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

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

The underlying concern here is likely qualitative issues regarding tone,
stability, sensory processing, airway maintenance and airway regulation
that in the end, impact intake. 

-----Original Message-----
From: pressmah@sjhmc.org [mailto:pressmah@sjhmc.org] 
Sent: Tuesday, June 28, 2005 1:23 PM
To: scott-dailey@uiowa.edu; smhjr@surfbest.net; Shaker, Catherine S.
Cc: hpressman@msn.com; Dysphagia@b9.com
Subject: RE: [Dysphagia] RE: [asha-div13] Prader Willi Syndrome


The interesting thing about this youngster is that when he does feed he
does so quite funtionally.  I will take another look in his mouth.
Thanks

-----Original Message-----
From: Dailey, Scott [mailto:scott-dailey@uiowa.edu]
Sent: Tuesday, June 28, 2005 1:45 PM
To: smhjr@surfbest.net; CShaker@covhealth.org
Cc: hpressman@msn.com; Dysphagia@b9.com
Subject: RE: [Dysphagia] RE: [asha-div13] Prader Willi Syndrome


Along with hypotonia, I have seen upper airway obstruction (large
adenoids and large tonsils) which can interfere with feeding and result
in obstructive sleep apnea. Hypotonia in the velum probably also
contributes to upper airway obstructions.   These kids have fatigued
quickly likely due to hypotonia and the airway issues.  ENT evaluations
and eventual adenoidectomies and tonsillectomies have helped but not
completely resolved feeding difficulties.

Scott Dailey, M.A., CCC-SLP
Speech-Language Pathologist II
University of Iowa Hospitals & Clinics
200 Hawkins Dr
Iowa City, IA 52242
(319)356-7030

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-----Original Message-----
From: dysphagia-bounces@b9.com [mailto:dysphagia-bounces@b9.com] On
Behalf Of smhjr@surfbest.net
Sent: Tuesday, June 28, 2005 12:37 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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