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 Childrens Hospital at Westmead, University of Sydney, Sydney, New South Wales, Australia
3
The Childrens Hospital at Westmead Clinical School, Discipline of Paediatrics & Child Health, University of Sydney, Sydney, New South Wales, Australia
INTRODUCTION
Cerebral palsy (CP) is a condition commonly seen in paediatric
practice. It is dened as a non-progressive disorder of motor
function and occurs in approximately 1 in 500 live births. European
gures suggest it is associated with comorbidities of severe
developmental delay/mental retardation in 31%, seizures in 21%,
severe developmental delay and non-ambulant status in 20% and
blindness in 11%.
1
Despite the fact that respiratory and sleep
problems are common in children with CP, there is comparatively
little in the literature to guide management of the everyday
problems of cough, pulmonary aspiration and sleep disruption.
LUNG DISEASE IN CHILDREN WITH CEREBRAL PALSY
Lung disease is an important cause of morbidity and mortality
in children with CP. Reddihough et al
2
published an audit of child
deaths from the CP Register in Victoria, Australia. The majority of
the 155 children who died between 1970 and 1995 had severe
spastic quadriplegia, intellectual disability and epilepsy. Infection
was felt to be the cause of death in almost half of the children with
pneumonia (including aspiration) in 61 (39.4%) and sepsis in a
further 7 (4.5%). In two publications, Baikie et al
3,4
studied 63
children with non-ambulant spastic quadriplegic CP to document
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
in 34% and snoring in 44% with documented apnoeas in 10%.
3
Focal
respiratory signs were present in 24% with crackles in 19% and
wheeze in 17%, while cough during a milk feed occurred in 44%.
3
Paediatric Respiratory Reviews 10 (2009) 1824
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.
A R T I C L E I N F O
Keywords:
Cerebral palsy
Pulmonary aspiration
Suppurative lung disease
Physiotherapy
Airway clearance techniques
Obstructive sleep apnoea
Sleep disordered breathing
S U M M A R Y
The major morbidity and mortality associated with cerebral palsy (CP) relates to respiratory compromise.
This manifests through repeated pulmonary aspiration, airway colonization with pathogenic bacteria,
the evolution of bronchiectasis and sleep disordered breathing. An accurate assessment involving a
multidisciplinary approach and relatively simple interventions for these conditions can lead to
signicant improvements in the quality of life of children with CP as well as their parents and carers. This
review highlights the more common problems and potential therapies with regard to suppurative lung
disease and sleep disordered breathing in children with CP.
2008 Elsevier Ltd All rights reserved.
* Corresponding author. Department of Respiratory Medicine, The Childrens
Hospital at Westmead, Locked Bag 4001, Westmead, NSW, Australia, 2145.
Tel.: +61 2 9845 3397; Fax: +61 2 9845 3396..
E-mail address: dominif2@chw.edu.au (D.A. Fitzgerald).
Contents lists available at ScienceDirect
Paediatric Respiratory Reviews
1526-0542/$ see front matter 2008 Elsevier Ltd All rights reserved.
doi:10.1016/j.prrv.2008.10.003
These studies serve to highlight the importance of recognizing
potential contributors to lung disease development in children
with CP and ensuring they are addressed in the management of
these children.
Potential contributors to lung disease development
A number of interrelated factors can potentially contribute to
the development of lung disease in children with CP (Table 1).
Lower motor ability, a history of asthma or cough and wheeze and
the presence of gastro-oesophageal reux (GOR) have been
demonstrated to predict radiological aspiration, and lower
adaptive ability, abnormal voice quality after feeds, lower scoliosis
angle and history of asthma, regurgitation or aspiration to
predict GOR in children with severe CP.
