Story of Pat Richardson (actress)- Natl Spokesperson for CurePSP

This will likely only interest the PSP folks though everyone might like to know that Patricia Richardson (an actress who was on “Home Improvement” and a few other TV shows) is the new national spokesperson for the PSP cause.  Of course the Parkinson’s community has been greatly aided by Michael J. Fox becoming a spokesperson for the cause.  Certainly all the diseases covered by our group would be aided by some famous person being associated with them.

Dudley Moore had PSP.  Because he was diagnosed fairly late in the disease course, there was only so much he could do as a spokesperson for the cause.  And, these days, very few people seem to have heard of Dudley Moore.  (I realize knowing who Dudley Moore is “shows my age.”)

In mid-December ’06, CurePSP, the new name for the Society for Progressive Supranuclear Palsy, sent out a letter announcing that actress Patricia Richardson has become its national spokesperson.  Her father had PSP; he died in December ’05.  I had never heard of her before.  Hopefully many of you have!  She played Jill Taylor in the TV show “Home Improvement,” Sheila Brooks in the TV show “The West Wing,” and Dr. Campbell in the TV show “Strong Medicine.”  Almost a year ago I was told that things were in the works for her to become the national spokesperson.  I guess it took awhile.

I don’t know if CurePSP’s December letter went out to past donors or everyone on their mailing list.  (If you aren’t on their mailing list, you should get on it.  Go to and sign up for the quarterly newsletter.)  Enclosed with the fundraising letter was Richardson’s poignant story.  In case you didn’t see it, I’m copying Richardson’s story below.


Letter from Patricia Richardson
CurePSP Fundraising Letter
December 2007

I am honored to be the National Spokesperson for CurePSP – the Society for Progressive Supranuclear Palsy.

My father, Laurence, had PSP, and when I tell people how he died they look surprised because they have never heard of PSP. I tell them it also killed Dudley Moore and that it’s a brain tangle disease like Parkinson’s… but you can’t really explain how terrible it is. The only person I ever met who had heard of it was the producer of the hit show ER, and that’s because of his many years of involvement with medical cases. CurePSP has asked me to write my father’s story, but it’s hard to do. Like many families of love ones with PSP, my family didn’t know he had the disease for the first few years. Like many of you reading this letter, we had our hearts broken as we watched Dad struggle with this cruel and bewildering disease.

My Dad was an intelligent, proud, funny, robust, active, and athletic man with many types of arthritic injuries and health problems that disguised the onslaught of PSP. We thought of him as being an unstoppable force – a tiger. My mother was the one with the bigger health problems, and he was taking care of her because she had heumatoid arthritis, high blood pressure, congestive heart failure, emphysema, three hip replacements, hand surgery, meningitis, and strokes! Mom and Dad were married for nearly 60 years and even after her death he continued to be vital, curious, and as independent as possible. Dad died of PSP in December 2005, only three years after Mom passed away. Dad didn’t have that extra time that we had expected him to have so that he could enjoy all the things he put on hold while taking care of Mom. Dad joined my mother before she died in assisted living and was falling down a lot. We thought, at that point, that Mom was really pulling him down when she was falling. We soon realized, however, that he was pulling her down when he lost his balance. My sisters and I didn’t live near my Dad when he retired. He and Mom didn’t want to be a burden to us. As a retired Naval officer, he wanted to be near a base and chose to move near to where his brother lived. So, my sisters and I took turns going to their home each time there was a surgery, a medical problem, a crisis, or for holidays. In addition to Mom’s problems, Dad had back surgery, hip surgery, open heart surgery, and a knee replacement. Because I was a well known TV star on Home Improvement, I often got a chance to see Dad as well as get some extra attention for him during his stays at the hospital.

When I look back, I think that his heart surgery was the thing that most disguised his PSP-driven decline, especially his thought processes. I read that there is sometimes a significant cognitive loss when the patient has been on a heart-lung machine for some hours. We all noticed quite a difference in his sharpness and quick thinking. Dad was also unable to benefit from physical therapy afterwards. He didn’t walk or work out enough. We attributed his stiffness to the osteoarthritis, the knee problems, and lack of exercise. Eventually, Dad began to speak less and less, and we noticed that he was saying “that’s right” in stead of using a more appropriate response. We didn’t realize at that time that this reply was his inability to form the words; a kind of aphasia. We also had to stop Dad from giving himself, and Mom, medication fearing that he would make some terribly dangerous mistakes. My sisters and I confronted him about that and he was furious. He soon realized that he was beginning to lose his independence. How painful it must have been for Dad to have his competence challenged especially by his own daughters.

Dad was having more and more minor car accidents, mostly on the grounds of the retirement community in which he and Mom lived. One time we were on vacation and he had my son Henry in the car with him. Dad backed into a police car on a parking lot! Fortunately, no one was hurt. At that time, everyone found it pretty amusing because in retrospect, it was very early in his illness and we just thought that “Mr. Perfect” made a mistake. Increasingly though, Dad couldn’t turn his head to look around behind him, so he went through stop signs and red lights. His driving terrified Mom and the sitter when he took them on various errands. Because we now had a private day time nursing assistant, we started to get daily reports about our parents’ problems and behavior. Dad wasn’t telling us about the accidents, and Mom couldn’t tell us because by that time she had a few strokes and was incoherent. We finally learned about the accidents and Dad’s other odd behaviors from a concerned neighbor and friend.

Eventually, Dad flunked his driving test and lost his license. This made him terribly angry and depressed. We discovered, however, that he was still driving on the grounds of the retirement community. Then we had to take his car away, which only added insult to injury!

The family wasn’t getting anywhere with a proper diagnosis of Dad’s PSP, so we decided to take him to a new doctor – Dr. John Patterson, a very kind physician who worked at the clinic at the retirement home. Finally, we got some answers and help.

