Ehlers-Danlos Syndrome: An Emerging Challenge for Pain Management – Editor’s Memo—September 2017 – By Forest Tennant, MD, DrPH
Thank you, Dr. Tenant, for drawing attention to this terribly painful genetic syndrome that is completely ignored by so many pain specialists, even as it clearly causes increasing physical pain.
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
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.
Recognizing Ehlers-Danlos Syndrome
EDS may be the most common genetic connective tissue disease to cause intractable pain, necessitating high-dose opioid therapy.
This disorder causes progressive deterioration and degeneration of connective tissue in joints, spine, eye, gums, teeth, internal organs, and central nervous system (CNS).
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.
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.
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.
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
Letters to the Editor: Ehlers-Danlos Syndrome – from Practical Pain Management
Treating for Ehlers-Danlos Syndrome
I am a primary care physician who wishes to find a better treatment for a patient with Ehlers-Danlos Syndrome (EDS). In particular, I would be interested in hearing your thoughts and/or experience with regard to the use of oxytocin for pain in these patients.
Dear Dr. Fedorowicz,
EDS is a progressive, degenerative connective tissue disorder that causes microtears in multiple organs.
It is common to see EDS patients entering their teens or twenties who then develop severe pain
I believe we have finally arrived at a point where we have sufficient understanding of the underlying pathology of EDS to treat it with a more informed approach.
Oxytocin appears to work on neurons in and around the spinal cord-brain region, making it an effective nonopioid therapy, which should be adopted more readily and more widely used.
To date, I have found oxytocin to be quite effective in relieving pain in patients with EDS.
In addition, oxytocin may confer a neuroprotective effect given its antineuroinflammatory properties.
If you decide to prescribe oxytocin for your current EDS patient, I suggest a starting dose of 20 units given sublingually.
Typically, the concentration I titrate up to is 40 units per milliliter. If necessary, the dosage can be increased up to about 80 units safely, and it can be given as often as every 4 to 6 hours.
Another therapeutic approach that has been beneficial in my clinic is the use of human chorionic gonadotropin, human growth hormone, and nandrolones in some EDS patients.
The purpose of these hormones is to not only inhibit deterioration but, hopefully, to provide regeneration of some tissues.