Ehlers-Danlos & Hypermobility

Ehlers-Danlos syndrome (EDS) is a group of 13 hereditary genetic disorders that weaken the body's connective tissues

Connective tissues act as the body's scaffolding—supporting skin, joints, blood vessels, and internal organs. Because EDS affects how the body produces or uses collagen and related proteins, these support structures become fragile or overly flexible

Hypermobility Spectrum (HSD) means that a person’s joints have a greater range of motion than is expected or usual. Some people have joint hypermobility that does not cause them pain or other issues. However, some people with joint hypermobility also have joint instability. Joint instability occurs when the bones of a joint aren’t held in place securely. This can lead to joint subluxations, dislocations, sprains, and other injuries. Joint instability can cause both acute and chronic pain and interfere with daily life.

Beighton Test for HSD

One way to assess generalized joint hypermobility is with the Beighton Score, which measures joint hypermobility on a 9-point scale. One point is given for each of the following joints that show hypermobility on exam:

Common Co-Morbidities

Dysautonomia

Also known as autonomic dysfunction, is a group of disorders that affect the autonomic nervous system (ANS). Many people with EDS or HSD also have a type of dysautonomia. Common signs of dysautonomia include:

Tachycardia (fast heart rate)
Hypotension (low blood pressure)
Dizziness/lightheadedness
Gastrointestinal dysmotility (problems moving food through the digestive system)
Disturbed bladder function
Temperature and sweating dysregulation
Sleep problems

MCAS: Mast Cell Activation Syndrome

It is a condition where mast cells—a vital part of your immune system that normally helps protect you from infections and heals wounds—become hyperactive or release chemical "mediators" (like histamine, tryptase, and prostaglandins) too easily or in excessive amounts

POTS: Positional Orthostatic Tachycardia Syndrome

POTS is diagnosed when a person has all of the following:

Frequent symptoms that get worse when standing (including lightheadedness, palpitations, tremor, generalized weakness, blurred vision, exercise intolerance, and fatigue)
An increase in heart rate of ≥ 30 beats per minute when moving from lying down to standing (or an increase of ≥ 40 beats per minute in people 12 to 19 years old)
Absence of orthostatic hypotension (meaning there is not a > 20 mm Hg drop in systolic blood pressure when moving from lying down to standing)