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Respiratory problems in children with neurodisability
SYMPOSIUM: SPECIAL NEEDS
Respiratory problems in children with neurodisability
associated difficulties with swallowing and the coordination of the swallowing reflex. From the same series of Belgian children dysphagia was reported in 54%, gastro-oesophageal reflux (GOR) in 53% and 43% had reported an episode of aspiration. 41% used a gastrostomy. While the majority had not required a respiratory-led hospital admission in the previous year paradoxical breathing, asymmetric breathing and absence of a spontaneous cough were associated with an increased risk. Those with severe scoliosis, dysphagia, GOR and previous aspiration were also more likely to require hospitalization for a respiratory problem. Hypoxia and hypercapnoea may be present at a late stage (Table 1 summarises mechanisms of hypoxaemia). It is therefore appropriate to consider each in turn with respect to identifying and managing such problems. Table 2 summarises some of the common clinical manifestations.
M Hurley Harish Vyas
Abstract Respiratory problems are very common in children with neurodisability being the most common cause of higher morbidity and death. The factors that contribute to these difficulties include poor cough and pooling of oropharyngeal sections. Gastro-oesophageal reflux, muscle weakness and development of kyphoscoliosis lead to chronic ongoing morbidity leading to recurrent infections and development of bronchiectasis. Obstructive sleep problems may require mechanical support. Common paediatric conditions such as asthma may co-exist. To optimise care of these complex children, a multidisciplinary team should include respiratory physician.
Aspiration Oropharyngeal aspiration is common in children with neurological impairment. Causes include impaired airway protective responses (cough and gag), dysfunctional swallowing and abnormal connections between the airway and gastrointestinal tract (e.g. trachea-oesophageal fistulae). Symptoms may include cough, wheezing and recurrent lung infection. Bronchiectasis is a significant complication of chronic aspiration. The irreversible damage to the bronchi and bronchioles may predispose to chronic lung infection, reduced lung function and increased morbidity and mortality that extends into adulthood. As the current gold standard technique for diagnosis is high-resolution chest CT (HRCT), many cases are not identified as the investigation is not often undertaken. Of 100 children aged between 6 months and 19 years attending an ‘aerodigestive and sleep centre’ all of whom had extensive investigation, 66% had bronchiectasis. Those with bronchiectasis were significantly more likely to have severe neurological impairment (OR 9.45 (95% CI 2.05e43.6)) and have reported symptoms of gastrooesophageal reflux (OR 3.36 (95% CI 1.08e10.43)). Interestingly in this population, although only a small proportion of patients had repeat HRCT, after intervention (feed modification, fundoplication, oral secretion reduction, airway clearance) 81% of those who did had stable or improved bronchiectasis. Diagnosing reflux aspiration may be problematic as there is no gold standard biomarker. The finding of lipid laden macrophages in bronchoalveolar lavage (BAL) fluid is not specific and the reference ranges for other putative biomarkers (such as pepsin, bile acids and alpha-amylase) have yet to be established. The decision to site a gastrostomy is complex and considerations include the nutritional status of the child, the frequency of respiratory infections and the presence of gut motility problems. The family and cultural context is also an essential consideration; for many families the risks of aspiration must be weighed against the undoubted pleasure that children with neurodisability (and their carers) gain from oral feeding. Unfortunately the evidence base to inform decisions about the need for gastrostomy is poor with the Cochrane review unable to find any randomised controlled trials. The general opinion of many specialists, supported by some low-level evidence, is that gastrostomy placement may be beneficial not only in terms of weight gain, but also in terms of social functioning and opinions of carers with the
Keywords adenotonsillectomy; aspiration; bronchoalveolar lavage; fundoplication; laryngomalacia; neurodisability; oximetry; prematurity; scoliosis
Introduction The respiratory complications of severe neurodisability occur as a consequence of immobility, posture and disorders of motor control. Of the children who died in England and Scotland between 2001 and 2010, and Wales between 2003 and 2010, 38.5% (9032 of 23438) of children died with neurological/sensory conditions, in many the final cause of death was respiratory failure. When assessing the respiratory health of a child with neurodisability it is important to consider the causes of the neurological impairment as this may help to tailor the treatment options. However, some respiratory complications are common to many with severe neurodisability. The role of the respiratory paediatrician is to provide the family with pragmatic advice and help the family to determine what treatments are acceptable. Unfortunately the evidence base that may guide difficult decisions is lacking.