Dr. Patterson had a small geriatric practice and was the type of doctor you pray for; the kind that goes above and beyond to help their patients. After concluding that Dad’s medication, Sinemet, wasn’t helping him with his symptoms, Dr. Patterson diagnosed that he really didn’t have Parkinson’s disease, but actually PSP. He then sent Dad to a neurologist to confirm his diagnosis. Sure enough, Dad’s lack of eye movement confirmed the sad diagnosis. We were shocked to realize that Dad hadn’t received the correct diagnosis of PSP for such a long time and not until just two years before his death. In hindsight, he had already had the disease for several years!

In addition to taking the lead on the PSP diagnosis, Dr. Patterson also suggested a treatment called VitalStim, a form of electrode therapy. Dad was losing his speech and starting to have real difficulty swallowing. The therapy worked well and after his first treatment he was able to speak much better. We were thrilled. Dad would have the treatments as needed, but no more frequently than four months apart. Because of the nature of the treatment and how it taps into specific brain cells, a patient can’t continue to have them for long. Unfortunately, Dad slowly lost his speech again. Toward the end of his treatment, he could only speak during the actual therapy and then eventually not at all. His ability to swallow, however, continued for another year until the stroke that preceded his death.

Never in a million years, would I have imagined that my father would have put up with the difficulties of his life with PSP. Yet he did put up with it, in bed, in a wheel chair and in an electric lifting recliner. That recliner became his cockpit and gave him some sort of control of his life. He had a TV with earphones which helped him hear without the sound blaring into the room. Toward the end of his life he seemed to lose interest in watching anything. We had pictures of his family across from his bed where he could see them any time he wanted. Dad now depended on his beloved caregiver, Alice, to bathe and dress him. He loved good food and wine and now seemed satisfied with thickened juice and meat even when it was ground up. Because of the eye problems caused by the PSP, he couldn’t read, and he watched TV without his glasses, often grimacing in pain from spasms that he would have in his arms and legs. At one point, we all assumed that Dad had lost his mind due to the medications or the disease but, we realized later that he was in fact cognitive. He was a man; now very impaired and trapped in a body that had once been handsome and vital.

Although Dad benefited from a high level of personal care that many PSP sufferers do not receive, he still didn’t have an easy time of it. Our father had been the president of a major corporation, but now he was frozen and helpless in a bed. It was a terrible thing to see him looking so frightened. Often, we didn’t know how much Dad was really with us as his moods got blacker and he seemed so far away. Sometimes he’d just stare into space or sleep. We all knew that it was the terrible effects of PSP. I would fly from the west coast to be with him for a few days, but he was barely awake. When he was awake, Dad would peer at me, but he didn’t seem to know me. Occasionally, he’d laugh while listening to us talk or, while watching a movie, he’d cry at the mention of his grandchildren. Sometimes I wasn’t sure if he was laughing or crying because they sounded so much alike.

For a while, Dad was still able to use one hand although most of his body was useless or contorted in muscle spasms. Sometimes muscle relaxants controlled the spasms and we treasured that one hand that seemed to work consistently. We tried to keep it open and useable by giving him a stuffed Scottish terrier dog to hold. (I knew he would like it because it was the same dog that his mother had). When Dad could no longer speak, he would give us the thumbs up or thumbs down sign with his one good hand to tell us yes or no to the vital question such as “do you want a vanilla milkshake?” Dad had been a Naval fighter pilot and test pilot, and was accustomed, as pilots do, to giving the thumbs up when everything was okay for take off. Dad was also famous in the health clinic for his strong, sometimes even painfully firm, handshake.

I can’t tell you what a loss it was when he no longer had the strength to command that thumb to go up or down or to just shake hands. I tried to get him to blink yes or no, but Dad would just stare in the way that PSP patients often do. Dad had a stroke when I was shooting an episode of The West Wing. Coincidently, it turned out to be the episode during which John Spenser (one of the actors on West Wing) died. I flew to Virginia to see Dad and soon learned that he was unable to swallow. We decided not to insert a feeding tube. Our decision was based on Dad’s medical directives and the fact that his health had so deteriorated in the last month. He had lost 20 pounds and the doctors said it was clear that he was near the end of his life. Dad had reached that part of the disease where he slept most of the time and was hardly eating. We felt that a feeding tube would really be prolonging his struggle and not improve his quality of life. Dad could no longer receive medication by mouth since he couldn’t swallow so he was provided with an intravenous tube for hydration and to make him comfortable. His serious lung problems were soon to follow, but I will spare you the painful details.

I flew back to the Los Angeles to finish an episode of The West Wing while my sisters stayed with Dad. They expected him to live for a few more weeks. When I came back east my sisters and I ended up sleeping in Dad’s room and staying with him around the clock for four nights. We slept with one eye open to watch him and listened to his breathing. We petted his brow and held his hand. We played guitar music for him as he had played the guitar for us as children. We talked to him, gave him permission to go, laughed and cried, prayed and waited. Dad gradually slipped into a coma but seemed to be aware of our comforting presence.

Alice, his caregiver, and the nurses who came in and out of his room explained to us what to expect in the process of dying. People are usually afraid to talk about death, but after this experience, I think that I am less afraid to talk about it. The more the nurse talked to us about what was going to happen, the less frightened we became. My sisters and I had been coming to the retirement community for years to visit our parents so we were all well known by the staff there. They brought us coffee and muffins in the mornings and blankets and pillows at night. Dad’s wonderful doctor – Dr. John Patterson had also become our friend and cried with us when he died. He had known and cared for both Mom and Dad for years.

We donated Dad’s brain for research to the Eloise H. Troxel Memorial Brain Bank supported by CurePSP, at the Mayo Clinic in Jacksonville, Florida. They confirmed the diagnosis of PSP. The areas of his brain that were most affected caused severe damage to motor areas in the brain. I found this so sad and wondered what did Dad do to deserve this kind of death? Why did he have to have such a tragic final chapter? But more importantly, what can I do now to help prevent others from suffering with PSP? What can we all do?

I hope that you will join me in the fight against PSP. CurePSP (Society for PSP) funds research and provides outreach and education to families dealing with PSP every day. It educates physicians and healthcare professionals worldwide on PSP diagnosis and symptoms. It was CurePSP’s website where I first found knowledge about my father’s disease. CurePSP receives no government funding and is solely dependent on gifts from generous people just like you. Please send in your gift today to help CurePSP’s vital programs and services.

