hedwyg’s chronic pain journey

I’ve been asked a few times for more information on my chronic illness, so I decided to put it all on a page here.

In November 2008, at age 36 and 8 months, I was definitively diagnosed with Ehlers-Danlos Syndrome: Classical Type.  The journey toward this diagnosis began in August 2006, when I first began seeking treatment for generalized pain, but it is a condition I’ve had my entire life.  Here are some of the ways in which EDS affects me:

  • Hypermobile joints: My joints move more and further than a “normal”
    Patient with Ehlers-Danlos syndrome. Courtesy of Enrico Ceccolini, MD, from http://emedicine.medscape.com/article/1114004-overview
    Patient with Ehlers-Danlos syndrome. Courtesy of Enrico Ceccolini, MD, from http://emedicine.medscape.com/article/1114004-overview

    range of motion.  My knees and elbows can bend backward, and I can do some of the joint tricks you see pictured on the EDS page on wikipedia.

  • Flexible skin: My skin is apparently very soft and velvety — I didn’t ever realize, it just always felt like my skin! — and is more flexible than typical.  I can’t do the circus-freak skin stretchy tricks, but this does affect how wounds heal and how scars act over time.
  • Easy bruising: My skin bruises really easy.  At the end of the day, I usually notice a new bruise or two, and I have no idea where they came from.  Sometimes it seems like you can give me a dirty look, and I’ll end up with a bruise.
  • Increased risk of injury: Hypermobile joints are easier to injure, and
    Girl with Ehlers-Danlos syndrome. From http://emedicine.medscape.com/article/1114004-overview, courtesy of Enrico Ceccolini, MD.
    Girl with Ehlers-Danlos syndrome. From http://emedicine.medscape.com/article/1114004-overview, courtesy of Enrico Ceccolini, MD.

    I’ve had more than a dozen sprains of each ankle, as well as sprains of other body parts.  When a joint moves out of position, it can also cause muscle spasms, muscle strains and tears, nerve impingement, or just plain old pain.  For example, one November I had an abscessed tooth and had to undergo a root canal, which failed, so then I had to have the tooth extracted.  A month later, my jaw finally felt like it was back in the right position, without causing painful clicks, pops, and spasms.

  • Early onset osteoarthritis: Arthritis is the eventual degradation of joints due to wear and tear.  The cartilage wears down so that bone scrapes on bone, and this causes pain.  My mother had both her knees replaced at age 50.  I’m looking at the possibility of knees, hips, and shoulders, probably in about two decades.
  • Reflux: With all of my connective tissue being weak, stretchy, and floppy, there are effects on the inside as well as on the outside.  I have mild reflux on a regular basis, and there are some activities that make this significantly worse.  I’ve noticed that singing (really singing, like in voice lessons or choir rehearsals) tends to trigger reflux, and I think it’s a combination of good breathing and a floppy epiglottis.
  • Asthma-like symptoms: I’ve had an asthma diagnosis for years, but the symptoms don’t respond to asthma management medications.  A rescue inhaler does provide help during a bad coughing episode, but the management medications don’t do much but cost a lot of money.  It turns out that weak connective tissue can be present in the airways, too, so that they get floppy and collapsey.  Think about a garden hose. Most of them are flexible but rigid and stay round all the time; this is like more normal airways.  Now imagine one made of soft rubber that collapses in on itself when the water isn’t flowing, so that it looks almost flat.  Now, for a hose that holds its shape, the water flows through freely; on the other hand, the hose that goes flat will require more pressure for the water to open the hose back up before it can flow through.  This is what my airways do to me.
  • and this is inherited: It’s an autosomal dominant trait, which means it
    Autosomal Dominant Inheritance, from Retina Australia NSW
    Autosomal Dominant Inheritance, from Retina Australia NSW

    isn’t passed on the sex chromosomes (X and Y).  My sister has it, my mother has it, my grandmother has it, my aunt probably has it along with her two daughters, and I’m watching my daughter like a hawk.  Each pregnancy carries a 50% risk of passing on Ehlers-Danlos Syndrome to the child, along with other significant risks to the mother.

While this isn’t necessarily a progressive condition, the symptoms related to hypermobile joints do tend to progress over time.  Joints wear down, and if I don’t constantly work to keep the surrounding muscles strong and toned to compensate for the flawed connective tissue, then I’ll have more pain and lose the use of those joints sooner.

Bug's Silver Ring Splints, from Our Journey with Ehlers-Danlos Syndrome
Bug’s Silver Ring Splints, from Our Journey with Ehlers-Danlos Syndrome

On the other hand, there really isn’t any getting better or being on the mend for someone with Ehlers-Danlos Syndrome.  An individual acute injury can mend (somewhat), but the connective tissue in my body won’t get better.  Treatment for EDS is management of symptoms; management of pain; avoidance of activities that are riskier for injury; taking lots of Vitamin C, Vitamin D, and calcium; and specific kinds of exercise and/or physical therapy.  I have splints and braces for many joints in the body; while I don’t use them all the time because this would end up weakening the joints further, I do use them when pain in the area is particularly bad.

Physical Therapy, by www.army.mil
Physical Therapy, by http://www.army.mil

I am under regular treatment from my primary family doctor, a physical therapist, and an orthopedic surgeon.  In the last few years, I’ve also had consultations with a geneticist, a neurologist, and a rheumatologist, and a neurosurgeon.  I’ve had five MRIs (shoulder, hand, ankle, brain, neck), an MRA (brain), a lumbar puncture, and an uncountable number of x-rays.  I had one rotator cuff surgically repaired and someday I will have a breast reduction.

