Goschi

Thanks, Hearts!!

I send you lots of strength too!!

Hi Raisin!

My hospital stay was planned, so I am in general happy to be here, hopefully getting some more answers.

I haven't had a hemodynamic test and I am not sure whether they can do this here - the docs never mentioned it.

But I guess, they are sure that I have blood pooling, it seems to be common with pots (?)

What dosage of midodrine do you take?

Do your leg's skin look very much mottled when you are upright?

Best wishes!!

I am in hospital right now (since 10 July). The original main purpose was/is to find the underlying cause of my POTS.

Up to now, the docs didn't really succeed, however some diseases were ruled out at least. (Morbus Fabry, Morbus Wilson, Multi System Atrophy, Thyroid issues).

Parallel to the tests, they started me on different therapies, in order to see how I react to them - this might also be of diagnostic value.

I now take 2,5mg midodrine 3 times/day and will start with 0,05mg Florinef today. In addition, a slight exercise program (recumbent bike) was started.

I have to say that the midodrine gave me some improvement. My "general malaise" is better, I have less brain fog and I can stand and walk for longer periods of time.

However, what I don't understand (docs don't either...) despite these improvememts, my legs and lower arms still show the typical, extreme mottled skin pattern. No doc has yet had a clear explanation for what this actually is, they still confine their statements to "must be a sort of autonomic dysfunction" (really, is it???)

Anyway, I am very curious (slightly optimistic) whether I will leave this hospital in a better state than I came in!

Hi blue,

thanks for your message - however, we are running into one of the world's most "famous" mistakes! ;-)))

I don't live in Australia, but in Austria!! No kangaroos and koalas here!

I will ask my GP about the nasal spray (the brand name here is "Vividrin"). It only contains liquid Cromolyn, so it should in fact be helpful! (even through the nose! ;-)).

I am not in the US, the brand Gastrocom isn't available where I live (Austria). I only found an anti-allergic nasal spray that contains Cromolyn. I wonder if this would be as effective. Is Gastrocom a liquid for internal use?

I know this phenomenon quite well too! The natural automatism of walking seemed to have disappeared!

However, since I am on Midodrine (5 days now) most of my normal feeling with walking has returned! So I guess in my case it was probably an issue of insufficient vasoconstriction in the legs(?). My legs (and arms!!) now also don't feel as heavy as before!

Before I was on my betablocker, my BP range was between 120/80 and spikes with 190/120. Since I take bystolic, my BP is always between 110/75 and 130/95. Docs regard this to be perfect, most "healthy" people have worse figures, I am always told.

Besides, I asked some of my healthy friends to take their BP regularly, as I really wanted to have some figures to compare. And in fact, most of them have extreme BP swings in the course of the day without realising and without feeling bad at all.

Hm, that sounds rather complicated, to be honest... In fact, finding out whether your son has small fiber neuropathy or not won't yet fix the problem. But SFN is again in principle secondary, which means that an underlying cause can (and should) be found! And, most important, Pots can possibly fixed, if the underlying cause of the SFN is treatable, though it may take some time for the small fibers to recover.

Anyway, SFN is a very typical comorbidity of Pots. Most Pots patients don't have it because of the two main causes (diabetes and alcohol), but rather because of some sort of autoimmune condition. And that's where you already are concerning your research, as far as I understood. Therefore, finding out about the SFN and it's cause could contribute to completing the puzzle (?)

Well - what to say? Some time ago I always grew very angry when I read such a kind of nonsense (or was told it by a doc personally).

But as a dysautonomia patient you slowly get used to the fact that a portion of medics simply doesn't know their business. Annoying, yes - but in fact not surprising.

You have this phenomenon in every scientific field, so why not in medicine?

So, just read it, shake your head, laugh it off - and forget it as soon as possible!

It's the only thing that does help. He has been tried on a few other medications but they will not keep his blood pressures up. He has had the QSART test at MAYO and it was negative. Our cardiologist does suspect neuropathy pain is a major problem but have not found a neurologist willing to do the biopsy for the other.

My skin biopsy was done by a dermatologist, maybe you can find one easier? However, the Interpretation should best be done by a specialised neuropathologist, but I am sure, Mayo should have one...?? The biopsy can be made and then sent to another place, so you don't have to travel to the lab that finally looks at it!

Becia, don't worry too much about Bystolic! For me, it actually lowered HR, not BP! Just be careful with the dosage, maybe start with 1,25mg and look what it does!

For me it was particularly helpful for supine HR - before Bystolic, I sometimes couldn't get my HR down after standing, Bystolic really solved this problem!

QSART can sometimes be false negative (mine was!) - skin biopsy is definitely the gold standard. However, even if he tests positive, it would be decisive to find the underlying cause for the SFN!

Hang on!!

I am on Bystolic too (1,25mg once per day) - together with the midodrine it seems to work well. I also have no side effects from it, it seems to be easy to tolerate (I know other patients who take it too).

I would also be rather careful about further ablations! Pots can definitely be treated with medications as a first step, next thing would be to try finding the underlying cause.

You will eventually get thru this rough time! I send you all the best wishes and tons of power!!

Very, very interesting with the Prednisone!! My Pots came in gradual onset since 2012. But I could handle it (though not knowing at all, what it actually was).

