Please Tape Me Back Together: What is EDS?

Ehlers-Danlos Syndrome, or EDS, is a group of inherited disorders that cause the body to make faulty collagen. Depending on the type of EDS, different organs are affected. For instance, in the hypermobility type, the most common type, pain and joint dislocations are among the top complaints. Classical type of EDS has more skin and organ involvement as well as pain and joint hypermobility. The Vascular type of EDS has a lot of organ involvement, pain, and hypermobility may be limited to small joints or it may appear in large joints as well. I will go into each of the most common types of EDS in their own separate pages. This page is an overview of EDS.

A few questions I've heard asked are:

Q: Does it hurt to stretch like that?
A: It may not when an EDSer is young, but every time we hyperextend our joints (extend our joints beyond the normal movement) we do damage. The older we get the more it starts hurting.

Q: Can you show me?
A: I have pictures up on the H-EDS page of myself. I shouldn't be stretching like that. I got a 7/9 on the Beighton Scale. The Beighton Scale is described at the end of this page. I've made plenty of YouTube videos about EDS, but they are educational videos or vlogs, so don't expect an Elasti-Girl video from me. :-)

Q: Do people with chronic pain just have a low tolerance for pain?
A: I can't answer for everyone, but my tolerance for pain is very high. I can walk around on a dislocated toe and not realize it sometimes. I've burnt myself, I've ripped my skin, and I've messed up joints without knowing it because my pain levels are so high. Most EDSers have a very high tolerance for pain because we are in pain all the time. I personally think that if a normal person were suddenly put into the shoes of an EDSer in extreme pain their brain would literally explode. (Okay, so that last sentence wasn't scientific. There might not be any exploding brains.)

Q: Is EDS real?
A: Yes! They have genetic tests available for most types of EDS.

Q: Is EDS just being stretchy and dislocating joints?
A: EDS is way more than that. EDS can affect many systems in your body, regardless of your type. Also, a lot of people are cross-typed, meaning that they genetically have more than one type of EDS.

Q: Can you catch EDS?
A: No, it is only passed down genetically. It can also start with someone (as it did me) whose genes messed up all on their own when they were growing from embryo to baby.

Q: What does it feel like to subluxate or dislocate a joint?
A: When it's a joint you just started dislocating it's painful. Weird. Scary. It gives me a feeling of helplessness and a sinking feeling in my tummy, but each person is different. Once I get used to dislocating that joint it is painful and annoying. If I can't get it back in, then I go back to the weird, scary, and sinking feeling in my tummy. I also tend to panic if it's been awhile and I can't get a joint back in.

Q: Is EDS very common?
A: EDS is listed as a rare disease by NORD (National Organization for Rare Disorders). According to the Genetics Home Reference Guide to Ehlers-Danlos, EDS affects approximately 1 in 5,000 people worldwide.

Q: What do EDSers call themselves zebras?
A: In medical school, students are taught "When you hear hoof beats think, horses, not zebras." Since EDS is a rare disease, we need doctors to think of zebras when they hear hoof beats.

Q: I've seen references online to being a "spoonie." What is a spoonie?
A: My own words cannot do the Spoon Theory justice. To sum it up in a few sentences, a spoonie is someone with a chronic invisible illness. This means an illness you can't see by looking at the person, like Chiari, EDS, fibromyalgia, ME/CFS, lupus, RA, arthritis, IBS, mental illness, chronic back pain, diabetes, etc. Your energy for the day is represented by spoons. If you say you have one spoon left for the day, you are saying that you have only a little energy left. Two spoons would mean you had more energy. For instance, on some days it may cost cost you one spoon to shower, while other days it takes three. I highly recommend going to the Spoon Theory link to understand what a spoonie is better.

This information is from Mayo Clinic's article on Ehlers-Danlos Syndrome.

Hypermobility type (formerly type III) EDS This type affects approximately 1 in 10,000 to 15,000 people. Signs and symptoms include:

Loose, unstable joints with many dislocations
Easy bruising
Muscle fatigue and pain
Chronic degenerative joint disease
Advanced premature osteoarthritis with chronic pain
Heart valve problems (mitral valve prolapse and aortic root dilation)

Classical type (formerly types I and II) EDS This type affects about 1 in 10,000 to 20,000 people. Signs and symptoms include:

Loose joints
Highly elastic, velvety skin
Fragile skin that bruises or tears easily
Redundant skin folds, such as on the eyelids
Slow and poor wound healing leading to wide scarring
Noncancerous fibrous growths on pressure areas, such as elbows and knees; fatty growths on the shins and forearms
Muscle fatigue and pain
Heart valve problems (mitral valve prolapse and aortic root dilation)

Vascular type (formerly type IV) EDS This type of EDS is rare, but it's one of the most serious. It affects an estimated 1 in 100,000 to 200,000 people. Signs and symptoms include:

Fragile blood vessels and organs that are prone to tearing (rupture)
Thin, translucent skin that bruises easily
Characteristic facial appearance, including protruding eyes, thin nose and lips, sunken cheeks and small chin
Collapsed lung (pneumothorax)
Heart valve problems (mitral valve prolapse and others)

Other types of EDSThe other types of EDS are extremely rare. Some have only been described in a few families. In addition, the relationship of some of these types to the syndrome as a whole isn't well defined.

Beighton Score (useful for diagnosing all types of EDS)