For those of us with EDS, this is a post we can show our doctors to explain all the various pains we have and to provide hard scientific evidence (from the National Institute of Health, no less) that our pain is absolutely “real”.
Because our pain moves and changes so much, we can seem a little crazy when we report all its varieties and change our reports from appointment to appointment.
Pain is common in Ehlers–Danlos syndrome (EDS) and may correlate with hypermobility, frequency of subluxations and dislocations, soft tissue injury, history of previous surgery, myalgias, and may become chronic.
- Pain may be musculoskeletal or widespread.
- It may be acute and/or chronic.
- The pain may interfere with socialization and activities of daily living.
- Overall, pain impacts health‐related quality of life.
Multiple pathologies likely contribute to pain in EDS since it is a disease of connective tissue, which is found in virtually every organ system (Table I).
Table I. Review of Literature of Types of Pain in hEDS
Manifestations Number of patients studied Incidence (%) References Generalized body pain >800 (cumulative) 90 Jerosch and Prymka ; Camerota et al. ; Hamonet et al. [2012, 2014]; Hamonet and Brock ; Scheper et al. ; Voermans and Knoop, 2011 Soft‐tissue pain >800 (cumulative) 90 Hudson et al. ; Hamonet et al. [2012, 2014]; Scheper et al.  Dislocations >800 (cumulative) 78 Voermans et al. ; Hamonet et al. [2012, 2014] Joint pain 28* Elbow (43)* Moore et al. ; Aktas et al. *; Sacheti et al. ; Tubiana ; Berglund et al. ; McCulloch and Redmond ; Hamonet et al. ^; Hamonet et al. ; Hamonet and Brock, 2015#]; Christopherson and Adams ; Scheper et al.  232# Shoulders (80)* 644^ Hands (75)* Knees (71)* Spine (67)* Fatigue 644 [cumulative] 95 Gulbahar et al. ; Voermans et al. [2009, 2010]; *Celletti et al. ; Hamonet et al.  11 [cumulative] 6 (55)* Bone loss 23 16 (70) Gulbahar et al.  Neuropathic pain 29* 68* DeGraaf ; Kass and Kayed ; Stoler and Oaklander *; Camerota et al. ; Voermans et al.  Loss of proprioception 18*, 32#, 22^ Significant P‐value Helliwell *; Ferrell et al. ; Fatoye et al. ; #Rombaut et al. ; Zarate et al. ; Celletti et al. ; Galli et al. ; ^Clayton et al. ; Smith et al. ; Deparcy  Headaches 28* 75* Sansur et al. ; Schievink et al. ; DeCoster et al. ; Henderson et al. [2005a]; Gulbahar et al. ; Milhorat et al. *; Bendik et al. ; Rozen ; Hamonet and Brock  Gastrointestinal pain 21* 85.7* Douglas and Douglas ; Petros and Swash ; Castori et al. *; Zarate et al. ; Dordoni et al. ; Hamonet and Brock ; Mohammed et al., 2010 Temporomandibular joint pain 42* 71.4* *DeCoster et al. [2004, 2005]; Hagberg et al.  Menorrhagia 387 77.57 Gompel  Dysmenorrhea 73.1 Vulvodynia/dyspareunia 387 42 Gompel 
An international group of physicians with experience in treating pain in EDS formed a working group under the auspices of the International Consortium on the Ehlers–Danlos Syndromes.
Sacheti et al. interviewed 51 patients with EDS of which 28 (55%) were diagnosed with EDS hypermobile type (hEDS). They reported that the incidence of pain in hEDS was 28 out of 28 (100%). In this population, the mean score on the Numerical Rating Scale was 8 out of 10 for all types of EDS. Out of the 28 patients with hEDS, 24 (85.7%) reported progressively worsening pain.
The authors concluded that moderate to severe pain is common in hEDS, starts early in life and progresses and evolves over time but that it is often complex and varied, frequently reporting pain at multiple locations.
In a more recent study of 273 patients with EDS by Voermans et al., 246 (90%) patients reported pain.
Musculoskeletal pain in EDS is influenced by external factors such as lifestyle, sport activities, trauma, surgery, and various co‐morbidities.
Many patients report their very first painful sensations acutely, in relation to joint traumas such as dislocations and sprains as well as “growing pains” mostly localized to the knees/thighs
Reading this, I recall an episode of waking up in the middle of the night with mysterious excruciating lower leg pain when I was in Junior High School. Instinctively, I seemed to know that cooling it would help, so I spent a long time sitting on the edge of the tub running cold water over my knee and lower leg, which finally eased it enough to go back to bed.
Approximately 30% of children with hEDS reported arthralgias, back pain, and myalgias. This rate increases to >80% in patients over 40 years of age
those over the age of 33 years, often have a “negative” Beighton score but yet pain symptomatology in all aspects continues and, in most cases, increases.
Neck pain is a common feature of hEDS and is frequently associated with headaches.