3,4
Baikie et al
4
also
investigated the use of various tests for aspiration in their cohort of
63 children and found that the salivagram was most frequently
positive (56%; CI 4368%) with barium videouoroscopy the next
most frequently positive test (39% CI 2653%), while radionuclide
scan was rarely positive (6%, CI 216%). However, there was poor
agreement between the tests with the best agreement being
between the salivagramand bariumvideouoroscopy. The authors
concluded that the salivagramwas probably the most useful test at
predicting who is at risk of aspiration in this population. An audit of
294 children with CP presenting to the dysphagia clinic at the
Childrens Hospital at Westmead demonstrated evidence of
pulmonary aspiration in 72 (41%) of the 174 videouoroscopy
studies performed, while 42 (67%) of the 62 CT scans performed
demonstrated bronchiectasis (Fig. 2), again highlighting pulmon-
ary aspiration as an important cause of lung disease in children
with CP (unpublished data). A number of factors are likely to
contribute to impaired mucociliary clearance in children with CP,
including recurrent pulmonary aspiration, poor nutrition, kyphos-
coliosis, motor weakness, upper and lower airway obstruction,
recurrent infection and bronchiectasis. In turn, impaired muco-
ciliary clearance may lead to the development of airway
obstruction and atelectasis, recurrent infection, bronchiectasis,
ventilation/perfusion inequality, reduced lung compliance and
eventually, respiratory failure in these children. Kyphoscoliosis is
also common in children with severe CP and occurred in 39% of
children in Baikies cohort.
3
This in turn may contribute to GOR and
pulmonary aspiration, impaired mucocilary clearance, structural
lower airway obstruction and the development of respiratory
failure. In a recent evaluation of 35 patients with severe CP, the
degree of spinal deformity, neck rotation deformity and severe
asymmetrical deformity posture, and the severity of motor
dysfunction were all found to predict respiratory dysfunction.
5
As highlighted previously, snoring is common in children with
CP
3
and upper airway obstruction can also occur at the supraglottic
level with redundant aryepiglottic folds causing a clinical picture
of laryngomalacia. In addition, asthma is also commonly
diagnosed in these children,
3
but is more likely to be due to some
of the other contributors to lung disease development, including
pulmonary aspiration, bronchiectasis, impaired mucociliary clear-
ance, kyphoscoliosis and structural upper and lower airway
obstruction. It is therefore important to be aware of the potential
for over-treatment with asthma medications in children with CP.
MANAGEMENT OF LUNG DISEASE INCHILDRENWITHCEREBRAL
PALSY
The management of lung disease in children with CP is
summarized in Table 2. While thickened feeds and antireux
therapy should be trialled initially, many children, particularly
those with severe CP, will require gastrostomy feeds combined
with fundoplication if there is signicant GOR. However, continu-
ing aspiration of saliva may be an ongoing problem. Antic-
holinergic therapy in the form of both benztropine
6
and
glycopyrrolate
7,8
has been documented in open label studies as
being effective in patients with CP and is generally favoured as
rst-line therapy.
9,10
Glycopyrrolate has the theoretical advantage
of having fewer side effects.
10
Botulinum toxin injections into the
salivary glands has been used as alternative treatment for the
management of sialorrhea
9
but trials have been limited.
10,11
Surgery remains an option for patients with persistent problems
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.
Table 1
Potential contributors to lung disease development
Pulmonary aspiration
Impaired mucociliary clearance
Recurrent infection leading to bronchiectasis
Kyphoscoliosis
Upper airway obstruction
Lower airway obstruction asthma
Figure 2. CT scan demonstrating early bronchiectasis in a 6-year-old child.
D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 1824 19
despite trials of medical therapy.
9,10
Children with impaired
mucociliary clearance will benet fromphysical therapies to assist
secretion removal (see below) and this may be combined with a
nebulized inhalation.
There should be a low threshold for antibiotic use for
intercurrent respiratory infections in children with CP, particularly
in those with evidence of suppurative lung disease. Ideally,
antibiotic choice should be based on sputum culture results and
prolonged courses (34 weeks) are usually required in these
patients. Immunization to protect against inuenza and pneumo-
coccus should also be administered.
While kyphoscoliosis may be a signicant contributing factor to
lung disease development inthese children, the potential benets of
surgery need to be carefully weighed up against the potential risks,
particularlyinchildrenwithestablishedlungdisease. As highlighted
previously, asthma is commonly diagnosed in these children but is
usually related to other causes so any use of asthma medication
should be seen as a trial and not continued or escalated unless there
is a convincing response. Finally, childrenwithsignicant structural
lower airway obstruction or those in respiratory failure may benet
from respiratory support in the form of non-invasive ventilation.
Physiotherapy interventions
Impaired mucociliary clearance may lead to the development of
recurrent chest infections, bronchiectasis and eventually, respira-
tory failure. Therefore, models of care are necessary to improve
mucociliary clearance and hence optimize the respiratory status of
children with CP.