I am looking forward to helping CurePSP through my involvement as the national spokesperson, in public awareness campaigns, guest appearances and much more. Please, join me in our fight to find a cure for PSP while we help those affected by this terrible disease.

On behalf of all those affected by PSP, and in memory of my Dad, Laurence Richardson, I thank you for your support.

Together, we will find a cure.

Warm regards,
Patricia Richardson
National Spokesperson

Gastroparesis – Symptoms, Evaluation, and Treatment

Gastroparesis, or delayed gastric emptying that causes bloating, regurgitation, and early satiety, can be a problem in the neurodegenerative disorders in our group, especially in MSA and LBD. Gastroparesis can lead to weight loss and dehydration.

One gentleman in our local support group with a clinical diagnosis of MSA but a pathologically confirmed diagnosis of LBD had gastroparesis.  It was the worst symptom he had.

Online friend Vera James posted to an MSA-related Yahoo!Group a link to a terrific article on gastroparesis.  Here’s a link to the article, available at no charge:

Motility and Functional Disorders of the Stomach: Diagnosis and Management of Functional Dyspepsia and Gastroparesis
Practical Gastroenterology
December 2006
by John M. Wo and Henry P. Parkman

See pages 37-45 especially.

The section on “Treatment” is particularly good. These points are discussed in detail:

* eating smaller, more frequent meals

* relying on liquid nutrient

* limiting fatty foods

* avoid salads, raw foods, and red meat

* antiemetic medications.  “The most commonly prescribed traditional antiemetic drugs include promethazine and prochlorperazine.”

* prokinetic medications such as metoclopramide, domperidone (not available in the US), and erythromycin.

* botulinum toxin injection

* gastric electrical stimulation

* jejunal feeding, rather than PEG feeding

I’ve copied below the “Gastroparesis Cardinal Symptom Index.”



Table 4.  The Gastroparesis Cardinal Symptom Index
1. Nausea (feeling sick to your stomach as if you were going to vomit or throw up)
2. Retching (heaving as if to vomit, but nothing comes up)
3. Vomiting
4. Stomach fullness
5. Not able to finish a normal-sized meal
6. Feeling excessively full after meals
7. Loss of appetite
8. Bloating (feeling like you need to loosen your clothes)
9. Stomach or belly visibly larger

Each symptom is graded by the patient on a 0 to 5 scale: 0=none; 1= very mild, 2=mild, 3=moderate, 4=severe, 5=very severe.

From Revicki DA, Rentz AM, Dubois D, Kahrilas P, Stanghellini V,  Talley NJ, Tack J. Aliment Pharm Ther, 2003;18:141.

LBDA on Surgery/Anesthesia

I saw this second post last week by Angela Taylor, the president of the Lewy Body Dementia Association, in a couple of places. She mentions these medications for which I’m adding the brand names (in parentheses): donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne, which used to be called Reminyl).


Anesthesia and LBD
Posted by: “jaektaylor”
Date: Thu Jan 11, 2007

We’ve all talked about how our loved ones respond poorly to most
anesthesia. The following verbiage is going to be included in the
next version of the LBDA’s informational pamphlet. Here it is for
you, before it’s even ‘hot off the presses!’

“When considering any surgery, caregivers should meet with the
anesthesiologist in advance. People with LBD often respond to certain
anesthetics and surgery with acute confusional states (delirium)
and/or may have a precipitous drop in functional abilities which may
or may not be permanent. The pros and cons of stopping donepezil,
rivastigmine, or galantamine should be carefully considered. If a
spinal block or regional block can be used instead of general
anesthesia, this would be preferred as those methods are less likely
to result in postoperative confusion.”

LBDA on Excessive Daytime Sleepiness + Use of Provigil

I saw this post last week by Angela Taylor, the president of the
Lewy Body Dementia Association, on the LBDcaregivers online
discussion group. The topics are excessive daytime sleepiness and
the use of the medication Provigil.



Treating excessive daytime sleepiness/Provigil
Posted by: “jaektaylor”
Date: Thu Jan 11, 2007 6:02 am ((PST))

….The LBDA’s Scientific Advisory Council is also writing a short
overview on the issue of excessive daytime sleepiness, and the use
of psychostimulants like Provigil. The paper isn’t ready for
release to the public yet, but here are a few things that I’ve come
to understand as a lay caregiver that may be of help.

1. The use of psychostimulants does not appear to be a common
treatment in LBD, and should be done with caution. A neurologist
who specializes in sleep disorders might be the best person to
manage this type of medication.

2. There are a number of reasons that patients may experience
excessive sleepiness, which should be addressed BEFORE considering
using psychostimulants, like depression, delirium, inactivity, etc.

3. With the use of psychostimulants, there are some risks of
increased psychosis in some patients. Fluctuating pulse and blood
pressure and falls is also a risk.

4. A full sleep evaluation is recommended before using
psychostimulants to address what other sleep disorders may be
contributing to the problem, that wouldn’t be resolved by the use of
a psychostimulant.

Hope that helps,

“Speech and swallowing in MSA and other autonomic disorders”

This article on “Speech and swallowing in MSA and other autonomic disorders” will certainly be of interest to the MSAers but some of the questions/answers will be of interest to everyone (especially #1, #7, #8, #9, #12, and #13). (My dad, with PSP, has many of the problems indicated.)

The Sarah Matheson Trust is a UK charitable organization focused on MSA. The Trust’s newsletter, SMarT News, had an article last year in which “speech and language therapist Tricia Gilpin report(ed) on the presentations of speech and swallowing problems and the therapists role in treating people with MSA.” Here’s a link to the Winter ’05 issue of the newsletter:
Editor’s Note: Link no longer available

There was one surprising sentence in the article: “It is important to realise that exercises will not improve the speech.” I thought that was the whole point of speech therapy. Other than that one quibble, I thought the article was a good introduction to what speech/language pathologists (our term in the US) do and what kinds of speech and swallowing problems there are with these disorders.

