Update on me.....got a diagnosis
 

Hi All,

I just thought I would let you all know that I actually got a diagnosis yesterday. I have been diagnosed with postural orthostatic tachycardia syndrome or Pots for short. I am going to try and explain very basically what it means as I am struggling myself with explaining it.

OK in normal healthy people on rising from supine position and standing your heart rate would increase by 10-15 Beats per min (bpm) and then in a few minutes it will settle back down as your body gets used to the pull of gravity on your blood supply, on standing up blood drops to abdomen and legs. In POTS our heart rates (hr) go up by at least 30 bpm and stay there. Mine hr goes up more than 30bpm. All I know is that during the test my hr went to over 150bpm and it also hovered around 130/140 bpm. In POTS you don't have a drop in blood pressure (BP) bp stays the same or rises.

POTS is treatable not curable and some people go into a spontaneous remission, some people don't.

The good thing about the diagnosis is that it opens the door for more testing for me. Its unlikely that I have primary pots as my symptoms were not triggered by pregnancy -don't have kids, trauma - no, surgery no not when symptoms started. Secondary pots runs alongside autoimmune disease like MG (funny that!) and ehler danlos syndrome- EDS (joint hyper mobility). I do have joint hypermobilty probably EDS - but my GP was saying I couldn't be referred to a specialist.

I only got my tilt table test as they wanted to humour me. They thought it wouldn't show anything and my GP told me POTS doesn't exist and its normal for your heart rate to go up over 30bpm when standing.......really?

The tilt table test is the gold standard. Not a pleasant test but then what test is.

So I just wanted you to know that I may not be posting regularly but my adventures continue. For those who are interested in my story and my latest appointment I have details on my Blog http://themyastheniakid.blogspot.com/

Thanks for all your support guys. Particularly Annieb3, Alice and my face book chums.

Love
Rach:hug:

I am so glad you have a diagnosis. :D Hooray!

I learned about POTS when I was going through major blood pressure swings and tachycardia and researching online what could be wrong with me. I saw that they use mestinon (of all things) for treatment. They had removed my mestinon to assess my "true strength" for thymectomy surgery prep and all these cardio symptoms started. My sx got better when we added back the mestinon!!

Are you using mestinon? If so, is it helping? I really hope so. If not, I hope they find something soon for you. It is awful to deal with those swings in heart rate....

debra

Hi Guys
 

Thanks for your messages.

Debra - yep I use mestinon although I haven't taken any for a few weeks as I wanted the tilt table test to be as dramatic as possible.

The problem is I don't tolerate mestinon too well even with the probanthine. I have developed symptoms of gastroparesis and as the mestinon stimulates my gut it gets very painful and I end up having to take morphine which makes me sleep! LOL

I have already upped my fluid intake, I drink a lot anyway over 3 litres a day as I have excessive thirst - not diabetic been checked numerous times. But I have found out excessive thirst is a symptom of autonomic dysfunction. Ive also increased my salt in take, as much as possible. So I just need to wait and see what the doc suggests in 3-4 weeks time.:hug:

Desert Flower - cheers, one diagnosis down, a couple more to go but Ive got my foot in the door now and hopefully I am no longer considered a nut case!:hug:

Love
Rach

:Dancing-Chilli: That's how I feel for you. How great is that, Rach? Like I told you, I looked out my window but couldn't see any pigs flying. They probably froze on their way over here.

I know this isn't the end of your medical struggles nor does it make you all better but at least there is definitive proof of this now. I imagine your next appointment might look like this. With you winning, of course.

:mf_swordfight:

I can almost hear you saying, "Now that wasn't so hard to do, was it? Just lay someone flat on a table and tilt them. Voila, problem solved."

I hope you check in from time to time. I still think you have an acetylcholine problem too.

I'm so glad you got "some" care. Now you need some more.

:hug:
Annie

Rach,
I'm glad you finally have a diagnosis, it's so frustrating when you know something is wrong and nobody can figure it out.

I took a peek at your blog, loe your dog!

Rachel

Hi Annie and Rachel
 

Thanks for those messages.

I will still be around as I have made too many friends here to leave!

I think you are right Annie I do have an acetylcoline problem, its probably autonomic neuropathy, and the something to do with the ganglionic (spelling) receptors in the brain. I sound like I know what I am talking about LOL

Rachel thanks my mad dog on my blog is Frankie! Thanks for looking at my blog.

