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Rosie (15 years) has Cri du Chat syndrome. She was admitted to hospital in April 2004 for elective spinal surgery. Two days later she presented with increasing respiratory distress (SaO2 80%, increased respiratory rate, requiring frequent suctioning), and was placed nil by mouth by her medical team. One week post-surgery she was continuing to deteriorate and subsequently was intubated and ventilated for four days. I saw her for speech and language therapy swallowing assessment six days after extubation. A swallow was present but cervical auscultation showed it was significantly delayed with severe pharyngeal residue. Rosie also had excessive oral and pharyngeal secretions and a decreased SaO2 / increased respiratory rate with feeding. This was demonstrated by vital signs which were monitored pre/during/post feeding trials. At this stage I recommended she was nil by mouth and referred Rosie for a videofluoroscopy. On videofluoroscopic examination four days later, Rosie took two teaspoons of pure. Poor oral skills were evident, with no sign of bolus formation or attempts at anterior-posterior transfer. There was delay in triggering of a swallow, with residue in the pyriform sinus for 20 seconds, incomplete airway closure, and aspiration before, during and after the swallow. There was no attempt to clear the pharyngeal residue, and only a weak and delayed cough in response to the episodes of aspiration. On the Rosenbek et al. (1996) penetration-aspiration classification, Rosie scored 7. The procedure was stopped at this point due to clinical risk. Following this, Rosie was continued nil by mouth and it was clear at that time to me and the medical team that the prospect of non-oral feeding would have to be discussed with her parents. This assessment process left me with several questions: 1) Rosie was ventilated via an Endotracheal tube for four days. Had the period of ventilation caused any of symptoms seen? This seemed unlikely at this stage post-extubation to be a significant causal factor. 2) What was the effect of Rosies underlying neurological diagnosis on her swallow? Cri du Chat is a chromosome disorder with characteristics which can include hypotonia / failure to thrive / learning difficulties. The literature regarding feeding disorders points towards nutrition as the main issue, although there are some reports of chronic aspiration in the syndrome. 3) How much was acute respiratory illness affecting Rosies swallow? 4) What was recovery potential? At this stage I was pessimistic and felt that Rosie would require long-term non-oral feeding due to the severity of her dysphagia. 5) Was this dysphagia primarily neurological or respiratory? My initial feelings were that Rosie had been chronically and silently aspirating for some time. I felt that the combination of this, her poor nutrition on admission and the anaesthetic had led to an acute aspiration episode that had resulted in her current respiratory difficulties. My feeling therefore was that her underlying neurological condition was the primary cause of her dysphagia, but that it had been compounded by respiratory difficulties. Rosie continued on nasogastric tube feeds. Gastrostomy was discussed with but refused by her parents as they felt oral feeding was of great importance to Rosie, so wanted it pursued if at all possible. We therefore agreed as a

team to do a repeat videofluoroscopy when her respiratory status improved to the extent that: 1. Suction / chest physiotherapy was no longer needed 2. Oxygen saturations were 90% or above in air 3. Secretions were clear in colouring. At this repeat videofluoroscopy (six weeks post-extubation / four weeks post-initial videofluoroscopy), I assessed Rosie with pure (x 4 teaspoons), soft solid (1 mouthful) and fluid (20mls @40% weight for volume). Oral intake was small because of Rosies limited ability to comply, but it was clear that her oral stage had improved, with good bolus formation and transition. Her swallow was initiated within normal limits and there was no aspiration or penetration (Rosenbek et al., 1996, classification = 1). The mild post-swallow residue in her pyriform sinus was cleared, and Rosie was started on a full oral diet and normal fluids as she had been prior to admission. She has had no further complications with regard to safety of feeding, although nutrition remains a concern. I now had some answers to my questions. Contrary to my initial thinking, Rosies dysphagia was primarily respiratory-based. Her recovery was full and correlated with respiratory recovery. So how do we ensure we take sufficient cognisance of the impact of respiratory disorders in older children? First, we need to familiarise ourselves with the anatomy and physiology of normal, spontaneous ventilation, and understand how this appears to change from infancy to adulthood. It is clear to me that children generally have less leeway in their system to enable them to cope without having their system compromised in the event of a respiratory problem. Next, we need to be aware of the aetiologies that put children at risk of aspiration. If a child has any of the following they are likely to have a degree of dysphagia and their respiratory status should be considered as contributing significantly to their potential dysphagia: Chronic lung disease Bronchopulmonary dysplasia Apnoea Airway Malacias Transoesophageal fistula Respiratory distress syndrome Central hypoventilation syndrome Spinal cord injury Neuromuscular myopathies. These disorders may result in the child needing a tracheostomy, ventilation or speaking valves. While a tracheostomy has not been found to cause aspiration (Leder & Ross, 2000), it should be considered as a potential exacerbating factor for these children. As more and more children are having ventilation needs met in the community, speech and language therapists need to develop basic understanding of the implications of ventilation modes, ranging from full ventilatory support, continuous positive airway pressure (CPAP) and bilevel positive airway pressure (BIPAP). Children who have high positive end expiratory pressure (PEEP) requirements also present their own challenges; further information about the importance of the timing of trial swallows with such children is in Bleile (1993). There is conflicting evidence about whether tube occlusion / speaking valves (specifically the Passy-Muir valve) are influential in reducing the level of aspiration in a patient with a tracheostomy (Leder et al., 2001; Suiter et al., 2003). However, my interpretation is that, if using a Passy-Muir valve with ventilated patients, we should at least discuss with the medical team the possibility of decreasing the level of positive end expiratory pressure to allow for the restoration of physiological PEEP that the valve may bring.