It is often difficult to segregate the two conditions. Loose ligaments in the cranio‐cervical junction and cervical spine may manifest as occipital‐atlanto and/or atlanto‐axial instability.
This is compounded by ligamentous laxity of the cervical spine.
In one study, the prevalence of chronic pain was 90% in patients with various types of EDS, with the highest scores on severity of pain found in hEDS.
Chronic pain is one of the major symptoms presented by patients with hEDS.
It often presents as diffuse body pain affecting almost every part of the body. It is common and may be severe.
Loss of proprioception in hEDS has been reported in the medical literature and is considered to be an important factor in hEDS‐related chronic pain
Often, hEDS may be misdiagnosed as fibromyalgia because of diffuse pain with a strong myofascial component. These are to be considered as two distinct conditions with very specific diagnostic criteria. They may co‐exist as two separate conditions but have different etiologies.
Pain and fatigue have a high prevalence in EDS, frequently manifesting as the predominant symptoms and as the most disabling features.
Management of chronic pain in hEDS is hindered by lack of evidence based studies that clearly demonstrate effectiveness of different modalities.
Most cases of chronic pain are an uneven mix of nociceptive and neuropathic pain.
Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint) and minimizing the sensation of pain.
Successful management of chronic pain requires a multidisciplinary approach.
Physiotherapy: available evidence suggests that patients who receive exercise intervention improve over time [Palmera et al., 2014]. Physical rehabilitation consists of core stabilizing and joint stabilizing and proprioception enhancing exercise coupled with general fitness program
They improve in some ways perhaps, but no amount of exercise can prevent our tendons and ligaments from deteriorating more and more rapidly since they are made almost entirely from collagen, the tissue that is defectively manufactured by our bodies.
Cognitive behavioral therapy: this approach is applicable to all patients especially those whose pain is intractable, life dominating and unresponsive to analgesics or other physical interventions. To date there have been no clinical trials to demonstrate its efficacy in EDS.
Non‐steroidal anti‐inflammatory drugs (NSAIDs): May be helpful if the pain is of inflammatory origin.
Chronic use of NSAIDs is frequently associated with gastrointestinal, renal, and hematologic consequences.
…and then they go right on to point out how damaging these medications are with chronic use, which is required for chronic pain.
Opioids: for acute, severe pain, opioids may be an option, but only for a short duration. There is good evidence that long‐term treatment with opiates is not a viable option and may lead to central pain sensitization.
Opioids have been a perfectly viable option for the last 25 of my 60 years. Because EDS pain is from an unending series of micro-tears and micro-injuries of my connective tissue, this is actually chronic acute pain, and it varies greatly in location, type, and intensity. It lacks the 24/7 chronicity of medically-defined chronic pain, which persists unchanging.
Options for neuropathic pain include low dose tricyclic anti‐depressants, anti‐convulsants, and selective norepinephrine reuptake inhibitors.
…and I take then all, literally. But I still need concurrent opioids for pain that cannot be sufficiently eased by any of these other medications.
Acetaminophen, to avoid hematologic consequences that could be associated with NSAIDs.
And yet, acetaminophen is poisonous if taken in high doses for long periods, leading to organ failure and death. (I witnessed this myself in a co-worker who died from taking too much Tylenol for her frequent migraines.)
- Tylenol/acetaminophen/APAP is a Dangerous Drug
- Acetaminophen Risks May Have Been Underestimated
- More Acetaminophen Warnings from FDA
- Potential Dangers of NSAIDs, Acetaminophen Often Ignored
And to top it all, it’s not even particularly effective, according to studies:
“In conclusion, there is little evidence to support the efficacy of acetaminophen treatment in patients with chronic pain conditions.”
Topical lidocaine for localized pain after subluxations as well as painful gingival tissue
Nefopam is a non‐morphine derived potent analgesic
For musculocutaneous pain: injections with 1% lidocaine into trigger points
Dysmenorrhea can be treated with NSAIDs as it is often time‐limited
Women with dysmenorrhea and whose overall symptoms worsen during the peri‐menstrual period may be aided by hormonal control with anti‐gonadotropic, hypoestrogenic progestins
This might be due to the fact that proprioceptive accuracy decreases during menses.
Transcutaneous neuro stimulator (TENS) to block pain signals via gate control theory
I tried this for months but found it mainly distracting instead of pain-relieving. When my pain we more severe, I had to turn it up so high that it was just as “painful” as the pain.
Treating the underlying proprioceptive disorder with compressive, that is, tight clothing, physiotherapy, and proprioceptive shoe inserts
Dystonia has been described in 54% of EDS patients in a cohort study of 626 patients. Treatment with Levodopa/carbidopa or Levodopa 50 mg/benserazide 12.5 may improve dystonia, pain, and fatigue
Fatigue and pain are linked when it comes to disability issues. They both diminish the quality of life and need to be addressed. Treating the fatigue treats the pain and vice versa.
Dysautonomia is a common factor in EDS and when treated may alleviate both fatigue and pain.