New Member

[Guest guest]

Maureen, Welcome, though sorry you are here. I live in Mass., but after

speaking with my husband a minute ago, I am around 2 hours from Pam.

My mother Joyce (71, died 11/5/00) had my father working at home. But he

wasn't right for the full-time care giving role (anyway, he was working full

time, too). They eventually (too late if you ask me) hired aides to care for

my mom. One thing they did was shower her everyday. She was always showered

and dressed (although that took most of the day). She was also lucky enough

to have friends in the area that came every day.

If she had been unable to hire aides, I am sure they would have brought in

hospice. It is important to get someone in there that appreciates Pam's

situation and basic needs. I am surprised that hospice is not meeting those

needs. If they don't do that, is it possible to hire an aide even for 2

hours/day to attend to these needs?

In addition to a hospital bed, my mother spent her time in a wheelchair. My

mother's head was flexed into her chest, so she was very bent over. Still,

the change to the wheelchair (and even the kitchen chair while eating) was a

relief for her. It was also important for my mother for her to get out. Is

there any possibility of doing this for your sister (if she wants to). I

know it is an all-day affair, but it makes the days more normal and

interesting. The cell phone is a great idea.

Good luck and stay on this list--you will find what you are looking for.

Debbie

New Member

Hello to all,

I have just joined your list. My sister is very ill with Shydrager and

has asked me to contact you in an effort to find out what she may expect

from this point. Although she has had Shydrager for a number of years, she

was only diagnosed in March, 2001. By that time she was extremely sick and

her doctors could no longer deny that something was seriously wrong. Before

that she had been treated with tranquilizers and antidepressants, neither

of which were at all helpful, accused of malingering and turned down by

disability in the early stages. In other words, she was generally

discredited. Since she was finally diagnosed she has found it almost

impossible to get information from her doctors, who are mostly uninformed,

or anyone else. All that she has I supplied to her - and her doctors - from

my research on the internet.

She is currently being cared for by my brother (55 and relatively

healthy although he is currently down with a bad back), my mother (84 and

almost completely disabled by arthritis) and more recently Hospice which I

arranged in a visit last August. Unfortunately they all live about 1400

miles away from me so I am unable to do much more than provide moral

support and information when I can get it.

My sister's name is Pam and she is extremely isolated. She has no

computer available to her and likely would not be able to use it if she had

one. I will be her conduit to those of you who feel that you can help her

in any way. She feels that she is close to death but has no objective

criteria to go by. She is miserably uncomfortable and in pain a great deal

of the time. She lives from medication to medication, but has very few

hours of the day when she feels even remotely normal. She is particularly

desirous of information which would help her to identify the last stages of

this illness and of any remedies which others have found to make the last

months of life less painful and distressing. She also would like to know

what is likely to be the determinant of when and of what she will likely

die other than the primary illness. How can she best prepare herself for

what lies ahead? Currently she is completely bedridden (Hospice has kindly

supplied a hospital bed which has made her more comfortable). She is not

being bathed often and finds that very burdensome. It has now been weeks.

Should I call Hospice and ask that someone assist her with this? My mother

currently cannot due to her own physical condition.

I am at such a distance from her and feel relatively helpless. All I can

do is ask for information from those who also live with this condition and

might be able to help me find solutions to add to her comfort both

physically and psychologically. I thank you for any which you can provide

so that I can help her. We are in contact frequently and I will transmit

any messages directly to her. Thanks to all for listening!

Best regards,

Maureen~

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[Guest guest]

Maureen,

Is pam on any medication for low blood pressure? I assume she has

orthostatic hypotension (BP falls at least 20 points when going from

sitting to standing.) There are a couple of meds that are used to

treat this, although not everyone can tolerate them. For those who

can(like my husband Rob, they make a huge difference.

The doc makes a good point about many meds lowering bp. Dealing with

this disease is a constant balancing act, but it is possible, with

good medical attention and monitoring. It sounds like Pam's pain is

from rigidity, and she might want to ask her doctor about baclofen,

which MIGHT help. The other medication for neuropathic pain that

many on the list have tried is neurontin. I don't know if either

would be appropriate for her, but she can mention them to her doctor

and see what he/she thinks.

town is about 2 hours from here, so it would be difficult for

us to " drop in " for a visit. One of our group members is from

Enfield, CT and sees a neurologist in Springfield, I think, that they

are very pleased with. That might be possible for Pam. If you are

interested I will get a name, or they may chime in when they see this

post.

Pam might also need to consider an antidepressant. Clinical

depression goes along with MSA -- not just being distrsught over the

disease, but actual chemical changes in the brain that affect

emotions. Again, someting to discuss with the doc.

Carol & Rob

> Dear Carol and Rob,

>

> In answer to your question, Pam is 52 and lives in town,

Mass. I

> will take a stab at the medications, but can clarify more after I

talk to

> her tonight. I believe she is on Synthroid as she also has Graves'

Disease.

