May is Ehlers-Danlos Syndrome (EDS) Awareness Month

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.

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.  Like I've said before, 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 wouldn't 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 or all 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:  Painful.  Weird.  Scary.  It gives me a feeling of helplessness, but each person is different.  It is even weirder to put them back in.  I usually have DH help me get my joints 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.


                                                    

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

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)

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)

Vascular type (formerly type IV) EDSThis 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)

Score 1 point if you can bend and place your hands flat on the floor without bending your knees

Score 1 point for each elbow that will bend backwards.

Score 1 point for each hand when you can bend the little finger back beyond 90 degrees

Score 1 point for each knee that will bend backwards

pictures from Hypermobility Syndrome Association - UK