Fit for assessment

Speech and language therapists should be able to make basic observations and judgements regarding a childs respiratory status and whether they are fit for a swallowing assessment. This should be considered by therapists working both in a hospital and home / school settings with respiratory-compromised children. Guidelines for establishing basic respiratory function are in figure 1 (nos.



1 and 2 reproduced by kind permission of Rachel Hufton, senior respiratory physiotherapist at Manchester Childrens Hospital). In my experience it is best to practise using a combination of patient observations, medical information and monitoring equipment in your own assessment - do not reply on one feature on its own. Figure 1 Establishing basic respiratory function
1. Obtain medical information (medical notes / nursing charts / parental reports) - How is handling tolerated: decreases in saturations / heart rate / respiratory rate? - How long does child take to recover from this? - Incidences of bradycardia (decreased heart rate) - Apnoeic spells (no respiration for > 20 secs /< 20 secs with bradycardia) - Is apnoea related to specific stimuli such as feeding / handling / position? - Temperature - < 36.5C is a contraindication for non-essential handling. 2. Observe for signs of respiratory distress - Recession: ribs and sternum pulled in on recession - Tachpnoea: respiratory rate > 60 - Grunting (attempts to expire against partially closed glottis to increase functional residual capacity of the lungs) - Neck extension - Nasal flaring - Stridor: due to inflammation / obstruction of larynx - Head bobbing: caused by use of accessory muscles of respiration - Pallor specifically reduced colour - Barrel-shaped chest: air trapping, as in bronchiolitis or asthma (NB: those in red are the most significant and are contraindications for a swallowing assessment) 3. Use monitoring equipment. Although this is mainly available in hospital, it can also be used with children at home. Normal values of note (Davies & Hassell, 2000) include: - Respiratory rate: 40-60 (newborn), 20-30 (1-6 years), 15-20 (>7 years) - Heart rate: 100-200 (newborn), 100-180 (1-3 years), 70-150 (>3 years) - Blood gases: ph 7.30-7.40 (newborn), 7.35-7.45 (>3 years) - Oxygen saturations: individual levels should be considered to ascertain what is an acceptable lower level. Do not rely on saturations as an independent indicator of respiratory status. - Cervical auscultation: to establish baseline sounds of respiration. It is essential that you undertake auscultation before oral intake, to provide a benchmark for auscultation during feeding.

