Ehlers Danlos

[helen.t]

Hi folks, I know that a lot of people here have EDS and that it can be associated with POTS. I have POTS but not EDS as far as I know but I do suffer a lot from joint pain at the top of my legs and pelvic floor muscles and also knees, back and ankles. Also my muscles are easily pulled out and I suffer from a lot of severe muscular pain and spasms at times. I was wondering if EDS affects muscles and ligaments? I am generally quite stiff and slow and not double jointed but just wanted to know if loose muscles/ligaments had anything to do with EDS. Thanks, Helen

[flop]

Hi,

EDS is an genetic condition that causes you to have faulty collagen. Usually the collagen is too stretchy. Collagen is the main structural protein in tendons and ligamants (having overly stretchy ligaments is what leads to having hypermobile joints). Collagen is also found in blood vessels (thought to be why EDS veins overdilate and allow blood pooling) and in muscles. There are different sub-types of EDS. The Ehlers-Danlos National Foundation has a good website - they have recently made it compulsory to register with them to be able to read the website but registering is free (not to be confused with their membership which is not free). There are other EDS sites also such as www.ehlers-danlos.org which is the UK support group.

Hope this helps,

Flop

[mkoven]

I have eds and didn't really consider myself flexible. The question is whether you feel stiff-- which is different froma ctually being stiff. I avoid using my full range of motion because it hurts-- but at the end ranges, my joints slip. and muscles spasm because they are working overtime to keep joints in place.

It took me a long time to get a diagnosis. You should see someone familiar with eds, as it can be subtle unless someone has seen a range of cases. not all my joints are pretzel-like, but some are. And my skin is on the stretchy side, but not extremely so. Right now, my ans doc thinks eds is the reason for my autonomic symptoms. www.ednf.org is a great resource.

[Maxine]

EDS affects the collagen/connective tissue. Depending on the type of EDS, it can affect joints, ligaments, anything that involves connective tissue.

Hypermobile form (type III) Joint features only

Classical mitis, type II: Joint features and mild skin features (the mildness is the skin features, not the joint features)

Classical gravis type I: Joint features and severe skin features

Vascular form, type IV: Joint, severe skin and vascular features.

Here's a link to the EDNF web site:

http://www.ednf.org/

Muscle spasms can happen with EDS, as this is the way your body tries to compensate for the loose joints and ligamants.

I'm still learning about EDS, as I didn't realize how much this can affect different parts of our body.

There is an EDS study going on right now at NIH, and Dr. Nazli McDonnell heads up this study. On April 1st, and 2nd. I participated in this study. My EDS diagnosis was changed from type III hyper mobile type, to type II classical. I still have joint hyper mobility, dislocations, and subluxations, but more of my tissue is affected, such as my skin, and now my intestines-----------and possibly more. I have poor vascular tone from stretchy vessels causing blood pooling. My POTs is secondary to the EDS. My cervical/cranial instability, other spine instabily, and scoliosis is also caused by my EDS---------this can also cause ANS dysfunction/POTS. I didn't like the news, but it does help explain a lot of my problems.

The best thing to do is see a geneticist who specializes is EDS/connective tissue disorders. They should be able to rule in or out a connective tissue disorder for you.

I hope you DON'T have EDS.

Take Care,

Maxine :0)

[helen.t]

Hi Thanks everybody for your reply's. Yes like you say, I don't really want another diagnosis but if it explains a lot of what is happening in my body then it might help me to deal with it. All your info has been helpful and I will look into it more as a lot of EDS seems make sense withsome of my symptoms. Can it get worse through time?

Thanks a million for all your support!! Nobody wants to be here really but it is good to know you're not struggling alone and having people who understand what it's like living with Dysautonomia has helped me keep my sanity!! Thanks again, Helen x

[flop]

Ehlers-Danlos Syndrome has, as Maxine posted, got several different types with different features. There is no simple list / set of rules to easily work out which type someone has (I am a member of an EDS board and several people there have had doctors change their diagnosis from one type to another).

I have the hypermobile type (previously called type III - the numbers system is no-longer used). The info that I have on the hypermobile type has a table of different signs and symptoms that the doctor gives you major or minor for and you need 2 major / 1 major + 2 minor / 4 minor criteria. The list includes scores for:

- Joint hypermobility

- Longstanding pain in multiple joints

- Several dislocations / subluxations of joints

- Muscular pains

- Marfanoid habitus (arm span > height, long hands and feet)

- Straie (stretch marks)

- Stretchy skin

- Thin "tissue-paper" scars

- Eye signs

- Varicose veins,

- Hernias

- Prolapses

I have quite a few skin signs - stretchy elastic skin, stretch marks, abnormal scars, easy bruising. My diagnosis was made by a Professor who specialises in EDS and related hypermobility problems.