Common features Severe neurodisability leads to a fairly redictable set of respiratory problems in children. Muscle weakness and immobility can lead to inadequate cough and kyphoscoliosis. Breathing control mechanisms are often reduced. In a series of 127 patients recruited from 10 multi-disciplinary teams in Belgium, 50% had an irregular breathing pattern and in 68% noisy breathing was reported with 31% snoring. Finally, it is common to find
M Hurley MRCP is Respiratory Registrar at Nottingham Children’s Hospital, Nottingham, UK. Conflict of interest: none declared. Harish Vyas DM(Notts) FRCP FRCPCH is Consultant PICU/Respiratory Medicine at Nottingham Children’s Hospital, Nottingham, UK. Conflict of interest: none declared.
PAEDIATRICS AND CHILD HEALTH --:-
1
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
studies in this regard is poor, however one prominent cohort study suggests an improvement in GOR with improving nutritional status and a corresponding reduction in the need for respiratory-led hospital admissions regardless of whether a fundoplication had also been performed. There is considerable doubt as to whether the routine addition of a fundoplication at the time of gastrostomy is an effective means of reducing GOR and there is certainly no robust clinical trial evidence to support its routine use.
Mechanisms of hypoxaemia in children with neurodisability Hypoventilation:
C
Ventilation-perfusion mismatch
C
Right-to-left shunt
C
Diffusion impairment
C
In neuromuscular conditions Both PaCO2 and PACO2 increase during hypoventilation Ventilatory support may be necessary in severe hypercapnoea PAO2 decrease: Responsive to oxygen Common in seizures, obstruction, interstitial disease PaO2 low, variable PaCO2 (normal or high) Due to pneumonia/ARDS Low PaO2 variable PaCO2 (normal or high) Interstitial disease/co-exist with V/Q mismatch PaCO2 normal
Respiratory muscles The respiratory ‘pump’ may be compromised either by respiratory muscle weakness (intercostal, accessory and diaphragmatic), incoordination, paradoxical breathing and inefficient angle of work due to scoliosis/kyphoscoliosis, or a combination of the above. These problems will, in turn, be exacerbated by poor nutritional status, which is probably the simplest problem to tackle. Scoliosis repair is often technically difficult to undertake, as is the decision regarding optimal timing. It is not uncommonly undertaken although even case series reports are limited to short-term outcomes and a relatively high complication rate. Those at particular risk of requiring intensive care following their procedure appear to be those with reduced lung function, a greater Cobb angle and higher number of vertebrae involved.
Table 1
qualitative studies that document a positive outcome in terms of the family unit appear to out-number those that identify concerns. One unresolved clinical issue with the siting of a gastrostomy is the concern that GOR will be exacerbated. Again, the quality of
Sleep Sleep disorders are common in children with cerebral palsy, particularly with regard to disorders of initiating and maintaining sleep (22%), sleep-breathing disorders (14%), disorders of arousal (10%), sleepewake transition disorders (15%), disorders of excessive somnolence (13%), and hyperhidrosis (7%). Overnight oximetry should be considered early in the assessment of a child with upper airway symptoms and should be repeated at intervals. In children with multi-system involvement formal polysomnography may also have a role. Non-invasive interventions with regard to sleep positioning and sleep systems have been used and a forthcoming Cochrane review will examine the evidence for these interventions.
Summary of problems and clinical manifestation Problem
Clinical consequence
Feeding problems Reflux: antegrade or retrograde
C
C C C C C C
C
Respiratory muscle weakness
C C C
Upper airways
C C C
C C C C
Sleep
C C
Kyphoscoliosis
C
Wheezing that is poorly responsive to appropriate beta-agonists or steroids Failure to thrive/Teeth erosion Atelectasis and bronchiectasis Recurrent pneumonia/cough Stridor or laryngitis/hoarseness Pulmonary abscess Apnoea/bradycardia/acute life-threatening events Recurrent tonsillitis/discharging ears Paradoxical breathing Poor secretory clearance Development of kyphoscoliosis Laryngomalacia, tracheomalacia Tonsil/Adenoids Pharyngeal collapse due to palatal hypotonia Macroglossia/small pharyngeal space Glossoptosis/Small mandible Sub-glottic stenosis Obesity Initiating or maintaining sleep Sleep breathing disorders Increasing
Upper airway Problems with the upper airway are very common in children with neurodisability. Upper airway musculature and laryngomalacia may contribute to impending respiratory failure due to upper airway obstruction. To an extent these can be alleviated by surgical approaches but any intervention runs the risk of both short and longer term complications. In general, simpler solutions higher up the airways, such as adenotonsillectomy are unlikely to result in longer term problems but are less likely to offer a complete solution whilst more definitive surgery such as tracheostomy requires significant lifelong postoperative care. Overnight oxygen or non-invasive ventilation may help but their initiation needs to be carefully planned in conjunction with the family. It is important to define an individualised management plan with clear goals of the outcomes to be achieved. Infection Because of the high aspiration risk empirical antibiotic use should cover gut organisms and so broad-spectrum agents (co-
Table 2
PAEDIATRICS AND CHILD HEALTH --:-
2
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
bleeding and perforation, hyperglycaemia, hypertension, hypertrophic cardiomyopathy and growth failure. It also documented increased risks of developmental delay (in one study), cerebral palsy and abnormal neurological examination in the limited number of included trials that reported late outcomes. Commentators have recommended against the use of early corticosteroids due to the higher risk of adverse events and reserving the use of late corticosteroids to those babies who cannot be weaned from ventilation.