> I believe she takes Sinemet. There may be another med also, but I

can't

> recall what it is.

>

> Her pain mainly is in one leg above the knee which is likely due

to

> spasm and/or rigidity. This is so painful that she spends a large

part of

> every day just struggling with the pain. She can only stand briefly

and

> with assistance and then is bent over double as her back muscles

long

> longer function properly. She also blacks out and has been injured

hitting

> her head on the floor, tub, toilet and other objects as she has

fallen. So

> she was a mass of bruises last time I saw her.

>

> Her doctor was very resistant to giving her any pain medication

because

> of her low blood pressure and what he said would be interference

with her

> primary medication. He finally gave her a patch of some kind, but

has told

> her he only wants her to use it for emergencies and she has thus

never

> dared to try it because of his admonitions. So she remains in pain.

>

> To date Hospice has never bathed her. That is what I must

arrange. But

> it may be that my mother or brother refused such services. They were

> totally against my arranging Hospice to begin with and have been

anything

> but cooperative to say the least. Pam is in a very negative

situation all

> around and is very depressed. She really has no emotional support

at all

> apart from me.

>

> Any suggestions are appreciated. I am conversant with the

illness and

> more than willing to learn as well.

>

> Thanks to all,

> Maureen~

[Guest guest]

Dear Debbie,

Thanks you for your so kind response. If only I were there and could

attend to these things for Pam! There is really no possibility of hiring

assistance for her. What help she receives must come through Hospice, I'm

afraid. Finances are tight, but I will speak to her about all the options

and possibilities anyway. She is receiving SSI now so maybe there is a

chance for something more.

Getting Pam into a wheelchair is only a once-in-a-while kind of thing

right now. My mother is completely incapable of this and my brother is

temporarily unable due to a back problem. He has not been out of bed

himself much since before Christmas. A friend had to come from 30 miles

away to bring groceries or they all would have starved. Pam used to do all

the cooking. My brother has tried, but it seems beyond him so now they are

mainly eating TV dinners and pizza delivered. When I was there in August I

started her on Ensure as she had lost more than 50 pounds and was not

getting anywhere near appropriate nutrition. It seems unlikely that an

outing of any kind is feasible for the time-being.

I hope to go up there again in the late Spring, but even then I cannot

stay for long enough to do much good. I did get her set up with Hospice

last time and got her on SSI. There is still much to be done, I think, to

make her more comfortable and make her life (such as it is) worth living.

She has been told that she will likely live only a few months longer, but

then no one knows for certain as this illness is so unpredictable. Right

now Pam would be delighted just to get a bath so she could wear some of the

new (already washed) nightgowns which my daughter and I sent her for

Christmas!

It helps me too to have someone to talk to about all of this.

Thanks again,

Maureen~

[Guest guest]

Hi Carol,

Pam is currently taking Florinef for low blood pressure although she

reports it does not work very well. Many times the nurse is unable to get a

recorded blood pressure and when she does succeed it ranges from 58/38 to

70/44. These are readings taken when she is lying in bed.

I have told her about the medication for neuropathic pain which you

mentioned and she will discuss it with her doctor. Before she only had pain

in her right leg above the knee. Now she is reporting pain elsewhere on her

right side and arm.

As far as her going to Springfield or elsewhere to a doctor, I don't

think it is possible any longer. Her doctor is in North and she can

no longer go even there. Her internist will begin seeing her at home later

this month. I think her neurologist will no longer see her as she cannot

come to his office.

I read your message to her so she will determine what she will discuss

with her doctor and her nurse from Hospice. I thank you for making the many

helpful suggestions you have.

Maureen~

[Guest guest]

Maureen:

Our experience with Ken was that when he passed out, it looked a lot like a

seizure but it was just plain old passing out or syncope. He did it sometimes

in the shower and every time he had a bowel movement. We learned to just hold

him until he came to. It is best if you can lay them down on their backs, but

Ken had this tendency to where it was impossible to move him at all, so we

just waited, and he always came to eventually. The trick in bathing is not to

let the patient sit still for too long and not to use water that is too warm.

We had a very sturdy shower seat that was provided by Hospice after he broke

the one we had getting rigid and passing out. I went to Home Depot first to

replace it, and he broke it the very first day, but our bath girl said that

Hospice could provide a sturdy one, and that one worked until his death.

Bathing should happen at least three times a week, and a good Hospice

Organization should be able to arrange it. As an aside, ken not only broke

shower seats passing out, he broke two toilets!