Basically EDS should be diagnosed by a doctor with a special interest in EDS, not all rheumatologists are interested in EDS.

Flop

[Maxine]

Even if your diagnosed with a specific type of EDS, you can have crossover features of another type.

The EDS study at NIH may help in discovering the mechanisms behind this.

It's hard for me to take it in sometimes, as I'm about to be 49 in a couple weeks, and how in the world did I go so long without knowing I had this? It's genetic, so how did my problems stay mild so long? Then BOOM------> the next thing I know my body starts falling apart AFTER I'm diagnosed with POTS, then chiari related/spine instability, then EDS LAST... I suspected EDS as soon as I learned about it, but some of my doctors that I had at the time just didn't take me seriously--------------even though some of my symptoms were obvious.

With the EDS the probable cause to all the rest of my problems, I just don't understand how it wasn't diagnosed first?

Maxine :0)

[MightyMouse]

I've been told so far, that I'm closer to hypermobile type--as flop mentioned, the numeric system has now been replaced. So, type III is now referred to as HEDS. Types I and II are now considered "classical type", etc.

Of the items flop listed for HEDS, I have most:

Joint hypermobility (I had much more mobile joints everywhere as a child, but as I've aged, damage to the joints has limited the mobility in some)

Longstanding pain in multiple joints (knees, fingers, feet since childhood--back and hips since teens)

Several dislocations / subluxations of joints (mostly jaw, fingers, toes, sometimes knees, hips, wrists)

Muscular pains (everywhere)

Straie (stretch marks on chest, hips, calves, thighs-despite never being more than about 30 lbs overweight, and never pregnant)

Eye signs (I have thin corneas)

Varicose veins (since age 16)

Hernias (sliding hiatal hernia)

My mother has the major skin involvement with the cigarrette paper scars, as well as stretch marks like you've probably never seen. Her c-section scars are very wide and odd looking (but if you've seen medical photos of classical type scars, hers match). She, however, is probably some type of overlap between classical and hypermobile.

My sisters have no symptoms other than keloids which are unusual to have repeated problems with in fair skinned folks.

Nina

[flop]

Ah - I've just learned something (I didn't know that keloid scars were related to EDS). I have nasty red keloid scar formation, but with the interesting twist of being tissue paper thin arround the lumpy bit!! The scar is on my chest from having my reveal device inserted and then replaced. The first time I thought it was a wound infection that caused the ugly scar but the repeat incision through the old scar has gone lumpy at one end too. Sorry, I'm digressing from Nina's point that there are a wide range of problems with each of the different types of EDS.

Flop

[MightyMouse]

My sister's keloids were a result of moles being removed--and then the keloid removal produced... bigger keloids. My sister had multiple revisions to the one on her face b/c, well, it was on her face.

[Megan]

Dr. Grubb diagnosed me with hypermobility syndrome. Is this the same thing or something different? I was under the impression that my flimsy veins and joints that make other people wince because it looks painful was just a POTS thing.

Meg

[Radha]

even if you are diagnosed with this, are there any medicines that actually improve the condition?

Radha

[mkoven]

I think no. It's a problem with the genes that produce collagen, and there isn't yet a way to make your body produce healthy collagen.

I think the current wisdom is to try to strengthen muscles to support weak joints and use proper body mechanics. I think doing enough exercise to challenge the tissues without so much that causes them to fail applies to us as to healthy people too. People with normal collagen also need enough regular input to keep bones, ligaments, and cartilage healthy-- so do we. It's just that our threshold of enough but not too much can be pretty low. So easy does it on increases in activity. Whenever I have a new pain that feels wrong, I back off till it settles down and I can figure out what's going on. I've personally found I can be a lot more active if I wear orthopedic braces. Myofascial release also helps muscle pain and joint problems-- since stretching on us can do more damage than good. But we still need to work out the kinks in our muscles-- just without overextending our joints. Having overly knotted up muscles can make it hard for muscles to fire normally.

But that's just the musculoskeletal end. As the others have said, collagen is everywhere in the body.