A copy of the article follows (note that the numbers in front of the questions were added by me).


Speech and swallowing in MSA and
other autonomic disorders

by Tricia Gilpin
Senior Speech and Language Therapist
SMarT News, Winter ’05 Issue, November 2005
Sarah Matheson Trust newsletter

#1 What does a Speech & Language
Therapist do?

Speech and Language Therapists are
involved with both children and adults
with many different types of disorders.
These include developmental difficulties,
stroke, head injury, brain
tumours and many different degenerative
neurological diseases.

The SLT will see patients with any
of the following problems:

* Dysarthria: a motor speech
disorder, where there is loss of
function or co-ordination in the
breathing mechanism, or in the lips,
tongue or soft palate

* Dysphasia: a disruption to the
language centre in the brain which
may affect understanding of the
spoken word, spoken output,
reading & writing

* Dysphonia: the loss of the voice
due to physical or psychological
difficulties. This must always be
checked by an Ear, Nose and Throat
specialist to eliminate the possibility
of structural damage to the larynx

* Dyspraxia: a motor speech
programming disorder. There is no
loss of movement in the lips,
tongue or soft palate, but the
messages from the brain to them
becomes disrupted

* Dysphagia: this is a disturbance in
the process of swallowing. A person
with dysphagia may report any of
the following signs:
– difficulty with hard, dry foods or
mixed consistencies
– problems with thin liquids
– coughing and choking when
eating and drinking
– food becoming stuck in the mouth
or falling out of the mouth
– extended meal times
– excessive saliva and/or dribbling
– having a ‘gurgly’ voice after
mealtimes or drinks
– difficulty opening the mouth
– weak cough
– weight loss
– chest infection

* Tracheostomy: SLTs also work
with patients who have a

#2 Classification of Multiple
System Atrophy

There are three different aspects to

* Parkinsonian or Extrapyramidal signs
* Cerebellar signs
* Autonomic Impairment

The disease can present in any of
these three ways, or often as a
combination of two or three of them.
The speech and swallowing
characteristics of the patient will vary
according to the clinical picture, and
may change over time.

#3 What speech characteristics
should I expect if the presentation
is mainly Parkinsonian?

This type of MSA is now called MSA-P.
The type of dysarthria seen in
Parkinson’s Disease, and in MSA-P is
called hypokinetic dysarthria. This is
characterised by:

* festination of speech — that is
difficulty getting started and then
speech coming out in a rush

* quiet speech

* slow and hesitant speech

* speaking in a monotone and at the
same pitch

#4 What should I expect if the
presentation is mainly

This used to be called Olivopontocerebellar
Atrophy but is now
called MSA-C.

This type of MSA involves the
cerebellum and brain stem and the type
of dysarthria seen is called ataxic or
cerebellar dysarthria. This is
characterised by:
* staccato (or chopped up) speech
* imprecise consonants
* slow and slurred speech

Unfortunately, this type of dysarthria
can make you sound as if you have been
drinking too much alcohol.

#5 What if it is the Autonomic

The third type of MSA is the
predominantly Autonomic presentation
where the patient often has postural
hypo-tension. This may lead to general
feelings of dizziness and exhaustion
and you may not feel very much like
talking at all.

A drop in the blood pressure can
result in a decrease in the volume of
the speech.

#6 Spastic Dysarthria

In addition some patients present with a
spastic dysarthria where the speech is
characterised by:
* imprecise consonants
* strained or strangled voice
* quiet voice

It is important to remember that speech
characteristics in MSA can be mixed
between the different types of
presentation and therefore the different
types of dysarthria.

A research study by Kluin et al in
1996 looked at 46 patients with MSA
and looked at the speech characteristics
and concluded that:

* Hypokinetic components
predominated in 48%
* Ataxic components predominated in
* Spastic components predominated
in 11%
* the remaining 6% were mixed

#7 What can the SLT do to assess
my speech and communication?

The SLT may decide to do a formal
dysarthria assessment. This consists of
looking at the different aspects of the
speech process:
* respiration (breathing for speech)
* phonation (production of sound)
* movements of the facial musculature
* prosody (intonation patterns — the
ability to change pitch in speech)
* articulation (ability to produce clear
* intelligibility

Alternatively, the SLT may assess you
in a more informal way by talking to
you and to your family.

#8 What can the SLT do to help me
with my speech and

Unfortunately, there is no cure for
the speech difficulties experienced in
MSA but the SLT can help by giving
information to the patient and to
the family.

Many people with MSA experience
other difficulties with their
communication in addition to the

* many people experience a lack of
facial expression

* others find making and maintaining
eye contact difficult

* some may have a forward head tilt
which hampers communication,
feeding and vision

* others will have displays of
inappropriate laughter or crying

* some people have difficulty
switching attention from one topic
to another

* some people will experience a
low mood

Often it is helpful just to be able to
discuss these matters and try to find a
way, with the therapist, to manage these
difficulties. It can be helpful to discuss
ways to avoid very noisy situations, or
to cut down on background noise when
trying to communicate (even turning off
the TV can be helpful).

For the family and friends of the
person with MSA it is important to try
to make time to communicate, even
though the process may be slower
than previously. It is often helpful to
be able to watch the face of the
person to help with understanding and
to ask for repetition in a different
way if something cannot be understood.
It is important not to pretend to
understand if you have not, and to ask
for clarification.

#9 Will the SLT give me exercises to
do to improve my speech?

It is important to realise that exercises
will not improve the speech. However,
sometimes the therapist will decide to
focus on a particular aspect of the
speech mechanism to try to maintain
and preserve speech for as long as
possible. It may be helpful to work on:

* exercises to improve facial

* breathing exercises to maintain an
adequate respiratory drive for speech

* relaxation exercises to reduce
tension in the muscles used for

* voice exercises to maximise volume

* speech exercises to improve overall
intelligibility and fluency

#10 Are there any Communication
Aids for people with MSA?

