Download Medical Conditions in Williams Syndrome

Medical Conditions in Williams
Syndrome
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The number of conditions present affects the
occurrence and severity of the feeding
disorder.
Hypotonia and GI dysmotility are prevalent in
the children identified with feeding problems.
Cardiac conditions contribute to feeding
difficulty
Feeding Problems Resulting from
Hypotonia
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Poor postural stability
Weak suck/swallow
Decreased oral sensory awareness/drooling
Low facial tone and strength for manipulation of
puree
Decreased strength and coordination for chewing
Difficulty advancing food texture
Field, Garland, & Williams, May, 2002
Feeding Problems Resulting from
GI Dysmotility/Gastroesophageal
Reflux
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Poor appetite
Food refusal
Limited interest in eating
Inadequate oral intake
Poor weight gain and growth
Field, Garland, & Williams, May, 2002
Twelve Month Old Female with
Hypotonia
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Low oral/facial tone
Poor liquid
manipulation/oral
containment
Inefficient oral transit
Drooling/saliva
management
Lengthy mealtimes
Positioning
Ten Month Old Female with Food
Refusal
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Slow weight gain
Spitting out food
Head turning,
swatting at spoon
Difficult mealtimes
Parental anxiety and
frustration
W.S. Infant s/p Cardiac Surgery
Pharyngeal Dysphagia
ƒ aspiration of liquid
ƒ tube feeding
ƒ oral aversion
Case History
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History
• Williams syndrome
• Cardiovascular (aortic)
repair
• Bilateral renal artery
stenosis
• Gastroesophageal
reflux
• Constipation
• Rapid breathing
• Hoarse vocal quality
„ Medical
Feeding History
• Dysphagia
• Refusal of bottle drinking
• Feeding difficulty
• Increased congestion
• Discontinuation of liquid
• Decreased respiratory rate
• Improvement in vocal quality
Refusal of spoon
Thickened Liquid from Cup
Acceptance
Behavioral Feeding Problems….
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Develop as a result of learned negative association
with eating
Occur when the food texture presented is
overwhelming for the child
May be symptomatic of pharyngeal dysphagia/silent
aspiration
Occur following surgical procedures contributing to
oral aversion
Emerge when the demand to eat is perceived as
intense
Cause stress and anxiety for the parent/caregiver
Stressful mealtimes are reported by the majority of
parents of children with Williams Syndrome in the
first year of life … in their attempts to increase food
intake and improve weight gain.
Feeding problems resolve by age four and children
with W.S. develop chewing skills and the ability to
eat table food. Low oral tone and malocclusion
continue to affect their ability to chew hard solids.
Evaluation
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medical history/feeding history/growth
pattern
non-nutritive movement
posture/overall tone
oral/facial tone
hunger/food seeking
oral structures/intra-oral structures
nutritive movement patterns
feeding trial/meal observation
Treatment Goals
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Evaluate swallowing function and safety
Improve oral motor coordination
Increase swallowing efficiency
Advance developmental food texture
progression
Establish acceptance and positive association
with mealtimes
Mealtime Behavior
Parent report/observation:
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mealtime structure
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refusal behaviors
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seating/positioning
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utensils
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food texture/liquid
Treatment Strategies
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Implement recommendations of MBSS
Jaw/cheek support during bottle drinking
Specialized bottle/nipple systems
Postural support during bottle drinking
Adequate seating & positioning
Modifications in food texture and consistency
Intensive behavioral treatment program
Conclusions
Experience with Williams Syndrome has provided
consistent information that emphasizes the need
for:
„ Early diagnosis
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Comprehensive medical management
Appropriate assessment and intervention of
feeding and swallowing function