[Dysphagia] RE: [asha-div13] Prader Willi Syndrome

[CShaker at covhealth.org (Shaker, Catherine S.)]

I would wonder if there is still a level of hypotonia that, while it is
not "overtly noticeable" as in some Praeder Willi infants, it is
interfering with function...what is his sound production like in terms
of differentiated intrinsic tongue muscle control? vowel variety?
strength of consonant contacts? laryngeal control for loudness, pitch
variability? does he babble and if so what is the quality in terms of CV
transitions for example. I find that often these observations in infants
provide insight into true oral-motor control.This then would impact
endurance for feeding (due to oral-motor fatigue, often seen in
conjunction with hypotonia) and precision/control/refinement of skills
with bottle, spoon and cup. 
 
If he required a soft nipple early on does that perhaps reflect some
decreased integrity in intrinsic tongue muscles? When this is the case,
I have typically seen it persist well beyond infancy with Praeder Willi.
 
Also has the laryngomalacia completely resolved per ENT flexible
fiberoptic laryngoscopy? I have seen some infants diagnosed with
laryngomalacia without overt audible signs. If we do not have objective
data from ENT showing resolution, there may be some compensations on the
infant's part to limit feeding due to stressing airway maintenance. 
 
The other piece may be GI issues. These babies often have associated GI
issues that reduce drive to eat, and impact GI motility (related to
proximal hypotonia) without again the presence of overt signs.
 
Regarding the Swallow Study, while it sounds like there was no
aspiration, was there any observed difference in swallowing physiology
related to hypotonia? Was there a built in fatigue factor observed?
(i.e. feeding off line and then re-starting fluoro similar to mealtime
length?) often these babies can do ok initially at a meal but then
through the course of a meal, over time, both skills and safety degrade.
If there were airway compromise, perhaps it may be then silent, due to
likely a decreased sensory responsiveness typically seen in the presence
of even subtle hypotonia.
 
Clinically, what is bolus control like with cup drinking? Is there
active thinning and cupping of the tongue with cup drinking? If not,
this again may reflect less overt but clear problems with intrinsic
tongue control. How effective are the lips at cleaning the spoon?  How
stable is the jaw with biting on toys or hard solids?
 
How is he developing otherwise, e.g. motor control and postural control?
often a decrease in postural control, even subtle, may impact functional
feeding skills in ways you describe.
 
What types of foods does he prefer? Flavors? The sensory "load" of the
foods he is being offered may not be sufficient for his system with a
level of hypotonia, as hypotonia and hyposensation often go hand in
hand.That may be an avenue of intervention as well.
 
 
Just some thoughts.
Keep us posted on this little guy!
 
Catherine
Catherine S. Shaker M.S./CCC, BRS-S 
Speech-Language Pathologist 
Board Recognized Specialist in Swallowing and Swallowing Disorders 


St. Joseph Regional Medical Center 
5000 West Chambers Street 
Milwaukee, WI  53210 
Phone:  414-447-2797 
Fax:      414-874-4104 

 
 
-----Original Message-----
From: Hilda Pressman [mailto:hpressman@msn.com] 
Sent: Monday, June 27, 2005 4:20 PM
To: Division 13 Discussion List
Subject: [asha-div13] Prader Willi Syndrome



I am treating a 10 month old with Prader Willi who had a GT placed at
birth.  I have followed him almost from the beginning.  He never had
significant oral hypotonia and was readily able to learn to suck from a
bottle using a soft nipple.  He initially had laryngomalacia which
interferred with PO feeding to some degree.  Swallow study at about 5
months of age was normal.  He has acquired skills for bottle drinking,
spoon feeding and cup drinking.  The issue is that he continues to take
limited quantities PO and to be dependent on the GT.  

I have been advised by two docs who have seen him that decreased
appetite is often an issue in the first year.  When I did a literature
search the only problem referred to was the hypotonia.  He is now
approaching a year with no significant improvement.  We know that he
will get there and that excessive intake will then be a problem but Mom
says that no one else on the Prader Willi listserve appears to present
with a similar problem.  Does anyone have any experience with this type
of presentation?




Hilda Pressman, MA, CCC SLP BRS-S
Board Recognized Specialist in Swallowing and Swallowing Disorders
Nutritional Management Associates, LLC 
www.nutritionalmanagement.org
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