ACQ Vol 11 No 1 2009

MULTICULTURALISM AND DYSPHAGIA

gastrointestinal problems, where a child receives a negative experience in response to eating or drinking. In addition, infants who have required tube feeding since infancy, or for prolonged periods, may not have fully established a relation­ ship between oral intake and a desire to eat, or may never have experienced hunger or thirst. Behavioural feeding dif­ ficulties can also be seen as part of the larger behavioural phenotype associated with this condition, with young children often preferring structure, routine and showing strong food preferences. A multidisciplinary approach is essential to rule out any ongoing organic reason for food refusal, prior to behavioural intervention. Oral motor development Delayed global motor development can lead to a delay in maturation of chewing skills, and thus a delay in moving on to more textured foods (Eicher et al., 2000). Anecdotal experience shows parents often report unchewed food pocketed in the mouth “hours” after a meal. Children tend to gain skills as their global motor development improves. Parental support regarding graded texture progression and awareness of encouraging the development of feeding milestones is often critical. Otolaryngologic issues Congenital airway and vascular anomalies are reported in patients with 22q11DS. Dyce et al. (2002) performed a retrospective medical review of 102 children with 22q11DS to determine presence of airway difficulties and found 14% to have laryngotracheal anomalies such as subglottic narrowing, vocal cord paralysis, tracheomalacia, and laryngeal cleft. Vascular problems such as vascular rings and aortic arches with tracheobronchial compression were also found. An absent, hoarse, or high-pitched cry indicates a need for onward referral to otolaryngology. The presence of any of the aforementioned difficulties significantly increases the risk of feeding or swallowing problems and a subsequent vulnerability to aspiration. A detailed swallowing assessment including objective measures should be undertaken where Swallowing difficulties are reported in approximately 10% of children with 22q11DS (Eicher et al., 2000). Difficulties at all phases of the swallow have been described in the literature, with particular attention paid to the crico-oesophageal phase. Eicher et al. (2000) and Rommell, Davidson, Cain, Hebbard and Omari (2008) describe difficulties with material passing smoothly from the pharynx into the oesophagus. The aetiology of this problem and appropriate management is unknown. Rommell et al. (2008) assessed four patients with crico- oesophageal phase swallowing difficulties, combining manometry with videofluoroscopy swallow study (VFSS). Each patient was reported to present with a unique pattern of cricopharyngeus movement, thus requiring different manage­ ment in each case. Assessment of pulmonary consequences of aspiration, both chronic and acute, can be difficult to determine due to potentially altered immunology, as outlined earlier. An objective assessment such as VFSS or fibre endoscopic evaluation of swallow should be carried out whenever aspiration is suspected. At Great Ormond Street Hospital, our clinical experience using VFSS indicates thin fluids and sticky textures prove more problematic for patients with 22q11DS. The use of thickened fluids, and avoidance of textured substances, often reduces aspiration risk. structural difficulties are reported. Swallowing difficulties

Palatal anomalies/velopharyngeal dysfunction (VPD)

The incidence of VPD in patients with 22q11DS is reported to be at least 70% (Solot et al., 2001). Within this group, there may be an obvious structural defect such as cleft palate (10%) or submucous cleft palate/bifid uvula (20%). In other cases, VPD exists because of multiple additional factors, resulting in an inability of the soft palate and the posterior pharyngeal wall to shut off effectively and with appropriate timing. The relationship between VPD in feeding and subsequent speech development is not known. Research has shown that palate movement during speech and swallowing differs Nohara, K., Kotani, Y., Ojima, M., Sasao, Y., Techimura, T., & Sakai, T. (2007), and one should thus not assume that VPD associated with feeding will automatically transfer to VPD during speech. Early feeding efficiency in patients experiencing VPD may be characterised by a lack of negative pressure in the oral cavity, leading to decreased sucking efficiency. As a result, only small boluses of milk are extruded from the nipple/teat per suck. The infant with feeding-related VPD may appear to feed vigorously over time but only manage small volumes, experiencing associated limited weight gain. The infant may also present as a frequent feeder, falling asleep regularly on the breast or bottle. Clinical assessment may show a fast suck­ ing rate or nasal regurgitation. Strategies which are commonly applied to the cleft population such as adapted bottles and positioning may increase feeding efficiency. Cardiac issues Approximately 75% of infants with 22q11DS experience some type of cardiac problem (McDonald-McGinn, 2004). It is widely acknowledged that infants with unresolved cardiac problems present as breathless, tiring quickly during feeds, and are frequently disorganised in terms of suck–swallow– breathe. Often problems can be resolved by presenting the infant with frequent, short, high energy feeds, although some­ times a period of tube feeding is required. Feeding problems should resolve as cardiac conditions improve. Persistent hoarse or absent voice post cardiac surgery, especially accompanied by deterioration in feeding skills, may indicate vocal fold damage or paralysis, as a result of intubation injuries or re­ current laryngeal nerve damage. The infant may therefore be more vulnerable to swallowing difficulties, and subsequent aspiration. Gastrointestinal issues There is general agreement in the literature that infants with 22q11DS are prone to various gastrointestinal difficulties, including reflux and constipation (Eicher et al., 2000). These difficulties can lead to smaller food volumes taken, poor weight gain, food refusal, and slower transition on to more textured food. Oesophageal atresia and tracheo-oesophageal fistula are reported. Referrals for a detailed gastroenterological examination are critical in patients where problems are suspected, in an attempt to minimise potential long-term feeding problems. Eicher et al. (2000) indicated that following the treatment of reflux, patients showed better progression with food textures, in contrast to oral motor therapy alone. Behavioural feeding difficulties Behavioural difficulties, or “fussy” or “aversive” feeding behaviours, are reported. These may occur as a result of complex medical interventions, where a child is repeatedly exposed to negative experiences around the face, or to

ACQ uiring knowledge in sp eech , language and hearing , Volume 11, Number 1 2009

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