Love, Barbara

[Guest guest]

Maureen:

Every Hospice Organization has a Social Worker whose job it is to see

that the patients and their families are getting all the services that are

available. This is the person you should speak to about your concerns. It is

hard if your mother and your brother are against their being there, but it is

the Chaplain of Hospice and the Social Worker who can help you in your effort

to get them to see that this is more than they can do alone. I suspect they

are at their wit's end and are afraid they will be criticized if they ask for

too much help. It really is the job oof the Chaplain and the Social Worker to

get through this denial. There are some very good Websites if you do a

Hospice Search. Your brother and your mother may think that bringing in

Hospice meant that your sister would not get any more medical treatment. Here

again, they are wrong, but the Hospice people are the ones who can help them

with that.

I pray that you and your mother and brother can get together in

planning your sister's care. Even in the condition she is, life can be

better, and there are resources out there to help.

Love, Barbara

[Guest guest]

Maureen:

What patients are often not told is that Florinef has to be used with large

quantities of salt, sometimes salt pills and much fluid intake. Also,

potassium levels have to be checked regularly and potassium supplements taken

if needed. Ken took both Florinef and Midodrine, he was the first patient in

Orange County to receive Midodrine and it enabled him to get back on his feet

when Florinef alone was no longer enough.

Barbara

[Guest guest]

Maureen:

From what you have told us, I suspect your Internist is a better doctor than

Pam's present Neurologist, and if he comes to the house, that will help, but

there is probably a public transport system for wheelchair bound patients

that she can qualify for in her area that can take her and a companion to the

doctor if you find a good Movement Disorder Specialist willing to see her.

Another question to ask the Hospice Social Worker.

Love again, Barbara

[Guest guest]

Marueen,

Florinef is the medication most often prescribed for low blood

pressure. It ONLY works if taken with large quantities of salt (for

instance, salt tablets (Thermotabs, available at CVS buy special

order) or instant bouillon, at least one cup a day. Also, Pam should

try to drink at least 2 liters of water a day. Hydration is

extremely important in MSA patients for maintenance of blood pressure

and also to prevent urinary tract infections/

There is another medication for low blood pressure called midodrine

(aka proamatine) which works well for low blood pressure. It is

quick acting lasts about 4 hours. The problem with this medication

in Pam's case is that it should not be taken if she will be lying

down, as it can cause the supine pressure to go too high.

The two meds work differently -- florinef works to increase blood

volume, midodrine works to constrict the blood vessels, so they can

be used together. This is something she might want to discuss with

her doctor, as if increasing water and salt with the florinef doesn't

help, adding midodrine when she is going to be getting up might help

her sustain a high enough bp not to pass out.

Please remember that I am not a doctor, and am only passing along

information I have learned here on the list and through managing my

husband's illness. Pam should discuss all this with her doctor.

Carol & Rob

> Hi Carol,

>

> Pam is currently taking Florinef for low blood pressure although

she

> reports it does not work very well. Many times the nurse is unable

to get a

> recorded blood pressure and when she does succeed it ranges from

58/38 to

> 70/44. These are readings taken when she is lying in bed.

>

> I have told her about the medication for neuropathic pain which

you

> mentioned and she will discuss it with her doctor. Before she only

had pain

> in her right leg above the knee. Now she is reporting pain

elsewhere on her

> right side and arm.

>

> As far as her going to Springfield or elsewhere to a doctor, I

don't

> think it is possible any longer. Her doctor is in North and

she can

> no longer go even there. Her internist will begin seeing her at

home later

> this month. I think her neurologist will no longer see her as she

cannot

> come to his office.

>

> I read your message to her so she will determine what she will

discuss

> with her doctor and her nurse from Hospice. I thank you for making

the many

> helpful suggestions you have.

>

> Maureen~

[Guest guest]

Hi Maureen,

If my atlas in correct town, MA is close to Albany, NY. Currently

there are no support meetings being held there but I'm sure Ellen would

be happy to talk to you or your sister.

Ellen Vitagliano

mev945@...

Contact for Albany, NY

I also suggest you call the Hospice people and demand to speak with a

supervisor. This lack of bathing issue is intolerable. Also she should be

given something to manage her pain.

As for Florida members there are several near Tampa and Sarasota. I can

give you a list of names.

Regards,

Pam

Re: New Member

> Hi Judy,

>

> I am in Southwest Florida and Pam is in town, Mass. As far as I

> know there is no support group in her area, not that she would be able to

> go anyway given her condition. She is so alone. That is one of the things

> that most bothers me. She lies in one room alone all of the time with only

> a TV for company. I just went out and bought a cell phone with free long

> distance service every night so that I can call her at least daily.

>

> Does anyone in the group live near either of us?

>

> Thanks,

> M~

>

> If you do not wish to belong to shydrager, you may

> unsubscribe by sending a blank email to

>

> shydrager-unsubscribe

>

>

>

>

>

[Guest guest]

Marueen,

Blood pressure medication varies for each patient, but here is what

Rob takes for blood pressure:

Florinef: - 3 0.1mg tablets once a day (at bedtime)

Proamatine (aka midodrine) - 2 5mg tablets 3 times a day, 8am,12n,4pm

Thermotabs (buffered salt tabs) - 1 4 times a day, 8am, 12n, 4pm and

bedtime

He also consumes 2 liters of water a day.