Physiotherapy intervention is part of a 24-h care plan, designed
to complement but not overwhelm what already can be a
considerable burden of care. If the child can cough effectively
and adequately clear their secretions, they may only need to be
positioned well and repositioned throughout the day. Good
positioning can be in an upright standing frame, a well supported
well tting seating system in a wheelchair or in bed with the head
raised slightly. Slumped sitting should be avoided, especially at
mealtimes, as it promotes GOR and increases respiratory effort.
However, if the child cannot cough effectively or clear their
secretions adequately, or develops an acute lower respiratory tract
infection they may need more active chest physiotherapy.
Chest physiotherapy
Chest physiotherapy is now referred to by the more generic
term of airways clearance techniques (ACTs).
12
There is a dearth
of evidence as to the most effective ACT for children with CP. The
degree of disability will inuence the most appropriate ACT so, as
with all treatment, it should be carefully individualized and
reviewed regularly as the child grows. Due to the limited evidence
for chest physiotherapy in CP children and adolescents, the
evidence discussed is mostly from research done in children and
adolescents with cystic brosis who also can have difculty with
excessive secretions and impaired mucociliary transport.
Until recentlythe goldstandard ACTfor all respiratoryproblems
requiring physiotherapy was considered to be postural drainage
(PD) with percussion and vibrations. These PD positions were
reported in Thorax in 1950.
13
There are 12 positions that place the
patient so that gravity can assist in draining the mucus from the
lungs. There is nowincreasing evidence that challenges the safety of
the traditional head down PDpositions. GOR is well recognized as a
common complication in children with CP, affecting their nutrition
and growth and perhaps their gross motor development.
14
A
number of studies in cystic brosis have demonstrated an increased
incidence of GOR when infants and children are tipped head
down.
1517
One long termstudy alsoshowedthat infants withcystic
brosis inwhomnoheaddowntilt was performedwiththeir PDhad
better lung function and less chest X-ray change after 5 years than
those who where tipped head down.
18
Other documented
complications of head down tip include increased dyspnoea
19
and
oxyhaemoglobin desaturation.
20
Severely disabled children with CP can also have marked
skeletal deformity and corresponding muscle dysfunction and
weakness. Effectively, they rely heavily on the use of the
diaphragm for respiration which, in the head down tipped
position, is at a mechanical disadvantage. Consequently, most
chest physiotherapy treatments for CP children are nowperformed
in modied PD positions.
15
These positions are:
Supine 308 head up;
Prone horizontal;
Left and right horizontal side lying;
Upright sitting for apical segment of upper lobe (this is done
leaning against the therapists/carers shoulder or sitting
upright).
Currently the limited available evidence
18
and our experience
has shown no adverse effects whenmodied PDis used. Percussion
and vibrations are used as an adjunct to modied PD, not
increasing the amount of secretions produced
21,22
but decreasing
the amount of time needed to remove secretions.
21
Percussion
produces an energy wave that is transmitted through the chest
wall to the airways and aims to mobilize sputum. It is a rhythmical
technique performed using a cupped hand with exion and
extension of the wrist with a frequency of approximately 36 Hz.
23
Vibrations are the shaking of the chest wall during expiration.
Vibrations increase the expiratory ow rate more than percussion
or oscillation so should increase mucous transport.
24
Once
secretions are loosened, then a cough is necessary to clear
secretions from the central airway to be expectorated or moved
to the back of the throat where they can be swallowed.
Practicalities of conventional therapies
The ACTs can produce a cough spontaneously and if the
secretions are not overwhelming then they can be swallowed
without adverse effect. If, however, they are excessive or the child
is having difculty clearing the secretions, then oropharyngeal (O/
P) and nasopharyngeal (N/P) suction may be necessary.