* some people find that a small speech
amplifier can be helpful

* there are other communication aids
such as Lightwriters which allow the
patient to type out their message and
the machine talks for them

* some people prefer to use a pen and
paper if they are finding speech

#11 I have heard that sometimes
people with MSA need to have a
tracheostomy. Is this true?

Unfortunately, some patients with MSA
may experience difficulty opening the
vocal cords in the larynx sufficiently to
allow the normal amount of airflow.

This may be due to vocal cord palsy and
can result in:
* excessive snoring
* inspirational stridor — a sound like
snoring but on the ‘in’ breath
* sleep difficulties and vivid dreams
* breathing problems, particularly at
* sleep apnoea, where the person stops
breathing for a short time during

In extreme cases the ENT surgeon may
recommend a tracheostomy. This is a
small tube which is fitted into the
neck below the level of the vocal cords,
allowing the patient to breathe

The Speech and Language Therapist
can assist in advising whether or not a
speaking valve is appropriate.

#12 What happens to the normal
swallow in people who have

The normal swallow is made up of 3
* the oral stage
* the pharyngeal stage
* the oesophageal stage

All three stages of the swallow may be
disrupted in MSA. A research study by
Smith & Bryan in 1992 looked at ten
patients with MSA and found the

Oral stage:
* 90% had decreased control of what
was in their mouth
* 40% had poor tongue movements

Pharyngeal stage:
* 80% had a delayed swallow
* 100% reported the feeling of
‘something stuck in the throat’
* 30% experienced ‘silent’ aspiration —
where food or drink goes ‘down the
wrong way’ and enters the lungs, but
the patient does not cough

Oesophageal stage:
* some patients with MSA or other
types of autonomic dysfunction
experience dysmotility of the gut
* this can result in constipation but
also in dysmotility of the
oesophagus with patients reporting a
feeling of ‘something stuck’ in the
centre of their chest

#13 How will the Speech and
Language Therapist assess my

The SLT will start by taking a full
history of the disease process and also
of the eating and swallowing problems
>from both the patient, and if possible,
>from the carers.

The SLT will then probably
complete the following assessments:

* a ‘bedside’ assessment of the
swallow including:
– assessing the cranial nerves of the
patient, looking at the functioning
of the face, tongue, lips, soft
palate etc.
– trial the patient on various foods
and drinks, observing their ability
to cope with different

* possibly perform a videofluoroscopy
(this is a moving x-ray of the
swallow which is filmed onto
video). This shows the safety and
assist the therapist in making
recommendations regarding:
– appropriate consistencies of food
– positioning
– possible modifications to the diet

It may be that the SLT, in consultation
with other members of the multidisciplinary
team, may suggest a PEG.
This is a Percutaneous Enderscopic
Gastrostomy which is a small tube
which is passed directly into the
stomach to allow the patient to be fed
with liquid feed, should this become

A PEG is often used in conjunction
with continuing to eat and drink. It may
be that the patient is finding eating and
drinking very time consuming, slow and
difficult and may find it much more
pleasurable to get all the necessary
hydration and nutrition through the PEG
leaving the opportunity to eat and/or
drink small amounts for pleasure.

#14 The Multi-Disciplinary Team and
the Speech and Language

The SLT may discuss a number of
complex issues with you regarding
your speech and swallowing and
also have discussions with you
regarding the placing of a PEG tube or
a tracheostomy.

It is important to remember that any
complex decision that has to be made
will always be after joint discussion
between members of the MDT, the
patient and his/her family. The MDT
may include the Neurologist, the GP, the
clinical nurse specialist for MSA, other
nurses, the dietician, the physiotherapist,
the occupational therapist and the speech
and language therapist.

In the discussions regarding the
placement of a PEG or a tracheostomy
it is vital to allow time for discussion
with the patient and his family and to
take into account the individuals right to
make decisions about his or her quality
of life.

New Zealand Pamphlet on MSA

On the Shy-Drager online support group today, Vera posted a link to a pamphlet done by the Parkinsonism Society of New Zealand on multiple system atrophy (MSA).  The link is:

Editor’s Note: Link is no longer active

This short pamphlet would be something you could send to family members to provide a general introduction to MSA.  Or it could be given to new caregivers.

Copied below is most of the inside page of the 2-page New Zealand pamphlet that gives details on MSA.



What is MSA?
Multiple system atrophy (MSA) is a rare
progressive neurological disorder presenting
with similar symptoms to Parkinson’s disease.
The condition is marked by a combination of
symptoms affecting movement, blood
pressure, and other body functions; hence the
label multiple system atrophy.

Various forms of MSA
Symptoms of MSA vary from person to
person. Because of this, three different
diseases were initially described to encompass
this range of symptoms: Shy-Drager
syndrome, striatonigral degeneration, and
olivopontocerebellar atrophy.

What causes MSA?
The cause of MSA is unknown.

Symptoms of MSA?
MSA can cause a wide range of symptoms,
.. orthostatic hypotension, or a significant fall
in blood pressure when standing, causing
dizziness, lightheadedness, fainting, or
blurred vision
.. male impotence
.. loss of control of bowel or bladder
.. stiffness or rigidity
.. freezing or slowed movements
.. postural instability; loss of balance; lack of
.. speech and swallowing difficulties blurred
.. changes in facial expression.

Who gets MSA?
MSA usually starts between the ages of 50-60
years, although it can affect people younger and
older than this. Around 4 in 100,000 people
are affected by MSA in New Zealand. MSA
does not appear to be hereditary and is not
infectious or contagious. It is a sporadic
disorder that occurs at random.

How is MSA diagnosed?
Often symptoms are vague and diagnosis is
difficult. MSA is often mistaken for Parkinson’s,
especially in the early stages of the condition.
Diagnosis should be made by a specialist,
usually a neurologist.

What is the treatment?
Currently there is no specific treatment for
MSA. A variety of medications, including some
drugs used for Parkinson’s, and other forms of
therapy can help control the symptoms.
Treatment may focus on alleviating the
symptoms, so people with MSA could benefit
>from working with physiotherapists, speech
therapists, dieticians, continence nurses, and
occupational therapists.