amoxiclav, third generation cephalosporins) are often used. Unfortunately for patients with poor airway clearance, bronchiectasis and chronic exposure to broad spectrum antibiotics, the lung microbiota is likely to be shifted and a niche for opportunistic organisms such as Pseudomonas aeruginosa may result. As with other areas of care the evidence supporting management of such problems are extrapolated from other areas and so, as with CF, isolation of P. aeruginosa is often treated with successive eradication regimens of varying durations of ciprofloxacin and nebulized colistin. Many patients receive prophylactic antibiotics by way of thrice-weekly azithromycin. Unfortunately as children with neurodisabilities often find it difficult to expectorate, reliable respiratory sampling is uncommon and so the sensitivity of upper airway cultures is questionable. The identification of pathogens may be delayed as a result. As with CF, whether upper isolates reflect lower airway infection is also controversial, however BAL is often reserved for those with chronic chest X-ray changes or lobar collapse.
Insult at birth A poor neurological outcome often results for children who require ventilation at birth, in one series 11/45 children died or suffered severe neurodisability. While babies who present with respiratory insufficiency are likely to have sustained a multiorgan insult, the sequelae of ventilation including upper airway complications (e.g. sub-glottic stenosis) must be also considered as should the suggestion that respiratory compromise may form the presenting feature of a neurological insult.
Specific conditions
Cerebral palsy Those with cerebral palsy not only have the effects of the primary brain lesion but are also likely to have problems of the associated co-morbid conditions including epilepsy, hearing impairment, abnormal muscle tone and posture and nutritional insufficiency. Those with cerebral palsy appear to be particularly severely affected with GOR, which may be particularly difficult to manage.
Prematurity The outcomes of babies born extremely prematurely have been well documented by the series of EPICure studies and deserve particular consideration, not least because children born extremely prematurely have significant risks for severe neurodisability but also with significant respiratory sequelae. In terms of short-term outcomes of those children born at between 22 and 26 weeks gestation in 2006, 68% had BPD and 13% had evidence of severe abnormality on cerebral ultrasonography at hospital discharge. Later at 11 years of age, those born extremely prematurely (25 weeks gestation) and with most of whom attending mainstream school, they had lower lung function than their age-matched control friends. Among those born extremely prematurely, those who had a past history of BPD had significantly higher rates of wheeze and bronchial hyper-reactivity. An association with impaired haemodynamics suggests that impaired lung physiology should be interpreted within the context of abnormal arterial stiffness, adding to the risk factors for morbidity in later life. Even relatively modest degrees of prematurity may be associated with poor later respiratory outcomes. The risk of emergency admission to hospital from birth to the age of 5 years decreases with each successive additional week in gestation up to 40e42 weeks (hazard ratio for respiratory admission after birth between 33 and 34 weeks gestation 1.59 (95% CI 1.50e1.68)). Managing the respiratory sequelae of preterm birth is controversial and the subject of a series of Cochrane reviews considering the use of corticosteroids both to prevent and treat chronic lung disease of prematurity. Corticosteroid administration is associated with reductions in the risk of failure to extubate and death or chronic lung disease at 28 days or 36 weeks corrected gestational age. The review considering late-administered corticosteroids reported a reduction in risk of clinically important impairments of forced expired volume in one second (FEV1) of children undertaking spirometry later in childhood. The review examining early-administered corticosteroids however reported a greater burden of adverse effects including gastrointestinal
PAEDIATRICS AND CHILD HEALTH --:-
Ataxia telangiectasia Ataxia telangiectasia (AT) is a progressive, neurodegenerative disease causing immunodeficiency and an increased risk of malignancy and respiratory disease that commonly includes chronic sinopulmonary infection, aspiration, bronchiectasis and interstitial lung disease. Infection drives much of the respiratory morbidity in AT with 74% of patients attending the UK national clinic being prescribed a chronic prophylactic antibiotic. Much of the evidence informing the treatment of these children is extrapolated from cystic fibrosis and accordingly, those with severe lung disease in AT are chronically infected with P. aeruginosa and Burkholderia cepacia complex. Myelomeningocele/Arnold-Chiari malformation Children with spina bifida may also have a Chiari II malformation, which may cause central apnoea and dysphagia. An excessive gag reflex can be confused with GOR and associated vocal cord dysfunction may require treatment with tracheostomy, the combination of all of the above make a gastrostomy a priority.