Table 2
Management of lung disease in children with cerebral palsy
Pulmonary aspiration
Thickened feeds and antireux treatment
Gastrostomy and fundoplication
Control of saliva
Anticholinergics
Botox injection
Surgery
Impaired mucociliary clearance
Inhalations normal/hypertonic saline or bronchodilators
Physical therapies to improve clearance
Infection
Antibiotics
Treatment vs prophylaxis
Guided by sputum results
Prolonged courses required
Immunization inuenza, pneumococcus
Scoliosis benet vs risk of surgery
Upper airway obstruction surgery vs CPAP
Lower airway obstruction
Trial of asthma treatment (cease if no response)
CPAP/BiPAP
D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 1824 20
In children the suction catheter is only passed as far as the
pharynx to stimulate a cough; then the secretions are produced
and suctioned out. Suction is a traumatic experience and there are
necessary precautions that need to be followed. Hands should be
washed and gloved. There is a risk of vomiting so the child should
be positioned on their side and routine suction following chest
physiotherapy should be performed when the child has an empty
stomach or at least 1 h after eating. Swaddling of the child may be
necessary to prevent unnecessary struggling and to decrease
trauma to the mouth or nose. The suction pressure (vacuum
pressure) should be sufcient to draw the secretions up the
catheter but no greater than 1020 kPa (75150 mmHg). Suction
pressure should only be applied on extraction of the catheter. The
catheter should not be too large, generally an 8 or 10 French
gauge.
25
Suction can cause adverse effects which include mucosal
trauma, hypoxia, tachycardia or bradycardia and bronchospasmso
close monitoring of the child is required.
26
An external tracheal stimulation may also be used to stimulate
a cough. This is done by applying manual pressure to the trachea in
the sternal notch below the cricoid cartilage. This should be done
with care and used judiciously as there are currently no guidelines,
consensus or research on this technique.
Alternative therapies
Due to the time consuming and physically demanding nature of
the modied PD with percussion and vibrations, many more ACTs
have been developed that allowindependence of the child and less
physical involvement of the carer. A group of therapists in Sweden
have performed research on the use of positive expiratory pressure
(PEP) on a small group of severely disabled CP children. They found
that the transcutaneous oxygen tension improved after PEP in the
short term but there was no change in the respiratory rate. The
children accepted the treatment without any problems. The long
term effect or amount of secretions produced were not men-
tioned.
27
It has also been suggested that the low ow Acapella
(oscillating PEP) may be an alternate ACT, however there is no
research in this area. The high frequency chest wall oscillation vest
has been effective in patients with cystic brosis in the US, helping
clear their secretions,
28
but due to its high cost and limited
availability it has not been widely used worldwide.
In conclusion, not all children with CP need daily chest
physiotherapy. If they have difculty clearing excessive secretions
of the lower respiratory tract then chest physiotherapy is
indicated, but the need should be reassessed regularly. There
are limited guidelines to inuence clinical practice.
29
The fact must
be recognized that ACTs impose an extra demand on carers who
already have a signicant workload in caring for a disabled child.
SLEEP BREATHING PROBLEMS
Children with CP have a higher prevalence of sleep breathing
problems than otherwise healthy children reported in community
based surveys.
30
The disruption of normal sleep patterns may have
an enormous impact upon the interactive and cognitive skills of the
child with CP, adversely affect their quality of life and interfere
with the normal sleeping patterns of siblings and parents. Indeed,
sleep problems in children with CP are under-reported, under-
recognized and under-treated.
Disturbed sleep patterns may involve fragmented sleep,
increased nocturnal awakenings and even inversion of the
sleepwake cycle.
30
A range of factors predispose children with
CP to sleep disordered breathing. Children with CP may be
vulnerable to sleep problems for a variety of reasons which may
include, but are by no means limited to:
Upper airway obstruction: glossoptosis, poor pharyngeal muscle
tone, adenoid and tonsillar enlargement;
Severe visual impairment: altered timing and maintenance of
sleep;
Brainstem dysfunction: cardiac and respiratory control may be
altered as well as level of alertness;
Postural limitations and discomfort: muscle spasms resulting
from dislocated hips, scoliosis and joint contractures together
with an inability to alter posture in response to discomfort;
Epilepsy treatment with anticonvulsants may have excessive
somnolence as a side effect which may disrupt normal
sleepwake patterns;
Pulmonary aspiration: micro-aspiration and cough will disrupt
sleep architecture.
Upper airway obstruction
This is an underappreciated issue in sleep in many children with
CPandmay not be considereduntil the questionof obstructive sleep
apnoea (OSA) is raised because of a lengthy history of snoring. A
recent retrospective small case series described eight children with
severe spastic quadriplegic CPpresentingover an8-monthperiodto
a large paediatric teaching hospital with upper airway obstruction.
Sevenrequiredadmissiontotheintensivecareunit for amedianstay
of 8 days.