What is the prognosis?
MSA is a progressive disorder, the rate of
progression differs in every person.

To the person with MSA and their
The diagnosis of MSA has significant impact
on those close to the patient. As the
condition progresses so does the need for
care. Carers/families may feel isolated,
frustrated and chronically tired. Infinite
patience is needed. Carers/families will
need support and should utilise support
services. Support services can be contacted
directly or referral can be made through
your doctor or health professional.

Warning for those with dementia about anticholinergics

Like Alzheimer’s Disease (AD), those with many other types of dementia have an imbalance of acetylcholine in the brain.  Anticholinergic drugs can be problematic for those with AD and non-AD dementias.

I saw this Q&A recently in my Dad’s AARP Health Care Options newsletter called fyi.



Ask Dr. Reed ([email protected])
AARP Health Care Options fyi (newsletter)
Fall 2006

Question:  My husband has Alzheimer’s disease.  His pharmacist told me that certain medicines could further worsen his memory problems.  Any advice?

Answer:  …You are wise to take steps to ensure that your husband’s mental status is not worsened by the effects of his medicines.  As we have mentioned in previous columns, many of us become more sensitive to medicines as we age.  As a result, a variety of medicines could produce unanticipated effects that could worsen mental status and overall function.

Alzheimer’s disease is characterized by low levels of a chemical that transmits signals between nerves called “acetylcholine.”  As a result, medicines called “anticholinergic” drugs that block the effects of this nerve chemical can be especially problematic for people with Alzheimer’s disease.  Unfortunately, these drugs are very common.  They include:

* Certain antihistamines such as diphenhydramine (Benadryl)
* Certain antidepressants such as amitriptyline (Elavil) and doxepin (Sinequan)
* Medicines for bladder problems such as oxybutynin (Ditropan)
* Muscle relaxants such as carisoprodol (Soma), cyclobenzaprine (Flexeril), and methocarbamol (Robaxin)

I always say to write down the name of every medicine that you or your loved one is taking, and review this list regularly with your doctor and your pharmacist…

Warning for those with dementia about anticholinergics

Like Alzheimer’s Disease (AD), those with many other types of dementia have an imbalance of acetylcholine in the brain.  Anticholinergic drugs can be problematic for those with AD and non-AD dementias.

I saw this Q&A recently in my Dad’s AARP Health Care Options newsletter called fyi.



Ask Dr. Reed ([email protected])
AARP Health Care Options fyi (newsletter)
Fall 2006

Question:  My husband has Alzheimer’s disease.  His pharmacist told me that certain medicines could further worsen his memory problems.  Any advice?

Answer:  …You are wise to take steps to ensure that your husband’s mental status is not worsened by the effects of his medicines.  As we have mentioned in previous columns, many of us become more sensitive to medicines as we age.  As a result, a variety of medicines could produce unanticipated effects that could worsen mental status and overall function.

Alzheimer’s disease is characterized by low levels of a chemical that transmits signals between nerves called “acetylcholine.”  As a result, medicines called “anticholinergic” drugs that block the effects of this nerve chemical can be especially problematic for people with Alzheimer’s disease.  Unfortunately, these drugs are very common.  They include:

* Certain antihistamines such as diphenhydramine (Benadryl)
* Certain antidepressants such as amitriptyline (Elavil) and doxepin (Sinequan)
* Medicines for bladder problems such as oxybutynin (Ditropan)
* Muscle relaxants such as carisoprodol (Soma), cyclobenzaprine (Flexeril), and methocarbamol (Robaxin)

I always say to write down the name of every medicine that you or your loved one is taking, and review this list regularly with your doctor and your pharmacist.

Caregiver Health (New Fact Sheet from Family Caregiver Alliance)

Family Caregiver Alliance has a good newsletter called “Update.”  The Fall ’06 issue has an interesting article on caregiver health, a topic we should all take seriously!  Here’s an excerpt:

“A large and growing body of evidence has revealed that providing care for a chronically ill person can have harmful physical, mental, and emotional consequences for the caregiver.  As families struggle to care for their loved ones, their own health is jeopardized.”

The article is about the availability of a Fact Sheet on this topic called “A Population At Risk.”  This link might work to get to the Fact Sheet:

This link might work to get to the article:

The article follows along with some highlights from the new Fact Sheet.


FCA Introduces New Fact Sheet on Caregiver Health
Update, a newsletter of Family Caregiver Alliance
Fall 2006, Volume 23, No. 4

A large and growing body of evidence has revealed that providing care for a chronically ill person can have harmful physical, mental, and emotional consequences for the caregiver. As families struggle to care for their loved ones, their own health is jeopardized.

This important public health issue has broad implications. Medical advances, shorter hospital stays, limited discharge planning, and expansion of home care technology have placed increased costs as well as increased care responsibilities on families, who are being asked to shoulder greater care burdens for longer periods of time. To make matters worse, caregivers are more likely to lack health insurance coverage due to time out of the workforce. These burdens and health risks can hinder the caregivers’ ability to provide care, lead to higher health care costs and affect their own quality of life as well as that of the care receivers.

FCA has introduced a new Fact Sheet discussing this significant issue. It describes the impact of caregiving on the mental and physical health of the caregiver, summarizes research from a variety of sources, and offers recommendations from a health and public policy perspective.

Studies indicate that caregivers:

  • suffer from high levels of stress and frustration
  • show higher levels of depression
  • may exhibit harmful behaviors
  • are in worse physical health than noncaregivers
  • may have increased risk of heart disease
  • have lower levels of self-care
  • may pay the ultimate price for providing care­increased mortality.

This new publication joins the FCA library of more than 60 Fact Sheets on caregiving issues, with many translated into Chinese and Spanish. All are available at no charge on the FCA website at, or send $2 for each copy to Family Caregiver Alliance, 180 Montgomery Street, Ste. 1100, San Francisco, CA 94104.


Excerpts from:

A Population at Risk
Fact Sheet of Family Caregiver Alliance
Fall 2006

Evidence shows that most caregivers are ill-prepared for their role and provide care with little or no support.