But in March 2013 I recieved a few Infusions of Prednisone (for a sudden conductive hearing loss) - and literally from one day to the other my Pots got into full blow - and didn't actually improve since then...

This is in fact a rather high dosage - I hope your son benefits a lot from it?

Btw, was he ever tested for small fiber neuropathy? I have it (in a rather mild form) and it definitely seems to play an important role with my blood pooling.

All the best wishes to your son and you!!

A couple of docs has taken a look at my mottled legs today - and they all agree that the pattern is very untypical and they are not yet sure what it exactely stands for.

When I am standing, about 70% of my leg's skin is in a rather bright red tone. The rest appears totally normal in colour. However, the red area is interspersed with numerous very white areas, almost all of them in the shape of regular circles in the size of a large coin.

As it is clear to the docs that this whole picture is of course reflecting abnormalities in blood circulation under the skin, they want to find out what exactely is going on.

Btw, taking midodrine makes me actually feel better when I am upright (pulse rate is lower too!) but the mottled appearance of the legs doesn't go away at all!

I was already checked for Lyme twice (Elisa, Westernblot and Igenex) all negative both times, but they will check another time, I guess.

Does midodrine help your son? Which dose is he taking?

Goschi : A PPI is a 'protone pump inhibitor' that is used in GERD and other gastro disturbances. It lowers the amount of acid that is produced.

Thanks! Have heard about such a device, but didn't know how it's called. If it has vasoconstrictive effect, would be worth a try!?

I am really sorry for having to ask - what is "ppi"...?

I am starting my urine testing for Morbus Wilson today, and they draw some more blood. Lots of other tests will continue on monday.

One neuro doc was in my room, telling me that the fact that midodrine makes me feel better doesn't point to any particular causes - just to the fact that more vasoconstriction is indeed helpful, but it wouldn't yet explain why I actually need it.

However, he also investigated my mottled legs (when I am standing) and seemed a little concerned. He took lots of photos of my legs and told me he had never seen my particular skin pattern. On monday, a team of 3 docs will again take a look at my legs.

At least Morbus Fabry is now excluded (for males the resp. blood test is definite).

And I start to like the midodrine more and more! While I am still not sure what it does for me concerning physical activity, it definitely helps with brain fog! As well as in any upright position as being supine! Haven't yet spoken to a doc about it, but anyway this must mean something to, I guess!

I am not yet really sure, it's still too early - but I take midodrine since 2 days and besides some other things I realised that my gastrointestinal issues (IBS-like) started to improve!

This leads me to the conclusion that my gastro-issues were in fact all due to abdominal pooling - otherwise midodrine wouldn't be able to resolve them, as it "only" works as a vasoconstrictor.

So maybe, midodrine could be a solution for you too?

They may not have too much experience with Dysautonomia as being the only illness in a patient. The symptoms of Dysautonomia can be symptoms of other autoimmune illnesses. I know you probably did not want to deal with anything else on top of dysautonomia but maybe it will go away if they figure out what might be the underlying cause. How many more days are they planning on keeping you?

Well, in fact I always thought my dysautonomia to be just a symptom on top of my actual disease (whatever it may be). Thus, what they are doing here is exactely my point of view.

And I never wanted anyone to simply treat my Pots-symptoms, I always regarded this to be a strategy that leads nowhere.

But here in this hospital I met docs who were surprised that I feel dizzy when upright and told me "can't be from POTS, this only makes you tachy. The dizziness must be from an other condition."

I will stay here for at least for the whole next week, maybe even longer. I try to make the best of it - and don't expect too much in order to not being too disappointed, just in case.

Goschi

Sounds like they are on the autoimmune track or looking for something gentic. Chorea can be connected to several conditions. Hang in there, it sounds like these doctors want to help find the underlying cause of your POTS. Keep us posted on your results.

Ctat

How are you doing with the midodrine? Did the doctor up your dosage?

Well, you are of course right! - I am really glad that they approach my whole condition, I was just a bit disappointed how little experience they have with dysautonomia...

Concerning the midodrine: I take 2,5mg three times a day, it could be upped, depending how I feel on it.

But in fact, I don't really know - I definitely feel "different", no doubt! I feel less fatigue, a bit more "awake" in general. This is certainly very pleasant!! But though this may sound contradictive: I still feel weak, clumsy, heavy etc. when I am upright, though on the other hand, I can walk for longer distances and feel less dizzy. Still don't know what to think about midodrine...

Well... Turns out they really try very hard to search for any underlying causes, some rare things no one ever considered before: Morbus Wilson, Morbus Fabry, Chorea Huntington, Multi system Atrophy (scary...).

On the other hand, I learned that they are not really familiar with POTS/dysautonomia. Therefore, they often don't understand my symptoms as typical POTS-related and instead desperately try to find the "cause". A bit frustrating, to be honest...

However, at least the midodrine actually changed "something", can't yet really describe what exactely.

And I learned that I don't tolerate compression stockings at all. They give me a weak and uncomfortable feeling inside my legs (not on the skin!). I was surprised about that result and the docs here actually baffled...

...I have a drumming lesson this afternoon that I'm saving my spoons for.

Good luck with it!! Beat the drums loudly, imagine "bully POTS" hearing it and getting very, very much afraid!