31
Seven underwent awake nasendoscopy which demon-
stratedenlargedadenoidsintwopatients. However, intheremaining
sixpatients, nostructural abnormalitywas foundat endoscopyof the
supraglottic regions, but collapse of the pharyngeal/supraglottic
structures was noted.
31
Only two of the eight subjects had been
referredforsleepstudies. Twoof thepatientshadtracheostomiesand
one had an adenotonsillectomy and continued on nocturnal CPAP.
The eighth patient, with a 7-month history of upper airway
obstruction, was shown to have a diffuse malignant brainstem
glioma, was managed palliatively and died 6 months later.
The interrelationship between awake upper airway obstruction
and OSA is not clear-cut. It is possible to have neither, one without
the other or indeed both. For those children with stridor and
obstructive symptoms when awake, but not in sleep, the
symptomatology has been attributed to laryngeal dystonia, and
reported in a small cohort of children with dystonic CP
32
or severe
laryngomalacia.
31
The laryngomalacia seen in older children with
CP reects the reduced tone of the supraglottic structures, which
on forceful inspiration collapse into the airway, reducing the
calibre of the airway, compromising airow and resulting in
stridor. The concurrent presence of a pseudobulbar palsy may
serve to predispose to this tendency.
31,32
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
and consider the morbidity that may follow from an under-
appreciation of upper airway obstruction and its management.
Altered sleep cycles
The establishment and maintenance of normal sleep patterns in
young children with CP is often challenging. In addition to the
negative impact upon the child with CP, altered sleep cycles are
frustrating for the parents and siblings as they are often disruptive
to their sleep requirements. Sleep may be affected by a seizure
disorder and its treatment, altered perception of light with visual
impairment as well as pain and physical discomfort.
Insomnia
The prevalence of difculties in settling off to sleep in the
general paediatric population is generally high and is higher again
D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 1824 21
in special needs populations, including children with CP.
33,34
Figures quoted relate to difculty settling and frequent night
awakenings in 40% of infants; delayed sleep onset and disruptive
night awakenings in up to 50% of preschoolers and bed-time
resistance in up to 27%of school-aged children.
34
These events may
be disruptive for the child whilst tiring and distressing for the
family.
34,35
Poor sleep quality will adversely impact upon
behaviour, mood, attention, cognition, memory and school
performance.
35
In children with CP, and indeed with many other
neurocognitive abnormalities, the mechanisms of sleep disruption
are not well understood.
Seizure disorders
Sleep patterns may be adversely affected by the existence and
treatment of a coexisting seizure disorder.
33,36
The interaction
between epilepsy and sleep disorders is not well understood, but
the most frequently reported problems are those of sleep
fragmentation with reduced sleep efciency and more frequent
arousals from sleep.
37
Conversely, poor sleep quality may also
increase seizure frequency.
38
Possible contributory factors include
associated psychological and mood disturbance and an alteration
in sleep architecture that may persist after seizures.
39
A child with frequent seizures may have post-ictal phases with
somnolence as the initial symptom. Alternatively, the treatment of
a difcult seizure disorder, often with a number of anticonvulsants,
may also result in somnolence as a side effect of the medications.
40
A range of antiepileptic medications have been shown to alter
sleep architecture.
41
The alteration and more typically reduction in
an anticonvulsant regimen may improve sleep-wake cycling and
reduce somnolence. This frequently will reduce sleep related
hypoventilation and perhaps concurrent OSA with a resulting
improvement in gas exchange.
Altered light perception
The presence of blindness or greatly reduced vision may impair
the benets of natural light and its facilitation of wakefulness
during the daytime. A reduction in the perception of natural light
will disrupt the natural secretion of the sleep-inducing hormone
melatonin, released from the pineal gland. The secretion of
melatonin is inhibited by light and enhanced during darkness.
42
In healthy human controls, melatonin secretion will increase soon
after dark, peak in the middle of the night and steadily decline
during the second half of the night.
43
The consequences of reduced
or absent light perception upon sleep can be dramatic. Children
with CP and blindness may have a free running circadian rhythmor
even an inverted sleep-wake cycle.
44
Pain and physical discomfort
The effect of physical discomfort and the impaired ability to
adjust position in sleep may also impact adversely upon sleep
quality. The children may present with increased tiredness,
restless sleep, night time crying or agitation. Initial responses
may be to offer the child sedation with antihistamines or
benzodiazepines, but such responses may overlook the underlying
problem such as a dislocated hip or pressure sores on dependent
areas. The physical examination should always consider these
possibilities. Interestingly, the sedative agents may also inuence
upper airway muscle tone, increasing snoring and problems with
the coordinated swallowing of oral secretions, which may further
complicate the assessment.