Caregivers show higher levels of depression.  Depressed caregivers are more likely to have coexisting anxiety disorders, substance abuse or dependence, and chronic disease.

Research shows that female caregivers (who comprise about two-thirds of all unpaid caregivers) fare worse than their male counterparts, reporting higher levels of depressive and anxiety symptoms and lower levels of subjective well-being, life satisfaction, and physical health than male caregivers.  According to one study, there is a dramatic increase in risk of mental health consequences among women who provide 36 or more hours per week of care to a spouse.

Caregivers suffer from high levels of stress and frustration.

Stressful caregiving situations may lead to harmful behaviors.  As a response to increased stress, caregivers are shown to have increased alcohol and other substance use. Several studies have shown that caregivers use prescription and psychotropic drugs more than noncaregivers.  Family caregivers are at greater risk for higher levels of hostility than noncaregivers.  Spousal caregivers who are at risk of clinical depression and are caring for a spouse with significant cognitive impairment and/or physical care needs are more likely to engage in harmful behavior toward their loved one.

High rates of depressive symptoms and mental health problems among caregivers, compounded with the physical strain of caring for someone who cannot perform activities of daily living (ADLs), such as bathing, grooming and other personal care activities, put many caregivers at serious risk for poor physical health outcomes. Indeed, the impact of providing care can lead to increased health care needs for the caregiver.

Caregivers are in worse health.  About one in ten (11%) caregivers report that caregiving has caused their physical health to get worse.
The physical stress of caregiving can affect the physical health of the caregiver, especially when providing care for someone who cannot transfer him/herself out of bed, walk or bathe without assistance. Ten percent of primary caregivers report that they are physically strained.  Caregivers have an increased risk of heart disease.  Caregivers exhibit exaggerated cardiovascular responses to stressful conditions which put them at greater risk than noncaregivers for the development of cardiovascular syndromes such as high blood pressure or heart disease.

Women who spend nine or more hours a week caring for an ill or disabled spouse increase their risk of heart disease two-fold.

Caregivers are less likely to engage in preventive health behaviors.  Caregivers’ self-care suffers because they lack the time and energy to prepare proper meals or to exercise. About six in ten caregivers in a national survey reported that their eating (63%) and exercising (58%) habits are worse than before.

Caregivers pay the ultimate price for providing care—increased mortality.  Elderly spousal caregivers (aged 66-96) who experience caregiving-related stress have a 63% higher mortality rate than noncaregivers of the same age.  in 2006, hospitalization of an elderly spouse was found to be associated with an increased risk of caregiver death.

“Strategies for Managing Home Care Workers” Article

This will be of interest only to those who use home care workers though I do think that many of the principles mentioned in the article below apply to “managing” caregivers at assisted living centers, residential care homes, and nursing homes…

Family Caregiver Alliance ( has a good newsletter called “Update.”  The Fall ’06 issue has an interesting article on managing home care workers.  At the end of the article, there’s a note about the availability of a Fact Sheet on “Hiring In-Home Help” and about an upcoming workshop on this topic in the winter.

This link might work to get to the “Hiring In-Home Help” Fact Sheet:

This link might work to get to the article:

The full article follows.


Working Successfully with Home Care Services
Update, a newsletter of Family Caregiver Alliance
Fall 2006, Volume 23, No. 4

You are the caregiver who has finally conceded that “outside” help is needed and you’ve taken the plunge. You’ve done your homework (or not) and hired home care workers. You’ve already sorted out whether to use an agency or to hire private contractors. You may have made your decisions with reams of information or with little information at all. Paid caregivers are now in place to help care for your loved one.

Yet, the situation still does not feel under control, and you feel stressed. Your loved one might even be delighted with the caregiver, but you’re not happy about some aspects of home care. What next? Whether the care receiver lives with you or alone or with other family, you may find that several more hats have been added to the ones you’ve already been wearing. On top of being a spouse, an adult child, a loving caregiver, perhaps you now feel you need nurse’s aide skills with a smattering of R.N. and M.D. skills; accounting skills with knowledge of employer responsibilities; and supervisory skills covering personal care, household care, and the special needs of people with dementia. And you’re asking: “Isn’t this why I hired help?”

The Team

There are many possible sources of stress in utilizing home care services. Family members might provide help in various ways, but some might have no role where home care is concerned. Family members who live with the care receiver probably have a role in home care, but how they relate to paid caregivers may be uncertain. If there’s an agency involved, does the agency have a role other than providing a worker? If there’s a professional care manager, what is that person’s role? Once you determine who is on the team, then you might ask, “who wears which hats?” Maybe the team does not expect you to do anything other than hire the home care worker. Or, maybe the team expects you to do everything. Sorting these issues out early in the home care venture (or adventure) can make the plan work more smoothly.

Family Caregiver As Care Manager

A second source of stress may arise when a care plan is too vague. In the “care management” model, the care manager follows a process which includes assessment; planning; implementation; and evaluation. If you have already hired home care workers, knowingly or unknowingly you’ve completed the first three steps. It’s expected, and more than okay, to evaluate your plan as often as needed. Assessing, planning, implementing and evaluating are a continual process. Care plans need to change from time to time. They also need flexibility. Both the care receivers and the caregivers (paid or family) will have some variation in their abilities and needs from day to day.

A care plan is a written plan which outlines the paid caregiver’s duties. A written schedule is useful as well. If there is more than one caregiver, it might even be essential in order to assure that all tasks are covered.

A written care plan is particularly helpful if last minute substitute care is needed, so everyone knows the plan for the day. Because consistency in routines is useful when working with someone with dementia, the care plan is a way to provide details of those routines. The plan may even provide hints on what strategy works in getting the care recipient to cooperate with bathing or other activities.


A third to way to minimize stress in home care involves communication. A notebook in the care receiver’s home is essential if the care receiver has dementia. The caregivers can communicate with each other or with you about how the plan is working. Some paid caregivers will bring new and useful ideas to you on how to care for your loved one. Some will think that they know more than they do. Read carefully. The comments of some paid caregivers may reveal that they need more training or are not suitable at all.

