The Good News: No Epilepsy. The Bad News: Autonomic Damage is Worse

[Carolina]

Hi Anita,

I know you have POTS and was hoping  you would comment.

1)  I haven't had the tilt table test.  I haven't seen an autonomic specialist.  But two cardiologists checked me, two years ago,  for falling BP, when standing, and I didn't have it at all.  This was before I developed the falling, which was just last spring. Don't ask why.  I had actually asked to be referred to check my SFN, and wound up with cardiologists.

2)  I don't pass out when I fall, I just crumple.

3)  I take medication for HIGH blood pressure.  With that med. my blood pressure is OK.

4)  So YOU have the shaking too?  Is it just orthostatic?  Mine is.  although I have some odd tremors and movements at rest, too.

5)  I have a self-use BP cuff, and my BP doesn't drop when I check it, either.

6)  I will try to get a referral to a doctor at Duke who specializes in autonomic problems.  But honestly, I've checked out all of the autonomic problems and I don't seem to 'fit' into any of the categories.  However autonomic problems seems to be more complex than just categories, or at least I think so.

7)  Right now I am just discouraged about this entire problem, and I'm also in the second week of the three-week bronchitis from Hades...so I'm not really ready to start on another quest for answers, quite frankly.

I am in a 'discouraged phase'.  That doesn't usually last very long.

Hugs,  Elaine

[anita]

The tilt table is the standard test for orthostatic hypotension...I'm surprised you haven't had it done.  They can check what IS happening when you 'crumble' by constant monitoring of your vital signs during the change in position.

There is an entire autonomic battery of tests that can be done to check various parts of your autonomic system.  I'm' sure Duke would have an autonomic specialist there to help determine what is happening.

Yes, I have the shaking one just one side.  It doesn't seem to be related to orthostatic though...but still just my left side shakes.

Now that you mention the med for HIGH BP, it is even more important that you NOT use the compression stockings until a doctor orders them...as they can increase your BP (because that is what they are designed to do).

Just so you know...falling BP when you stand does NOT happen every time (even for most patients with orthostatic hypotension).   Just like you don't 'crumble' EVERY time you stand.  BP drops can happen frequently or infrequently in any patient, so sometimes hard to Dx this...especially by just having the patient stand and take their BP to see if it drops (like cardiologists do in the office).  Why the tilt table is a good test...as they can tell what is happening DURING the change of position, not just after you stand.

[MAT51]

Dear Carolina. I'm really sorry about what you are going through. But I'm also very grateful to you for setting out what you've been told in such a clear way. My late mum had very similar symptoms for the last five years of her life before dropping dead suddenly at 73. My dad had diabetes and also had many autonimic features even as a young man. He would suddenly drop while crossing a road. He broke his ankle this way and walked with a cane for decades prior to his first heart attack - caused by late onset diabetes. He too died suddenly at 73.  It transpired that my mum had severe atherosclerosis that had never been discovered by the few medics she sought out. Both had quite rational explanations for their own physical declines but nevertheless it's quite an inheritance! So I'm grateful for your post because it gives me a different perspective. Neither of my parents had Sjogrens or any other connective tissue diseases.

Your description of symptoms is so like advanced MS and even MND. And yet Sjogrens is still, at best, only really known about by the public at large as a disease suffered by Venus Williams?!

I can see the bewilderment in my GP's eyes when I describe my latest symptoms and ask if these might relate to my Sjogrens. They half heartedly affirm with expressions that tell me they really only know Sjogrens as a disease that causes dry eyes and mouth, at best. I can tell that if I didn't have this confirmed diagnosis it would all be classed as "functional" - as was starting to occur prior to my lip biopsy.

And really, from all you say, there isn't a lot of difference in the way it's treated. But there's a huge difference psychologically in knowing the cause and understanding the very rudiments of the mechanisms and being realistic about the prognosis. I'm very glad that you have such a supportive husband and can access complimentary therapies and physio that help you live with this level of disability at least.

I have similar symptoms to a very small extent - worst part affected is currently my GI system - which doesn't seem to be very functional at the bottom end at all. I don't have your quakes but I do have the SFN and because of you, Irish and Anita and others, I'm much more aware of where this might lead than I would have been otherwise. As a pragmatist this is good because forewarned is forearmed!

Mine has been described by my neurologist as "presumed ganglionopathy of Sjogrens". I would like to be tested by an autonomic specialist to have clarrification but, with the NHS currently in tatters - and no healthcare insurance (in common with the majority of UK residents still) there's not much chance of this. I have twitches and resting weakness in my limbs and heat and cold intolerance with no sweating and proprioceptio issues, all mildly and all probably down to Sjogrens. My BP is usually just rather high despite BP meds and I don't have your falls yet very thankfully.

