Eds What Are The Symptoms?

[brethor9]

Hi all

Just curious since I read it in so many posts....what exactly is EDS and what are the symptoms most people suffer?

Thanks

Bren

[sugartwin]

EDS is a catchall name for six different genetic connective tissue disorders that share hyperflexible joints as their main manifestation. In its most common forms (the forms that tend to cause autonomic problems) it affects joints, causing subluxations or sometimes outright dislocations. There are often skin manifestations, with the skin tending to be stretchy, soft, prone to easy bruising and easily damaged while being slow to heal. Autonomic disruptions are common, affecting between 33-77% of sufferers of Classical and Hypermobility types (the common forms I mentioned earlier.)

I'd say the main symptom is PAIN. I think one study found 97% of people with Hypermobility type (the most common type) suffer from some pain. I'm sitting here in pain right now, typing this. There are also a lot of distinctive little things like easy bruising, premature arthritis, flat feet, recurring tendonitis/bursitis, etc. And of course many people with EDS has POTS or some other form of autonomic dysfunction.

I'm sure other people will chime in since I'm sure I've forgotten some things.

[rubytuesday]

It not only affects joints. Connective tissue holds all things within the body together and even affects the veins. In some, not only veins but the vascular system (in those the life span is less than my age I was told). It also affects the GI, manifesting in IBS, delayed gastric emptying, gastritis,GERD, hernias, adenomas, subluxations or dislocations of joints, tendons and ligaments. Some exhibit widen scars and surgical wounds that dehicse (sp)--split open. Wounds are often slow to heal. There is a common thread of immune compromisation in many--especially sinus. On one continuum of connective tissue disorder is Marfans with dilated aortas (congenital) and such. Skin is often very stretchy and has velvety feel/transluscent in places where veins are very visible. In hypermobility, the joints that were once able to move beyond normal limits (like 90 degrees), as one ages they become very stiff and less mobile, exhibiting more pain and complications. Another not so uncommon feature found in EDSers is Chiari I malformation. The glue that is holding everything in the body (even the eyes) is very lax and the body fights to keep itself held together (to put in simple terms).

It's a genetic defect in the connective tissue. that provides structural support to most of the body including muscles, joints and ligaments. Collagen is the proteini that acts as the glue giving the connective tissue strength and elasticity. The fragile skin and faulty joints found in EDS results from faulty collagen.EDS is a varied group of heritable connective tissue disorders that are characterized by articular hypermobility (joints that move or bend more than normal), skin softness and stretchiness, and tissue fragility.There are at least six major types of EDS which are classified by their manifestation of distinct signs and symptoms. Each EDS type is thought to involve a unique defect in connective tissue, although not all of the genes responsible for causing EDS have been identified.EDS affects men and women of all racial and ethnic backgrounds. To learn more about EDS, please visit our Website at www.ednf.org.

The later was taken from support group forum to which I belong.

[abnel]

My POTS specialist believes all POTS patients have joint hypermobility disorder.

I think he is outright wrong and have expressed reservations to him openly, but he is very dogmatic about it.

Because of his theory, he believes I must also be hypermobile even thought I cannot perform any of the tests he wanted me to succeed in (touching tongue to nose, flexing thumbs back to touch forearms, placing palms flat on floor without bending knees, etc).

Which leads to a rather obvious but stupid question.... is it possible to have JHD or EDS without being hypermobile in our joints?? Say, with vascular EDS??

For example, could we simply have hypermobile connective tissue in our organs and blood vessels but this NOT be present in our joints (elbows, knees, etc)???

[rubytuesday]

My POTS specialist believes all POTS patients have joint hypermobility disorder.

I think he is outright wrong and have expressed reservations to him openly, but he is very dogmatic about it.

Because of his theory, he believes I must also be hypermobile even thought I cannot perform any of the tests he wanted me to succeed in (touching tongue to nose, flexing thumbs back to touch forearms, placing palms flat on floor without bending knees, etc).

Which leads to a rather obvious but stupid question.... is it possible to have JHD or EDS without being hypermobile in our joints?? Say, with vascular EDS??

For example, could we simply have hypermobile connective tissue in our organs and blood vessels but this NOT be present in our joints (elbows, knees, etc)???