The notebook is also a way to track variations in the schedule: “John was pacing all day and refused to shower.” “Ann asked for a shower today.” In addition to getting a sense of how your loved one is functioning, you will also get a sense about whether certain workers have more difficulty getting your loved one to cooperate (and whether, in general, they are skilled in taking care of someone with dementia).

The notebook will also provide you with other useful information about your loved one. When care receivers are unable to report reliably about essential bodily functions, the communication book is useful way to note whether the care receiver eats and drinks enough; whether he or she has adequate elimination; whether there are any new concerns about mobility; whether there are any skin problems; and the like.

However, the communication notebook does not replace the need to check in personally with the home care worker.

Checking In

Some of your stress may be related to having set up no plan for supervision or checking in. It may be difficult to start this practice after a problem arises. Both you and the home care worker will probably benefit from regularly scheduled meetings or telephone conversations. The communication notebook will not cover it all.

If you’ve been lucky, you’ve hired someone who stepped right in and took over without a problem. A really good home care worker will even have skills to take care of you as well as your loved one. Be prepared for the possibility of wearing the “being taken care of” hat. If your loved one lives with you, your paid caregiver may want to cook for you or clean for you. You will need to decide what you are comfortable with. The home care worker may be sympathetic about how difficult it was for you to be up all night with the care receiver. This same home care worker will want to hear positive feedback from you about the care that she is providing. This home care worker may even want to coach you about how to provide care. Yet, if some part of her care provision is a problem, this same experienced home care worker must get that message as well.

Thus, it is useful, early on in the home care venture, to set up a plan for checking in with the worker regularly, either face to face or on the telephone. It is useful to create a safe space where the home care worker can share her expertise with you and where you can share your preferences for care for yourself and your loved one.

It is also essential to sometimes be at the care receiver’s home to do “odd jobs” while the paid caregiver is there. While you are occupied with the “odd jobs,” you will have an opportunity to see and hear how the caregiver interacts with your loved one. If your loved one is responsive and involved when this caregiver is around, you may be able to overlook the fact that this caregiver is not neat about making a bed. If you know that your loved one argues with you every time you try to give a shower and you see that the caregiver has a way to gain cooperation, then you are reassured that you have hired well. On the other hand, you may overhear another paid caregiver arguing with your loved one about resistance to taking a shower. Then you must decide if that worker needs further training or has crossed a line into unacceptable behavior. In all likelihood, your loved one with dementia cannot give you reliable feedback about each paid caregiver.

You protest, “I am not a manager or a supervisor.” If you feel you truly are not a good supervisor, can anyone else on the team do this job? If you have hired through an agency, clarify whether the agency supervises its employees on a regular basis at the care recipient’s home. Determine whether the agency supervisor has the skills to supervise your loved one’s care. Is the plan for supervision detailed in the agency’s contract? If you have hired a private contractor or have hired through an agency that does not provide regular supervision, would a private care manager be useful and affordable for you? It is guaranteed that the home care plan will not run itself. If you are not a supervisor, then you’ll probably end up as chairman of the board. You won’t escape involvement unless the rest of the team bails you out somehow.

What Else Is Essential?

Many aspects of home care need close attention. There are really good home care workers and unfortunately some really unsatisfactory home care workers. However, most are neither stars nor failures. Most home care workers bring a mix of talents to the job. Your responsibility is to determine whether their strengths outweigh their weaknesses in meeting your loved one’s needs. It is useful to contract with any home care worker on a trial basis. It takes two or three weeks to determine if your loved one, the home care worker, and you are a good match for each other. Personalities and individual styles make a difference. Whether it is important to match up with other team members also needs to be determined.

The Essentials


  1. The home care worker should be able to make a connection with your loved one and provide companionship. If your loved one is no longer able to connect with others, does the worker demonstrate gentleness and respect in working with him or her?
  2. Tasks which involve the health or safety of your loved one should be demonstrated to the worker, be demonstrated back to you by them, and reviewed from time to time. For example, your Dad, who is a big man and is paralyzed from a stroke, transfers bed to chair in a specific way. A good worker will be able to safely maintain this routine. If your loved one uses oxygen or a Hoyer lift or a transfer bench, after demonstration by you and redemonstration by the worker, the worker should understand how to use the equipment safely and appropriately. If there are medical treatment plans to be followed (such as routine medications, daily weights, therapeutic diets) the worker should follow your instructions exactly. The worker should also follow your directions on what to do if, for any reason, the plan cannot be completed as directed.
  3. It is essential to discuss emergency procedures with the home care worker. You should ascertain the worker’s understanding of how and when to call 911. Some workers are new to this country and are unfamiliar with many aspects of day-to-day life in the United States. Do not assume that the worker knows these procedures. If your loved one has a written “Do Not Resuscitate” directive, does the worker know where it is? Does the worker know which part of the team needs to be called about the emergency? If a situation is not an emergency but is urgent, how do you want the worker to handle this? Do you want the worker to take messages from the doctor or the visiting nurse? Is your contact information up-to-date?
  4. Accountability is essential and needs to be discussed even before the start of care. If your loved one cannot be left alone, coverage for absences must be clarified. Will the agency or private contractor provide back-up? Is this acceptable to you? Sometimes the answer is yes and sometimes no. Care receivers will have problems despite the best of care. And care receivers will definitely have problems from substandard care. There are times when workers need to be fired. There are times when the worker needs to be reassured that she did the best that she could. And by all means, there are times for praise.

A Final Word

A home care plan does not totally relieve you of your involvement in your loved one’s care. The situation will still be stressful in some ways. If the plan you have developed cannot be fixed, change it. Change the duties; change the worker; get professional advice. Allow that things will not go exactly as you envision. Focus on the priorities. Is your loved one safe? Is your loved one’s health being maintained? Is your loved one connected to the home care worker? If you answer yes to these three questions, then maybe some of the rest is not so important.

For more information, see the FCA Fact Sheet, Hiring In-home Help. (Robin’s Note:  the link to this fact sheet might be  The link given in the print version of Update is correct but the link given in the online version of the article is incorrect.)