Thanks again

Love
Rach

This is such wonderful news!! You have been such an incredible trooper in your search for answers. Just to get a dx has to be a delicious victory!!

I'm extremely impressed by POTS' causing your ptosis. ;)

If you don't mind answering, what kinds of tests did you get for familial myasthenia gravis and congenital myasthenic syndromes when they did all of your testing?

Rach73, I will be interested in how this works out for you. My internist told me I have POTS and likely low blood volume which can go with it. This came as a response to my daughter telling them how I get so much better when I get IV fluids...salt and water. The info on it from the medical site Update is really good and my doc gave it to me to educate me. It tell about the mestinon use at 35-45 mg a day for treatment. Do you know that is all it takes? You wouldnt have to take the 60mgs and such for MG. Dr Low at Mayo is the one who did the researcxh on this and his study I think is still in progress.

Unfortuantely in trying to see a specialist for this in January I got the first of a string of docs at the university that has me in this place I am in unable to get treated there. I was stunned when I read his notes. He said he felt I was bringing dizziness on my self by withholding water. Given I have Sjogrens and have to drink to even swallow well first thing in morn that is rediculous!! he added that he also thought the symptoms were in part from me hyperventalating. Damn! I have never hyperventilated in mylife. He totally when down the blame the patient route. Unfortuntely he was a neuro so this really hurt. I had to counter his acusations with even my lung guy who I saw after this and thankfully was prepared to do the mestinon challenge that time which proved how great I do on my pulm tests with mestinon in me so he didnt take that docs stuff seriously after that. But others have.

I have alot of symptoms of autonomic dysfunction. Sadly some of the symtpms are anxiety and breatrhing stuff that make it too easy for docs to want to go to the mental route.

I hope you get a good turn out.

Annie59

Rachel, I'm happy you have a diagnosis. Is this in addition to the MG or instead of the MG? I hope I'm not out of line in asking.

I did read some of your blog. It's always interesting to me to read others stories. It sounds like you've had a long road on your way to being diagnosed.

Good luck with everything.

Hi Guys
 

Thanks for all your messages.

Mestinon - Unfortunately just one 30mg dose is enough for me to be in agony. So I do try to avoid taking it now as my stomach problems are so much worse. I do take it when I experience problems with breathing (elephant on the chest sensation) or if I have ptosis that is threatening to close both eyes.

Fluids= Like you Annie59, I drink excessively over 3 litres a day, think its called Polydypsia (probably have spelt that wrong), Ive always drunk loads even as a baby. Ive had more tests for diabetes than Ive had hot dinners and they always come back fine. I suffer with dry eyes and mouth and have raised this with my GP, but it was ignored. Perhaps now with a diagnosis of POTS I may get him to listen. I need to email him with the results of the test (TTT) but every time I start I end up getting angry writing rude things and not sending it. LOL! Will have to calm down.

MG or Not MG Ive had the achr blood tests, Musk tests, SFEMG x 4 all come back negative. However my clinical signs on examination by a neuro ophthalmologist led him to believe I have MG. The only test I am positive on is the ice pack test which shows that there are issues with the NMJ. Ive had no genetic testing for CMS. One tensilon test was positive one was negative.
Who's to say I don't have ocular MG? My eyes don't move properly, one pupil drifts out to the left when I am tired and I can not look up without both eyelids dropping? I also have double vision which resolves if I close one eye So to me the jury is still out......thats why I wont leave the party LOL

Ptosis Low blood pressure / Pots in rare cases can cause ptosis and facial droop, like you have had a stroke. There was a case reported on in the New York Times which forum members on DINET forwarded to me. When I have ptosis my BP can be low or low normal. Again the jury is out. What complicates the issue is that mestinon raises the blood pressure, so does my ptosis resolve because of my blood pressure rising or is it because I have MG?

So there are a lot of questions I don't have answers to but hopefully this will be the start of me being taken seriously or at the very least getting treatment for POTS, which is leaving me pretty much bed bound at the moment. I only feel OK when I am lying down.

Thanks again for all your messages xxxx

Love
Rach

congrats! i am glad to see progress, especially in the form of diagnosis.