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Ehlers-Danlos Syndrome: An Emerging Challenge for Pain Management

key information

source: Vertical Health

year: 2017

authors: Tennant Forest, MD, DrPH

summary/abstract:

EDS may be the most common genetic connective tissue disease to cause intractable pain, necessitating high-dose opioid therapy.  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 (i.e, 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 upholds 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.

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