jjb

Yes, orthostatic hypotension can occur with POTS.

I have both.

Chiari is somewhat common in EDS ... in fact I think the chiari institute now automatically screens all of their cases for EDS.

I have actaully been wondering if it is in fact chiari that is the reason for autonomic issues in folks w/ EDS.

Now I will be looking around for info on folks that had both chiari and aut issues and went on to do the decompression surgery.

I wonder how many had improvements.

I have chiari symptoms, but I have said I would never iot for the surgery. I have heard it is not always has helpful in EDS cases.

Who are you seeing for the chiari?

Who did you see for the EDS?

Hi,

I have to finish getting around for an appmt, but thought I would quickly post...

I think they are referring to HSAN 1 & 2 . For info. google hereditary sensory (and) autonomic neuropathy. I think they probably ruled the others out by that pain response is intact and that she does have the ability to thermoregulate (sweat).

Hope that helps!

Yes, I assume they are referring to HSAN and have googled some.

Looking for info from the expereinced.

Ava does have response to pain, but has no distal sweating and no facial sweating ... and has other autonomic quirks.

I realize this is a milder form of HSAN (thankfully) but am wondering if it is progressive.

My mother is only able to sweat along her hairline. I don't think I have adequate distal sweating but have tons of torso & neck sweating.

I have lots of sensory quriks and am gradually losing some sensation (or at least am getting numbing) at the finger tips.

First I apologize for starting two threads at once.

Ava and I had our testing this past April. We still have not had our follow up and it may be a ways away, but I briefly spoke w/ the aut neuro this morning as Ava has a five year check up tomorrow and I am certain pedi will be asking me about the test results.

Anyway, the skin biopsy was normal but the neuro says there it is likely hereditary autonomic neuropathy.

I guess I knew this, but he said it could be type one or type two.

I did not realize there were different types.

Anyone know what the difference is?

I am still trying to figure out my daughters episodes.

She had some scary episodes last week where her o2 dropped into the 80s several times and she becomes tahcycardic. Sometimes she just becomes tachycardic. Once bradycardic (scary)

Anyway, she does have a very mild sleep apnea in general, but her o2 only drops like this once a monht.

She cycles.

We still have not figured out if these are autonomic seizures ... or autonomic activatio without seizures.

I am wondering if anyone here has any ideas or info on hypoxia or transient apnea events due to autonomic activation.

Years ago I was DXed with ortho hypotension and would get the usual feelings of light headedness and presyncope and so forth.

So anytime I'd get these symptoms I had assumed it was my BP dropping.

Well, this past April I did a ttt. I got he usual light headed feelings along w/ nausea. BUT my BP was fine. It the tachy that was causing the symptoms.

Hello,

I am wondering if anyone can share thoughts or experiences... I often feel light-headed and like there is not enough blood going to my head, but my peripheral BP meaures in a normal range. Do others frequently experience this as well? Any ideas what causes it or how to help it? Thank you kindly.

~ Broken_Shell

Ava, my mom and myself are all sensitive to the sun.

Ava will get hives & fatigue. Some of the time the fatigue is from heat intolerance (she does not sweat well)

But sometimes I think it is directly related to sun exposure even when it is cool.

Her rashes only occur some of the time. Sometimes they are a red bumpy rash and sometimes welty hives.

She can get hives even if it is cool out.

I am less sensitive but occasionally get both the rash and fatigue. Think it depends on if there is something else going on in my system.

I get sun poison easily.

My mom hates the sun. She does not get rashy, but gets very fatigued and irritable.

I tend to think it is somehow related to the autonomic issues.

It does sound like it can be a vision issue. I think Motion sickness is.

Here is a link to vision therapy:

http://www.visiontherapystories.org/motion..._dizziness.html

I get this too.

I also get nausea in my head, neck and more recently my upper back in between my shoulders.

When I was at the NIH ... I was told it sounded like chiari malformation or a CSF block.

I often get a lot of morning nausea ... well anytime nausea really, but sometimes I go through periods where I have more morning nausea.

My cardiologist always though I have vagla nerve issues. Maybe so, but the geneticist thought I had chiari malformation.

Chiari is somewhat common in EDS. Both EDS and chiari can be a cause of dys.

Chiari can cause lots of nausea. Do any of you have EDS and/or chiari?

Here an interesting blog that talks about EDS, chiari & dys

http://chiariandsyringomyeliaincanada.blog.../12/nausea.html

"I ask because Dr. Cohen said that in his experience, those who have dysautonomia due to a mitochondrial disease can't be diagnosed with a muscle biopsy."

Really? We are getting ready to go down the mito path and I thought dysautonomia was pretty common with mito in general which is often DXed through the muscle biopsy (which I won't do right now).

" With dysautonomia due to mito it is the nerves that are the most effected, thus the skin biopsy (taken under the arm where there are more nerves because of sweat glands) is more likely to show mito than a muscle biopsy."

Two questions:

1. So does the skin biopsy test for mito issues specifically, or are they looking for something else and they assume a mito issue based on results.

2. Is under the arm the only place to test?

At our exam, the took the sample for biopsy on our leg. For me it was lower leg a few inches above ankle and on the thigh.

FOr Ava they just did lower leg. And this is where they placed the electrodes for sweat test too.

But I know for this biopsy they were looking for neuropathy ... can they tell if there is an abnormality of mitochondria with this same biopsy?

Thanks,

Jen

Ava will be seeing a mito neurologist this summer.