Summary Unfortunately for children with neurodisability robust evidence to guide decisions about optimal respiratory care is lacking. Particularly difficult decisions regarding the use of intensive care and long-term ventilation must be taken in partnership with the patient’s family and with clear goals of what is expected to be achieved. The use of gastrostomies appears to be accompanied by improvement of nutritional status while the routine accompaniment of fundoplication is controversial. The assessment of
3
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
11 Vernon-Roberts A, Sullivan PB. Fundoplication versus postoperative medication for gastro-oesophageal reflux in children with neurological impairment undergoing gastrostomy. Cochrane Database Syst Rev 2013. Issue 8. Art. No.:CD006151. 12 Romeo DM, Brogna C, Quintiliani M, et al. Sleep disorders in children with cerebral palsy: neurodevelopmental and behavioral correlates. Sleep Med 2014; 15: 213e8. 13 Wood NS, Marlow N, Costeloe K, et al. Neurologic and developmental disability after extremely preterm birth. EPICure Study Group. N Engl J Med 2000; 343: 378e84. 14 Fawke J, Lum S, Kirkby J, et al. Lung function and respiratory symptoms at 11 years in children born extremely preterm: the EPICure study. Am J Respir Crit Care Med 2010; 182: 237e45. 15 Seddon PC, Khan Y. Respiratory problems in children with neurological impairment. Arch Dis Child 2003; 88: 75e8.
children with neurodisability is multiprofessional and due to the nature of the condition interval assessments are often warranted. A FURTHER READING 1 Hardelid P, Dattani N, Gilbert R, et al. Estimating the prevalence of chronic conditions in children who die in England, Scotland and Wales: a data linkage cohort study. BMJ Open 2014; 4: e005331. 2 Proesmans M, Vreys M, Huenaerts E, et al. Respiratory morbidity in children with profound intellectual and multiple disability. Pediatr Pulmonol 2014; http://dx.doi.org/10.1002/ppul.23114. 3 Weir KA, McMahon S, Taylor S, et al. Oropharyngeal aspiration and silent aspiration in children. Chest 2011; 140: 589e97. 4 Trinick R, Johnston N, Dalzell AM, et al. Reflux aspiration in children with neurodisabilityea significant problem, but can we measure it? J Pediatr Surg 2012; 47: 291e8. 5 Gantasala S, Sullivan PB, Thomas AG. Gastrostomy feeding versus oral feeding alone for children with cerebral palsy. Cochrane Database Syst Rev 2013. Issue 7. Art. No.:CD003943. 6 Sullivan PB, Juszczak E, Bachlet AM, et al. Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study. Dev Med Child Neurol 2005; 47: 77e85. 7 Sullivan PB, Morrice JS, Vernon-Roberts A, et al. Does gastrostomy tube feeding in children with cerebral palsy increase the risk of respiratory morbidity? Arch Dis Child 2006; 91: 478e82. 8 Srinivasan R, Irvine T, Dalzell M. Indications for percutaneous endoscopic gastrostomy and procedure-related outcome. J Pediatr Gastroenterol Nutr 2009; 49: 584e8. 9 Townsend JL, Craig G, Lawson M, et al. Cost-effectiveness of gastrostomy placement for children with neurodevelopmental disability. Arch Dis Child 2008; 93: 873e7. 10 Hauer JM. Does Nissen fundoplication decrease reflux-related problems including respiratory illness in children with neurological impairment? JAMA Pediatr 2014; 168: 188.
PAEDIATRICS AND CHILD HEALTH --:-
Practice points C
C
C C
C
C
C
4
All children with any degree of neurodisability should be reviewed by respiratory paediatrician to optimise care and reduce longterm complications Oropharyngeal aspiration is common in children with feeding difficulties and may lead to bronchiectasis Diagnosing reflux aspiration may be problematic The effectiveness of routine addition of a fundoplication at the time of gastrostomy as an effective means of reducing GOR is still uncertain Overnight oximetry should be considered early in the assessment of a child with upper airway symptoms and should be repeated at intervals An ENT review and intervention may be necessary if the sleep study demonstrates significant obstructive picture Azithromycin prophylactic antibiotics and pseudomonas clearance may reduce hospital admissions
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
Respiratory problems in children with neurodisability
associated difficulties with swallowing and the coordination of the swallowing reflex. From the same series of Belgian children dysphagia was reported in 54%, gastro-oesophageal reflux (GOR) in 53% and 43% had reported an episode of aspiration. 41% used a gastrostomy. While the majority had not required a respiratory-led hospital admission in the previous year paradoxical breathing, asymmetric breathing and absence of a spontaneous cough were associated with an increased risk. Those with severe scoliosis, dysphagia, GOR and previous aspiration were also more likely to require hospitalization for a respiratory problem. Hypoxia and hypercapnoea may be present at a late stage (Table 1 summarises mechanisms of hypoxaemia). It is therefore appropriate to consider each in turn with respect to identifying and managing such problems. Table 2 summarises some of the common clinical manifestations.