But more than anything your post makes me feel how urgently more research is needed into Sjogrens and autonomic dysfunction - even if it's a relatively rare presentation of this disease as I'm told. I read Facebook posts and posts here and wonder if it's quite as rare to have Sjogrens affecting the whole body and autonomic nervous system as my doctors suggest? I keep hoping that the research done by Birnbaum at the John Hopkins will at last filter down more into the rheumatology and Neurology communities here in the U.K but I fear that in these financially straightened times this is very unlikely to happen. There tends to be eye rolling from medical professionals here about the kind of testing done in the US I'm afraid - and IViG is not on the menu here at all unless things get a whole lot worse. There is no protocol for its use for Sjogrens here.

So your experience and realistic resignation about your prospects remind me to utilise my physical faculties while I can. And I do hope like anything that more is done for those with Sjogrens related PoTS than can be done at present. Scary stuff really -but i admire your pragmatism and hope that you overcome your bronchitis soon and your choice of car works out well. Take care and best of luck.

[irish]

caroline, I am just sitting here thinking of you and trying to make sense of your attacks. It would probably be wise to do the testing for autonomic disease but I know it gets old running to the doctor all the time.

I did wonder a bit if you have had your carotid arteries checked what with your history of artery disease. This can cause some interesting symptoms also. Do you fall every time you arise or periodically??? Just curious as it really does sound like a positional thing that could have many causes. Good luck in your qwest. Irish

[MAT51]

I'm with Irish, Carolina. My mum died suddenly after years of ignoring sudden crumpling and the beginning of vascular dementia. It turned out that all her symptoms related to severe atherosclerosis and a carotid duplex rather than a brain CT (she was given on her Bupa insurance!) would have shown this. The rest was peripheral artery disease which she also failed to get checked for. Coroner said all could have Beeb adddessed.X

[Nymph]

I agree with Anita, sounds like a complex dysautonomia. There are quite a few people with dysautonomia with high BP. Treatments may be quite different. There is an autonomic specialist at Duke. Some people really like her and others not so much. Camille Frazier-Mills. She has a syncope clinic. I believe that they can do a complex tilt there with catecholamine monitoring. If not then there is always Mayo (Rochester) or Vanderbilt. For extensive reviews of any of these clinics see DINET forums, where people talk about their experiences going to each one.

Right... DON'T wear compression with high BP unless a doctor approves. It could backfire. Some people do but usually they have labile, not high, bp. I hope that you figure something out and find someone who really knows their stuff.

hugs,

Nicole

[Carolina]

I saw Camille Frazier-Mills about three years ago, before the falling and jerking/tremors, started.  She did the standard sit down stand up test, and my blood pressure was fine.

I've asked for a referral from the two neurologists I've seen, and now I know that Dr. Frazier-Mills is the doctor I should see.

I FELL AGAIN YESTERDAY.  I hit my head on the bed post, but not badly.   It is really really upsetting when I collapse.  I didn't even know it was happening until I was almost all the way down.

What I need, probably, is things to do to prevent the lack of blood flow to my head (my husband says that is the problem, why I fall).

Maybe sitting down and standing up several times before I start walking?  OR not sitting for more than 10 minutes without getting up and moving?  I could set a timer on my phone to go off every 10 minutes.

That's what my hair stylist insisted I do while I was waiting for my hair color to 'take', because I had fallen in her salon in late December, with both her and her assistant holding my arms!

I need mechanisms to make this less likely.  I don't think a medication is available for this

YES, Irish, I had  my CAROTIDS scanned in 2014, after my first arm jerking experience.  They were fine then.

I cannot express enough my gratitude for your caring and attention.  I am really have a problem with this falling, emotionally as well as physically.

And of course still in the second week of the three week bronchitis.  Feeling better.  Will get back to exercise soon, and that will help my 'sense of well being' for sure.

Hugs, Elaine

[gurs]

I was diagnosed with POTS few years back. Think Ive always had some form of this, pretty mild until my hysterectomy over 10 years ago when It just blew up after. No fainting, but extremely low BP, problems with going to the bathroom, Hypermobility, dizziness, sweating/cold etc. The doc didnt want to put me through the tilt table, convinced I did have POTS. I ate more salt, lots of potato chips and still have not been able to take baths or showers. I sit it the tub and sponge bathe basically. Extreme temp changes send me for a loop. Summer is horrible to deal with. Anyways, was managing until my sinus surgery last august. I woke up feel super dizzy, problems breathing, anxiety, depression, seems the POTS got alot worse. I thought it was temporary like my docs said, but still having these issues. Guess what!!! a week after surgery I developed high blood pressure??? not too bad 135/90, and still cant get it to go down. My sodium/potassium levels low too. I saw my POTS doc about 2 months ago and he said my ANS was likely damaged, and that most autoimmune patients wont recover. There is no medication etc and the fact the side effects of most medications just make it worse. I guess my point is, even after going to these specialist, what can be done? not much from what I understand. My gastroparesis is alot worse. I can barely get anything down and my intestines dont work either. Its very, very, upsetting and scary. Im going to try rituxan again because there is no other options.

