Video Update- 1yr2mo post in Life with Ehlers Danlos

  • April 28, 2021, 5:39 p.m.
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The Ehlers-Danlos syndromes are a group of connective tissue disorders that are genetic and are varied both in how they affect the body. They are generally characterized by joint/ ligament hypermobility ( stretching further than normal), skin hyperextensibility (skin that can be stretched further than normal), tissue fragility, and lastly issues affecting blood vessels. Hypermobility does not always equal flexibility, since the muscular system tends to overcompensate for inappropriate tissue and subsequent organ movement. Symptoms and co morbidities and severity of such varies so much between the types and even between people that have the same types. Those who do not meet the 2017 criteria for hypermobile EDS (hEDS) and are symptomatic fall under the category of Hypermobility spectrum disorder (HSD) which can be just as debilitating and alter the persons quality of life just as much as an EDS diagnosis.

The Ehlers-Danlos syndromes (EDS) are currently classified into thirteen subtypes. Each EDS subtype has a set of clinical criteria and known genes that are responsible for causing symptoms. All but hypermobile EDS (hEDS)—have a genetic test for diagnosis. Research is still needed to determine which genes are affected in hEDS and HSD. Diagnosis is made based on physical criteria as well as looking at a persons whole body symptoms which can sometimes be very insideous and do not always present with obvious joint dislocations.

12 of the 13 types of EDS are considered rare diseases. hEDS and HSD is believed to be present as commonly as 1 in 2,000 people but those numbers could change based on research and awareness. The average age of diagnosis is somewhere in a person’s 40’s, if a person gets diagnosed at all.

What is Maythurners Syndrome?

May-Thurner syndrome (MTS) — also called iliocaval compression syndrome, Cockett syndrome or iliac vein compression syndrome — occurs secondary to compression of the left iliac vein by the overriding right iliac artery. Most MTS patients present with left leg swelling, and are higher risk for DVT (deep vein thrombosis) /blood clots. However many people with left sided pelvic pain and absence of leg swelling may have symptoms of pain and discomfort if their vein compression is greater than 50%.

What is pudendal Neuralgia?

PN is pain, discomfort or numbness in the distribution of the pudendal nerve. The pudendal nerve is a peripheral nerve that is the main nerve of the pelvis/ pelvic floor. The pudendal nerve emerges from the S2, S3, and S4 roots’ ventral rami of the sacral plexus. It carries sensory, motor, and autonomic fibers, however an injury to the pudendal nerve causes sensory deficits more than motor. These areas include the rectum, anus, urethra, perineum, and genital area. In women this includes the clitoris, mons pubis, vulva, lower 1/3 of the vagina, and labia. In men this includes the penis and scrotum. But often pain is referred to nearby areas in the pelvis. The symptoms can start suddenly or develop slowly over time. Typically pain gets worse as the day progresses and is worse with sitting. The pain can be on one or both sides depending on which nerve fibers and which nerve branches are affected. The skin in these areas may be hypersensitive to touch or pressure (hyperesthesia or allodynia). This is a chronic/ lifelong issue that can severely affect a persons quality of life and there are no simple treatment options.

What is pudendal nerve entrapment syndrome?

Pudendal nerve entrapment or PNE… is pain caused by a mechanical compression/ entanglement of the pudendal nerve between ligaments or scar tissue. Usually the sacrotuberous ligament and or the sacrospinous ligament. The only way to really definitively tell if you have a nerve entrapment is via surgery although some other specified imaging techniques which are able to image/ doppler where there is a hold up/ issue with nerve within scar tissue. This phenomenon is still debated and under studied. Pudendal decompression surgery is how this issue is diagnosed/seen. There are a few doctors who perform the surgery in the USA, but in Europe there are more options in terms of surgical techniques and experience. Please visit for more information.

The pudendal nerve entrapment syndromes subdivide into four types based on the level of compression.
Type I - Entrapment below the piriformis muscle as the pudendal nerve exits greater sciatic notch.
Type II - Entrapment between sacrospinous and sacrotuberous ligaments - this is the most common cause of nerve entrapment.
Type III - Entrapment in the Alcock canal.
Type IV - Entrapment of terminal branches.

Last updated April 29, 2021

music & dogs & wine April 28, 2021

You sound like a doctor!! Wow! So many things to learn about!

My friend has the "robot back" as I call it! She had it put in I think 3 years ago? It has helped her a TON. Majority of her issues are in the L4/L5 region. We always say she needs a full body transplant, cause she has lots of other issues too. I think you should get a full body transplant too :) Wouldn't that be nice!

AnOrangeZebra music & dogs & wine ⋅ April 28, 2021

Yep. Nerves and blood vessels are my main issue. If they could figure out how to reprogram Cells with the correct DNA I in theory could have my collagen fixed. Taking suppliments for my body is like tryiing to give it all the parts to a car without an instruction book I wont have anything to drive.

I appreciate the compliment also. Many people have asked me if I have a medical background. This illness forces you to do a lot of research and talk to people on your own. And years in PT has helped so much. I have learned how to put vocabulary to my pain which is invalueable. When I was a child trying to explain this shit was super stressing and now I can which I am so so thankful for.

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