3. Residue This may present both via vital signs ( saturations, heart rate / respiratory rate) as well as being audible on cervical auscultation. The residue may develop cumulatively or appear suddenly. The ability to clear this residue should be monitored during the assessment as well as its effect on respiratory status. In addition, videofluroscopy assessment enables more detailed analysis of the oral and pharyngeal stages, specifically triggering of swallow, aspiration / penetration status and pharyngeal function. Features observed may include: 4. Triggering of the swallow The swallow may be triggered appropriately with hyoid elevation beginning when the bolus is at the level of faucial arches valleculae. Research has indicated that elicitation of the swallow reflex is not dependent on stimulation of the anterior tonsillar pillars (Ali et al.,1996). Therefore, in my clinical practice, I accept triggering of the swallow at the level of the valleculae as within normal limits. In my clinical experience, it is likely that triggering of the swallow will be affected, and that the degree of this delay seems to be commensurate with the degree of respiratory impairment that is, patients with severely impaired respiratory function can show significant delay, to the level of the laryngeal vestibule. 5. Incomplete airway closure Alongside delay in initiation of the swallow there is often incomplete airway closure leading to deep penetration (aspirate penetrating below the top of the arytenoids and the top of the thyroid cartilage above the true vocal cords), and aspiration. Patients I have seen with acute respiratory disorders consistently show incomplete closure. 6. Pharyngeal residue This is caused by poor pharyngeal motility / sensation and can lead to aspiration, usually after the swallow. An inability to clear or to recognise this residue has been seen in children with acute respiratory disorders. The experience of working with Rosie and reading up on respiratory disorders has taught me several things: 1. Never underestimate the impact of respiratory disorders - whether acute or chronic - on swallowing. 2. Consider the timing of instrumental assessments, as results obtained when a patient is compromised may be misleading in terms of cause of disorder and prognosis. 3. Characteristics of swallow dysfunction that have a high correlation with neurological impairment are also seen in respiratory-compromised patients. 4. Respiratory dysphagia can exist independent of other factors (for example neurological ones), but has similar clinical features to neurological dysphagia. Consideration of the impact of respiratory status is essential in making an appropriate prognosis and management plan. Rebecca Howarth is a senior specialist speech and language therapist at Manchester Childrens Hospital / Central Manchester PCT.
Ali, G., Laundl, T.M, Wallace, K.L., deCarle, D.J. & Cook, I.J. (1996) Influence of cold stimulation on the normal pharyngeal swallow response, Dysphagia 11(1), pp. 2-8. Bleile, K. (1993) The care of children with long term tracheostomies. San Diego: Singular Publishing Group. Davies, J. & Hassell, L. (2000) Children in Intensive Care: A Nurses Survival Guide. London: Churchill Livingtone. Leder, S.B. & Ross, D.A. (2000) Investigation of the causal relationship between tracheostomy and aspiration in the acute care setting, Laryngoscope 110(4), pp. 641-644. Leder, S.B., Jo, J.K., Hill, S.E. & Traube, M. (2001) Effect of tracheotomy tube occlusion on upper esophageal sphincter and pharyngeal pressures in aspirating and non- aspirating patients, Dysphagia 16(2), pp. 79-82. Rosen, C.L., Glaze, D.G. & Frost, J.D. (1984) Hypoxemia associated with feeding in preterm infant and full term neonate, American Journal Diseases in Children 138, pp. 623-628. Rosenbek, J.C., Robbins, J.A., Roecker, E.B., Coyle, J.L. & Wood, J.L. (1996) A penetrationaspiration scale, Dysphagia 11(2), pp. 93-98. Suiter, D.M., McCullough, G.H. & Powell, P.W. (2003) Effects of cuff deflation and one way tracheostomy speaking valve placement on swallow physiology, Dysphagia 18(4), pp. 284-292.

Common characteristics
In the young infant, coordination and regulation of breathing and feeding is vital to ensure successful feeding (Rosen et al., 1984). Less well documented is the relationship between respiration and swallowing in the older child. Here, I will attempt to clarify the assessment process and identify dysphagic characteristics that from my experience are common in respiratory-compromised children. It may be that respiratory recovery brings with it recovery from the dysphagia. Before assessment / observation of feeding, it is useful to make yourself aware of typical respiratory parameters. By this I mean what are the acceptable limits for the child in terms of vital signs, pallor and effort of breathing? This information can be determined via monitoring equipment or obtained from parents / carers. The following assessments can be made without the use of videofluoroscopy (at home / in the community), using observation, monitoring equipment, carer information and cervical auscultation: 1. Swallow fatigue This can present as changes in vital signs, decrease in strength and mistiming of the swallow on cervical auscultation. It can also show through the development of pharyngeal residue as heard on cervical auscultation and typically presenting as a deterioration in skills. Such children show normal swallowing features at the beginning of the meal that deteriorate either as quickly as the initial two to three swallows, or develop over a longer period such as a ten minute meal. 2. Variable swallow strength This is most evident with the use of cervical auscultation when a variance in the strength of swallow sounds may be heard. This occurs even though the child is taking consistent volumes during the feed, and can be most evident when assessing fluids (Cichero et al., 1998).



For further information about Cri du Chat syndrome and support groups, see www.cafamily.org.uk/Direct/c81.html