Gursie

[anita]

One of the things they always tell people that fall after standing, is to sit up on the edge of chair, bed etc first, then wait a couple minutes. Then stand slowly, but next to something to hold...wait again.  Then start walking.  It gives your body time to adjust to the change in position.

One thing that is VERY common is blood pooling in the feet of those with autonomic dysfunction....hence the stockings.  But you must be confirmed in having this (and blood flowing away from the brain) before using the stokcings...due to possible increase in BP (especially if you already have high BP).

The BP drops other experience can be very quick...and most immediately wake up or are ok within seconds.  It seems for you  it only takes a small amount of blood leaving the brain to cause a reaction and make you drop.  They will also need to look at your current BP medication ,now that you are having this problem. 

The sit down BP then stand up BP is NOT the same as the tilt table.  It is similar but nearly as precise.  I'm sure you will be asked to have this done, so they can find out exactly what is making you collapse.  I know they said it was autonomic, but they need to find out WHICH part of your autonomic system is causing this...like BP, heart rate, etc.

[Carolina]

Hi Anita,

At this point what I am doing when I stand up is this:  I stand up, then sit down, then stand up, then sit down and put my head between my knees. 

The odd thing is that holding on to something doesn't help prevent the collapse, since I lose all muscle tone, and let go, even of my walker.  This is why I wonder if my collapse isn't something neurological rather than from blood flow problems?    Brain tumor? NOT epilepsy....  just so odd

The standing and sitting and standing and sitting and head down behavior should insure that blood is flowing to my brain if that is the problem.

While I asked for a referral to the clinic at Duke that tests for autonomic/dysautonomic conditions, I don't think I'll go.

What would be given to me (medication) if I'm found to have problems with blood flow to my brain (or within my brain for that matter)?

If there isn't something I can take to eliminate my falling, I might as well just 'do this procedure'' and hope to prevent falls.

You can see that I'm discouraged.  The folks on the Dysautonomia FB page are not enthralled with the Duke Clinic (and they are Carolina people).  They suggest Mayo.

I think that at almost 75, and with a long history of conditions, I should try to prevent the falls with careful standing up and exercises before embarking on walking. 

Worth a try.

I was so shaken up by the fall...and it is the complete collapse with no warning that is so unnerving, that I just panicked. 

Hugs,  Elaine.

[Nymph]

Elaine, I'm not surprised that you panicked. It is really difficult when you have so little control. There ARE meds that help some people, though. It does seems to be different meds for different people. I have had success with a simple (but pricey) OTC supplement, but many others try numerous meds at various doses to find something that helps. I guess that my message is, Don't give up! Your safety, mobility, and happiness are worth it. I know that you've said that you're on bonus time, but 75 is not that old! Just my dad's age! Hang in there. I am rooting for you.

[anita]

Well, see how it goes Elaine.  However, that type of action (up/down) will not make it easy for BP to keep up in the brain.  The slow process of standing up (taking up to 5 full minutes) is what they recommend for those with orthostatic issues.   But everyone is different.  Try your theory and see what happens.  If it doesn't work then try the standard slow process I suggested.

You should go ahead with the testing.  There are several medications to help...but it all depends on WHY the blood is not staying in the brain (BP, HR, etc).  You have to be tested to determine that...it is not a guessing process.  It may just be an adjustment of your current BP med.  Let the doctors do their job and figure it out for you.

it will likely take a while to get the appointments for the testing, so you can try your standing routines while you wait for your appointments.  If they work and you have no more problems, then cancel.

[SjoGirl]

Dear Carolina,

I am so very sorry to hear of the latest bad news. I have myoclonus though at night. I too was tested for epilepsy (negatory) and MS (also negative). The first two times I experienced myclonus (involuntary jerking) it was related to Plaquenil. That said, it returned even after I stopped take Plaquenil.

Along with the myoclonus I developed occular migraines, neuropathy in hands and feet and mild neuropathy along my left side (my right side has not been tested). My neurologist, rhuematologist and physical therapists all chalk the neuropathy to my neck/spine disc problems but also to Sjogrens.

As for the myoclonus the cause is unclear. I have been taking Gabapentin now for about three to four months to control the jerking, deal with the migraines and to address tremendous nerve pain (again from discs, but docs say probably also Sjs).

I live with concern that these  issues, particularly the neuropathy, will progress, but neuropathy is something docs know little about and there is no treatment. I hope for you that Anita is correct and that you can find some relief, this is no way to live!

Hugs.

[irish]

Do you recover from this quickly or does it take time and maybe nap to get you feeling better. I am thinking of Tran Ischemic Attacks that can cause unexpected decrease blood to the brain. This usually ends up with people falling. They don't know it is going to happen until it is over or almost over.

Probably not the culprit, but the fact that this occurs when you change positions is bugging me. Good luck. Irish

[gurs]

So sad after reading these stories about what alot of us are dealing with. They never really mention this when referring to SS. Its horrific.
Hope you can find some relief and answers.

Gursie