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

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

The sound patterns you describe likely reflect decreased intrinsic
muscle control. How differentiated are his vowels? at his age he should
have a range of clear high to low vowels that are distinct. And the
lingual contacts he seems to be missing for consonant production, to
facilitate babbling, are likely absent due to the hypotonia as well.
Direct work on the tongue and BOT, and use of  biters and teethers and
blow toys would likely help.
 
One thing to think about regarding the red premie nipple. It flows very
fast and does not require good suction; flow will be released with
merely up-down "compression" of the tongue. So he could be using that
nipple without having effective intrinsic tongue control. Likely due to
the hypotonia. He would likely do better with a nipple that offers more
proprioceptive input, i.e.. a firmer one, and one that has a more
controlled flow. Another issue is that higher flow, which the premie
nipple delivers, has been shown to increase work of breathing. The added
work of needing to swallow more often with such a nipple, and therefore
have less time to breathe, actually increases fatigue.What about a
Habermann feeder for flow control and  central grooving? 
 
I'd also suggest moving toward more "weighted" heavier pureeds by spoon
that offer a higher sensory "load" than thin purees like yogurt. They
will also facilitate more intrinsic tongue control and differentiation
of the intrinsic tongue muscle groups. There are also direct
interventions to the base of tongue and blade of tongue that can
increase stability and control as well for more efficient movement.
 
May want to try "slurping" thickened liquids off the side edge of a
spoon to start. Sounds to me like he is wise to refuse the cup right
now, as he seems to lack the motor prerequisites at this time. While
age-wise mom may want him to try a cup, he does not sound ready.
Thickened liquids offered by spoon to get him ready will provide the
weighted fluid again necessary to facilitate intrinsic tongue muscle
control and the sensory "load" to help facilitate central grooving and
tongue cupping.
 
Overall postural control is likely playing a roll too. At his age he
should be able to start moving out of sitting and having some balance
reactions. He may be more "stuck" in sitting as some of these kids are,
due to poor postural stability. This then provides a poor base of
stability, proximally, for the distal work of feeding.
 
Does he receive OT? May be helpful to help organize the sensory motor
system and work more specifically on head/neck/shoulder girdle, ribcage
for better sound production (along with SLP work on this too :-)
 
Lots of issues for sure, but great for problem-solving!
 
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: Tuesday, June 28, 2005 7:32 PM
To: Shaker, Catherine S.; asha-div13@lists.asha.org
Cc: dysphagia@b9.com
Subject: RE: [asha-div13] Prader Willi Syndrome



Thank you for your very detailed response.  He does have some hypotonia
but less than I have seen in other Prader Willi babies that I have
evaluated.  I've never had the opportunity to follow any of them.  He
does not have much sound production.  Still primarily open vowels and
even these are not frequent.  Until recently he almost never cried.  I
can certainly understand your point that this would impact endurance for
feeding.  Endurance is an issue.  He often takes 1-2 oz. PO and the rest
is given by tube.  He also demonstrates difficulty in getting organized
to start sucking and sometimes will not take anything.  We did use a red
preemie nipple and he continues to use these.  We haven't changed
because we did not want to add another aspect to his refusal.  When he
sucks it is adequately strong and well coordinated.  

He has not seen an ENT re the laryngomalacia.  It was very apparent
early on and the distress then disappeared.  I assumed that it was no
longer a problem but your suggestion about an ENT follow up is a good
one.  

On the GI front, he has had significant reflux from early on and we have
gotten him onto Prevacid which appears to resolve the problem ie no more
spitting up and no more GI distress which was quite apparent in the
beginning.  Delayed stomach emptying is a good thought.  Mom does not
test for residuals.  We may be able to do this or do a GI workup.  He is
fed q3 hours.  I"ve just asked  Mom to go to q4 during the day, to see
if this changes his acceptance.  I just spoke with her and when she
tried to increase him from 5.5 to 6oz. he spit up.

I was involved in the swallow study and will need to pull it up again to
see if I noted or now note on review any of the problems tha tyou list.
We did not look at the fatigue factor.  It was done during the period
when he had the laryngomalacia.  

He has refused cup drinking.  He does close to clear the spoon and there
is minimal dribbling.  I do, however suspect that he is not cupping his
tongue and will look at that.

He sits very well.  Brings toys to his mouth.  Is not crawling or
pulling to stand.  Received PT through EIP weekly.  He has been on
growth hormone since about 2months of age.  This is reported to improve
muscle as well as growth.

He has been on breast milk which does change flavors in a way that
formula cannot.  In spoon feeding he initially seemed to have a
preference for bananas but is now more hesitant.  Presently likes yogurt
(not sure what flavor it was when I saw him the other day.  

Lots of ideas to follow up on.  Mom was very excited when I told her
about the many responses.  She is a social worker by training.  I plan
to see her to discuss all of the comments and to assess more of the
issues once I digest all of these wonderful comments.  Thanks to all.


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




>From: "Shaker, Catherine S." <CShaker@covhealth.org>
>To: "Hilda Pressman" <hpressman@msn.com>,"Division 13 Discussion List"
<asha-div13@lists.asha.org>
>CC: <dysphagia@b9.com>
>Subject: 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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