I also have a deficiency of Vitamin D that requires supplementing with

Mr. Sunshine - ♥, by Warm 'n' Fuzzy
Mr. Sunshine – ♥, by Warm ‘n’ Fuzzy

prescription-strength doses.  It appears that my body cannot convert sunlight into Vitamin D, and I just can’t absorb enough from foods and regular over-the-counter supplements.  The levels of CRP in my blood remain elevated at a fairly stable level, reflecting the chronic inflammation of my joints.

In the last two years, we’ve ruled out rheumatoid arthritis, thyroid disorders, multiple sclerosis, lupus, fibromyalgia, Chiari malformation, pseudotumor cerebri, brain aneurysm, and probably a host of things the doctors didn’t even tell me about.  When I brought the geneticist’s diagnosis back to the pain management specialist, she celebrated.  Her explanation was: When doctors see a patient like you, they automatically think, No one person can possibly have so many problems.  But you don’t have many problems.  You have one problem, and that’s with your connective tissue. It made a lot of sense, and it really gathers together so many symptoms and questioned diagnoses and times when doctors just threw up their hands and gave up.

Between 2008 and 2013, I was very fortunate to work for a company that allowed me to telework.  This gave me the flexibility to rest when the pain became overwhelming, to take the more intense medications without having to worry about driving home later, and to minimize time away due to doctor visits and physical therapy sessions.  I spent much of 2011 to 2013 on full or partial disability leave, due to chronic pain and Chronic Fatigue Syndrome.  When I returned to full-time work in 2013, I was very anxious wondering whether I had the physical and emotional strength and stamina to maintain the schedule.  Good news: it has been fine!

Virginia handicapped placard
Virginia handicapped placard

I have handicapped license plates, so I don’t have to worry about safety when crossing busy parking lots, though I do endure many dirty looks from people who don’t understand invisible illness.  I’ve learned to count my spoons, because even the simplest things — like being at my daughter’s birthday party where my jobs were to take photos and to sit at the table and watch everyone’s purse while they roller-skated — can take an incredible toll on my body.

Just for fun, I thought I’d make a list of some of the normal activities that we do everyday and take for granted… but that have caused one or more of my joints to sublux (partially dislocate), sprain, or fully dislocate.  I’m not making any of these up; each of these represents an injury that I have actually experienced, on at least one occasion.

  • Unlocking the front door of the house (wrist)
  • Sliding open the door to a battery compartment of a book light (wrist)
  • Helping my daughter put on socks (tendons on the back of my hand)
  • Using a mouse (hand, wrist, elbow, shoulder, neck)
  • Using a “real” touchpad (hand, shoulder, neck)
  • Sitting at my (custom designed for little persons) desk (neck, shoulders, entire back)
  • Sitting in a “normal” chair, like at a restaurant or in a waiting room (SI joints, hips, entire back, knees, ankles)
  • Sleeping (jaw, neck, shoulder)
  • Walking (ankle, knee)
  • Squeezing conditioner from the bottle (wrists, fingers)
  • Washing my hair (shoulders, wrists, fingers, upper back, neck)
  • Shaving my legs (torsion, or twisting, of the sacro-iliac joint)
  • Shaving my underarms (shoulder, neck, wrist)
  • Going to church (hip, sacro-iliac joint)
  • Standing for more than 3 or 4 minutes (sacro-iliac joint, lower back, middle back)
  • Doing laundry (fingers, wrists, shoulders, neck, back)
  • Holding a book (fingers, wrist, elbow, shoulder)
  • Wheeling the garbage to the curb (shoulder, elbow, wrist)
  • Shoulder-checking before a lane change (neck)
  • Driving (sacro-iliac, knee, ankle, neck, back)
  • Standing up from my chair (wrist, knee, back)
  • Lifting a pot or pan (wrist)
  • Mixing a recipe (wrist, shoulder, upper back)
  • Wheeling my suitcase in the airport (shoulder, elbow)
  • Hooking a bra (shoulder, fingers)
  • Putting on a coat (shoulder, spine, wrist)
  • Fastening buttons (fingers)
  • Exercising (any joint!)
  • Coughing, sneezing (ribs)
  • Stretching out my pinky to reach the backspace key (tissue across the back of my hand)
  • Pressing the “sleep” button on my phone (thumb and wrist)
  • Trying to imitate the position a fellow EDSer has posted online to share a dislocation/subluxation (whatever joint I’m stupid enough to try it out on)
  • … more to follow, when I remember them or find new ones!

Now, these injuries don’t happen every time I do each activity, and I won’t even say that they happen most times (though some do).  But the risk is there in just about anything I do, that I’ll end up with an injury as a result.

You can find posts on the blog related to my journey with this illness through the tags.  Here are some of the most frequent ones.

Peace be with you on your own journey.

Updated October 16, 2014
Updated November 29, 1012
Updated May 3, 2010
Updated March 18, 2010
Updated March 16, 2010
Updated December 28, 2008

Page created December 27, 2008


2 thoughts on “hedwyg’s chronic pain journey

  1. Thank you for explaining the never ending trial and tribulation of EDS. I have the hypermobility type of ES, diagnosed in the 70’s, so you can imagine the frustration I went through, knowing something was not right, but never having a clue.

    Wish you the best.



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