Where To Start?

[4everOptimistic]

Hello! As a new member, let me just say that the info I have received here so far is invaluable. Thank you! Although I have been suffering the symptoms of dysautonomia for a couple years, I just recently received a diagnosis . Or at least, it is what my endocrinologist suspects I am suffering from since I have most of the symptoms but have not gone through testing to diagnose me specifically for dysautonomia. But man oh man, have I had testing!!! I think you all can relate.

I am currently diagnosed with PCOS (Polycystic Ovarian Syndrome), insulin resistant, pre-diabetic. I get all the related blood-work done 2X a year. At least. More like 8-10 times a year! Last year after biopsies of my hands and feet, it was discovered that I suffer from severe pernio. I think I saw that a few of you do, as well. I would love to hear from you. I now wonder if it is related to dysautonomia.

What I am wondering (and hoping you all can guide me) is where do I go from here? I have an appt at the Cleveland Clinic January 30th and am so thankful they got me in quickly. I am new to the diagnosis and am not sure what all the terms and testing mean. (I don't want to waste the Dr. time explaining everything to my husband and me.) Help!! What is one piece of advice you each would offer?

Thanks!

[jump]

Hello and welcome!

I've never been to the Cleveland Clinic, but it's my understanding that they're very good so they'll probably understand exactly what you need.

However, I've found that it's always helpful to bring a document detailing your symptoms, their frequency, when they started, what makes them worse, what makes them better, and your general medical history. Sometimes on the day of the testing you may have contradictory tests or inconclusive tests (although hopefully that doesn't happen as much at Cleveland!) and if your doctor has a clear picture of what your day-to-day symptoms are he or she will have an easier time sorting out what your tests could mean and which tests to take.

Also, you mentioned you've had a lot of tests already -- you should bring copies of any that are relevant to your autonomic dysfunction, and be familiar with what they say. For example, I had a 24-hour holt monitor test that showed my tachycardias upon standing and my bradycardia upon sitting; when I consulted a doctor about it, he had a copy of the test, but just read the brief section that said I had no harmful arrythmias. If I hadn't read the test myself and known what was in it, he might have missed my POTS symptoms. Just because they have copies of your tests doesn't mean they are reading them carefully!

Good luck and I hope you find some relief soon!

[iheartcats]

Agreed. Copies of tests - no need to be retested for stuff you've had recently. Good luck!

[goldicedance]

Good luck at the CC.

BTW, what is pernio?

Lois

[janiedelite]

Last year after biopsies of my hands and feet, it was discovered that I suffer from severe pernio. I think I saw that a few of you do, as well. I would love to hear from you. I now wonder if it is related to dysautonomia.

Thanks!

ME TOO!!! My first dysautonomia symptom was tachycardia in 10/06 but I noticed itchy red bumps on my toes that winter. They were only on 1-2 toes and I ignored them. I think I put hydrocortisone on them. But the winter of '07-'08 I had purple toes, red bumps, swollen feet for almost 5 months! A biopsy came back as pernio. My dermatologist accused me of putting my feet in ice water!

I know of at least one other member here who has Raynauds. My feet are either freezing cold (even if the rest of my body is hot) and purple/white/ or blotchy red, or in summer they are bright red and burning. I did get chilblains on my fingers this winter too. I've been to a dermatologist, podiatrist, vascular surgeon, etc. and no one can tell me why my blood vessels do this. I'm a nurse and have taken care of folks with Raynauds. It seems like Raynauds is more episodic, but in my case my feet are cold for hours on end. But at night, they warm up and turn red and burn again! I can't even have sheets on them because the burning is so tender.

Another wierd thing is that I always experience venous pooling in my feet (they'll turn purple and blotchy) and they can be stone cold at the same time. It seems like my arteries constrict while my veins dilate maybe????

I am flying to Mayo in Rochester next Tuesday. I hope they can shed some light on this because there are times when my feet turn completely blue and I can't even feel pulses. I look forward to hearing how things go for you at the Cleveland Clinic!

[ramakentesh]

I know of at least one other member here who has Raynauds. My feet are either freezing cold (even if the rest of my body is hot) and purple/white/ or blotchy red, or in summer they are bright red and burning. I did get chilblains on my fingers this winter too. I've been to a dermatologist, podiatrist, vascular surgeon, etc. and no one can tell me why my blood vessels do this. I'm a nurse and have taken care of folks with Raynauds. It seems like Raynauds is more episodic, but in my case my feet are cold for hours on end. But at night, they warm up and turn red and burn again! I can't even have sheets on them because the burning is so tender.

This is a symptom of POTS - excessive peripheral constriction is one of the things the body tries to do to increase blood flow to your thorax and brain. Normal flow POTS patients exhibit this after orthostatic stress, while low flow POTS patients exhibit this when supine.