M Hurley Harish Vyas
Abstract Respiratory problems are very common in children with neurodisability being the most common cause of higher morbidity and death. The factors that contribute to these difficulties include poor cough and pooling of oropharyngeal sections. Gastro-oesophageal reflux, muscle weakness and development of kyphoscoliosis lead to chronic ongoing morbidity leading to recurrent infections and development of bronchiectasis. Obstructive sleep problems may require mechanical support. Common paediatric conditions such as asthma may co-exist. To optimise care of these complex children, a multidisciplinary team should include respiratory physician.
Aspiration Oropharyngeal aspiration is common in children with neurological impairment. Causes include impaired airway protective responses (cough and gag), dysfunctional swallowing and abnormal connections between the airway and gastrointestinal tract (e.g. trachea-oesophageal fistulae). Symptoms may include cough, wheezing and recurrent lung infection. Bronchiectasis is a significant complication of chronic aspiration. The irreversible damage to the bronchi and bronchioles may predispose to chronic lung infection, reduced lung function and increased morbidity and mortality that extends into adulthood. As the current gold standard technique for diagnosis is high-resolution chest CT (HRCT), many cases are not identified as the investigation is not often undertaken. Of 100 children aged between 6 months and 19 years attending an ‘aerodigestive and sleep centre’ all of whom had extensive investigation, 66% had bronchiectasis. Those with bronchiectasis were significantly more likely to have severe neurological impairment (OR 9.45 (95% CI 2.05e43.6)) and have reported symptoms of gastrooesophageal reflux (OR 3.36 (95% CI 1.08e10.43)). Interestingly in this population, although only a small proportion of patients had repeat HRCT, after intervention (feed modification, fundoplication, oral secretion reduction, airway clearance) 81% of those who did had stable or improved bronchiectasis. Diagnosing reflux aspiration may be problematic as there is no gold standard biomarker. The finding of lipid laden macrophages in bronchoalveolar lavage (BAL) fluid is not specific and the reference ranges for other putative biomarkers (such as pepsin, bile acids and alpha-amylase) have yet to be established. The decision to site a gastrostomy is complex and considerations include the nutritional status of the child, the frequency of respiratory infections and the presence of gut motility problems. The family and cultural context is also an essential consideration; for many families the risks of aspiration must be weighed against the undoubted pleasure that children with neurodisability (and their carers) gain from oral feeding. Unfortunately the evidence base to inform decisions about the need for gastrostomy is poor with the Cochrane review unable to find any randomised controlled trials. The general opinion of many specialists, supported by some low-level evidence, is that gastrostomy placement may be beneficial not only in terms of weight gain, but also in terms of social functioning and opinions of carers with the
Keywords adenotonsillectomy; aspiration; bronchoalveolar lavage; fundoplication; laryngomalacia; neurodisability; oximetry; prematurity; scoliosis
Introduction The respiratory complications of severe neurodisability occur as a consequence of immobility, posture and disorders of motor control. Of the children who died in England and Scotland between 2001 and 2010, and Wales between 2003 and 2010, 38.5% (9032 of 23438) of children died with neurological/sensory conditions, in many the final cause of death was respiratory failure. When assessing the respiratory health of a child with neurodisability it is important to consider the causes of the neurological impairment as this may help to tailor the treatment options. However, some respiratory complications are common to many with severe neurodisability. The role of the respiratory paediatrician is to provide the family with pragmatic advice and help the family to determine what treatments are acceptable. Unfortunately the evidence base that may guide difficult decisions is lacking.
Common features Severe neurodisability leads to a fairly redictable set of respiratory problems in children. Muscle weakness and immobility can lead to inadequate cough and kyphoscoliosis. Breathing control mechanisms are often reduced. In a series of 127 patients recruited from 10 multi-disciplinary teams in Belgium, 50% had an irregular breathing pattern and in 68% noisy breathing was reported with 31% snoring. Finally, it is common to find
M Hurley MRCP is Respiratory Registrar at Nottingham Children’s Hospital, Nottingham, UK. Conflict of interest: none declared. Harish Vyas DM(Notts) FRCP FRCPCH is Consultant PICU/Respiratory Medicine at Nottingham Children’s Hospital, Nottingham, UK. Conflict of interest: none declared.
