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ACQ
uiring knowledge
in
sp eech
,
language and hearing
, Volume 11, Number 1 2009
51
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
structural difficulties are reported.
Swallowing difficulties
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.
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