Cough and pulmonary aspiration
This has been highlighted earlier with regard to the develop-
ment of lung disease. Interestingly, the symptoms may be more
apparent in the supine position during sleep and therefore a full
history of sleep breathing concerns should include consideration of
cough, its aetiology, GOR and oral secretion management.
MANAGEMENT OF SLEEP PROBLEMS IN CHILDREN WITH
CEREBRAL PALSY
After considering the history of sleep breathing concerns and
completing a physical examination of the chest and upper airway,
it is likely that the possible problems will fall into either
obstructive breathing patterns or altered sleep cycles which have
resulted in fragmented sleep. The presence of snoring on the
majority of nights, with or without a clear history of witnessed
apnoea, would favour the possibility of OSA. Conversely, the
presence of predominantly lengthy periods of wakefulness and
some daytime sleepiness may favour a problem with altered sleep
cycles. Certainly, both problems can occur concurrently but the
approach to their management differs.
Management of obstructive sleep apnoea
For children who snore, it would be reasonable to consider a
lateral airways radiograph to assess the size of adenoidal tissue
in relation to the airway. If there was a lengthy history of
witnessed apnoea, it would be appropriate to obtain a venous
blood gas or serum bicarbonate as a quick screen for carbon
dioxide retention. The denitive test is a polysomnogram to
characterize the presence and severity of sleep disordered
breathing. This will also demonstrate the presence of excessive
central apnoea (oxygen desaturation), a more common nding in
children with central neurological abnormalities. Where poly-
somnography is not available, the clinician can rely on a
combination of clinical judgement, the lateral airways radiograph,
a measure of serum bicarbonate (if not a blood gas) and perhaps
oximetry during sleep.
The therapeutic response to the presence of signicant
obstructive apnoea (an obstructive apnoea index > 5 episodes/h
with oxygen desaturation of > 3%) would be adenoidectomy and
tonsillectomy. In more severe cases, or in those children deemed to
have too great an operative risk, a period of non-invasive
respiratory support with nasal CPAP during sleep will improve
gas exchange, reset altered chemoreceptors and provide a bridge to
surgery to remove enlarged tonsils or adenoids. In children
without enlarged tonsils or adenoids, and presumably low
pharyngeal muscle tone in sleep, the use of CPAP in sleep should
overcome upper airway obstruction. Those with altered respira-
tory drive may benet from a more complex form of non-invasive
respiratory support with bi-level pressure devices which can be set
at a guaranteed rate, generally of up to 30 breaths/min. Both
devices can be established at the bedside with an approximation of
the pressures required to overcome snoring and minimize
paradoxical respirations. Importantly, ne tuning of the pressures
and rate would take place during further polysomnography as a
titration study. With growth over time and alterations in airway
dimensions, such as after surgery on adenoids or tonsils, the need
for ongoing respiratory support will need reassessment.
Management of altered sleep cycles
It is useful to begin with a sleep diary, lled in by the parents
over a 1014-day period which will demonstrate the sleeping
patterns, length of sleep and duration of overnight awakenings.
This can serve as a useful baseline and can be repeated at
subsequent intervals if needed to demonstrate progress. Once the
sleep pattern is documented, the use of sleep settling routines
D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 1824 22
should be the rst action. This is designed to make the
environment, usually the childs bedroom, more conducive to
sleep. A regular meal time, bathing routine and a lack of
stimulation from computers, television or DVDs in the pre-bed
hours is often helpful. Typically, whilst helpful, these measures are
insufcient to normalize the sleeping patterns of a child with a
delayed sleep phase or an inverted sleep cycle.
Cognitive behavioural therapies have been shown to be
successful in children with insomnia.
45
In a survey of American
community-based paediatricians, Owens and colleagues reported
that the prescription of sedatives and hypnotics is common for
treating paediatric insomnia despite reservations regarding this
practice and the lack of specic clinical guidelines in this area.
45
Over-the-counter sedating antihistamines are widely recom-
mended followed by melatonin.
The use of melatonin has become common for the treatment of
circadian rhythm disorders (especially delayed sleep phase
syndrome).
