Monday, August 8, 2016

What Is Ehlers Danlos Syndrome?

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Unless you've been diagnosed with it or know someone who has, you probably haven't heard of Ehlers Danlos Syndrome.  Among those who have heard of it, many don't actually know what it is.

What is Ehlers Danlos Syndrome?

Ehlers Danlos Syndrome (pronounced ay-lers dan-lohse) is a collection of genetic connective tissue disorders.  It affects the body's collagen which can lead to symptoms in every part of the body.  Collagen is the glue that holds the body together and when it's faulty, the body can literally fall apart.  Collagen can be found in the skin, eyes, muscles, ligaments, blood vessels, organs, gums, etc. which means that all of those systems can have issues due to EDS.

There are thirteen types of Ehlers Danlos Syndrome.  Each type runs "true" in a family which means that if the parent has the Vascular EDS, their child will have the same type, not a different one.  There can be crossovers between the types as well which means that someone with one type of EDS may also have features that are associated with another type.  Ehlers Danlos is also sometimes referred to as joint hypermobility syndrome. 

All thirteen types of EDS, except for the Hypermobility type, are diagnosed by a genetic blood test.  As the gene for Hypermobility Type III has not been identified yet, it is usually diagnosed by a geneticist using clinical evaluation, the Beighton Score and the Brighton Criteria.

The Beighton Score tests hypermobility using a 9 point system.  You can see more information and an illustration of the Beighton Score here.  The Brighton Criteria involves two major criteria and 8 minor criteria and can be seen here.

EDS affects everyone differently.  Some people have very few symptoms and can live a relatively normal life, while others have symptoms and pain so severe that they end up needing feeding tubes, wheelchairs or other mobility devices, etc.  With the exception of the vascular type, which can cause sudden organ or vessel rupture, the life expectancy for someone with EDS is usually the same as it is for someone who doesn't have it.

What is Ehlers Danlos Syndrome? Find out more here:

EDS causes joint hypermobility which can result in loose and unstable joints.  This means that dislocations and subluxations (partial dislocations) can easily happen and joint pain is the norm.  Many people with EDS have soft, velvety skin that tears, bruises, and scars easily.  Wounds often take a long time to heal.  Gastrointestinal problems are common in EDS as well.  However, everyone with EDS is affected differently so while these are common features of the syndrome, not everyone has them.

People with EDS are likely to have additional disorders and diseases (also known as comorbid conditions) such as IBS, POTS, dysautonomia, Raynaud's Syndrome, Chiari Malformation, scoliosis, migraines, asthma, eczema, mast cell activiation disorders, gastroparesis and many more.

There is no cure for Ehlers Danlos Syndrome, but if treated correctly, the symptoms can sometimes be managed.  It's estimated that 1 in 2,500 to 5,000 people have EDS, but because it's widely undiagnosed or misdiagnosed, those numbers may be much higher.  A parent with EDS has a 50% chance of passing it on to each of their children.
The symbol for Ehlers Danlos Syndrome is a zebra.  Students in medical school are taught that when they hear hoofbeats, they should think of horses, not zebras.  In other words, look for the obvious answer when diagnosing a health issue because that's probably what it is.  But people with EDS and other rare diseases are zebras in the medical sense of the word.  Also, every zebra's stripes are different, just as EDS affects each person differently.


Raising awareness for Ehlers Danlos Syndrome is very important.  One of the reasons for this is that so few people have heard of it or know what it is that people who have EDS often feel misunderstood and disconnected from others.  Another reason is that EDS frequently goes undiagnosed or is misdiagnosed as something else.  It's estimated that 50% of people with fibromyalgia actually have Ehlers Danlos Syndrome.  Getting a diagnosis is important so that it can be treated correctly and for a patient's mental well-being.

The more people who receive a diagnosis of Ehlers Danlos, the more likely the syndrome is to get funding for research-another reason why awareness is so important.  Very little is known about EDS at this time.
Help raise awareness for Ehlers Danlos Syndrome with these silicone bracelets!

You can help raise awareness of Ehlers Danlos Syndrome with these silicone bracelets which are available for purchase right here on Sunshine and Spoons.

Ehlers Danlos Syndrome awareness bracelets

If you think you or a loved one may have Ehlers Danlos Syndrome, ask your doctor about it.  Diagnosis is important! 

If you know someone who has Ehlers Danlos Syndrome, please try to show them grace as they are living with a chronic, debilitating disease.

The Ehlers Danlos Society (website)
The Ehlers Danlos Society (Facebook page)

Facebook groups (you can also find many local groups on Facebook):
Ehlers Danlos Support Worldwide EDS/POTS/MCAD/Chiari
Raising Children With EDS-Ehlers Danlos Syndrome
Good North American Doctors for POTS/OI/EDS/ME/cfs/Chiari/MCAD/RA/GP/Hashis
Ehlers Danlos Syndrome Zebras


  1. Wow that is intense! How bad do you have it? Did all your kids get it too? I appreciate you spreading the word of it and educating us on it.

    1. I don't have it as bad as lot of people I know, but it is definitely getting worse as I get older (my geneticist said that most people with EDS start declining around age 30...I'm 31). I have had pain my whole life and am starting to have problems with mobility due to my hips and knees being so unstable. Unfortunately, I passed it on to 3 of my 4 kids :( My 2 boys seem to have it worse than me, my youngest to the point of being medically complex and needing a feeding tube until he was 18 months old.

  2. My diagnosing physician, UW genetic pathologist, predicted that the symptoms would improve with age. That was 15 years ago and at 62 the dislocations are less frequent but the pain is screaming. But that kind doctor also said, "doctors know nothing about EDS, really" 62 and wiser.

  3. My diagnosing physician, UW genetic pathologist, predicted that the symptoms would improve with age. That was 15 years ago and at 62 the dislocations are less frequent but the pain is screaming. But that kind doctor also said, "doctors know nothing about EDS, really" 62 and wiser.

    1. I've never heard anyone say that EDS would get better with age! My geneticist said it is a degenerative disease. It makes sense that there might be less dislocations though since your joints tend to stiffen naturally as you get older.

  4. I've never heard of this! My brother has POTS and Dysautonomia, and I have JRA, Myotonia, Myesthinia Gravis and a lot of other things.

    1. Have you seen my post on how to tell if you might have EDS? You and your brother might want to check it out!

    2. I have. I did check it out!

  5. We had multiple doctors visits, er,hospitalization before our 14yo son got an EDS/JHS AT UMMS this past January. We had to leave our pediatrician as he kept negating my concerns for 2 years! Our son now has a feeding tube. He did have a POTS diagnosis when he was 11. He was 8/9 on the beighton scale, has pectus excavatum, severe weight loss due to GERD and gastroparesis. Hence the need for a feeding tube. He is 5'7" and reduced to the four weeks he's had the tube He has gained back 4 lbs.we have had a wonderful cardiologist, surgeon,geneticist and physical therapist. Terrible gastroenterologist s!!! They inferred it was "all in his head", not so bad, he didn't really have EDS. IDIOT DOCTORS.made my 14yo son cry after every visit. They were condescending and mean. It has been so hard to watch him go through this.

    1. I'm so sorry that your son has gone through all of that. I truly believe that the worst part of having EDS is dealing with doctors who don't understand, believe, or want to help. My family is blessed to have a team of doctors who have been amazing with diagnosis and treatment, but it took many years to find them!


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