[Dysphagia] end stage Shy Drager Syndrome

[bonnieh4455 at sbcglobal.net (Bonnie Heintskill)]

I do home care and I evaluated an end stage Shy Drager Syndrome Patient 2
weeks ago. She also has dysautonomia which affects her blood pressure
regulation and Parkinson symptoms. I looked on the net for information on
the disease.

  She had been in the hospital, had a VFSS which showed aspiration, was put
on thickened liquids and pureed solids, and sent home (after having gone to
the hospital for CHF incident and pneumonia).  I referred patient to hospice
after talking with her primary MD. No matter how much I thickened her
liquids (she refused anything thicker than nectar), she was aspirating on
subsequent swallows due to extreme muscle fatigue. Referring this lady to
hospice was very hard for me, but I tried to be realistic. I saw her the
next day to do more family education, more eval with different techniques
and again found her fatiguing quicker. My recommendations were small amounts
frequently during the day, try Ensure due to it having higher nutritional
value (family complied) and more viscosity (although not as thick as cans in
SNF's), taught the live-in caregiver and family what to watch for per
aspiration (sometimes she would cough, sometimes not, increased
respirations, clavicular breathing, etc). Even tried supraglottic swallows
without success, chin tuck, straws (not successful). Coordinating swallows
with supraglottic swallow was very difficult for her.

She's had the disease for a good 6-7 years.

Hospice is now requesting a consult/eval along with OT later this week.

Son and daughter are/were medical professionals and understand the disease
process. Caregiver followed through on teachings too.

She has advanced directives for no feeding tubes which would have been the
obvious suggestion. Her MD also stated patient didn't want this either.

Am I missing anything I should be doing for this lady?

Thanks in advance for any help.

Bonnie Heintskill, MS, CCC/SLP


Info from net on Shy Drager
http://www.mayoclinic.com/invoke.cfm?id=AN00616
Shy-Drager syndrome is a rare and progressive degenerative disorder of the
nervous system. It's considered a form of multiple system atrophy (MSA),
which is characterized by:

Progressive damage to the autonomic nervous system, which controls
involuntary functions such as blood pressure, heart rate, sweating,
breathing and digestion
Muscle tremors and rigidity
Slow movement
The main characteristic of Shy-Drager is a severe drop in blood pressure on
standing up or standing motionless (orthostatic hypotension). This can lead
to dizziness and fainting. Also, blood pressure may be very high when lying
down. Other symptoms of Shy-Drager may include:

Reduced sweating, which leads to heat intolerance
Loss of control of bladder or bowels (incontinence)
Constipation
Speech problems such as slow speaking and difficulty speaking
Impaired breathing due to vocal cord paralysis
Difficulty swallowing
There's no cure for this disorder. Treatment is directed at managing the
signs and symptoms. Death usually follows in seven to 10 years after the
diagnosis, due to breathing problems or cardiopulmonary failure.