First Impressions: EDS Defined

First Impressions: EDS Defined

ImageWhen meeting someone new, be it a potential employer, significant other, or your new doctor, the impression you make will directly influence your relationship moving forward.  So today’s blog is an effort to provide you with a first impression of Ehlers-Danlos Syndrome, defined by my own experiences with the condition.

Ehlers-Danlos Syndrome is a connective tissue disorder, and specifically it is a genetic defect that impairs the body’s ability to produce collagen.  (Bear with me, I’ll explain everything!)  Connective tissue (CT) is essentially the glue that holds the body together, and collagen is a protein that is the main component of CT.  Connective tissue is found in virtually every tissue, from skin to eyes to bones and blood vessels.

What this means for sufferers of EDS is that, because our “glue” isn’t the best quality, our bodies tend to stretch and sometimes fall apart more easily.

I have EDS – Hypermobility Type, formerly known as Type III.  The number one manifestation of this type is being extremely flexible, which is a direct result of the collagen defect.  Most people assume this just means I have cool party tricks, like bending my fingers backwards or bending at the waist and resting my wrists on the floor.  But the implications of this hypermobility are much worse.

DISCLAIMER: I can’t speak for everyone who suffers from EDS, so I’m going to focus on the symptoms I experience.  Please note that this is not a complete biography of the condition, only my personal account.

JOINTS

I grew up with more “growing pains” than the normal kid, but half the time I think my parents just thought I was complaining too much.  I was the youngest of five kids, so I did milk every scrape and bruise.  As I got involved in sports, these pains began to include chronic ankle sprains and neck pain.  After my cheerleading accident, these pains worsened severely.

The growing pains, aches, and eventual injuries I incurred (and continue to experience) are caused by, you guessed it, hypermobility.  Because the tissues supporting my joints are weaker than the average person, there is less strength available to protect my joints from injury.  Where a normal person might be just a little sore after jumping and landing wrong on their ankle, tripping on a step or twisting their knee during a dance, my joints can’t sustain the force applied to them and instead I receive larger stretches and tears in those supporting ligaments.

Think of it as a radio tower:  a regular tower or antenna has cables, guy-wires, attached to the ground to keep it upright.  In my case, my joints are like a free-standing tower that has broken or missing cables.  A strong enough gust of wind could easily knock it down without that extra support.

Not only does this hypermobility cause problems during exercise, but it also causes problems during normal daily functions.  I have a difficult time carrying dishes because of weakness in my fingers, and I have often had acute pain during writing for school that becomes chronic over time.

The hypermobility in my back is the most severe, with chronic pain in virtually every level of my spine and dysfunction in my neck, ribs, and sacroiliac (SI) joints that require frequent physical therapy to keep from subluxating (partially dislocating).

While my joint problems are the most severe of my symptoms, EDS plays a role in the entire body and these are just a few of the many problems caused by the condition.

SKIN

You’ll often find people with EDS have pale, nearly translucent skin, that allows you to see a lot of veins near the surface of the skin.  In my case, I have large purple and blue streaks along my chest, arms and legs because the skin in those areas is so thin and clear that you can see the veins.  Other skin complications include easy bruising and tearing, poor wound healing, and irregular scarring, which can cause complications following surgery.

EYES & MOUTH

Another group of key symptoms in my case are the eye problems I deal with.  I got glasses in the 2nd grade and am legally blind without glasses or contacts.  I have very thick lenses and an astigmatism severe enough in one eye that I wear a special contact lens, called a toric lens, that is specially designed to stay in place.

I’ve also suffered from dry eye severe enough that I use Restasis prescription drops daily.  I also have a condition that I first believed to be Keratoconus, a degenerative eye condition connected to EDS, but discovered it is actually an unusual condition called Thygeson’s Superficial Punctate Keratitis.  Perhaps someday we’ll discover a link between it and EDS, but as of now little is known about it.

A less serious but just as annoying eye problem I experience is photophobia, or hypersensitivity to light.  I often need sunglasses even when it’s raining, and when my Thygeson’s is acting up I cannot go outside without them.  This often results in my signature one-eyed squint in all outdoor photos, and this is the case for many EDSers!

Along with the dry eye problems, I suffer from dry mouth which puts me at a higher risk for gingivitis and cavities.  The only ways I’ve found to help combat this are drinking LOTS of water, chewing gum and using a dry mouth mouthwash.  Other problems we experience in the dental region are TMJ pain (grinding or gritting teeth), and hyperextensibility of the jaw, which makes dental visits very uncomfortable.

CARDIOVASCULAR

Many EDSers are born with heart murmurs, an extra “woosh” sound detected when listening to the heartbeat.  In many cases, including mine as far as we can tell as of now, this is an innocent murmur, meaning there are no defects causing the sound.  But for some, this can be caused by various heart defects, including Mitral Valve Prolapse or a hole somewhere in the heart.  In those cases, attention is needed to prevent ruptures, aneurysms and other life-threatening complications.

EDS manifests itself in many ways in the cardiovascular system, and in my case it is with autonomic dystonia (aka POTS, although I have yet to receive diagnosis).  This means I experience large, fast drops in blood pressure when I change positions too fast (i.e. standing up from working in the garden).  In some cases this has resulted in my nearly passing out.  I also have problems breathing, especially in humid weather, that have yet to be explained but almost positively relate to the EDS.

