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

[pressmah at sjhmc.org (pressmah@sjhmc.org)]

No cleft palate in this case

-----Original Message-----
From: smhjr@surfbest.net [mailto:smhjr@surfbest.net]
Sent: Tuesday, June 28, 2005 1:37 PM
To: CShaker@covhealth.org
Cc: hpressman@msn.com; Dysphagia@b9.com
Subject: RE: [Dysphagia] RE: [asha-div13] Prader Willi Syndrome


I have seen Prader Willi children with submuccousal clefts. Could
this combined with some hypotonia be causing the child swallowing
problems?

---- Original Message ----
From: CShaker@covhealth.org
To: hpressman@msn.com, asha-div13@lists.asha.org
Subject: RE: [Dysphagia] RE: [asha-div13] Prader Willi Syndrome
Date: Tue, 28 Jun 2005 05:41:40 -0500

>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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