Anyone you ask with EDS could have very different manifestations as there are 6 categories but a continuum of connective tissue disorders, some of which include Marfans (like manifestation of congenital dilated aorta) or autoimmune. As a lot of us age, our once very hypermobile joints become racked with pain and very restricted in mobility. Not everyone with EDS is hypermobile. However hypermobility EDS III can also be exhibited in EDS I (classic). But not all EDS I (classics) are hypermobile. There are currently no genetic tests explicit for EDS I and EDS III. EDS IV affects the vasculature structures much moreso than the other EDS classes altho EDS affects veinous/arterial structures. My cardiac specialist in dysautonomia told me when I questioned if I could have EDS IV and III (and I) because I am a hemorrhager, he said that I could not have the EDS IV or would have been dead before mid-50s. My hemorrhaging it was found stems from Delta Granule Storage Pool Deficiency. The hemeoncologist I was referred to for that had lot of questions about my parents/immune deficiencies before he referred me on to 2 more of his collegues. So I am wondering if at least with the immune component is genetic as well (many EDSers have problems with immunodeficiencies but that is not in and of itself exclusive to EDS.

One can be hypermobile without having EDS (i.e. an acrobat or a dancer or a gymnist--someone who conditions and exercises to stretch those musles to control those musles, to get the long lines and the funkey or beautiful moves)

[abnel]

Thanks so much Rubytuesday. You mentioned that EDS 1 (the classical type) is the type where people often aren't hypermobile.

I've never been hypermobile in my life and to date I haven't suffered from aches and pains. The only pain I ever experience in my joints, back or neck is when I sprain them by accident - and this happens very rarely. I wonder if EDS1 would still be a possibility for me?

Suppose I should try and see a Rheumatologist or Geneticist to get it ruled in or out given my current POTS specialist is **** bent on diagnosing JHD with all of his POTS/CFS and dysautonomia patients!

[sugartwin]

Thanks so much Rubytuesday. You mentioned that EDS 1 (the classical type) is the type where people often aren't hypermobile.

That's not true. Joint hypermobility is a necessary feature for the diagnosis of classical type, whether type 1 or 2. It's true that as a hypermobile person ages they stiffen and often develop arthritis thus losing their flexibility but Classical with no history of hypermobility can't be diagnosed; it's one of the major diagnostic criteria.

There are types with much less dramatic joint hypermobility like the Vascular type, where it can be limited to the small joints (like the hands). This type is rarer and comes with more severe manifestations as rubytuesday was saying. Life span is often limited because of the aneurysms that result from the weakened type III collagen. Dysautonomia is not as common in this type.

[rubytuesday]

Thanks so much Rubytuesday. You mentioned that EDS 1 (the classical type) is the type where people often aren't hypermobile.

That's not true. Joint hypermobility is a necessary feature for the diagnosis of classical type, whether type 1 or 2. It's true that as a hypermobile person ages they stiffen and often develop arthritis thus losing their flexibility but Classical with no history of hypermobility can't be diagnosed; it's one of the major diagnostic criteria.

There are types with much less dramatic joint hypermobility like the Vascular type, where it can be limited to the small joints (like the hands). This type is rarer and comes with more severe manifestations as rubytuesday was saying. Life span is often limited because of the aneurysms that result from the weakened type III collagen. Dysautonomia is not as common in this type.

Yes, you are correct sugartwin.

I stand corrected. Thank you.

[kmichaelson]

RubyTuesday, I see that you mentioned sinus issues as a common thread. I've had severe sinus problems for years and years, have had two sinus surgeries to no avail, and have not been able to treat my sinus problems in any way, so that's really interesting to hear.

I also have patellar tracking disorder (kneecap slides around too much), tendonitis in my elbow, and stiff painful hands (maybe early arthritis since I'm only 34). Could anyone tell me if I wanted to see if I do have EDS based on this, what kind of doctor would I see? Do others who have this condition think it's worth checking out based on what I've said, or would my symptoms be more obvious if I had this?

[rubytuesday]

RubyTuesday, I see that you mentioned sinus issues as a common thread. I've had severe sinus problems for years and years, have had two sinus surgeries to no avail, and have not been able to treat my sinus problems in any way, so that's really interesting to hear.

I also have patellar tracking disorder (kneecap slides around too much), tendonitis in my elbow, and stiff painful hands (maybe early arthritis since I'm only 34). Could anyone tell me if I wanted to see if I do have EDS based on this, what kind of doctor would I see? Do others who have this condition think it's worth checking out based on what I've said, or would my symptoms be more obvious if I had this?

My first EDS III diagnosis was made by a rheume who specialized in fibromyalgia that my orthopede referred me too. Cardiac specialist in dysautonomia concurred with the EDS III. Altho there is no genetic testing for Classic EDS I or EDS III, genetecists who are well versed in EDS are considered to provede the 'Good Housekeeping Seal of Approval' expert opinion/diagnosis. Depending on you health insurance plan, you may be able to contact genetecist office and ask for appointment (without a referral from your PCP).