower respiratory tract infections complicating consider may include: cerebral palsy: a. Enlarged tonsils and adenoids in the presence of snoring a. The major infective cause of death in children with would raise the possibility of obstructive sleep apnoea. cerebral palsy is pneumonia. b. The concomitant use of multiple anticonvulsants for b. Recurrent pulmonary aspiration often presents as cough coexisting seizure disorders may contribute to excessive without wheeze but is often inappropriately labelled and daytime somnolence. treated as asthma. c. Severe visual impairment has minimal effect upon sleep– c. A salivagram is not a useful test for the assessment of wake cycles. pulmonary aspiration. d. Children with significantly impaired mobility may have d. Children with gastro-oesophageal reflux and recurrent disrupted sleep without a history of snoring. pulmonary aspiration should be considered for gastro- e. Repeated pulmonary aspiration may interrupt sleep stomy feeds and a fundoplication. patterns. e. A sputum sample is of no benefit in the assessment of 5. Chest physiotherapy is appropriate and is best undertaken: children with suppurative lung disease complicating a. When the child has an upper respiratory tract infection. cerebral palsy. b. When the child has excessive secretions that they are 2. Factors to consider in the management of children with having difficulty managing. impaired mucociliary clearance complicating cerebral palsy c. When the child has an acute lower respiratory tract include: infection. a. The presence of altered swallowing dynamics and drooling d. Routine chest physiotherapy should be undertaken at least of saliva. an hour after the child has eaten. b. The use of inhaled or oral anticholinergic therapy has no e. Most physiotherapy treatments for children with cerebral role in the management of excessive oral secretions. palsy use modified postural drainage.