[firewatcher]

I know of at least one other member here who has Raynauds. My feet are either freezing cold (even if the rest of my body is hot) and purple/white/ or blotchy red, or in summer they are bright red and burning. I did get chilblains on my fingers this winter too. I've been to a dermatologist, podiatrist, vascular surgeon, etc. and no one can tell me why my blood vessels do this. I'm a nurse and have taken care of folks with Raynauds. It seems like Raynauds is more episodic, but in my case my feet are cold for hours on end. But at night, they warm up and turn red and burn again! I can't even have sheets on them because the burning is so tender.

This is a symptom of POTS - excessive peripheral constriction is one of the things the body tries to do to increase blood flow to your thorax and brain. Normal flow POTS patients exhibit this after orthostatic stress, while low flow POTS patients exhibit this when supine.

Which flow exhibits both? I can send my husband shooting out of bed with my hands and feet...he looks like a scared cat with his hair on end. The nurse at Vanderbilt said they were the coldest hands she had ever felt. When I had the cold-pressor test, the hand out of the water was sweating and just as cold as the one that had just come out of the ice.

[Guest tearose]

I've suffered with painful hands and toes for years and only last year was Raynaud's diagnosed. I had always thought it was something we just had to live with. I am curious, what is the difference between Pernio and Raynaud's ?

tearose

[Maxine]

Welcome, and good luck at the Cleveland Clinic. I have had some luck there, and they confirmed that I was hypermobile, which eventually lead me to a geneticist for definate diagnosis of EDS.

They are really good at getting people in fairly soon at the CC.

This is the defination of pernio:

Sometimes there is another underlying disease or disorder, and pernio can be secondary.

http://emedicine.medscape.com/article/1087946-overview

Maxine :0)

[janiedelite]

I've been tested for everything that could have caused my pernio (autoimmune, blood cancer, vasculitis - I did have a trace positive cryoglobulin level but I have no hepatitis or any other problems with vasculitis).

So it sounds like Dr. Stewart's low-flow POTS patients continually have peripheral vasoconstriction.

"Local vascular responses affecting blood flow in postural tachycardia syndrome

Julian M. Stewart,1,2 Marvin S. Medow,1,2 and Leslie D. Montgomery

Department of 1Pediatrics and 2Physiology, New York Medical College, Valhalla, New York 10595; and LDM Associates, San Jose, California 95126

Submitted 6 May 2003 ; accepted in final form 7 August 2003

Postural tachycardia syndrome (POTS) is defined by orthostatic intolerance associated with abnormal upright tachycardia. Some patients have defective peripheral vasoconstriction and increased calf blood flow. Others have increased peripheral arterial resistance and decreased blood flow. In 14 POTS patients (13?19 yr) evenly subdivided among low-flow POTS (LFP) and high-flow POTS (HFP) we tested the hypothesis that myogenic, venoarteriolar, and reactive hyperemic responses are abnormal. We used venous occlusion plethysmography to measure calf venous pressure and blood flow in the supine position and when the calf was lowered by 40 cm to evoke myogenic and venoarteriolar responses and during venous hypertension by 40-mmHg occlusion to evoke the venoarteriolar response. We measured calf reactive hyperemia with plethysmography and cutaneous laser-Doppler flowmetry. Baseline blood flow in LFP was reduced compared with HFP and control subjects (0.8 ? 0.2 vs. 4.4 ? 0.5 and 2.7 ? 0.4 ml ? min?1 ? 100 ml?1) but increased during leg lowering (1.2 ? 0.5 ml ? min?1 ? 100 ml?1) while decreasing in the others. Baseline peripheral arterial resistance was increased in LFP and decreased in HFP compared with control subjects (39 ? 13 vs. 15 ? 3 and 22 ? 5 mmHg ? ml?1 ? 100 ml ? min) but decreased to 29 ? 13 mmHg ? ml?1 ? 100 ml ? min in LFP during venous hypertension. Resistance increased in the other groups. Maximum calf hyperemic flow and cutaneous flow were similar in all subjects. The duration of hyperemic blood flow was curtailed in LFP compared with either control or HFP subjects (plethysmographic time constant = 20 ? 2 vs. 29 ? 4 and 28 ? 4 s; cutaneous time constant = 60 ? 25 vs. 149 ? 53 s in controls). Local blood flow regulation in low-flow POTS is impaired.

When I took midodrine, my feet slowly turned blue, cold, and painful over the several days I took it. It makes sense because midodrine works to peripherally vasoconstrict. I wonder if Mayo is incorporating Dr. Stewart's research into their testing??? It just seems that I am classic low flow POTS. Is this the same type that Mayo has described as hyperadrenergic? I hope that Mayo can address my problems with peripheral vasoconstriction.