44
Generally administered in doses of 36 mg as a
liquid or tablet 30 min before desired sleep onset at night, the
medication has become popular and has been considered
efcacious.
46
The only reservation may relate to an inconsistent
decrease in seizure control.
30,47
The effect is to reduce the time to
sleep onset (typically by 3060 min), increase total sleep time
(typically by 4560 min) and decrease the frequency of night
awakenings. The medication has been used continuously in clinical
studies for 6 months with no suggestion of tachyphylaxis.
Other therapies that have been used in children include
alternative medicines such as herbal preparations, chloral hydrate,
benzodiazepines, barbiturates, phenothiazines and diphenhydra-
mine.
45
It is presumed that these medications are also being used
in children with CP and neurocognitive decits, again with little in
the way of evidence to guide practice.
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PRACTICE POINTS
Lung disease is common particularly in children with
severe CP.
The aetiology is multifactorial, including pulmonary
aspiration, decreased mucociliary clearance, suppuration,
kyphoscoliosis and airway obstruction.
Asthma is over-diagnosed and over-treated with wheeze
usually due to other aetiologies.
CP is also commonly associated with sleep disordered
breathing that is under-recognized and under-treated.
Management needs to address the relatively predictable
lung and sleep breathing problems.
Recurrent suppurative lung disease is often amenable to
simple and inexpensive therapies that will improve the
quality of life of the person with CP and their family and
carers.
A range of airway clearance techniques are available for
children with CP.
Non-invasive respiratory support is useful for airway
clearance and sleep disordered breathing.
Life expectancy for children with CP is improving due in
part to a more aggressive approach to the use of
respiratory therapies.
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controlled release melatonin in the treatment of delayed sleep phase syndrome and
impaired sleep maintenance in children with neurodevelopmental disabilities. J Pineal
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CME SECTION
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administrative charge).
Educational questions
Answer true or false.
1. Regarding lower respiratory tract infections complicating
cerebral palsy:
a. The major infective cause of death in children with
cerebral palsy is pneumonia.
b. Recurrent pulmonary aspiration often presents as cough
without wheeze but is often inappropriately labelled and
treated as asthma.
c. A salivagram is not a useful test for the assessment of
pulmonary aspiration.
d. Children with gastro-oesophageal reux and recurrent
pulmonary aspiration should be considered for gastro-
stomy feeds and a fundoplication.
e. A sputum sample is of no benet in the assessment of
children with suppurative lung disease complicating
cerebral palsy.
2. Factors to consider in the management of children with
impaired mucociliary clearance complicating cerebral palsy
include:
a. The presence of altered swallowing dynamics and drooling
of saliva.
b. The use of inhaled or oral anticholinergic therapy has no
role in the management of excessive oral secretions.
c. Non-invasive respiratory support has a role in improving
impaired mucociliary clearance.
d. Cough in infants with cerebral palsy does not require
intervention.
e. The use of nebulized hypertonic saline may be of benet in
children with suppurative lung disease complicating
cerebral palsy.
3. Children with cerebral palsy have difculties with insomnia.
Aspects of their care to consider include:
a. Environmental issues such as light perception.
b. Pain from pressure areas or musculoskeletal problems
such as a dislocated hip.
c. Circadian rhythm disturbances.
d. Short sleep requirements.
e. Disrupted melatonin secretion.
4. Children with cerebral palsy have more frequent sleep
problems than other children. Contributing factors to
consider may include:
a. Enlarged tonsils and adenoids in the presence of snoring
would raise the possibility of obstructive sleep apnoea.
b. The concomitant use of multiple anticonvulsants for
coexisting seizure disorders may contribute to excessive
daytime somnolence.
c. Severe visual impairment has minimal effect upon sleep
wake cycles.
d. Children with signicantly impaired mobility may have
disrupted sleep without a history of snoring.
e. Repeated pulmonary aspiration may interrupt sleep
patterns.
5. Chest physiotherapy is appropriate and is best undertaken:
a. When the child has an upper respiratory tract infection.
b. When the child has excessive secretions that they are
having difculty managing.
c. When the child has an acute lower respiratory tract
infection.
d. Routine chest physiotherapy should be undertaken at least
an hour after the child has eaten.
e. Most physiotherapy treatments for children with cerebral
palsy use modied postural drainage.
D.A. Fitzgerald et al. / Paediatric Respiratory Reviews 10 (2009) 1824 24