Management of chronic pain in EDS – part 2

Management of chronic pain in EDS – Nov  2018 – part 2

This post continues from the first part, with more detailed descriptions of various types of pain that arise from our tissue fragility and from consequences of related genetic changes.

3. Discussion

3.1 Chronic pain and EDS overview  (covered in previous post)

3.1.1 Musculoskeletal pain

Nociceptive, joint pain is usually the first manifestation of pain in EDS. 

I remember cases of this from grade school and, of course, my pains grew more severe over my lifetime.

Patients most commonly report their very first painful sensations acutely, as a result from joint traumas such as dislocations and sprains as well as “growing pains.”

In junior high school, I can remember sometimes being woken in the night by an intense deep ache in my lower leg.

Because my parents weren’t available (long story), I figured out on my own that my body craved something cold, so I tried running water.

I ended up sitting on the rim of the bathtub running cold water over it for what seemed like hours before the pain was tolerable enough to sleep. The next morning, there was no trace of it.

This shows how at least some people with chronic pain can find their own best treatments by listening to their bodies and experimenting with treatments. We know pretty darn quickly when a treatment is not working, so it’s frustrating when doctors insist we long trials of ineffective therapies.

The most commonly involved joints are the

  • shoulder (80%),
  • hands (75%), and
  • knees (71%).

I’m surprised not to see the sacroiliac joint (or at least low back) on this list because that joint is the base of our spine and must carry and control all upper body movement.

The pain of a misalignment (subluxation) in the sacroiliac joint can radiate from the low back and buttocks and down the legs, so I suspect that most of my pain in hips and thighs is likely to originate there.

…foot pain is an additional strongly debilitating pain.

By my college years, my metatarsal arches (crosswise instead of lengthwise) had collapsed. That can cause “Morton’s neuroma” as the metatarsal bones get squished together and pinch the nerve running to the toe.

Luckily, I’ve found an orthotic that elevates the center of the ball of the foot where the pain is. For me, the first step into my Birkenstock sandals always solved the problem, but I didn’t want them to be the only shoes I could wear.

One day I was in a shoe store and saw Birkenstock insoles, which I bought immediately (~$50 and worth every penny). They help tremendously and I use them in all my sneakers now.

I also discovered the unusual “Hoka One-One” brand of sneakers that I can walk in comfortably because they have a “rocker bottom”. Regular sneakers only have thick padding at the heel, but these shoes also have significant padding in the center and a little in the front part of the sole too.

This creates a subtle front-to-back rounding which makes my steps less abrupt. The thick padding under the ball of my foot allows me to walk even on concrete (which I’ve noticed is significantly harder than asphalt).

Hands (especially thumb) and arm pain results in significant disability with writing and holding objects.

I’ve been lucky with this because, due to my early-onset low back pain, I was always concerned with holding (or at least trying to hold) my body in a good, mechanically efficient posture.

My early pains were a blessing in disguise because they spurred me to an awareness of my body’s inner workings – and also motivated me to do the work of changing how I habitually use my body.

Though I’m extremely sensitive to and pained by repetitive motions, I managed to avoid the carpal tunnel syndrome that plagued so many other programmers. I tried to keep a proper keyboarding position and took frequent breaks, which later became the standard advice for people suffering from carpal tunnel syndrome. I kept my wrists straight and comfortable until just a few years before I had to stop working.

Patients with EDS describe themselves as “Klutzy” often dropping objects and falling.

This is something I’ve long noticed and that has lately become a little dangerous.

As I move, I see an object I’m trying to avoid, make an effort to avoid it, and then still run into it. I blame this on our poor proprioception – we just don’t know where our bodies are in space.

I’ve become dangerous with knives and almost cut off a finger while I was honing a blade as I’ve done countless times before. This made me set a new policy for myself: when a knife is in my hand, I won’t let my mind wander and I will move slowly and deliberately. No more “flick of the wrist” when there’s a knife involved.

TMJ pain can also be a significant source in disability in 70% of Ehlers–Danlos patients

The recurrent dislocations due to joint laxity and associated trauma are further amplified by poor proprioception.

Severe chronic fatigue is now considered a common accompanying feature of EDS. It is reported in up to 95% of patients and in many cases was reported by patients to be more debilitating than pain. The frequency of fatigue is influenced by age with a rate of 28% in the first decade of life to 90% in adults over 40 years of age.

I’ve definitely noticed a significant loss of energy over the years – far more than any of my friends and coworkers (or bicycling cohort). I assume this is because we have to use far more muscular effort to hold ourselves upright with our “loose” joints.

3.1.2 Neuropathic pain [NP] and central sensitization [CS]

Approximately 68% of chronic pain patients with EDS can be considered to have NP

Compression and axonal neuropathies have been suggested as a potential cause of paresthesia, numbness, and dysesthesia in EDS patients.

Additionally, subluxation of the ulnar nerve occurs more commonly in EDS

Many patients describe NP also at rest and with strongly bilateral presentation which is not usually in keeping with compression neuropathies. This could be due to generalized small fiber neuropathy or evidence of CS.

…irrespective of EDS subtype, patients with clinical features suggestive of NP may show decreased intraepidermal nerve fiber density indicative of small fiber neuropathy.

Development of CS is usually at a later stage due to the persistent nociceptive input caused by joint abnormalities or changes within the extracellular matrix due to repeated injuries.

This is what I’ve suspected for a long time, so I’m gratified to see it’s now more commonly believed.

When a body is sent pain signals from, for example, a “bad knee”, how long before the “pain perception switch” finally gets stuck in the “on position”?

Features suggestive of CS include

A body that consistently experiences pain would become ever more wary of any indication that it needs to be careful and try not to provoke more pain.

Fibromyalgia, a widespread musculoskeletal pain syndrome commonly associated with CS, occurs in 42% of adults EDS.

Some suggest that many Fibromyalgia patients actually have EDS:

…postulate that EDS can predispose to the development of CRPS due to

  • stretch injury to nerves,
  • increased fragility of nerve connective tissue, or
  • nerve trauma from more frequent surgeries

3.1.3 Pain and psychological issues

Psychological distress, fatigue, and sleep disturbances are common comorbidities and their presence may negatively affect pain manifestations and evolution, and ultimately contributing to chronic pain and disability.

The literature review conducted by the international consortium for EDS highlights a high prevalence of anxiety (70%) and panic disorder in patients with EDS.

This connection has been proven in various scientific studies:

There is also growing evidence to suggest its association with depression and demonstrate a statistically significant link with

  • bipolar disorder,
  • autism spectrum disorder, and
  • attention deficit hyperactivity disorder.

The underlying mechanisms behind this association include

  • genetic risks,
  • autonomic nervous system dysfunction, and
  • decreased proprioception.

There’s a clear link between the biology of EDS and the biology of mental or mood disorders:

Many of these “mental disorders” might be less related to EDS than they are to chronic pain itself: Psychiatric disorders in EDS strongly associated with pain

Maladaptive behaviors such as pain catastrophizing, kinesiophobia, and fear avoidance are also prevalent in this population and can be a barrier to effective pain control as was present in Case 1.

Terms like “maladaptive behaviors” have strong connotations of being voluntary and deliberate and have been used to support the idea that we are “catastrophizing” about our pain. (See my numerous posts on this atrocity of nomenclature)

I’d like to see the researchers who come up with this term suffer extreme pain of their own and then not exhibit “maladaptive behaviors”, like wincing, crying out, asking for help, or showing worry about it. Those are absolutely normal behaviors.

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