PAEDIATRICS AND CHILD HEALTH --:-
1
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
studies in this regard is poor, however one prominent cohort study suggests an improvement in GOR with improving nutritional status and a corresponding reduction in the need for respiratory-led hospital admissions regardless of whether a fundoplication had also been performed. There is considerable doubt as to whether the routine addition of a fundoplication at the time of gastrostomy is an effective means of reducing GOR and there is certainly no robust clinical trial evidence to support its routine use.
Mechanisms of hypoxaemia in children with neurodisability Hypoventilation:
C
Ventilation-perfusion mismatch
C
Right-to-left shunt
C
Diffusion impairment
C
In neuromuscular conditions Both PaCO2 and PACO2 increase during hypoventilation Ventilatory support may be necessary in severe hypercapnoea PAO2 decrease: Responsive to oxygen Common in seizures, obstruction, interstitial disease PaO2 low, variable PaCO2 (normal or high) Due to pneumonia/ARDS Low PaO2 variable PaCO2 (normal or high) Interstitial disease/co-exist with V/Q mismatch PaCO2 normal
Respiratory muscles The respiratory ‘pump’ may be compromised either by respiratory muscle weakness (intercostal, accessory and diaphragmatic), incoordination, paradoxical breathing and inefficient angle of work due to scoliosis/kyphoscoliosis, or a combination of the above. These problems will, in turn, be exacerbated by poor nutritional status, which is probably the simplest problem to tackle. Scoliosis repair is often technically difficult to undertake, as is the decision regarding optimal timing. It is not uncommonly undertaken although even case series reports are limited to short-term outcomes and a relatively high complication rate. Those at particular risk of requiring intensive care following their procedure appear to be those with reduced lung function, a greater Cobb angle and higher number of vertebrae involved.
Table 1
qualitative studies that document a positive outcome in terms of the family unit appear to out-number those that identify concerns. One unresolved clinical issue with the siting of a gastrostomy is the concern that GOR will be exacerbated. Again, the quality of
Sleep Sleep disorders are common in children with cerebral palsy, particularly with regard to disorders of initiating and maintaining sleep (22%), sleep-breathing disorders (14%), disorders of arousal (10%), sleepewake transition disorders (15%), disorders of excessive somnolence (13%), and hyperhidrosis (7%). Overnight oximetry should be considered early in the assessment of a child with upper airway symptoms and should be repeated at intervals. In children with multi-system involvement formal polysomnography may also have a role. Non-invasive interventions with regard to sleep positioning and sleep systems have been used and a forthcoming Cochrane review will examine the evidence for these interventions.
Summary of problems and clinical manifestation Problem
Clinical consequence
Feeding problems Reflux: antegrade or retrograde
C
C C C C C C
C
Respiratory muscle weakness
C C C
Upper airways
C C C
C C C C
Sleep
C C
Kyphoscoliosis
C
Wheezing that is poorly responsive to appropriate beta-agonists or steroids Failure to thrive/Teeth erosion Atelectasis and bronchiectasis Recurrent pneumonia/cough Stridor or laryngitis/hoarseness Pulmonary abscess Apnoea/bradycardia/acute life-threatening events Recurrent tonsillitis/discharging ears Paradoxical breathing Poor secretory clearance Development of kyphoscoliosis Laryngomalacia, tracheomalacia Tonsil/Adenoids Pharyngeal collapse due to palatal hypotonia Macroglossia/small pharyngeal space Glossoptosis/Small mandible Sub-glottic stenosis Obesity Initiating or maintaining sleep Sleep breathing disorders Increasing
Upper airway Problems with the upper airway are very common in children with neurodisability. Upper airway musculature and laryngomalacia may contribute to impending respiratory failure due to upper airway obstruction. To an extent these can be alleviated by surgical approaches but any intervention runs the risk of both short and longer term complications. In general, simpler solutions higher up the airways, such as adenotonsillectomy are unlikely to result in longer term problems but are less likely to offer a complete solution whilst more definitive surgery such as tracheostomy requires significant lifelong postoperative care. Overnight oxygen or non-invasive ventilation may help but their initiation needs to be carefully planned in conjunction with the family. It is important to define an individualised management plan with clear goals of the outcomes to be achieved. Infection Because of the high aspiration risk empirical antibiotic use should cover gut organisms and so broad-spectrum agents (co-
Table 2
PAEDIATRICS AND CHILD HEALTH --:-
2
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
bleeding and perforation, hyperglycaemia, hypertension, hypertrophic cardiomyopathy and growth failure. It also documented increased risks of developmental delay (in one study), cerebral palsy and abnormal neurological examination in the limited number of included trials that reported late outcomes. Commentators have recommended against the use of early corticosteroids due to the higher risk of adverse events and reserving the use of late corticosteroids to those babies who cannot be weaned from ventilation.