Please remember that he started at lower doses and worked up to these

over time as he needed to. And I don't want to overstate how well he

is -- he still feels loousy sometimes and there are days that he just

needs to lay low. But, he still functions fairly well, albiet

slowly, most of the time.

Based on Pam's low bp, I'd say that if adding salt and water doesn't

help she might benefit from increasing the florinef and/or ading

midodrine. Hopefully this will give her some information to discuss

with her doctor. By the way, Rob sees Dr. Roy Freeman at Beth Israel

Deaconess Hospital in Boston. He is an autonomic specialist and I'm

sure would be happy to speak to Pam's doctor on the phone if that

would help. Let me know if you want the number.

Your mother's reaction sounds like denial. It's only natural to think

that Pam is young and she (your mother) is old, so Pam should be more

healthy. Hang in there!

Carol & Rob

> Dear Carol,

>

> Wow! That medicine sounds like a real posibility. Even if it

worked just

> for a short time each day, Pam would be thrilled. I do realize it

may not,

> but at this point anything is worth a trial.

>

> Your husband takes 37 pills a day? That sounds immense when

contrasted

> with the few that Pam is taking. Currently she is on Sinemet (150

mg 3

> times a day) and Florinef (.1mg 1 time a day). She also takes

Synthroid

> once a day for the Graves' Disease. That is the entire regimen.

> Occasionally she takes an additional Sinemet 1/2 or full dosage,

but feels

> very guilty about it as the doctor is so strict about what and how

much she

> may take. It sounds to me as though there is room for improvement

here

> somehow.

>

> You are right about the necessity for some physical therapy

should she

> be able to get up again. I have suggested it and Hospice has

offered it,

> but, due to the family situation, it has thus far been refused. I

do not

> understand how my mother can stand in the way of these things, but

she

> does. Her retort is that if Pam needs this much attention she can

go to a

> nursing home. It seems to me that there is more that can be done

for Pam if

> I can get some cooperation. It won't be easy and I will not be

popular, but

> I am surely going to try to alter this situation.

>

> Thanks for the encouragement. I am so glad that Rob is doing so

well!

> That gives me hope.

>

> Maureen~

[Guest guest]

My 6 yr old son has a complex one and 4 defect in OxPhos which is a type of mitochondrial disease. When they tested him for Pearson syndrome his DNA did not show that he had it. However, his doctors refer to him as having "phenotypic" Pearson syndrome - meaning he has every sign of it even though his DNA test was negative for it. he has profound bone marrow failure and is dependent on red cells and platelets and other blood products. he has the renal tubular acidosis and liver failure often seen with pearsons. he also has the classic vacuoles and sideroblasts on bone marrow biopsy. he is currently being treated with desferol for the iron overload that is secondary for the transfusions and sideroblastic anemia. he was diagnosed with mito in infancy but his marrow did not fail until he was 2 years old. Though he has many many medical issues he is a bright and vibrant little boy who has more resilience and spunk than anyone I know. Anne New MemberHello everyone,My name is Ellen Mollineaux. I have a two year old son who wasdiagnosed last year with Pearson's Syndrome. I found this site and HAD to join. Pearson's is SO rare I was hopeful that someone who belongs to this groupo would have someinfo to help me and my family. I have done alot of research and have seen manydoctors. What I need is the info from parents because clearly, we often know a greatdeal more than the so called "experts". I would appreciate any help at all.Thank youEllen

[Guest guest]

---HI, Thank for writing me back. I am really glad I found this

group. My son's syndrome is called Pearsons syndrome. Right now,

his only symptoms is that he is transfusion dependent. Pearsons

supposedly will become Kearns Sayer syndrome in the future. We

started my son on Aranesp shots in April and they have helped

tremendously!!!

Thanks for listening,

Ellen

In Mito , " gazubich " wrote:

> If you could describe some of the symptoms your son is having,

maybe

> someone would have some experience in that area.

>

> Geri-Anne and Wyatt, Complex I-

>

> -- In Mito , " ellenmollineaux "

> wrote:

> > Hello everyone,

> > My name is Ellen Mollineaux. I have a two year old son who was

> > diagnosed last year

> > with Pearson's Syndrome. I found this site and HAD to join.

> > Pearson's is SO rare I

> > was hopeful that someone who belongs to this groupo would have

some

> > info to help

> > me and my family. I have done alot of research and have seen many

> > doctors. What I

> > need is the info from parents because clearly, we often know a

> great

> > deal more than

> > the so called " experts " . I would appreciate any help at all.

> >

> > Thank you

> > Ellen