“No other disease in the history of modern medicine has been neglected in such a way as Ehlers-Danlos Syndrome.” Dr. Rodney Grahame
What is Ehlers-Danlos Syndrome?
Ehlers-Danlos syndrome (EDS) is actually the name given to a group of inherited disorders of connective tissue. The underlying problem in all these disorders is an abnormal structure or function of collagen and other connective tissue proteins.
The most common characteristic of an individual with EDS is joint hypermobility, which means their joints move further than normal range, and also joint instability, meaning partial separation of a joint, dislocations, scoliosis, and other joint deformities. Their skin may be able to be stretched further than normal and scar abnormally. The unusual collagen can also cause other problems such as hernias and organ prolapse through the pelvic floor. In some cases, EDS can cause serious gum and dental disease, eye disease, cardiac valve and aortic root disorders, and life-threatening abdominal organ, uterine, or blood vessel rupture.
EDS has 13 subtypes, each with their own symptoms and presentation. There is:
· Classical EDS (cEDS)
· Classical-like EDS (clEDS)
· Cardiac-valvular EDS (cvEDS)
· Vascular EDS (vEDS)
· Hypermobile EDS
· Arthrochalasia EDS (aEDS)
· Dermatosparaxis EDS (dEDS)
· Kyphoscoliotic EDS (kEDS)
· Brittle Cornea Syndrome (BCS)
· Spondylodysplastic EDS (spEDS)
· Musculocontractural EDS (mcEDS)
· Myopathic EDS (mEDS)
· Periodontal EDS (pEDS)
Diagnosing EDS is challenging for a number of reasons. There is no clear-cut diagnostic test and the symptoms are so various that when physicians are faced with these patient, they often have no idea where to begin the diagnostic process. Some of the symptoms, such as pain and dizziness, occur without an easily identifiable cause, leading some providers to believe the patient’s problems are psychological rather than physical. The mistrust often grows when negative diagnostic tests disappoint the patient, who just wants to find a name for what they are enduring. Diagnosing EDS takes an average of 14 years, and can commonly take 20 years.
Pathways To Trust has added provider- and patient-facing communication trainings tailored to meet the challenges of EDS. Check out our Time to Listen to EDS page to learn more.