It seems so WIERD that I can have such problems with arterial constriction but still have the venous pooling, tachycardia, and other POTS symptoms from low upper-body perfusion. Janie

[jump]

Did you guys have tests done to determine Raynaud's or was it just a clinical diagnosis? I know there's a test for pernio...

My doctor diagnosed me with Raynauds, but she just looked at my cold, purple hands, said "Do your feet do this too?" and I said yes, and told me it was Raynaud's and that I should wear mittens (Ha! as if I would ever go anywhere without them...). I've heard of a test where they stick your hand in ice-water but I thought that was archaic. Am I mistaken? It sounds like you all had testing done.

I wonder because although my hands and feet are often unbelievably cold, and they do occasionally turn kind of purplish and my nails turn purple, it doesn't sound nearly as severe as what several of you have described. I wonder if I DON'T have Raynaud's, if maybe I just have cold hands and feet.

[janiedelite]

Did you guys have tests done to determine Raynaud's or was it just a clinical diagnosis? I know there's a test for pernio...

Hi Jump, I had a PPG (which just checks for blood clots). The vascular doc I saw said that it's just a standard thing he orders whenever Raynauds is suspected. He did say I could go to OHSU (the local research hospital) and have the cold water test done that you described, but instead he just diagnosed me based on my symptoms. He gave me a script for procardia which my EP cardio said not to take because it would make my pooling and tachycardia worse. This was last summer, and I had gone to several docs and showed them my purple feet. They weren't really cold at the time, but the nails turned purple like you described. My capillary refill was fine. I think they didn't have any other name for what was wrong with me so they called it Raynauds. You may indeed have Raynauds, but our purple feet seem to be a more constant gradual lack of blood flow. Raynauds patients get quick spasms of their blood vessels in cold or stress which result in acute, episodic purple, blue or white extremities. Is that right???

But in cold temps, my feet are usually very white with purple tips or have a bluish tinge and they are very cold for hours on end unless I keep on my wool slippers or keep them under a heating pad. The capillary refill is very slow and sometimes I can't feel pulses in my feet. In the winter I develop the chilblains and chronic pernio symptoms, although I did get pernio once from sitting in a cold doctor's office for an hour in the summer.

When my feet or hands get cold, my toes ache so badly! But if I am too warm (like in summertime or cooking over a hot stove), then my hands and feet (and sometimes my whole body) turn flaming red and burn horribly. In warm temps, they burn nonstop and the only thing that helps is a cool rag on my skin in front of a fan. It's like my blood vessels are over-reacting to any cold or heat. Sorry if this is too much info about my feet, but it's been one of my most visible and annoying symptoms

[4everOptimistic]

Thanks all for your replies!!! And for the pernio definition, Maxine!

I'm currently working on getting all my records, including films, x-rays, etc. Talk about a process! I hope I can get it all done by Friday. LOL Has anyone had to get slides of their biopsies?!?! Is this even possible if it's been over a year? Ugh.

Thankful, thanks for all the excellent info on peripheral vasoconstriction. I'll have to look into that all further. I never made a connection between my summer and winter symptoms! Very interesting! My feet were always swelling and turning red this past summer. I kept hiding them.

You know, the curious thing is that I do not suffer from Raynauds. My daughter (age 6) was diagnosed last year, though. I only have pernio symptoms. From what I understand (and I'm still trying to figure it out), the blood vessels constrict during cold conditions and then cause the blisters and redness upon 'thawing.' This is usually about a 12 hour process for me so it doesn't always show up right away. But I get really cold easily. For those of you wondering about the difference between pernio and Raynauds, I'm not entirely sure what the clinical difference may be. I can tell you based on the experience in our household. My daughter had a diagnosis based on symptoms. Her feet go from ghost white to pink to black/purple progressively. For me, pernio is painful because the blood escapes through the skin and presents itself as redness, blisters and even pustules. I can't even walk when it gets too severe. I wear mittens and boots as soon as the temperature drops below 60 degrees.

BTW, I had extensive testing done after the pernio diagnosis and they determined that my pernio was not caused by an underlying illness (ruled out Autoimmune, blood disease, cancer, Lupus, etc.) Now, I'm not so sure. I'm thinking it is related to some type of dysautonomia.

Pernio can be secondary to Lupus but usually presents itself in the form of a rash on the face and nose area.

[janiedelite]

I am picking up a biopsy slide Monday from my dermatologist. They usually destroy the slides after a year. Mine was done in March '08.

What you described happening to your feet is exactly what happens to mine, too. It's comforting to see someone else have this problem without any underlying illness, except dysautonomia.