May is Ehlers Danlos Syndrome Awareness Month, and I want to do my part in talking about it. This article will tell you what it is and how to get treatment, and I’ll follow with an article about how it’s affected me personally.
What is Ehlers Danlos Syndrome?
Ehlers-Danlos Syndrome, often called EDS, is a group of inherited disorders that affect the body’s connective tissues. Connective tissue acts like the body’s scaffolding and support wiring, holding together the skin, joints, blood vessels, organs, and muscles. In people with EDS, this support system is weaker than it should be because of problems with collagen, one of the body’s most important structural proteins. But it isn’t just problems with joints. There are 13 subtypes of EDS and some of them can actually be life-threatening.
People with EDS often experience unusually flexible joints, frequent sprains or dislocations, chronic pain, fatigue, and soft or fragile skin. Some people bruise easily or heal slowly after injuries. Others may struggle with digestive issues, dizziness, heart problems, or severe exhaustion that can affect everyday life.
Because EDS affects connective tissue throughout the entire body, symptoms can appear almost anywhere. For many patients, it can feel like living in a body built from overstretched rubber bands and fraying threads. (Great analogy!)
A simple activity such as walking through a grocery store, climbing stairs, or opening a jar may cause pain or injury. I cannot tell you how many silly ways I have severely injured myself, but I think the prize winner is dislocating my shoulder and injuring my neck while doing a jigsaw puzzle.
EDS is genetic. Diagnosis can be difficult because many symptoms overlap with other conditions, and some patients spend years searching for answers. It was definitely the case for me, and when I finally received a diagnosis, it shed light on things that had happened my whole life that I’d never been able to explain before. I thought I was just “weird” or “extra clumsy.) I’m perhas rather weird, but the clumsiness – holy cow, so embarrassing.
Subscribe to our newsletter
Trying to figure out how to stock up while prices keep climbing? We can help with our free guide and newsletter!
The 13 Subtypes of Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome is not a single condition but a family of related disorders. In 2017, an international classification system officially recognized 13 distinct subtypes of EDS. Each subtype has its own pattern of symptoms, genetic cause, and level of severity, although many overlap like tangled threads in the same fraying tapestry.
1. Hypermobile EDS (hEDS)
The most common subtype, hEDS is marked by joint hypermobility, chronic pain, fatigue, frequent injuries, and joint instability. Unlike most other forms of EDS, no single genetic marker has yet been identified for hEDS. This is the type that I have.
2. Classical EDS (cEDS)
People with classical EDS often have very stretchy, fragile skin and widened scars that resemble thin cigarette paper. Joint hypermobility is also common.
3. Classical-like EDS (clEDS)
This subtype resembles classical EDS but lacks the severe scarring typically seen in cEDS. Skin fragility and loose joints are common features. (A friend of mine with this type of EDS gets big keloid scars whenever they take blood at the hospital.)
4. Vascular EDS (vEDS)
One of the most life-threatening forms, vascular EDS affects blood vessels and internal organs. Arteries, intestines, or the uterus may rupture unexpectedly, making careful medical monitoring essential.
5. Cardiac-Valvular EDS (cvEDS)
This rare subtype primarily affects the heart valves, especially the aortic and mitral valves, leading to progressive heart problems over time. This type can also be life-threatening.
6. Arthrochalasia EDS (aEDS)
Characterized by extreme joint looseness and congenital hip dislocations, this subtype often causes severe mobility challenges from birth.
7. Dermatosparaxis EDS (dEDS)
This very rare form causes extremely fragile, sagging skin that bruises and tears easily.
8. Kyphoscoliotic EDS (kEDS)
People with kEDS often develop severe scoliosis, muscle weakness, and fragile tissues beginning in infancy or childhood. (I do have scoliosis but I don’t have the genetic markers for this kind of EDS.
9. Brittle Cornea Syndrome (BCS)
This subtype mainly affects the eyes. The cornea becomes dangerously thin and fragile, increasing the risk of rupture and vision loss.
10. Spondylodysplastic EDS (spEDS)
This form can cause short stature, muscle weakness, skeletal abnormalities, and joint hypermobility.
11. Musculocontractural EDS (mcEDS)
People with mcEDS may be born with characteristic facial features, skin fragility, and congenital contractures that limit joint movement.
12. Myopathic EDS (mEDS)
This subtype combines muscle weakness with joint hypermobility and contractures. Symptoms may range from mild to severe.
13. Periodontal EDS (pEDS)
Periodontal EDS strongly affects the gums and teeth, often causing severe gum disease and tooth loss at an unusually young age.
The thing that ties this together into one group
Although the subtypes differ, they all stem from the same core issue: problems involving connective tissue and collagen. For many patients, obtaining an accurate diagnosis is like finally turning on a lighthouse after years spent drifting through medical fog. This was certainly the case for me.
Common Comorbidities
Ehlers Danlos Syndrome is not a standalone issue with bendy joints and frequent dislocations. Connective tissues are found throughout the body, which means there are many comorbidities. It can create a domino effect that impacts multiple organ systems at once.
One of the most common comorbidities is Postural Orthostatic Tachycardia Syndrome, often called POTS. This condition affects the autonomic nervous system, which controls automatic body functions such as heart rate and blood pressure. People with POTS may experience dizziness, fainting, rapid heartbeat, brain fog, and exhaustion, especially when standing.