amoxiclav, third generation cephalosporins) are often used. Unfortunately for patients with poor airway clearance, bronchiectasis and chronic exposure to broad spectrum antibiotics, the lung microbiota is likely to be shifted and a niche for opportunistic organisms such as Pseudomonas aeruginosa may result. As with other areas of care the evidence supporting management of such problems are extrapolated from other areas and so, as with CF, isolation of P. aeruginosa is often treated with successive eradication regimens of varying durations of ciprofloxacin and nebulized colistin. Many patients receive prophylactic antibiotics by way of thrice-weekly azithromycin. Unfortunately as children with neurodisabilities often find it difficult to expectorate, reliable respiratory sampling is uncommon and so the sensitivity of upper airway cultures is questionable. The identification of pathogens may be delayed as a result. As with CF, whether upper isolates reflect lower airway infection is also controversial, however BAL is often reserved for those with chronic chest X-ray changes or lobar collapse.
Insult at birth A poor neurological outcome often results for children who require ventilation at birth, in one series 11/45 children died or suffered severe neurodisability. While babies who present with respiratory insufficiency are likely to have sustained a multiorgan insult, the sequelae of ventilation including upper airway complications (e.g. sub-glottic stenosis) must be also considered as should the suggestion that respiratory compromise may form the presenting feature of a neurological insult.
Specific conditions
Cerebral palsy Those with cerebral palsy not only have the effects of the primary brain lesion but are also likely to have problems of the associated co-morbid conditions including epilepsy, hearing impairment, abnormal muscle tone and posture and nutritional insufficiency. Those with cerebral palsy appear to be particularly severely affected with GOR, which may be particularly difficult to manage.
Prematurity The outcomes of babies born extremely prematurely have been well documented by the series of EPICure studies and deserve particular consideration, not least because children born extremely prematurely have significant risks for severe neurodisability but also with significant respiratory sequelae. In terms of short-term outcomes of those children born at between 22 and 26 weeks gestation in 2006, 68% had BPD and 13% had evidence of severe abnormality on cerebral ultrasonography at hospital discharge. Later at 11 years of age, those born extremely prematurely (25 weeks gestation) and with most of whom attending mainstream school, they had lower lung function than their age-matched control friends. Among those born extremely prematurely, those who had a past history of BPD had significantly higher rates of wheeze and bronchial hyper-reactivity. An association with impaired haemodynamics suggests that impaired lung physiology should be interpreted within the context of abnormal arterial stiffness, adding to the risk factors for morbidity in later life. Even relatively modest degrees of prematurity may be associated with poor later respiratory outcomes. The risk of emergency admission to hospital from birth to the age of 5 years decreases with each successive additional week in gestation up to 40e42 weeks (hazard ratio for respiratory admission after birth between 33 and 34 weeks gestation 1.59 (95% CI 1.50e1.68)). Managing the respiratory sequelae of preterm birth is controversial and the subject of a series of Cochrane reviews considering the use of corticosteroids both to prevent and treat chronic lung disease of prematurity. Corticosteroid administration is associated with reductions in the risk of failure to extubate and death or chronic lung disease at 28 days or 36 weeks corrected gestational age. The review considering late-administered corticosteroids reported a reduction in risk of clinically important impairments of forced expired volume in one second (FEV1) of children undertaking spirometry later in childhood. The review examining early-administered corticosteroids however reported a greater burden of adverse effects including gastrointestinal
PAEDIATRICS AND CHILD HEALTH --:-
Ataxia telangiectasia Ataxia telangiectasia (AT) is a progressive, neurodegenerative disease causing immunodeficiency and an increased risk of malignancy and respiratory disease that commonly includes chronic sinopulmonary infection, aspiration, bronchiectasis and interstitial lung disease. Infection drives much of the respiratory morbidity in AT with 74% of patients attending the UK national clinic being prescribed a chronic prophylactic antibiotic. Much of the evidence informing the treatment of these children is extrapolated from cystic fibrosis and accordingly, those with severe lung disease in AT are chronically infected with P. aeruginosa and Burkholderia cepacia complex. Myelomeningocele/Arnold-Chiari malformation Children with spina bifida may also have a Chiari II malformation, which may cause central apnoea and dysphagia. An excessive gag reflex can be confused with GOR and associated vocal cord dysfunction may require treatment with tracheostomy, the combination of all of the above make a gastrostomy a priority.
