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Ehlers-Danlos Syndrome: An Emerging Challenge for Pain Management

Editor's Memo—September 2017

Until recently, Ehlers-Danlos Syndrome (EDS) was a name that elicited little relevance or urgency in the pain world. Little did I realize that I had been treating more cases of EDS in patients who had been referred to my practice initially for more commonly recognized diagnoses such as fibromyalgia, spine degeneration, and resistant migraine. A diagnosis of EDS has become more evident as more patients present to pain practices across the United States. In fact, the National Institutes of Health offers an estimate of 1 in 10,000 to 1 in 15,000 people worldwide.1 There are some characteristics and clinical manifestations about this condition that all pain practitioners should be well versed in, ready to identify, properly diagnose, and treat.

Ehlers-Danlos Syndrome is an emerging pain disorder that requires attentiveness to diagnose and treat.

Recognizing Ehlers-Danlos Syndrome

EDS may be the most common genetic connective tissue disease to cause intractable pain, necessitating high-dose opioid therapy.2 It has also been known as the “hypermobility syndrome” (in circus terms—"the rubber man”) since these patients can displace their body extremities out of their joints (ie, double-jointed). This disorder causes progressive deterioration and degeneration of connective tissue in joints, spine, eye, gums, teeth, internal organs, and central nervous system (CNS).

Patients with the severest form of this genetic disease seldom live past 50 years of age. Unfortunately, patients typically reach a point at which their connective tissue begins to fail in its ability and duty to stay attached to and uphold organs including tendons, ligaments, cartilage, spinal cord, brain, eye, teeth, and peripheral nerves. As the connective tissue deteriorates, painful micro-tears affect the organs and cause severe pain, essentially head-to-toe. Consequently, unremitting pain sets in, causing disability to such an extent that patients become bedridden or couch-bound.

At this stage, caretaker assistance is needed. Also, the connective tissue has often deteriorated so significantly, compromising the patient’s overall health such that a catastrophic, life-threatening event may occur. For example, catastrophic events common in patients with EDS include cerebellar herniation, spinal cord tethering, adhesive arachnoiditis, aorta or cerebral vascular rupture, uterine rupture, and visceroptosis.  

It has become more evident that some patients who present with a diagnosis other than EDS as the cause of their symptoms may have been incorrectly assessed, and are not, therefore, receiving the most appropriate and sufficient pain treatment. In fact, many of these patients may not even relate their disease to their pain. This points to the need for all pain practitioners to become well-versed in the presentation of EDS and to be prepared to screen for it.3-6

EDS Requires Aggressive, High-Dose Pain Therapy

At this time, the most meaningful treatment for symptomatic patients is aggressive, humanitarian pain relief.

Why does EDS require high-dose opioid therapy? 
Given the progressive centralized breakdown in connective tissue, patients develop intractable pain that leaves them unable to function. Typically, the need for analgesia rises. The treatment approach that I advocate in my practice is shared in a letter to the editor, Treating Ehlers-Danlos Syndrome, in the current issue.  

The easiest initial screen is to have the patient complete a brief, 5-question survey (Figure 1). If the patient answers “yes” to 3 of these questions, the next step would be to conduct a Brighton test (Figure 2).2 Should a diagnosis of EDS appear likely based on the Brighton score, a final step is to check the diagnostic criteria published—and recently updated—by the International Consortium or Ehlers-Danlos Syndrome and Related Disorders.5

In my experience, these criteria are almost always met when the patient has constant pain and a positive Brighton Score.

A major reason to screen for, and make a proper diagnosis of EDS is that more encouraging reports are indicating that high-dose opioid treatment with supplemental hormones (as needed on an individual basis) may be an effective means of pain control. Reports of hormonal treatments appear to slow deterioration and restore connective tissue. In particular, the hormones showing the greatest promise include human growth hormone, human chorionic gonadotropin, oxytocin, and nandrolone.

In addition, patients can be directed to a number of exercises and physical supports that have been developed for EDS; these resources are available through EDS support groups

Last updated on: September 27, 2017
Continue Reading:
Letters to the Editor: Arachnoiditis, Hormone Testing, Ehlers-Danlos Syndrome
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