Another frequently associated condition is Mast Cell Activation Syndrome, or MCAS. In MCAS, mast cells release excessive chemicals that can trigger allergic-type reactions throughout the body. Symptoms may include hives, flushing, itching, digestive distress, breathing difficulties, and reactions to foods, medications, or environmental triggers.
Digestive problems are also extremely common in EDS patients. Conditions such as gastroparesis, acid reflux, irritable bowel syndrome, chronic constipation, and nausea can significantly affect nutrition and quality of life. For some people, eating becomes less of a pleasure and more like negotiating with an unpredictable machine built from tangled wires and loose screws. (The gastroparesis had been affecting me for years, but I just brushed off the symptoms thinking, “I ate too much” or that I had a delicate digestive system and some new food was not agreeing.
Chronic pain disorders often overlap with EDS as well. Fibromyalgia, migraines, temporomandibular joint dysfunction (TMJ), and nerve pain are frequently reported. Repeated joint injuries and instability can also contribute to arthritis at an earlier age than expected.
Many people with EDS experience fatigue severe enough to interfere with work, school, and daily activities. Sleep disorders, including insomnia and non-restorative sleep, may worsen exhaustion.
Mental health conditions are also common, particularly anxiety and depression. Living with chronic pain, medical uncertainty, and physical limitations can place a heavy emotional burden on patients.
Other possible comorbidities include scoliosis, pelvic floor dysfunction, bladder issues, tethered cord syndrome, Chiari malformation, and craniocervical instability. Not every person with EDS will develop these conditions, but the overlap is significant enough that many specialists now view EDS less like a single diagnosis and more like an interconnected constellation of systemic disorders. I have craniocervical insufficiency, but it’s well managed. When it acts up, I truly cannot get out of bed because my head feels too heavy for my neck, like a bobblehead doll.
I also have degenerative arthritis, which links to my main diagnosis.
One of my daughters has many symptoms linked to Ehlers Danlos Syndrome, and a high likelihood of having it due to it being a genetic disorder.
The outlook
The outlook is less about “life expectancy” and more about quality of life, symptom burden, and preserving independence over time.
Unlike other types of EDS, hypermobile EDS is not generally considered life-shortening on its own. However, the cumulative wear-and-tear of decades spent in unstable joints and poorly supported connective tissue can become substantial by the late 50s and beyond. Many people describe it as reaching the point where the body’s compensation systems finally run out of spare parts. The joints that once bent too easily may become stiff, arthritic, painful, and mechanically unreliable.
Severe gastroparesis can become one of the most limiting aspects of the condition. Malnutrition, dehydration, medication absorption problems, weight instability, and exhaustion can dramatically affect daily functioning. Maintaining nutrition often becomes a central medical priority. Some patients improve significantly with careful dietary management, medications, feeding tubes, or surgical interventions, while others continue to cycle through flares and periods of instability. I’m hoping to have surgery approved by my insurance company soon.
Scoliosis and degenerative arthritis together can create a compounding effect. Scoliosis changes body mechanics, which increases strain on unstable joints and muscles already working overtime to compensate for lax connective tissue. (For example, during my first pregnancy with my eldest, the OG noticed that my uterus was pushed quite far over to the side. It wasn’t harmful and actually looked funny as my belly got bigger because I looked so lopsided.
Over time, EDS may lead to chronic pain, reduced mobility, nerve compression, and increasing reliance on mobility aids such as braces, canes, rollators – This is the one I have and yes, it’s worth it. I am making payments on it., wheelchairs, or adjustable furniture. For many patients, energy becomes a precious currency spent carefully throughout the day. A product called Visible is supposed to help with pacing. (I haven’t tried it but lots of folks like it.) I will provide more recommendations tomorrow.
That said, outlook is highly individual. Some people with severe hEDS remain active and independent well into older age, especially when they have good symptom management, supportive physicians, physical therapy tailored to hypermobility, adequate pain control, mobility accommodations, and strong social support. Others experience progressive disability.
In conclusion…
The biggest predictors of a better long-term outcome are often practical rather than dramatic: maintaining nutrition and hydration, protecting joints from repeated injury, pacing activity to avoid crashes, treating pain aggressively but safely, preserving muscle strength where possible, and adapting the environment to reduce physical strain.
If you get a doctor who will go along with diagnosing you, the first test you will receive is an in-office Beighton Scale.
This is not to say that everyone with hEDS will end up becoming disabled. Everyone’s situation is different.
If these symptoms sound familiar, consider talking to your Primary Care Physician. He or she can order the genetic tests needed to figure out which type, if any, you or your child may have. I believe my daughter’s outlook will improve because her job keeps her active and she knows what she’s dealing with. I can’t tell you how many times people told me to “just push through it” or “it’s always something with you.”
Your doctor may want to send you to a rheumatologist for testing. You might also be referred to physical therapy, which is great as long as the therapist is EDS literate. The problem is, PT cannot officially diagnose you.
This disease is real and has affected my life dramatically. I’ll write about that tomorrow so I can provide a first-person account. That makes it real. I think it’s extremely important to get a diagnosis, and I hope this information helps you to understand it a little bit better.
About Daisy
One Response
Thank you for this article! Although I do not have EDS, the article led me to another article regarding MCAS. MCAS hit the nail on the head. I have been fighting with all the symptoms of MCAS for several months, but no doctor (not even my allergist) put it all together. So I will be making an appointment to talk to my doctor about some testing.