Summary Unfortunately for children with neurodisability robust evidence to guide decisions about optimal respiratory care is lacking. Particularly difficult decisions regarding the use of intensive care and long-term ventilation must be taken in partnership with the patient’s family and with clear goals of what is expected to be achieved. The use of gastrostomies appears to be accompanied by improvement of nutritional status while the routine accompaniment of fundoplication is controversial. The assessment of
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Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013
SYMPOSIUM: SPECIAL NEEDS
11 Vernon-Roberts A, Sullivan PB. Fundoplication versus postoperative medication for gastro-oesophageal reflux in children with neurological impairment undergoing gastrostomy. Cochrane Database Syst Rev 2013. Issue 8. Art. No.:CD006151. 12 Romeo DM, Brogna C, Quintiliani M, et al. Sleep disorders in children with cerebral palsy: neurodevelopmental and behavioral correlates. Sleep Med 2014; 15: 213e8. 13 Wood NS, Marlow N, Costeloe K, et al. Neurologic and developmental disability after extremely preterm birth. EPICure Study Group. N Engl J Med 2000; 343: 378e84. 14 Fawke J, Lum S, Kirkby J, et al. Lung function and respiratory symptoms at 11 years in children born extremely preterm: the EPICure study. Am J Respir Crit Care Med 2010; 182: 237e45. 15 Seddon PC, Khan Y. Respiratory problems in children with neurological impairment. Arch Dis Child 2003; 88: 75e8.
children with neurodisability is multiprofessional and due to the nature of the condition interval assessments are often warranted. A FURTHER READING 1 Hardelid P, Dattani N, Gilbert R, et al. Estimating the prevalence of chronic conditions in children who die in England, Scotland and Wales: a data linkage cohort study. BMJ Open 2014; 4: e005331. 2 Proesmans M, Vreys M, Huenaerts E, et al. Respiratory morbidity in children with profound intellectual and multiple disability. Pediatr Pulmonol 2014; http://dx.doi.org/10.1002/ppul.23114. 3 Weir KA, McMahon S, Taylor S, et al. Oropharyngeal aspiration and silent aspiration in children. Chest 2011; 140: 589e97. 4 Trinick R, Johnston N, Dalzell AM, et al. Reflux aspiration in children with neurodisabilityea significant problem, but can we measure it? J Pediatr Surg 2012; 47: 291e8. 5 Gantasala S, Sullivan PB, Thomas AG. Gastrostomy feeding versus oral feeding alone for children with cerebral palsy. Cochrane Database Syst Rev 2013. Issue 7. Art. No.:CD003943. 6 Sullivan PB, Juszczak E, Bachlet AM, et al. Gastrostomy tube feeding in children with cerebral palsy: a prospective, longitudinal study. Dev Med Child Neurol 2005; 47: 77e85. 7 Sullivan PB, Morrice JS, Vernon-Roberts A, et al. Does gastrostomy tube feeding in children with cerebral palsy increase the risk of respiratory morbidity? Arch Dis Child 2006; 91: 478e82. 8 Srinivasan R, Irvine T, Dalzell M. Indications for percutaneous endoscopic gastrostomy and procedure-related outcome. J Pediatr Gastroenterol Nutr 2009; 49: 584e8. 9 Townsend JL, Craig G, Lawson M, et al. Cost-effectiveness of gastrostomy placement for children with neurodevelopmental disability. Arch Dis Child 2008; 93: 873e7. 10 Hauer JM. Does Nissen fundoplication decrease reflux-related problems including respiratory illness in children with neurological impairment? JAMA Pediatr 2014; 168: 188.
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All children with any degree of neurodisability should be reviewed by respiratory paediatrician to optimise care and reduce longterm complications Oropharyngeal aspiration is common in children with feeding difficulties and may lead to bronchiectasis Diagnosing reflux aspiration may be problematic The effectiveness of routine addition of a fundoplication at the time of gastrostomy as an effective means of reducing GOR is still uncertain Overnight oximetry should be considered early in the assessment of a child with upper airway symptoms and should be repeated at intervals An ENT review and intervention may be necessary if the sleep study demonstrates significant obstructive picture Azithromycin prophylactic antibiotics and pseudomonas clearance may reduce hospital admissions
Ó 2015 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Hurley M, Vyas H, Respiratory problems in children with neurodisability, Paediatrics and Child Health (2015), http://dx.doi.org/10.1016/j.paed.2015.06.013