NEUROLOGICAL

People with EDS often suffer from headaches, neuropathy (numbness or tingling in extremities), balance problems and something called “brain fog” (it’s like a prolonged, more frustrating brain fart!).  One of the best methods I’ve found for combating these symptoms is the practice of Tai Chi.

Many, including myself, also have a reduced sensitivity to lidocaine and other numbing agents often used during surgical procedures such as nerve blocks and dental procedures.  In my case this has led to requiring general anesthesia for a spinal procedure I have done periodically in my neck.

GASTROINTESTINAL

Digestive problems are yet another issue for many EDSers, and often involve irritable bowel syndrome (IBS), acid reflux and GERD.

PAIN

Much of the pain experienced with EDS is “idiopathic,” meaning the cause is unknown.  Note that this is the only time I will ever use this word, because I consider it a poor excuse for real pain whose cause can’t be pinpointed.  Idiopathic does not equal imaginary, contrary to popular belief.

In coming posts I will talk about the modalities I use to treat the pain and other symptoms I experience, with links to some really wonderful products I use every day!

EMOTIONAL

EDS can and does also have an emotional impact on patients.  At some point everyone questions whether their pain is real, and when friends, family and physicians question you, it puts a lot of pressure on your sanity.  Without a good support system and proper intervention, EDSers often fall victim to depression.

So, this turned out way longer than I expected, and to be honest I’m probably not going to go back and proof everything because writing this is mentally exhausting.  I hope this brief (ha!) overview of EDS has answered some questions you may have had and that it has been an accurate and comprehensible introduction to Ehlers-Danlos Syndrome.  Just know that this is the tip of the iceberg, and in the coming months I’ll be able to do so much more to raise awareness while telling my story 🙂

http://dailypost.wordpress.com/2013/08/22/daily-prompt-first/

Photo by buddawiggi

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9 thoughts on “First Impressions: EDS Defined

  1. I also have EDS and Thygeson’s. I’ve been night blind for years because of the huge pupils from my POTS. I can’t go anywhere without “movie-star sunglasses” (including my own living room on some days). 9 out of 10 new doctors I see think I’m stoned. This explains why I was very popular in undergrad.

    I didn’t realize that we didn’t do well with nerve blocks. I have to have them in my legs and feet every three months, and they NEVER work. Last time I went in I managed to upend the table with the doctor’s equipment (after he’d already given me two blocks and was giving me a third because I was still screaming), break off the needle in my foot, and kick him in the face. (All reflex–I actually love this guy, he’s willing to work with me because he knows how difficult it is for me to live with dysautonomia and EDS).

    1. I’ve never met anyone else with Thygeson’s! It was the bane of my existence for about a year but I’ve been in remission since fall 2013!

      I feel you on the nerve blocks! In my case, my PM&R doc and I have found that Bupvacaine works better than lidocaine, but it’s still not a strong effect. I just grin and bear it during my more basic SI nerve block, epidural or subacromial injection, but during the neurotomies it’s utterly unbearable without conscious sedation. For me, the lack of response to the blocks is mainly when it’s just a local injection; I’ve had a successful brachial block during shoulder surgery. Hang in there! Some of these treatments we seek are almost worse than the pain itself, but it’s so worth it for even a few days of relief 🙂

      1. I’m doing everything under conscious sedation now, and I’ve completely stopped the lumbar nerve blocks. They made me feel great for about a week and a half, but when they wore off the pain was much worse. My doctor said my body had just gotten used to feeling good, so coming off the block was “shocking” to my system. (He might have mentioned this in the first place). This is the same doctor who treated my migraines with occipital ablutions (sticking metal wires in your brain and frying your nerves) because he doesn’t “do Botox.” I also endured a week in the hospital on a ketamine infusion for pain (I wasn’t supposed to remember any of it, but I remember every minute, and it was like a living nightmare), which also didn’t work.

        I now have a very competent Dr. at the Mayo Clinic who is NOT a sadist, and who came up with an amazing treatment protocol for me back in June. However, I live in NM, and after finally getting into a neurologist last week (it took me 8 months to get into Dr. Frankenstein the pain doctor), I was told that my case was too complex and escorted from the office without a referral because, “It’s rare that we see Ehlers Danlos around here.” So yes, I’ve got a great treatment regimen, but no one will follow it.

        My family doctor wouldn’t prescribe the pain med the doctor at Mayo wants me on because it’s a “controlled substance” or set up the recommended steroid infusion once a month, but immediately asked me if I’d be interested in trying cannabis (which, last time I looked, is a controlled substance).

        I’ve been off work since August, which is ridiculous, because the biggest obstacle to my well-being is my health, and I literally have the solution to that written down step by step by one of the best neurologists in the country (and the irony is that I was referred to Mayo in the first place by three different NM doctors who couldn’t figure out what was wrong with me). Unfortunately, that was two years and 36 doctors ago (we have a high turnover here in NM), but I finally have the answers AND the treatment, but no one to administer it.

        Do you have to travel to get treatment, or do you live in a place where it’s readily available? And if you do travel, does your insurance pay for flight/lodging/lost wages? Mayo is in-network for me, despite being out of state. Since three doctors referred me, and I can’t find the prescribed treatment here, I wonder if insurance is liable to get me back and forth for treatment?

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