Have you been diagnosed with one or more of these conditions? Wondering how they all fit together?
The trifecta describes the triad of chronic conditions including hypermobile Ehlers-Danlos syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS) and Mast Cell Activation Syndrome (MCAS).
Why do these three conditions like to show up together?Research indicates that there may be a genetic mutation that when triggered, could flip the switch, triggering all 3 conditions to occur together. Each persons presentation may be different depending on epigenetic expression.
Let’s review the three conditions.
Mast cells are a type of white blood cell found in connective tissues throughout the entire body including just beneath the skin, near blood and lymph vessels, in nerves, lungs and the intestines. They are present in high numbers anywhere the outside environment interacts with our body so think mouth, sinuses, esophagus, lungs, GI tract, and bowel/bladder. They are intended to warn us of danger, but sometimes they become overreactive and send false danger signals.
Hypermobile Ehlers-Danlos Syndrome is a connective tissue disorder where there is increased laxity in the connective tissue, including ligaments. This results in joint hypermobility and instability.
Postural Orthostatic Tachycardia Syndrome is a dysfunction of the autonomic nervous system, resulting in autonomic dysregulation with the cardinal sign being increased heart rate upon standing.
How do they all relate? Let’s dive into the connections between the pairs of conditions.
One theory that connects MCAS and hEDS is that MCAS creates an immune response to pathogens, which releases mediators (such as histamine or tryptase) and this can affect the collagen in the connective tissue. This can lead to ligamentous laxity and joint hypermobility.
Another theory is that collagen, which plays a role in the connective tissues of the body, is located inside the extracellular matrix (ECM) and mast cells stick to this ECM. EDS could cause a change in the ECM enough to alter the behavior of the mast cells, leading to over activation.
hEDS contributes to laxity of the connective tissue, which includes blood vessels. This makes blood vessels “floppy” and more likely to vasodilate. POTS occurs when blood vessels remain dilated and blood pools, causing the heart to beat faster. There is disrupted communication between the autonomic nervous system and the vascular system.
hEDS can also lead to cranial cervical instability along with brainstem compression that could compromise the vagus nerve. The vagus nerve is involved in autonomic nervous system function and may cause dysautonomia or POTS.
Mast cell over activation can act on the blood vessels and cause vasodilation, which causes pooling of blood in the legs and abdomen as seen in POTS. Mast cells can interact with neurons, including autonomic nerve fibers, which can lead to POTS.
Increased norepinephrine, which is secreted in response to standing (and in higher levels with POTS) may cause mast cell degranulation, releasing mediators and causing symptoms.
More research is needed, but this is why it is important to view the body from a holistic lens because treating the one part of the trifecta may improve the others.
Wondering about holistic treatment of the trifecta and where to start?
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