I probably will not go through extensive testing, but will probably give the mito cocktail a try since a very similar cocktail ise also used to treat EDS symptoms.

When I was at the NIH for EDS, the doc said she thought I had chiari malformation. Chiari is common in folks with EDS.

Chiari can cause swallowing problems.

Chiari is an extremely controversial diagnosis - particularly when it is suggested to be the cause of POTS or related symptoms.

Yes, it is contraversial and is one reason why I would ONLY go to the chiari institute in NY.

They have worked along side Nazli McDonnel Clare Francomano. The institute understands the chiari EDS relation.

We tried the cooling items that have the crystals, but I find the three-layer evaporative cooling fabric to work better and longer.

I am going to contact Silver Eagle to see if I can just purchase the fabric in bulk and make some items myself.

Hi there. I have this problem too.

When you have multiple health issues it is hard to know what is what.

When I was at the NIH for EDS, the doc said she thought I had chiari malformation. Chiari is common in folks with EDS.

Chiari can cause swallowing problems.

Vagus nerve issues can also cause swallowing problems.

I would imagine low tone in the neck and joint hypermobility itself can cause swallowing and uncomfortable sensations as well.

Hope you figure it out soon and get some relief.

Here is a link to Silver Eagle cooling stuff

http://www.coolingapparel.com/shop/vests.htm

My primary doctor even went so far as to tell me that I was going to end up living a life full of pain and end up having multiple surgeries.

This doctor is foolish! He does not know this. Yes, please be sure to see a doctor that knows about this.

In general, a person with EDS has got to be very careful when it comes to surgeries.

If you go to the National Ehlers Danlos site you can get a CD that is full of great medical information.

I have trouble with all of my joints. My shoulders are in a subluxed position all of the time. When I get an joint injury, and I have injured my should a few times, I find it is best to let it heal and keep it immobilized if possible. I find letting someone work on it ... will just irritate it even more.

If you don't have an injury, I think it is good if you can try and strengthen the area around the joints to help prevent subluxes and dislocations. You do though have to be careful though and know what exercises help and what can hurt.

Yahoo has a great EDS forum.

I see an osteopath that helps with both my joint pain issues as well as some of the autonomic stuff. She also does something called prolotherapy.

Prolo can be an effective treatment for EDS joint issues.

Now a

I have never seen them at stores but have purchased from Silver Eagle online and can say I recommend them.

My daughter does therapeutic horse back riding and I also bought a head cooler to put under her hat/helmet.

Works great!

Has anyone been able to purchase their cooling vest at a retail store, or are they all from online? I'd love to have Chrissy try one on b/f buying. I've tried calling sporting goods stores, medical supply places, etc. I've heard they're used by people with MS and many football players, mascots, characters at amusement parks--do they all purchase them online too? Just wondering.

Patti

My daughter has a very rough time in the heat. In fact she is sick right now and I am suspecting too much heat and excitment.

She has a cooling vest too. This year we alos bought the cooling cap. Have only used the cap once and so far it seems to have helped more that the vest.

I have to try them both out more though to know for sure.

Have you tried the cap?

Subluxes are simply partial dislocations.

When I can feel them, they feel similar to how you describe. A joint out of place and I have to move around to get them back into place. Sometimes I do not feel it coming out of place ... but do feel it going back in.

This has happened to most of my joints and often my ribs. My shoulders are in a subluxed position all of the time ... this I never knew until I was evaluated at the NIH.

I LOVE red a nice dry red wine ... unfortunately I think I have a true intolerance. After only a few sips I flush. I become intoxicated very easily. The next day, even if I only have one glass ... I get sick. Vomiting and headache.

I also get more ortho intolerance the next day and sometimes some o2 desatting.

I get tis a lot. Seems as though I have come across info on the cholinergic nerves and cognitive issues.

Have you been tested fr small fiber neuropathy?

Hi there. We (my daughter and myself) had our testing last month and the doc suspects sfn. The way he tested though was with a skin biopsy.

It takes a month or so for the results to be in.

I think if you suspect this, it is a good idea to be tested as it can be an indicator of other illness.

One is diabetes, some others are autoimmune.

Joy, were you formally tested for fructose malabsorption?

I think cane sugar has just a little bit more fructose in it than glucose.

What tests did you have for the gluten & casien sensitivity?

1. Do you believe -or has a doctor told you - that you experience blood pooling mainly in your legs?

My hands almost as much as my legs

2. Have you ever been diagnosed with EDS or suspect that you may have it?

Yes, DXed at the NIH

3. Do you experience symptoms when lying down even after a night of sleep in your bed?

YES!

4. Have you ever been diagnosed or suspect that you have what has been called the Hyperadrenergic form of POTS?

Yes.

5. Have you ever been informed by a doctor that you have low blood volume?

No

6. Do you experience stomach-related symptoms similar to what has been described as Irritable Bowel?

Yes, IBS, gastroparesis and frequent N&V

7. Do you believe you experience excessive vasoconstriction of your hands and feet after standing or when symptomatic - which MAY be demonstrated through a feeling of them being cold or without visible veins?

Yes

8. Did your POTS arrive suddenly?

No

9. Is your skin pale?

In general, we are on the pale side ... but flush easily

10. Please the top 6 worst symptoms that you experience with POTS:

I don't think of POTS the primary issue here, but dysfunction of the ANS in general.

My worse symptoms are the times I have complete white outs and cannot see and cannot hear due to orthostatic intolerance.